CONVULSIVE DISORDERS

Introduction:

Epilepsy is one of the most frequent, chronic neurologic disorders of

childhood. The WHO estimates that 3-10 per 1000 of the total world population
have epilepsy. The frequency of seizures in the epileptic population varies widely.
Some epileptics experience only two seizures in a life time, and others may suffer
hundreds of attacks daily.

Definitions:

Epilepsy:

A neurologic condition characterized by recurring seizures.

Epilepsy is a neurologic sign of underlying nervous disorders.

Epilepsy is recurrent, episodic, paroxysmal transient disturbances of brain function

due to abnormal electrical activity of neurons.

Seizure:

A sudden attack of altered cerebral neurons.

Convulsion

Convulsion is the involuntary contraction or series of contractions of the

voluntary muscles. It occurs due to disturbances of the brain functions resulting
from abnormal excessive electrical discharge from the brain. It is manifested by
involuntary motor, sensory, autonomic or psychic phenomenon, alone or in
combination.
Incidence:

- Convulsions are more commonly found in infants and children.

- The overall incidence in childhood is about 8%.
- It is more commonly found along with cerebral palsy (35%) and mental
retardation (20%).
Causes of convulsive disorders in children

Neonatal period:

- Birth asphyxia, hypoxia, birth injury, IVH

- Hypoglycemia, hypocalcaemia, hypo or hypernatremia, hypomagnesaemia.
- Narcotic drug withdrawal, accidental injection of local anesthetic drug into
fetal scalp.
- Septicemia, Kernicterus, Meningitis, tetanus neonatorum.
- Congenital malformations-microcephaly, arteriovenous fistula.
- Pyridoxin deficiency, inborn errors of metabolism.
- Intrauterine infections such as TORCH infections.
In infants and young children:

- Febrile convulsions.
- CNS infections-meningitis, encephalitis, cerebral malaria, tetanus, Reye’s
syndrome and intrauterine infections.
- Post infections or Post-vaccinal encephalopathy-following acute viral
infections (mumps encephalopathy, measles encephalopathy), pertusis
vaccination, sub- acute sclerosing pan-encephalitis.
- Metabolic causes: Dehydration, alkalosis, dyselectrolytemia, hypocalcaemia,
hypomagnesaemia and inborn errors of metabolism.
 - Space occupying lesions in the brain: brain tumor, brain abscess,
tuberculoma, Cysticercosis.
- Traumatic: Accidental and non-accidental injury
- Vascular: Intracranial hemorrhage, DIC, arteriovenous malformations,
hypertension.
- Drugs and poisons: Phenothiazine, Diphenyl hydantoin salycilate, carbon
monoxide etc.
- Miscellaneous causes: heat stroke, acute brain swelling, poisoning, lead
encephalopathy, allergy, renal disease, breath holding spells, birth asphyxia,
degenerative disorders.
- Idiopathic epilepsy.
Febrile convulsions

Febrile convulsions refer to the seizures associated with fever but excluding
those related to CNS infections.

It is the commonest cause of convulsions in early childhood. It is related to

abrupt increase in body temperature rather than degree of temperature rise.

Types:

The febrile convulsions can be

- Simple benign typical type

- Atypical complex type

Simple benign typical febrile convulsions:

The fits occur within 24 hours of the onset of fever, last less than 10
minutes, usually single per febrile episode.
 Convulsions are generalized, a small proportion (4-18%) cases may show
focal convulsions. It is usually fund in children between 6 months and 5 years of
age.

Family history of convulsions is frequently present. Higher incidence occurs

in twins and children of consanguinous parents.

Atypical febrile convulsions:

They predispose to idiopathic epilepsy. The children may have focal

convulsions of more than 20 minutes duration even without significant fever.

They may have abnormal EEG for two weeks after the attack.

Management:

Febrile convulsions are managed by prompt reduction of temperature with

antipyretics or hydrotherapy.

The main goals of management are:

- To control convulsions
- To reduce increased body temperature
- To treat the cause of fever, usually ARI.
Medications:

- Anticonvulsive drugs are indicated in prolonged convulsions. Diazepam 0.3

mg/kg IV or 0.5 mg/kg per rectum or phenobarbitol 5 mg/kg IM can be
administered.
- Antipyretics (Paracetamol, Mefanamic acid) and tepid sponge should be
given to treat fever.
- Hydration and nutritional status to be maintained
 - Clearing of airways and oxygen therapy may be needed for some children
- Rest, comfortable position and hygienic measures to be provided.
- Explanation and emotional support to the parents are important along with
necessary health teaching.
Prevention of febrile convulsions:

- Antipyretics, hydrotherapy and temperature recording are essential for all

febrile children.
- Intermittent prophylaxis with antiepileptic drugs is indicated to prevent
recurrence of convulsive episodes.
- Continuous prophylaxis can be given in case of failure of intermittent
therapy or in atypical convulsions or in family history of epilepsy.
- Sodium valproate (10-20 mg/kg/day) or Phenobarbital (3-5 mg/kg/day) are
effective for febrile convulsion prophylaxis.
Prognosis:

- In typical febrile convulsions prognosis is good.

- Recurrence is more commonly found in younger age, females, presence of
risk factors and in atypical prolonged episodes.

EPILEPSY

Definition:

Epilepsy is recurrent, episodic, paroxysmal transient disturbances of brain

function due to abnormal electrical activity of the neurons.

It is manifested as abnormal motor, sensory or psychomotor phenomena and

often with impaired or loss of consciousness.
Incidence:

- One percent of all children have epilepsy with highest incidence in the
preschool years.
- Family history is commonly present with probable genetic predisposition.
Classification of epilepsy:

Clinically epilepsy can be broadly classified into two groups i.e. generalized
or partial.

Generalized Seizures:

a. Tonic-clonic seizures (grand mal)

b. Absence seizures –
i. typical (petitmal)
ii. Atypical
c. Atopic seizures
d. Myoclonic seizures
Partial seizures

a. Simple partial seizures

i) With motor signs (Jacksonians or focal motor)

ii) With somato- sensory or special sensory i.e. visual or auditory

iii) With autonomic manifestations

b. Complex partial seizures – Manifested with impaired consciousness and with

automatism. It includes psychomotor or temporal lobe seizures.

Patho physiology:
Prolonged depolarization

Brain cells become overactive

Discharge electrical energy in a sudden violent disorderly manner

Electrical energy spreads to adjacent areas of the brain and may jump to distant
areas of CNS

Seizures

Clinical manifestations:

Generalized tonic-clonic seizures (Grand mal type)

- It is most frequent form of childhood epilepsy.

- Onset is abrupt.
The classical form has four phases i.e. an aura, tonic clonic phase and
postictal phase.

i. Aura: A transitory premonitory symptom or aura heralds the onset of a

seizure. The child may recognize the impending seizure by the aura and
 adopt measures for self protection. Aura may be sensory, visceral, motor
or autonomic.
ii. Tonic phase: During this phase, skeletal muscles go into a sustained
spasm. Spasm of the laryngeal muscles forces the air in the lungs out
through a partially closed glottis resulting in a shrill cry.
- The muscular rigidity is most marked in the antigravity muscles, such as
flexors of arms and extensors of lower extremities.
- The child becomes unconscious and falls on the ground.
- The face appears pale, pupils are dilated and eyes are rolled either upward or
to the side.
- There is frothing from the mouth.
- Urine or stools may be passed involuntarily
- This phase lasts for about thirty seconds.
iii. Clonic phase: The clonic phase is characterized by rhythmic alternating
contractions of muscle groups, which persist for a few minutes to even a
few hours.
iv. Postictal phase: The child may complain of headache, confusion,
perform automatic actions of which he has little recollection later.
- Rarely the child develops a transient paresis.
- In children tonic clonic fits may not occur and may be predominantly tonic
or clonic.
- EEG shows generalized burst of spikes and irregular 4-6 Hz spike-wave
complex.
Absence seizures (Petitmal):

Absence seizures rarely appear before 5 years of age. It is manifested as the

following:
 - The child may lose contact with environment for a few seconds.
- The child may appear as staring or day dreaming.
- The child may discontinue the activity suddenly (eg. reading, writing) and
may resume the same activity when the seizure is over.
- Atypical absence seizure may present as rolling of the eyes, nodding of the
head, slight hand movements and smacking of lips.
- The child appears normal and not aware of having the episode in the post
seizure state.
- The duration is usually 5-10 seconds.
- The frequency varies from one to two per month to hundred times each day.
- The precipitating factors include hyperventilation, fatigue, hypoglycemia
and stress situation.
Status Epilepticus

Status epilepticus is condition where seizure lasts beyond 30 minutes or

seizures are repetitive, prolonged and the patient remains unconscious in
between the seizures. If the child becomes conscious in- between the attacks, it
is called status epilepticus.

Status epilepticus is a serious medical emergency with a mortality rate of

about 4 percent. During the attack the child may go into cardiac and respiratory
depression or arrest. The vomitus may be aspirated. Residual neurological deficits
occur in about 9 percent of cases and result from cerebral anoxia, hypoglycemia
and hyperthermia leading to cerebral necrosis. Damage may be related more to the
underlying etiology of status rather that to duration of seizures alone.

MANAGEMENT OF STATUS EPILEPTICUS

The child is rapidly assessed especially with respect to cardio respiratory status,
head injury, neuroinfections, drug noncompliance and etiology.

- Intravenous route should be established with 10 percent dextrose solution

- Best drug to use is lorazepam in a dose of 0.1 mg/kg by IV push and

repeated after 5 minutes. (Not available immediately means)

- Diazepam is given in a dose of 0.3 mg /kg /by IV push. Seizure may recur
after 15-30 minutes since half- life of diazepam is short.

- Diazepam is followed by phenytoin in a dose of 10- 15 mg /kg IV,

- If seizure continues an additional amount of 10mg /kg may be given.

- If fits are not controlled even with this, 20mg/kg of Phenobarbitone may be
given IV at a rate of 1mg/kg minute.

- Maintain adequate oxygenation, ventilation antiedema measures and care of

body temperature.

- Other therapeutic choices include paraldehyde, diazepam drip,

midazolam, and lidocaine.

- Necessary investigations and treatment of specific cause should be attended

simultaneously.

Partial seizures:

Partial seizures account for 60% of seizures in childhood.

The common causes are:

- Inflammatory granulomas
 - Atrophic lesions
- Birth asphyxia
- Head trauma
- Neoplasms
Partial seizures can be classified as

a. Simple partial seizures (20%) – without loss of consciousness, with motor,

sensory, autonomic or mixed symptoms.
b. Complex partial (25%) – With impairment of consciousness and
automatism, psychomotor or limbic system symptoms.
c. Partial with secondary generalization (15%).

Myoclonic seizures/ West’s syndrome (Infantile spasm)

This seizure occurs in infants usually between 3-8 months of life. It is

characterized by a combination of salaam spells (sudden droping of the head and
flexion of arms), mental retardation and hypsarrhythmic pattern EEG (diffuse high
voltage slow spike and chaotic cavity)

Causes: The common causes are

i. hypoxic ischemic encephalopathy

ii. neurocutaneous syndromes especially tuberous sclerosis
iii. perinatal infections
iv. hemorrhage
v. injury
vi. metabolic disorders
vii. localized structural malformations
Management:
Anti-epileptic drugs i.e. valproic acid, benzodiazepines.

Neonatal seizures:

Seizures are most frequent during neonatal life due to poor myelination and
incomplete dendritic arborization.

Neonatal seizures present as

a. Subtle
b. Multifocal clonic
c. focal clonic
d. generalized tonic
e. Myoclonic types
Subtle seizures may present with eye blinking, fluttering and buccolingual
movements. There may be pedaling or automatic movements.

The common cause of subtle seizures is perinatal asphyxia, sepsis and

bacterial meningitis.

- Focal clonic seizures are caused by metabolic disorders like hypoglycemia,

hypocalcemia and intracranial bleeding.
- Tonic and Myoclonic develops due to malformations and dysgenetic state.
- About one-third are multifactorial and idiopathic.
- Phenobarbitone (15 mg/kg IV) is the drug of choice.

Diagnosis of convulsive disorders

- Careful history with description of the convulsive episodes

- Detailed physical and neurological examination
 - Laboratory examination of blood , urine and CSF specially in the basis of
suspected cause
- electroencephalography [ EEG] is the most useful investigation
- Cranial imaging like X- ray skull, CT scan , PET or SPECT scan and MRI
- Metabolic or cytogenetic studies in case of suspected inborn error of
metabolism
Management of epilepsy\ convulsive disorders

Management depends upon the identified cause. The management mainly done is -
Drug therapy, diet therapy, surgical treatment if indicated. Emotional support,
psychosocial therapy, rehabilitation and vocational guidance are important aspect
of management.

Long term management may continue over 1 to 4 years and needs

supervision and explanation for treatment compliance.

DRUG THERAPY

The selection of antiepileptic drugs depends upon age, type of seizure and
economical status. The commonly used drugs are—

1) Phenobarbital- 3 to 5 mg/ kg/day in 1 or 2 divided doses, indicated in

Tonic –clonic, partial, akinetic and febrile convulsions.

2) Diphenyl hydantoin – 5 to 8 mg/kg/day in 2 divided doses indicated in tonic-

tonic, atonic, akinetic and partial seizures.
3) Carbamazepine – 10 to 20 mg/kg/day in 2 to 3 divided doses and indicated
in tonic- clonic, atonic, akinetic and partial seizures.
4) Diazepam – 0.2mg/kg/dose IV or per rectal is indicated in status epilepticus.
 5) Sodium valproate- 15 to 20mg/kg/day in 3 to 4 divided doses is indicated as
broad spectrum anticonvulsive agent
6) Ethosuximide- 10 to 20mg/kg/day in 2 divided doses is indicated in absence
seizure.
Duration of treatment may vary, usually 2 to 4 years, but mentally
retarded children may need longer duration

Side effects of the drugs should be observed and special precautions

to be followed during administration and thereafter.

Diet therapy

Ketogenic diet may be given to raise the seizure threshold with calculated
amount of proteins and fats without carbohydrates. This diet makes the child
ketotic as fat is used for energy production rather than carbohydrate. It seems
that ketones may inhibit the seizure.

The child should not be given IV fluid with dextrose and strict fluid
restriction to be maintained.

Surgical management

Neurosurgery is indicated in some cases of convulsive disorders, especially

in anatomical lesions like brain tumor, hematoma and in medically intractable
seizure disorders.

Possible surgical interventions include

- Corpus callostomy
- Focal resection of parts of cerebral cortex such as temporal lobe,
- Extra- temporal regions involved as epileptogenic foci.
Nursing management

Nursing Assessment:

- Detailed subjective and objective data to be collected

Nursing Diagnosis:

1. Ineffective breathing pattern related to spasms of respiratory muscles

2. Risk for injury related to convulsive episodes
3. Impaired self esteem related to lack of control over seizures.
4. Social isolations related to misconceptions
5. Knowledge deficit related to long term care of seizures disorder.
Nursing interventions

1. Ensuring safety during seizures

a) Provide preventive measures to protect the child from injury by
removal of hard objects, sharp things or toys from the child and
placing child on floor or bed
b) Side rails of the bed or crib to be padded
c) Removing oropharyngeal secretions by suctioning and turning head to
one side. Suction machine to be kept ready before hand.
d) Oxygen therapy to be given and all emergency equipments to be kept
ready to manage cardio respiratory problems.
e) Close observation and frequent monitoring of child’s condition for
vital signs, airway, breathing patterns, presence of aura, types of
movements during seizures, sites of contractions or twitching, eye
movements, pupil size, bladder incontinence, pallor/ cyanosis/
flushing, teeth clenched , tongue bite , frothy discharge / vomitus
 aspirations, level of consciousness , neurological status and postictal
events
f) Administering prescribed medications, IV/ IM or per rectal or oral as
indicated.
g) Special instructions about diet, rest and activities
2. Preventing respiratory arrest and aspiration
i) Loosen the clothing around neck and placing the child flat
ii) Avoid restraining the child and not to give anything in between
teeth or in the mouth, when the teeth are clenched during
convulsions.
iii) Clear airway, removes secretions, and turn head to one side during
seizures and on side lying position in postictal stage.
iv) Record the events in details.

3. Promoting socialization:
Instructing the parent to allow the child to perform normal life as possible
with some restricted activities like, not to climb high places, or to avoid
swimming and exertional activities. An identity card should be kept with the
child

4. Strengthening self esteem:

Explanation, reassurance, encouraging to discuss about feeling, promoting
independence in self care and family counseling are important for child and
parents to improve their self esteem.

5. Providing health teaching: Necessary related health teaching to be given

with special emphasis on continuation of medications, care during
 convulsions, diet therapy, restricted activities, misconception regarding the
disease and follow up.

CONCLUSION

Epilepsy is one of the most frequent, chronic neurologic disorders of

childhood. Some epileptics experience only two seizures in a life time, and others
may suffer hundreds of attacks daily. Management depends upon the identified
cause. The management mainly done is - Drug therapy, diet therapy, surgical
treatment if indicated. Emotional support, psychosocial therapy, rehabilitation and
vocational guidance are important aspect of management.

MENINGITIS
INTRODUCTION:

The one of most dreadful emergencies met within Peadiatric condition and
major cause for hospitalization of children with high mortality rate. The
Neurological disorder which is potentially fatal disease and may be a cause of
significant illness in Peadiatric population is condition known as meningitis.

DEFINITION

Meningitis is the inflammation of the meninges, the covering membrane of

the brain and spinal cord.

Classification:

- Pyogenic or bacterial meningitis

- Tuberculosis meningitis
 - Aseptic meningitis caused by virus, fungus or protozoa (toxoplasmosis,
amoebic)

PYOGENIC MENINGITIS (Bacterial meningitis)

Bacterial meningitis is caused by bacterial infections. Acute bacterial

meningitis is more common in neonates and infants due to immature immune
mechanism and poor phagocytic functions.

Etiology:

Infection may occur due to extension of local bacterial infections from sinusitis,

Mastoiditis or otitis media or from bacteremia through hematogenous spread.

- It may spread from pneumonia, empyema, pyoderma and osteomyelitis.

- Injury and penetrating wound

- Diagnostic procedures and surgical intervention

- Low immunodeficiency

- High risk neonates are vulnerable to neonatal meningitis

The causative organisms of meningitis in neonates are:

E-coli, Streptococcus Pneumoniae

- Streptococcus aureus

- Streptococcus fecalis

In infants and young children

H.Influenzae, S.Pneumoniae and meningococci

- Pneumococci is common beyond 3 years of age

- N.Meningitidis infection occurs in all ages.

- Meningococcus causes meningitis in epidemic.

Patho physiology:

Inflammatory cell infiltrate (leptomeninges)

Cortex becomes edematous and exudative with proliferation of microglia

Destruction of ependymal cells

Collection of purulent exudate at the base of the brain

Subarachnoid space is filled with a cloudy and opaque fluid and exudate

Blocking of foramina of lushka and magnedie

Internal hydrocephalus

Thrombophlebitis of cerebral vessels

Infarction and neurological complications

Tissue necrosis, Infarction and hydrocephalus

Permanent neurological damage

Fatal endotoxic shock may occur in cases of meningococcal meningitis.

Cerebral edema, change in composition of CSF, increased ICP and meningeal
irritation due to inflammatory process produce the clinical manifestations.

Clinical manifestations:
 - The onset is usually sudden with high fever, headache, malaise, vomiting,
restlessness, irritability and convulsions.
- The child will complain severe headache either diffuse or in the frontal
region spreading to the neck and eyeballs.
- Neonates present with insidious onset of refusal of feeds, high pitched shrill
cry, hypothermia, seizures, jaundice, lethargy and bulging fontanelle.
- Mental confusion with varying degree of alteration of level of
consciousness.
- Photophobia
- Generalized hypertonia and marked neck rigidity
- Cheyne-Stroke respiration with features of shock
On examination meningeal signs are found such as:

- Neck stiffness (difficult to flex due to spasm)

- Positive Kerning’s sign
- Positive Brudzinki’s sign
- Papilledema
- Neurological deficits like hemi paresis, cranial nerve palsies and
hemianopia.
Complications:

- Shock
- Myocarditis
- Status Epilepticus
- SIADH (Syndrome of Inappropriate ADH secretions)
- Subdural empyema or effusion
- Ventriculitis
- Arachnoiditis
 - Hydrocephalus and Brain abscess
- Convulsive disorders
- Mental retardation
- Neurological deficits like hemiplegia, blindness, speech problem, deafness
- Obesity and precocious puberty

Diagnostic evaluation:

- Clinical manifestations are important diagnostic clue

- The lumbar puncture and CSF study
 The CSF examination shows turbid appearance with increased cell
count mainly polymorph nuclear leukocytes.
 There is increased protein level – 100mg/dl and decreased sugar
below 40mg/dl in CSF.
 Elevation of CSF pressure
- CT Scan and X-ray skull and chest
- Rapid diagnostic tests (counter current Immuno Electro phoresis, latex
agglutination
- Non-specific tests like C-reactive protein, CSF-Lactic acid level
- Polymerase chain reaction
- Routine examination of blood and urine analysis.

Management:

- Specific antimicrobial therapy

 Penicillin 4-5 lac units/kg/day hourly
 Cefotaxime 200mg/kg/day 8 hourly IV
  Ceftriaxone 150mg/kg/day b.d
 Ampicillin, gentamycin, amikacin or chloramphenicol can be used
depending upon the causative organisms
 Antibiotics can be given intrathecal in neonatal meningitis and in
advanced cases.
- Corticosteroids, dexamethasone 0.15 mg/kg IV 6 hourly for 5 days is
recommended. This helps to reduce neurological complications
- Osmotic diuretic therapy is given with Mannitol (20%) 0.5 mg/kg every 4-6
hours IV for maximum 6 doses to reduce the increased ICP.
- Anticonvulsive drugs, diazepam 0.3 mg/kg is given to manage convulsions
like Phenobarbitone or phenytoin.
- Vasopressor (dopamine) is used in case of hypotension.

Other supportive management includes:

- Maintenance of fluid-electrolyte balance by IV fluid therapy

- Antipyretics
- Nasogastric tube feeding
- Vitamin supplementation
- Parent’s emotional support and necessary information for continuation of
care at home, follow-up and rehabilitation.

Prognosis:

- Prognosis depends upon initiation of treatment by early diagnosis

- Patient with bacterial meningitis show distinct improvement in 10 days with
appropriate antimicrobials agents. Some patients need more than 10-14 days.
 - Delayed management leads to serious neurological complications.
- With the use of newer antibiotics early diagnosis and treatment facilities
prognosis becomes favorable.

Nursing management:

- Provide rest and comfortable position in rail cot bed with calm quiet dim
lighted noise free environment.
- Change the position frequently
- Clear the airway by removing oropharyngeal secretion
- Oxygen therapy
- Tepid sponge in case of fever for reduction of body temperature.
- Maintenance of IV fluid therapy
- Naso-gastric tube feeding
- Dietary support and administration of prescribed medications
- Maintenance of personal hygiene (skin care, mouth care and eye care)
- Maintain proper bladder-bowel functions
- Prevent injury and monitor the patient condition continuously
- Provide care during convulsions and also teach the parents about
continuation of care after discharge
- Provide emotional support of parents

TUBERCULOUS MENINGITIS (TBM)

Tuberculous meningitis (TBM) is the inflammation of the meninges from

tubercular infection caused by mycobacterium tuberculosis.

It is a serious complication of childhood tuberculosis.

Incidence:

It may occur at any age but is most common between 6 and 24 months of
age, usually within a year of the primary infection with tuberculosis or may
accompany with miliary tuberculosis.

Path physiology:

Through Hematogenous route or intracranial lymphatic or cervical lymph nodes

Formation of submeningeal tubercular foci

Discharge tubercle bacilli into the subarachnoid space

Proliferation of bacilli

Perivascular exudation, caseation, gliosis and giant cell formation

Small tubercles scatterly develop over the convexity of the brain or per ventricular
area

Obliteration of Subarachnoid space and arachnoid villi

CSF is poorly absorbed

Increased amount of CSF and dilation of ventricles

Hydrocephalus

Inflammatory changes

Edema of brain
 Tubercular encephalopathy

Vascular occlusion

Infarction of brain tissue

Clinical manifestations:

- The onset of TBM is usually insidious

- The clinical course of illness may be divided into three stages

1. Prodormal stage: This is the first stage as illness and the stage of
invasion. It is presented with vague features like

 Low grade fever

 Anorexia, drowsiness, headache, vomiting
 Apathy, irritability
 Disturbed sleep, restlessness
 Constipation, loss of weight
 Photophobia
 Convulsions
2. The transitional stage: It is second stage of illness and also termed as
stage of meningitis.
  Increased ICP and meningeal irritation
 Positive Kerning’s sign
 Neck rigidity along with fever
 Bradycardia, drowsiness
 Delirium, headache, vomiting
 respiratory disturbance
 unconsciousness
 Increased muscle tone and convulsions
 Neurological deficits like monoplegia, hemiplegia, loss of sphincter
control
In infants

 Anterior fontanelle may be bulging

 Ocular paralysis, strabismus, Nystagmus and contracted pupils
3. The terminal or third stage: It is the stage of coma. This is manifested
by

Paralysis and coma.

o The child with fever, irregular respiration and bradycardia

o Pupils are dilated and fixed
o Ptosis and opthalmoplegia
o Hydrocephalus develops in small children and infants

Diagnostic Evaluation:

- History of illness in details with family history

- History of contact with TB cases
 - Physical examination
- The most important diagnostic procedure is CSF study by lumbar puncture
Increased CSF pressure, clear or slight turbid appearance of CSF, increased
cell count with presence of lymphocytes and cobweb formation in CSF.

- Identification of Tuberculous bacilli in the CSF smear and culture helps to

confirm diagnosis
- Biochemical study of CSF shows increased proteins but sugar and chlorides
are reduced.
- Other supportive investigations are
 chest X-ray to detect primary focus
 X-ray skull
 CT Scan
 Mantoux test
 Routine blood examination especially ESR and newer diagnostic
approaches like biochemical markers
 Serodiagnosis and molecular diagnosis
 Test for HIV infection for all suspected and risk patients.

Management:

- Antitubercular drugs should be given for 12 months

Initially four drugs are given with INH, rifampicin, pyrazinamide and
ethambutol or streptomycin for 2 months. In continuation phase, 3 drugs are
given with INH, rifampicin and ethambutol for 10 months.

- Parentral corticosteroid therapy with dexamethasone for 1-2 weeks is useful

to reduce cerebral edema and prevention of Arachnoiditis with fibrosis.
 - Later, oral steroids should be continued with prednisolone for 6-8 weeks and
then gradually tapered off.
- Other symptomatic management includes
 Mannitol/glycerol/hypertonic glucose therapy to reduce increased ICP
 Anticonvulsive drugs (diazepam, Phenobarbitone) to control
convulsions and IV fluid therapy to treat dyselectrolytemia and to
maintain fluid electrolyte balance
 Pyridoxine (20mg) orally once a day to be given to prevent side
effects of INH.

Complications:

- Hydrocephalus
- Mental retardation
- Spasticity
- Cranial nerve paralysis
- Convulsive disorders
- Neurological deficits ( hemiplegia and quadriplegia)
- Endocrinal disturbances
- Precocious puberty
- Obesity, bladder-bowel dysfunction
- Optic atrophy and visual complications
Prognosis:

- Early diagnosis, prompt and adequate treatment improves the prognosis

- The prognosis is poorer in younger children, malnourished children and
advanced cases
 - About 2/3rd of the TBM cases have long term sequelae. Relapse may occur
in incomplete Antitubercular drug therapy

Nursing management:

- Provide rest and comfortable position in rail cot bed with calm quiet dim
lighted noise free environment.
- Change the position frequently
- Clear the airway by removing oropharyngeal secretion
- Oxygen therapy
- Tepid sponge in case of fever for reduction of body temperature.
- Maintenance of IV fluid therapy
- Naso-gastric tube feeding
- Dietary support and administration of prescribed medications
- Maintenance of personal hygiene (skin care, mouth care and eye care)
- Maintain proper bladder-bowel functions
- Prevent injury and monitor the patient condition continuously
- Provide care during convulsions and also teach the parents about
continuation of care after discharge
- Measure the head circumference daily to detect hydrocephalus.
- Maintenance of nutritional requirements
- Monitor for features of complications
- Provide emotional support of parents
Conclusion

Meningitis is the inflammation of the meninges, the covering membrane of

the brain and spinal cord. The type of meningitis is Pyogenic or bacterial
meningitis, tuberculous meningitis, Aseptic meningitis. Bacterial meningitis is
caused by bacterial infections. Acute bacterial meningitis is more common in
neonates and infants due to immature immune mechanism and poor phagocytic
functions. Tuberculous meningitis (TBM) is the inflammation of the meninges
from tubercular infection caused by mycobacterium tuberculosis. It is a serious
complication of childhood tuberculosis.

ENCEPHALITIS

INTRODUCTION

Neurological disorders show a wide and more diverse spectrum in infancy

and childhood and then in adulthood. Among them encephalitis is one of the most
common disorder which develop due to direct infection or via hematogenous route.

DEFINITION:

Encephalitis is defined as an inflammatory process of the central nervous

system with dysfunction of the brain.

ETIOLOGY:

Encephalitis may be found in epidemics. In India some types of encephalitis

occur in sporadic or endemic form.

The vast majority of cases of encephalitis are due to viral infections.

I. The common viral agents are

- RNA viruses (Mumps, Measles, Rubella, Enterovirus

- DNA viruses (Herpes simplex, Cytomegalovirus, Epstein Barr, Equine

viruses)
 - Arthropod borne (Japanese B, West Nile, Russian spring summer,

Equine viruses)

- Rabies and lymphocytic choriomeningitis

- Dengue

II. The non-viral agents

- Rickettsia

- Fungi (Cryptococcus)

- Protozoa (Toxoplasma Gondii)

- Bacteria (Tuberculosis meningitis, Typhoid, Shigella, Leptospirosis)

- Reye’s syndrome

- Helminths (Cysticercosis, hydatid disease)

PATHOPHYSIOLOGY:

Diffuse cerebral edema, congestion and hemorrhages

Necrosis and degeneration of neurons

Meningeal congestion with mononuclear infiltration, Perivascular

tissue
 Necrosis and myelin breakdown

Glial proliferation

Type of infecting agent

Demyelization, vascular and Perivascular destruction and cerebral cortical

Involvement

In case of rabies and herpes simplex infection, specific inclusions are

identified. Characteristic pathological changes are found in Falciparum malasia.

CLINICAL MANIFESTATIONS:

The signs and symptoms of encephalitis depend on

I. Severity of infection

II. Susceptibility of the host

III. Localisation of the agent

IV. Presence of raised intracranial pressure

 - The features may be apparent or may be found as mild abortive type of
illness or

Severe encephalomyelitis

- The onset of illness is generally sudden but may at times be gradual

- Initial symptoms:

- High fever, headache, vomiting, mental confusion, irritability

- Apathy or loss of consciousness often associated with seizures

- Sudden severe rise of ICP may result in decerebration,

- Cardio-respiratory insufficiency, hyperventilation and autonomic

Dysfunction.

Clinical features include

-Increased Intracranial pressure

-Tense bulging fontanelle

-Distended scalp veins

-Papilledema with evidence of brain stem dysfunction

-Meningeal involvement and neurological deficits like occular palsies,

Hemiplegias are usually present

-Hyperventilation
 -Cheyne-stroke respiration and bradycardia

-Herniation of cerebellum through foramen magnum causes distortion and

Compression leading to respiratory or cardiac arrest.

DIAGNOSTIC EVALUATION:

- Careful health history and physical examination

- CSF study helps to differentiate the condition from meningitis

- Blood examination for sugar, urea, electrolytes and metabolic products

- Urine examination, toxicologic study and virological study

- CT Scan

MANAGEMENT:

Treatment aims

- To save life,

- prevent neurological residue,

- relieve symptoms and

-provide specific treatment of the cause.

Emergency treatment:

- Airway clearance is the prime important by positioning and frequent

suctioning.

- Oxygenation to be provided by nasal cannula or bag mask technique.

Mechanical ventilation is necessary in cardio-respiratory insufficiency.

- Hydrotherapy and antipyretics are administered to get relieved from

hyperpyrexia.

- IV fluid therapy and dopamine to be given to treat shock and fluid-electrolyte

Imbalance.

- Anticonvulsive drugs may be required to control convulsions.

- Mannitol or glycerol may be needed to reduce ICP.

- Steroid (dexamethasone) therapy when role of drug is not established.

- Antiviral (Acyclovir-30mg/kg/day in 3 divided doses)

- Specific detoxication therapy is given in lead or insecticide poisoning.

- Antibiotics to be prescribed to prevent secondary infections.

- Vitamin and mineral supplementation

- Human immunoglobulin (IVIG) may be administered 200-500 mg/kg in early

stage,

Which reduces mortality rate.

COMPLICATIONS:

- Shock

- Cardio-respiratory disorders

- Epilepsy and paralysis

- Cerebellar ataxia
 - Mental retardation

- Obesity and behavioral problems

PROGNOSIS:

- The prognosis is variable

- The patient may recover completely without any sequelae in case of minor
illness

- The illness may be self-limiting

- The mortality rate is usually high in complicated cases.

Nursing Management:

Nursing Assessment

- Detail health history

- Physical and neurological assessment along with review of diagnostic findings.

Nursing Diagnosis

1. Ineffective airway clearance related to upper airway obstruction as evidenced

by breathing difficulty.

2. Hyperthermia related to disturbance of brain functions as evidenced by

increased temperature.

3. Impaired tissue integrity of cornea related to diminished corneal reflex as

evidenced by difficulty in vision.

4. Risk for impaired skin integrity related to immobility.

5. Impaired nutritional status less than body requirement related to decreased food
intake.

6. Impaired urinary pattern (incontinence) related to unconsciousness.

7. Risk for injury related to convulsions and alteration of consciousness.

8. Ineffective family coping related life threatening condition.

Nurses role

- Special attention to be given on skin care.

- Assess for continuous bladder drainage.

- Change the position and monitor of patient’s condition with features of

complications.

- Provide care of eyes.

- Nasogastric tube feeding followed by gradual acceptance and intake of nutritional

diet.

CONCLUSION:

Encephalitis is an inflammatory process of the central nervous system with

dysfunction of the brain, may be found in epidemics. The vast majority of cases of
encephalitis are due to viral infections. Supportive and symptomatic management
is important for better prognosis of these conditions.

GUILLAIN-BARRÉ SYNDROME
Guillain-Barré syndrome is a postinfectious polyneuropathy involving mainly
motor but sometimes also sensory and autonomic nerves. This syndrome affects
people of all ages and is not hereditary. The disorder closely resembles
experimental allergic polyneuritis in animals. Most patients have a demyelinating
neuropathy, but primarily axonal degeneration is documented in some cases.
Clinical Manifestations.
The paralysis usually follows a nonspecific viral infection by about 10 days. The
original infection may have caused only gastrointestinal (especially Campylobacter
jejuni) or respiratory tract (especially Mycoplasma pneumoniae) symptoms.
Weakness begins usually in the lower extremities and progressively involves the
trunk, the upper limbs, and finally the bulbar muscles, a pattern known as Landry
ascending paralysis. Proximal and distal muscles are involved relatively
symmetrically, but asymmetry is found in 9% of patients. The onset is gradual and
progresses over days or weeks. Particularly in cases with an abrupt onset,
tenderness on palpation and pain in muscles is common in the initial stages.
Affected children are irritable. Weakness may progress to inability or refusal to
walk and later to flaccid tetraplegia. Paresthesias occur in some cases.
Bulbar involvement occurs in about half of cases. Respiratory insufficiency may
result. Dysphagia and facial weakness are often impending signs of respiratory
failure.
They interfere with eating and increase the risk of aspiration. The facial nerves
may be involved. Some young patients may exhibit symptoms of viral meningitis
or meningoencephalitis. Extraocular muscle involvement is rare, but in an
uncommon variant, oculomotor and other cranial neuropathies are severe early in
the course.
The Miller-Fisher syndrome consists of acute external ophthalmoplegia, ataxia,
and areflexia. Papilledema is found in some cases, although visual impairment is
not clinically evident. Urinary incontinence or retention of urine is a complication
in about 20% of cases but is usually transient. The Miller-Fisher syndrome
overlaps with Bickerstaff brainstem encephalitis, which also shares many features
with Guillain-Barré syndrome with lower motor neuron involvement and may
indeed be the same basic disease.
Tendon reflexes are lost, usually early in the course, but are sometimes preserved
until later. This variability may cause confusion when attempting early diagnosis.
The autonomic nervous system may also be involved in some cases. Lability of
blood pressure and cardiac rate, postural hypotension, episodes of profound
bradycardia, and occasional asystole occur. Cardiovascular monitoring is
important. A few patients require insertion of a temporary venous cardiac
pacemaker.
Prognosis.
The clinical course is usually benign, and spontaneous recovery begins within 2–3
wk. Most patients regain full muscular strength, although some are left with
residual weakness. The tendon reflexes are usually the last function to recover.
Improvement usually follows a gradient inverse to the direction of involvement,
with recovery of bulbar function first and lower extremity weakness resolving last.
Bulbar and respiratory muscle involvement may lead to death if the syndrome is
not recognized and treated. Although prognosis is generally good with the majority
of children recovering completely, three clinical features are predictive of poor
outcome with sequelae: cranial nerve involvement, intubation, and maximum
disability at the time of presentation. An electrophysiologic feature of conduction
block, by contrast, is predictive of good outcome.
Chronic relapsing polyradiculoneuropathy (sometimes called chronic
inflammatory demyelinating polyradiculoneuropathy) or chronic unremitting
polyradiculoneuropathy are chronic varieties of Guillain-Barré syndrome that
recur intermittently or do not improve for a period of months or years. About 7%
of children with Guillain-Barré syndrome suffer relapse. Patients are usually
severely weak and may have a flaccid tetraplegia with or without bulbar and
respiratory muscle involvement.
Congenital Guillain-Barré syndrome is described rarely, presenting as generalized
hypotonia, weakness, and areflexia in an affected neonate, fulfilling all
electrophysiologic and cerebrospinal fluid (CSF) criteria, and in the absence of
maternal neuromuscular disease. Treatment may not be required, and there is
gradual improvement over the first few months and no evidence of residual disease
by a year of age. In one case, the mother had ulcerative colitis treated with
prednisone and mesalamine from the 7th month until delivery at term.
Laboratory Findings and Diagnosis.
CSF studies are essential for diagnosis. The CSF protein is elevated to more than
twice the upper limit of normal, glucose level is normal, and there is no
pleocytosis.
Fewer than 10 white blood cells/mm3 are found. The results of bacterial cultures
are negative, and viral cultures rarely isolate specific viruses. The dissociation
between high CSF protein and a lack of cellular response in a patient with an acute
or subacute polyneuropathy is diagnostic of Guillain-Barré syndrome.
Motor nerve conduction velocities are greatly reduced, and sensory nerve
conduction time is often slow. An electromyogram shows evidence of acute
denervation of muscle. Serum creatine phosphokinase (CK) level may be mildly
elevated or normal. Antiganglioside antibodies, mainly against GM1 and GD1, are
sometimes elevated in the serum in Guillain-Barré syndrome, particularly in cases
with primarily axonal rather than demyelinating neuropathy, and suggest that they
may play a role in disease propagation and/or recovery in some cases. Muscle
biopsy is not usually required for diagnosis; specimens appear normal in early
stages and show evidence of denervation atrophy in chronic stages. Sural nerve
biopsy tissue shows segmental demyelination, focal inflammation, and wallerian
degeneration but also is usually not required for diagnosis.
Serologic testing for Campylobacter infection helps establish the cause if results
are positive but does not alter the course of treatment. Results of stool cultures are
rarely positive because the infection is self-limited and only occurs for about 3
days, and the neuropathy follows the acute gastroenteritis.
Treatment.
Patients in early stages of this acute disease should be admitted to the hospital for
observation because the ascending paralysis may rapidly involve respiratory
muscles during the next 24?hr. Patients with slow progression may simply be
observed for stabilization and spontaneous remission without treatment. Rapidly
progressive ascending paralysis is treated with intravenous immunoglobulin
(IVIG), administered for 2, 3, or 5 days. Plasmapheresis, steroids, and/or
immunosuppressive drugs are alternatives, if IVIG is ineffective. Combined
administration of immunoglobulin and interferon is effective in some patients.
Supportive care, such as respiratory support, prevention of decubiti in children
with flaccid tetraplegia, and treatment of secondary bacterial infections, is
important.
Chronic relapsing polyradiculoneuropathy or unremitting chronic neuropathy is
also treated with IVIG. Plasma exchange, sometimes requiring as many as 10
exchanges daily, is an alternative. Remission in these cases may be sustained, but
relapses may occur within days, weeks, or even after many months; relapses
usually respond to another course of plasmapheresis. Steroid and
immunosuppressive drugs are another alternative, but their effectiveness is less
predictable.
High-dose pulsed methylprednisolone given intravenously is successful in some
cases. The prognosis in chronic forms of the Guillain-Barré syndrome is more
guarded than in the acute form, and many patients are left with major residual
handicaps.
Even if Campylobacter jejuni infection is documented by stool culture or serologic
tests, treatment of the infection is not necessary because it is self-limited, and the
use of antibiotics does not alter the course of the polyneuropathy.

ANOMALIES OF NERVOUS SYSTEM

SPINA BIFIDA

Spina bifida (Latin: "split spine") the neural tube defect: incomplete closure of
the embryonic neural tube results in an incompletely formed spinal cord. In
addition, the bones of the spine (vertebrae) overlying the open portion of the spinal
cord do not fully form and remain unfused and open.

Definition

Spina bifida, which literally means “cleft spine,” is characterized by the

incomplete development of the brain, spinal cord, and/or meninges.

Incomplete closure of the embryonic neural tube results in an incompletely

formed spinal cord. In addition, the bones of the spine (vertebrae) overlying the
open portion of the spinal cord do not fully form and remain unfused and open.
This allows the abnormal portion of the spinal cord to protrude through the
opening in the bones

Spina bifida malformations fall into three categories: spina bifida occulta, spina
bifida cystica (myelomeningocele), and meningocele. The most common location
of the malformations is the lumbar and sacral areas of the spinal cord.
Myelomeningocele is the most significant form and is that which leads to disability
in most affected individuals. The terms spina bifida and myelomeningocele are
usually used interchangeably.

Spina bifida can be surgically closed after birth, but this does not restore normal
function to the affected part of the spinal cord and an individual with this condition
will have dysfunction of the spinal cord and associated nerves from the point of the
open defect and below. Intrauterine surgery for spina bifida has also been
performed and the safety and efficacy of this procedure is currently being
investigated

Incidence

Spina bifida is one of the most common birth defects, with an average worldwide
incidence of 1-2 cases per 1000 births, but certain populations have a significantly
greater risk.

In the United States, the average incidence is 0.7 per 1000 live births. The
incidence is higher on the East Coast than on the West Coast, and higher in whites
(1 case per 1000 live births) than in blacks (0.1-0.4 case per 1000 live births).
The highest incidence rates worldwide were found in parts of the British Isles,
mainly Ireland and Wales, where 3-4 cases of myelomeningocele per 1000
population have been reported during the 1970s, along with more than 6 cases of
anencephaly (both live births and stillbirths) per 1000 population. The reported
overall incidence of myelomeningocele in the British Isles is 2-3.5 cases per 1000
births. Since then, the rate has fallen dramatically with 0.15 per 1000 live births
reported in 1998.

Parents of children with spina bifida have an increased risk of having a second
child with a neural tube defect.

Symptoms and complications

These vary with the extent of the spinal defect, and differ between the subtypes
described below.

The most common location of the malformations is the lumbar and sacral areas of
the spinal cord. The lumbar nerves control the muscles in the hip, leg, knee and
foot, and help to keep the body erect. The sacral nerves control some of the
muscles in the feet, bowel and urinary bladder, and the ability to have an erection.
Some degree of impairment can be expected in these areas, resulting in varying
degrees of paralysis, absence of skin sensation, and poor or absent bowel and/or
bladder control, curvature of the spine (scoliosis), depending on the severity and
location of the lesion damage on the spine. Although these individuals are rarely
mentally retarded, in most cases there are cognitive problems.

Tethered Spinal Cord syndrome, with symptoms such as lower body pain, leg
weakness, incontinence, curvature of the spine (scoliosis), numbness, is a common
problem associated with spina bifida. Indeed 100% of spina bifida
myelomeningocele patients have Tethered Cord on imaging studies such as
Magnetic resonance imaging, but not all will develop symptoms. A tethered cord is
thought to result from scar tissue which forms following the initial surgery to close
the open defect. Symptoms caused by a tethered cord are rare in infancy and early
childhood. Once symptoms develop it is important to make the diagnosis early,
before permanent damage is done to the spinal cord and nerves.

According to the Spina Bifida Association of America (SBAA), over 73% of

people with spina bifida develop an allergy to latex, ranging from mild to life-
threatening. The common use of latex in medical facilities makes this a particularly
serious concern. The most common approach is to try to avoid development of the
allergy by avoiding contact with latex containing products such as examination
gloves, catheters (tubes used in the management of bladder dysfunction, inserted
through the urethra to evacuate urine), and many of the products used by dentists.

Presentation of the different types

Spina bifida occulta

Occulta is the mildest and most common form in which one or more vertebrae are
malformed Occulta is Latin for "hidden." This is one of the mildest forms of
spina bifida although the degree of disability can vary depending upon the
location of the lesion.

In occulta there is no opening of the back, but the outer part of some of the
vertebrae are not completely closed. The split in the vertebrae is so small that the
spinal cord does not protrude. The skin at the site of the lesion may be normal, or it
may have some hair growing from it; there may be a dimple in the skin, a lipoma, a
dermal sinus or a birthmark.
Many people with the mildest form of this type of spina bifida do not even know
they have it, or symptoms do not appear until later in life. People with spina bifida
occulta may suffer from a tethered cord (diastematomyelia), when the spinal cord
gets trapped below the affected level of the growing spine. This may cause
neurological problems of the legs and bladder.

A systematic review of radiographic research studies found no relationship

between spina bifida occulta and back pain. More recent studies not included in the
review support the negative findings.

However, other studies suggest spina bifida occulta is not always harmless. One
study found that among patients with back pain, severity is worse if spina bifida
occulta is present. Another study found that spina bifida occulta may predispose to
disk herniation.

Spina bifida cystica (myelomeningocele)

In this, the most serious form, the unfused portion of the spinal column allows the
spinal cord to protrude through an opening in the overlying vertebrae. The
meningeal membranes that cover the spinal cord may or may not form a sac
enclosing the spinal elements. Superficially, the cyst may resemble an unrelated
defect, sacrococcygeal teratoma. Spina bifida with myeloschisis is the most severe
form of spina bifida cystica. In this defect, the neural folds fail to meet and fuse
leaving the spinal cord open and the involved area represented by a flattened,
plate-like mass of nervous tissue with no overlying skin or membrane. The unfused
elements of the spinal cord can be surgically closed along with the overlying
muscle and skin shortly after birth (see treatment section below).
The incompletely closed portion of the spinal cord and the nerves which originate
at that level of the cord are damaged or not properly developed. As a result, there is
usually some degree of paralysis and loss of sensation below the level of the spinal
cord defect. Thus, the higher the level of the defect the more severe the associated
nerve dysfunction and resultant paralysis. People may have ambulatory problems,
loss of sensation, deformities of the hips, knees or feet and loss of muscle tone.
Depending on the location of the lesion, intense pain may occur originating in the
lower back, and continuing down the leg to the back of the knee.

Most children and adults with this condition experience problems with bowel and
bladder control since the nerves which control these functions originate at the
lowest part of the spinal cord. This may result in urinary incontinence from
neurogenic bladder.

Many individuals with spina bifida will have an associated abnormality of the back
of the brain, or cerebellum, called the Arnold Chiari II malformation. In affected
individuals the back portion of the brain is displaced from the back of the skull
down into the upper neck. In approximately 90% of the people with
myelomeningocele, hydrocephalus —extra fluid in the ventricles of the brain—
will also occur because the displaced cerebellum interferes with the normal flow of
cerebrospinal fluid.

The myelomeningocele (or perhaps the scarring due to surgery) tethers the spinal
cord to the enveloping vertebra. In some individuals this causes significant traction
on the spinal cord and can lead to a worsening of the paralysis, curvature of the
spine, also known as scoliosis, back pain, or worsening bowel and/or bladder
function.
Meningocele

The least common form of spina bifida is a posterior meningocele (or meningeal
cyst).

In a posterior meningocele, the outer faces of some vertebrae are open (unfused)
and the meninges are damaged and pushed out through the opening, appearing as a
sac or cyst which contains cerebrospinal fluid. The spinal cord and nerves are not
involved and their function is normal.

In an anterior meningocele, the inner faces of vertebrae are affected and the cyst
protrudes into the retroperitoneum or the presacral space.

Apart from spina bifida, causes of meningocele include teratoma and other tumors
of the sacrococcyx and of the presacral space, and Currarino syndrome. Usually a
meningocele has no negative long-term effects, although there are reports of
tethered cord.

Closed neural tube defects make up the second type of spina bifida. This form
consists of a diverse group of spinal defects in which the spinal cord is marked
by a malformation of fat, bone, or membranes. In some patients there are few
or no symptoms; in others the malformation causes incomplete paralysis with
urinary and bowel dysfunction.

Etiology

 The exact cause of Spina bifida remains a mystery. No one knows what
disrupts complete closure of the neural tube, causing a malformation to
develop.

 Scientists suspect genetic, nutritional, and environmental factors play a

role.

 Research studies indicate that insufficient intake of folic acid—a common B

vitamin
Pathophysiology

Spina bifida is caused by the failure of the neural tube to close during the first
month of embryonic development (often before the mother knows she is pregnant).

Normally the closure of the neural tube occurs around the twenty-eighth day after
fertilization.[3] However, if something interferes and the tube fails to close properly,
a neural tube defect will occur. Medications such as some anticonvulsants,
diabetes, having a relative with spina bifida, obesity, and an increased body
temperature from fever or external sources such as hot tubs and electric blankets
can increase the chances a woman will conceive a baby with a spina bifida.
However, most women who give birth to babies with spina bifida have none of
these risk factors, and so in spite of much research, it is still unknown what causes
the majority of cases.

The varying prevalence of spina bifida in different human populations and

extensive evidence from mouse strains with spina bifida suggests a genetic basis
for the condition. As with other human diseases such as cancer, hypertension and
atherosclerosis (coronary artery disease), spina bifida likely results from the
interaction of multiple genes and environmental factors.

Research has shown that lack of folic acid (folate) is a contributing factor in the
pathogenesis of neural tube defects, including spina bifida. Supplementation of the
mother's diet with folate can reduce the incidence of neural tube defects by about
70%, and can also decrease the severity of these defects when they occur. [10][11][12]
As yet it is unknown how or why folic acid has this effect.

Spina bifida does not follow direct patterns of heredity like muscular dystrophy or
haemophilia. Studies show that a woman who has had one child with a neural tube
defects such as spina bifida, have about a 3% risk to have another child with a
neural tube defect. This risk can be reduced to about 1% if the woman takes high
doses (4 mg/day) of folic acid before and during pregnancy. For the general
population, low-dose folic acid supplements are advised (0.4 mg/day).

Signs and symptoms of Spina bifida

 The symptoms of Spina bifida vary from person to person, depending on the
type. Often, individuals with occulta have no outward signs of the disorder.

 Closed neural tube defects are often recognized early in life due to small dimple
or birthmark on the skin at the site of the spinal malformation.

 Meningocele and myelomeningocele generally involve a fluid-filled sac visible

on the back protruding from the spinal cord.
 In Meningocele, the sac may be covered by a thin layer of skin, whereas in most
cases of myelomeningocele, there is no layer of skin covering the sac and a
section of spinal cord tissue usually is exposed.

Diagnosis

In most cases, Spina bifida is diagnosed prenatally, or before birth. However,

some mild cases may go unnoticed until after birth, or postnatally. Very mild
cases, in which there are no symptoms, may never be detected.

1. Prenatal Diagnosis

 Maternal serum alpha fetoprotein (MSAFP) screening, finding high levels of

AFP may indicate that the disorder is present.

 Fetal ultrasound.

 The MSAFP screen measures the level of a protein called AFP. During
pregnancy, a small amount of AFP normally crosses the placenta and enters the
mother’s bloodstream. But if abnormally high levels of this protein appear in
the mother’s bloodstream it may indicate that the fetus has a neural tube defect.
The MSAFP test, however, is not specific for Spina bifida.

 Amniocentesis—an exam in which the doctor removes samples of fluid from

the amniotic sac that surrounds the fetus may also be used to diagnose spina
 bifida. Although amniocentesis cannot reveal the severity of Spina bifida
Postnatal Diagnosis

 Mild cases of Spina bifida not diagnosed during prenatal testing may be
detected postnatally by X-ray during a routine examination. Doctors may use
magnetic resonance imaging (MRI) or a computed tomography (CT) scan to get
a clearer view of the spine and vertebrae. Individuals with the more severe
forms of Spina bifida often have muscle weakness in their feet, hips, and
legs. If hydrocephalus is suspected, the doctor may request a CT scan
and/or X-ray of the skull to look for extra fluid inside the brain.

Treatment

There is no cure for nerve damage due to spina bifida. To prevent further damage
of the nervous tissue and to prevent infection pediatric neurosurgeons operate to
close the opening on the back. During the operation, the spinal cord and its nerve
roots are put back inside the spine and covered with meninges, muscle and skin. In
addition, a shunt may be surgically installed to provide a continuous drain for the
cerebrospinal fluid produced in the brain, as happens with hydrocephalus. Shunts
most commonly drain into the abdomen.

Most individuals with myelomeningocele will need periodic evaluations by

specialists including orthopedists to check on their bones and muscles,
neurosurgeons to evaluate the brain and spinal cord and urologists for the kidneys
and bladder. Such care is best begun immediately after birth. Most affected
individuals will require braces, crutches, walkers or wheelchairs to maximize their
mobility. The higher the level of the spina bifida defect the more severe the
paralysis. Thus, those with low levels may need only short leg braces while those
with higher levels do best with a wheelchair. Many will need to manage their
urinary system with a program of catheterization. They will need to insert a tube
into their bladders to drain urine, of which the intervals vary from person to
person, and may need medications to improve their dryness. Most will also require
some sort of bowel management program.

Clinical trial

Management of Myelomeningocele Study (MOMS)[13] is a phase III clinical trial

to evaluate the safety and efficacy of fetal surgery to close a myelomeningocele.
This involves surgically opening the pregnant mother's abdomen and uterus to
operate on the fetus. Fetal skin grafts are used to cover the exposed spinal cord, to
protect it from further damage caused by prolonged exposure to amniotic fluid.
The fetal surgery may decrease some of the damaging effects of the spina bifida.

Pregnant women who consent to participate and meet all eligibility criteria are
referred to one of three centers in the United States, located in California,
Tennessee, and Pennsylvania. The centers provide final evaluation, randomization,
surgery and follow-up assessments. Participants' travel, lodging, relevant medical
and surgical procedures, and related costs are covered by the clinical trial, not by
the participants' medical insurance.

Major Eligibility Criteria:

 Gestational age at randomization: 19 – 25 weeks

 Myelomeningocele defect must start at: T1-S1 (may extend below S1)
 Fetal hindbrain herniation (Arnold-Chiari malformation type II)
 Maternal Body Mass Index < 35
 United States resident

Prevention
There is no single cause of spina bifida nor any known way to prevent it entirely.
However, dietary supplementation with folic acid (folate) has been shown to be
helpful in preventing spina bifida (see above). Sources of folic acid include: whole
grains, fortified breakfast cereals, dried beans, leaf vegetables and fruits.[14]

Folate fortification of enriched grain products has been mandatory in the United
States since 1998. The FDA and UK recommended amount of folic acid for
women of childbearing age and women planning to become pregnant is at least 0.4
mg/day of folic acid from at least three months before conception, and continued
for the first 12 weeks of pregnancy. [15] Women who have already had a baby with
spina bifida or other type of neural tube defect, or are taking anticonvulsant
medication should take a higher dose of 4–5 mg/day.[15]

Recent (as of 2007) research has shown that there is a specific gene that causes
spina bifida, allowing parents to identify embryos that may develop the condition.
[16]

Pregnancy screening

Neural tube defects can usually be detected during pregnancy by testing the
mother's blood (AFP screening) or a detailed fetal ultrasound. Spina bifida may be
associated with other malformations as in dysmorphic syndromes, often resulting
in spontaneous miscarriage. However, in the majority of cases spina bifida is an
isolated malformation.

Genetic counseling and further genetic testing, such as amniocentesis, may be

offered during the pregnancy as some neural tube defects are associated with
genetic disorders such as trisomy 18. Ultrasound screening for spina bifida is partly
responsible for the decline in new cases, because many pregnancies are terminated
out of fear that a newborn might have a poor future quality of life. With modern
medical care, the quality of life of patients has greatly improved

Prognosis
Children with Spina bifida can lead relatively active lives. Prognosis depends on
the number and severity of abnormalities and associated complications. Most
children with the disorder have normal intelligence and can walk, usually with
assistive devices. If learning problems develop, early educational intervention is
helpful

Nursing Management:

Assessment:

1. Assess sensory and motor response of lower extremities

2. Assess ability to void spontaneously, retention of urine, and symptoms of
UTI.
3. Assess usual stooling patterns, need for medication to facilitate elimination.
4. Assess mobility and care of braces, casts and other special equipments.

Nursing Diagnosis:

Pre-operative care (Neonatal period)

1. Risk for impaired skin integrity related to impaired motor and sensory
function.
2. Risk for infection related to contamination of the myelomeningocele site.
3. Altered urinary elimination related to neurological deficits.
4. Altered cerebral tissue perfusion related to potential hydrocephalus.
5. Fear (parents) related to neonate with neurological disorder and surgery.
Post-operative care (Infancy and childhood)

1. Ineffective thermoregulation following surgery

2. Reflex incontinence related to sacral denervation.
3. Bowel incontinence/constipation related to impaired innervations of anal
sphincter and bowel musculature.
4. Body image disturbance related to the child’s appearance difficulties with
locomotion and lack of control over excretory functions
Prevention

 Folic acid, an important vitamin in the development of a healthy fetus.

Although taking this vitamin cannot guarantee having a healthy baby, it can
help. Recent studies recommended that all women of childbearing age
consume 400 micrograms of folic acid daily.

 Foods high in folic acid include dark green vegetables, egg yolks, and some
fruits. Many foods—such as some breakfast cereals, enriched breads, flours,
pastas, rice, and other grain products—are now fortified with folic acid. A lot
of multivitamins contain the recommended dosage of folic acid as well.

 Folate fortification of enriched grain products has been mandatory. The FDA
and UK recommended amount of folic acid for women of childbearing age and
women planning to become pregnant is at least 0.4 mg/day of folic acid from at
least three months before conception, and continued for the first 12 weeks of
pregnancy.

 Women who have already had a baby with spina bifida or other type of neural
tube defect, or are taking anticonvulsant medication should take a higher dose
of 4–5 mg/day.

 Recent (as of 2007) research has shown that there is a specific gene that causes
spina bifida, allowing parents to identify embryos that may develop the
condition.

Pregnancy screening

- Neural tube defects can usually be detected during pregnancy by testing the
mother's blood (AFP screening) or a detailed fetal ultrasound. Spina bifida may be
associated with other malformations as in dysmorphic syndromes, often resulting
in spontaneous miscarriage. However, in the majority of cases Spina bifida is an
isolated malformation.

- Genetic counseling and further genetic testing, such as amniocentesis, may be

offered during the pregnancy as some neural tube defects are associated with
genetic disorders such as Trisomy 18. Ultrasound screening for Spina bifida is
partly responsible for the decline in new cases, because many pregnancies are
terminated out of fear that a newborn might have a poor future quality of life. With
modern medicall care, the quality of life of patients has greatly improved
Complications

 Complications of Spina bifida can range from minor physical problems to

severe physical and mental disabilities.

 Nerve damage and loss of muscle function (paralysis) and sensation.

 Chiari II malformation—a rare condition (but common in children with

myelomeningocele) in which the brainstem and the cerebellum, or rear portion
of the brain, protrude downward into the spinal canal or neck area. This
condition can lead to compression of the spinal cord and cause a variety of
symptoms including difficulties with feeding, swallowing, and breathing;
choking; and arm stiffness.

 Chiari II malformation may also result in a blockage of cerebrospinal fluid,

causing a condition called hydrocephalus, which is an abnormal buildup of
cerebrospinal fluid in the brain.

 Some newborns with myelomeningocele may develop meningitis, an infection

in the meninges. Meningitis may cause brain injury and can be life-
threatening.

 Additional problems such as latex allergies, skin problems, gastrointestinal

conditions, and depression may occur as children with spina bifida get
older.

Health education to the parents:

- Prepare the parents to feed, hold and stimulate the infant as naturally as
possible.
- Teach the parents the special techniques that may be required for holding
and positioning, feeding, caring for the incision, emptying the bladder and
exercising muscles.
- Alert the parents to safety needs of the child with decreased sensation, such
as protection from prolonged pressure, the risk of burns due to bath water
that is too warm and avoidance of trauma from contact with sharp objects.
- Urge continued follow-up and health maintenance including immunization
and evaluation of growth and development.
- Advice parents that children with paralysis are at risk for becoming
overweight due to inactivity, so they should provide a low-fat, balanced diet,
control snacking and encourage as much activity as possible.
 NEURAL TUBE DEFECTS

Introduction:

Neural tube defects are responsible for overwhelming developmental

anomalies of the CNS. They result from a failure of the neural tube to close in
utero between third and fourth week of gestation.

Meaning of Neural tube defect:

Any type of congenital malformations involving defects in the skull and

spinal column that are caused primarily by the failure of the neural tube to close
during embryonic development.

Incidence:

Risk of defects is 1-5 in 1000 live births. Risk in subsequent pregnancies is

as high as 50 in 1000 live births.

Causes/etiology:

- Primarily disclosure of the neural tube during embryonic development.

- Factors as maternal radiation, drugs (valporic acid) and chemicals.

- Malnutrition (Folic acid deficiency) and genetic determinates alone or in

one or the other combination adversely affect the normal development of the
neural tube there by causing the defect.
Types of Neural tube defect:

1. Spina bifida occulta

It is a defective closure of the posterior arch and laminae of the vertebrae

usually L5 and S1. It is the most frequent and most benign neural tube defect.

Clinical manifestations:

- Most of the cases are asymptomatic.

- Occasional cases: - Loss of bladder and bowel control

-Cavus deformities of feet develop as the child grows.

2. Meningocele

It is a fluctuant midline sac of meninges that herniates through a defect in

the posterior vertebral arch, generally in the lower back (sacrum).

Clinical manifestations:

- Usually it is asymptomatic and well covered with skin.

- Skin covering is thin or CSF leak is present.

3. Meningomyelocele (Myolomeningocele)

It is a midline cystic sac of meninges and spinal tissue that herniates through
a defect in the posterior vertebral arch, usually in the lumbosacral region though it
may be located anywhere along the neuroaxis.

Clinical manifestations:

- It is covered with only a thin skin.

- Usually accompanied by a neurological deficit (flaccid paralysis, absent
drop reflexes and absent sensations)
- Postural abnormalities as club foot and dislocation of the hips.
- Hydrocephalus is a common association.
- Risk of rupture of the sac with super added infection and meningitis is high.

4. Encephalocele

It is a meningeal sac together with cerebral cortex, cerebellum or portions of

the brain stem herniating through a bony defect in the skull (cranium bifidum)
usually in the occipital region.

Clinical manifestations:

- The size may vary from small to as big as it exceeds the cranium.
- High risk of developing hydrocephalus due to aqueduct stenosis and Dandy
walker syndrome.
 - Meckel-Gruber syndrome – It is association of occipital Encephalocele with
cleft lip or cleft palate, microcephaly, abnormal genitalia, congenital
nephrosis and polydactyl.
Diagnostic Evaluation:

Prenatal diagnosis is possible by estimation of Alpha-feto protein level and

biparietal diameter on ultrasonography.

Prognosis:

Prognosis is poor; most of the patients develop visual problems, seizures,

mental retardation and microcephaly.

5. Anencephaly

It is a rudimentary brain with a large defect of the calvarium meninges and

scalp. Death usually occurs within a week or two weeks of birth.

6. Diastematomyelia

It is a projection of bony or fibro cartilaginous septum from the posterior

vertebral body that divides the spinal cord into two halves. The commonest site is
L1, L2 and L3.

Clinical manifestations:

- Presence of a localized midline, tuff of hair

- Dermal sinus, hemangioma
- Weakness and muscle atrophy in lower limbs and urinary incontinence
- Excision of the bony spur and lysis of surrounding adhesions is indicated in
symptomatic cases.
7. Syringomelia

It is a cystic cavity within the spinal cord. It may communicate with the CSF
pathway (syringe bulbia).

Types of Syringomelia:

I. Communicating Syringomelia: It is usually complicated by chiari type I

malformation. In it CSF passes caudally on sneezing or coughing producing
dilatation of the central canal.
ii. Non communicating Syringomelia: It is complicated by cord tumors, trauma,
vascular accidents and Arachnoiditis.

Clinical manifestations:

- Syringomelia progresses slowly, producing symptoms in later childhood or

adulthood.
- Progressive scoliosis
- Dissociation of sensations (loss of pain, temperature, preservation of light
touch)
- Tropic ulcers
- Muscle wasting
- Absent doop reflexes in upper limbs and upper motor neuron signs in lower
limbs.
Management:

Treatment includes decompression, plugging the open end of the central

canal, percutaneous aspiration and draining of the cystic cavity into Subarachnoid
space.

8. Schizencephaly: It denotes presence of clefts with in the cerebral hemisphere

which may be fused or unfused unilateral or bilateral.

Clinical manifestations:

- Severe mental retardation – seizures

- Microcephaly and spastic quadriparesis.
Diagnosis:

CT scan is diagnostic in delineating the size and shape of the cleft.

9. Porencephaly

Presence of cavities or cysts within the brain in the region of sylvian fissure.
They communicate with Subarachnoid space, the ventricular system or both.

Clinical manifestations:

- Microcephaly
- Mental retardation
- Seizures
- Quadriparesis and optic atrophy
10. Mobius syndrome

Agenesis of cranial nerves II, III, V, VIII, IX, X, XI and XII nerves. There is
facial weakness (bilateral) and feeding difficulty presenting in neonatal period.

VI. Diagnostic Evaluation:

Prenatal diagnosis

a. Amniocentesis for alpha feto protein and acetyl cholinesterase in the

amniotic fluid in early pregnancy has accuracy of 97%.
b. Estimating alpha-fetoprotein level in the maternal blood between 14-16
weeks of gestation has accuracy of 60-70%.
c. Ultrasound pick up rate is around 100% i.e., ultrasound of head and sac.
d. X-ray spine and CVP
e. Culture from lesion and draining CSF
f. Complete blood counts, serum electrolytes and blood for cross match.
VII. Management:

Management of neural tube defects requires a team approach with the co-
operation of pediatrician, neurologist, neuro surgeon, urologist and orthopaedic
surgeon with assistance from physiotherapist, social worker and psychiatrist.

- Parents should be counselled actively

- The degree of paralysis, presence of hydrocephalus, kyphosis, congenital
malformations, evidence of infections of nervous system influence
decisions.
a. Pre-operative management:

- Keep the child nil per orally and maintain temperature.

- Cover the lesions by sterile saline moist dressings
- Nurse the baby prone or lateral
- Rule out other cardiac pulmonary, genito – urinary and gastro-intestinal
malformations.
b. Surgical management:

- Early closure prevents neurological deterioration

- Open lesions draining CSF should be closed within 24 hours and closed
lesions within 48 hours of birth.
- If the lesion is infected, administer parentral and intra-thecal antibiotics.
This is to be followed by surgery.
I. Lorber’s criteria:

Surgery is not done if there is

- Severe paraplegia at or above L3

- Kyphosis or scoliosis
- Gross hydrocephalus
- Associated gross congenital anomalies
- Intra cerebral birth injuries
- Pre-existing Ventriculitis

Nursing Management:

Assessment:

5. Assess sensory and motor response of lower extremities

6. Assess ability to void spontaneously, retension of urine, and symptoms of
UTI.
7. Assess usual stooling patterns, need for medication to facilitate elimination.
8. Assess mobility and care of braces, casts and other special equipments.
Nursing Diagnosis:

Pre-operative care (Neonatal period)

6. Risk for impaired skin integrity related to impaired motor and sensory
function.
7. Risk for infection related to contamination of the myelomeningocele site.
8. Altered urinary elimination related to neurological deficits.
9. Altered cerebral tissue perfusion related to potential hydrocephalus.
10.Fear (parents) related to neonate with neurological disorder and surgery.
Post-operative care (Infancy and childhood)

5. Ineffective thermoregulation following surgery

6. Reflex incontinence related to sacral denervation.
7. Bowel incontinence/constipation related to impaired innervations of anal
sphincter and bowel musculature.
8. Body image disturbance related to the child’s appearance difficulties with
locomotion and lack of control over excretory functions.
VIII. Complications:

- Delay in intervention may result in worsening of neurologic deficit.

 - Local or Ventriculitis infection and progressive hydrocephalus
- Late complications include:
- Urinary tract problems

- Fecal incontinence or constipation

- Congenital dislocation of hip

- Sexual dysfunction

- Intellectual deterioration

IX. Prognosis:

- Epilepsy in 10-30%
- Occular problems in 30%
- Shunt infection in 25%
- Psycho-social problems
- 2% die during initial hospitalization and 15% by ten years of age.

X. Prevention:

- Primary prevention includes folic acid supplementation (400mg) to all

prospective mothers i.e., all married women between 18-44 years of age.
- Counseling of family with a previous child with neural tube defect is
essential
- Advise peri-conceptional folic acid (5mg/day) and offer prenatal diagnosis
in mothers who have previously given birth to a child with neural tube
defect. Minimum duration of supplementation is 2 months before and 3
months after conception. It reduces the recurrence risk by 70%.

XI. Health education to the parents:

- Prepare the parents to feed, hold and stimulate the infant as naturally as
possible.
- Teach the parents the special techniques that may be required for holding
and positioning, feeding, caring for the incision, emptying the bladder and
exercising muscles.
 - Alert the parents to safety needs of the child with decreased sensation, such
as protection from prolonged pressure, the risk of burns due to bath water
that is too warm and avoidance of trauma from contact with sharp objects.
- Urge continued follow-up and health maintenance including immunization
and evaluation of growth and development.
- Advice parents that children with paralysis are at risk for becoming
overweight due to inactivity, so they should provide a low-fat, balanced diet,
control snacking and encourage as much activity as possible.

HYDROCEPHALUS

Introduction:

Hydrocephalus is characterized by dilation of the cerebral ventricles, increased

CSF is predominantly (80-90%) formed by the choroid plexus of the lateral, third
or fourth ventricles by an active transport process across the endothelium of
capillaries in the villus process of the choroid plexus. The arachnoid villus is the
primary site of CSF absorption.

Meaning:

The Greek term ‘hydrocephalus’ literally means water logging of the head, refers
to the enlargement of the head as a result of abnormally high accumulation of CSF
in the intracranial spaces.

Incidence:

It occurs approximately 3-4 cases per 1000births including those associated with
spina bifida.

Etiology:

- Increased production (obstructive hydrocephalus) eg. pseudotumor cerebri,

choroid plexus papilloma.
 - Obstruction to the flow (communication hydrocephalus) eg.Inflammatory
adhesions, developmental obstructive lesions.
- Interference with absorption eg. cavernous sinus thrombosis
- Clinically the causes are:
1. Congenital hydrocephalus:

i. Arnold-chiari malformation: In which there is a displacement of the brain

stem and cerebellum through foramen magnum, into upper cervical part of
the spine. It is generally associated with spina bifida and meningomyelocele.

ii. Dandy-walker anomaly: In which congenital septa or membrane block the

outlet of the fourth ventricle.

iii. Malformations or stenotic lesions of aqueduct cerebri.

iv. Malformations of arachnoid villi.

2. Acquired hydrocephalus:

i. Inflammatory: Meningitis occasionally encephalitis in first few months of

life.

ii. Traumatic: Birth trauma, head injury, intracranial hemorrhage.

iii. Neoplastic: Space occupying lesions like tuberculoma, subdural

hematoma or abscess gliomas etc.

iv. Chemical: Hypervitaminosis A

v. Connective tissue disorders: Achondroplasia.

Types of hydrocephalus:
1. Congenital hydrocephalus:

It is present right at birth or becomes apparent in the first few months of life.

2. Acquired hydrocephalus:

Develops later in association with or as a sequele to the causative factor.

3. Types depending on the site of obstructions

a. Communicating hydrocephalus:

The ventricular system is patent and the site of block is in the basal
subarachnoid

sulci or at the arachnoid villi . Meningitis is the most common cause of

communicating hydrocephalus.

b. Non-communicating hydrocephalus:

There is a blockage of CSF pathway at or proximal to the outlet foramina of

the

fourth ventricle the cause is usually congenital.

c. Arrested hydrocephalus:

A large proportion of both congenital and acquired hydrocephalus may

undergo

spontaneous arrest and may not require active surgical intervention.

Pathophysiology:
Ventricular system becomes distended and dilated

Increased ventricular prssure

thinning of cerebral cortex and cranial bones

ependymal lining of ventricles is disrupted

periventricular ooze

Subependymal edema occurs and white matter is compressed

Downward bulging of third ventricle compresses the optic nerves and hypophysis
cerebri with dilation of sella turcica
Atrophy of choroid plexus and cortex occurs

Clinical manifestations:

These depend on age at onset, duration and severity.

Early onset (0-2 years):

- 50% of children may be asymptomatic.

- Vomiting, drowsiness, failure to thrive, shrill cry.
- Delayed motor milestones are common symptoms
- Progressive increase in head circumference
- Head shape is abnormal and overshadows face
- Frontal bossing and triangular facias
- The skin of scalp is shiny and tense with dilated veins and sparse hair
- The anterior fontanelle is open, large and non-pulsatile
- Open squamo-parietal suture beyond the first month is an early sign of
hydrocephalus
- Ocular signs such as 6th nerve palsy
- Sunset sign, ptosis and nystagmus may be present
- Spasticity of lower limbs due to compression of periventricular white matter
may develop
- Pseudo bulsar palsy may be present and results in regurgitation, dysphonia
and stridor
Late onset (2-10 years):
 - Papilledema, headache and vomiting are usual presenting features
- Increasing head size is present in only 605
- McEwen’s sign (crack pot resonance on percussion of the skull)
- Psychomotor retardation and gait anomaly in 30% and epilepsy in 20%

Diagnostic evaluation:

- Physical examination and neurological assessment

- Accurate serial recording of the head circumference is helpful to diagnose
the condition
- Increase in head circumference in first 3 months of life more than 1 cm
every fortnight should arouse suspicion of hydrocephalus
- Persistent widening of squamo-parietal sutures should arouse suspicion of
hydrocephalous
- Positive transillumination of infant head, typical cracked pot sound
(Macewen’s sign) of the skull bone
- Ophthalmoscopy
- MRI, CTScan, cranial ultrasonography and X-ray skull is necessary to
determine the site of obstruction and in congenital hydrocephalus to identify
associated malformations.

Management:

Main goals are

1. decreasing intracranial pressure to safe level

2. Maximizing the potential for neurological development by preserving brain
parenchymal thickness.
Medical management:

Medical therapy requires careful follow-up to assess the ventriculomegaly,

mental and cognitive development and fundus changes.

1. Carbonic anhydrase inhibitors like Acetazolamide (Diamox) in doses of

50mg/kg/day to reduce CSF production in slow progressive hydrocephalus.
2. Oral glycerol and Isosoribide is also used to reduce CSF production.
Surgical Management:

Hydrocephalus can be treated through a variety of surgical procedures including

direct operation on the lesion causing the obstruction, such as tumor.

- Intracranial shunts for selected cases of non-communicating hydrocephalus

to divert fluid from the obstructed segment of the ventricular system to the
subarachnoid space.
- Extracranial shunts (most common) to divert fluid from the ventricular
system to an extracranial compartments, frequently the peritoneum or right
atrium.

EXTRA CRANIAL SHUNT PROCEDURES:

1. Ventriculo peritoneal shunt (V-P shunt):

- Diverts CSF from a lateral ventricle or the spinal subarachnoid space to the
peritoneal cavity.
- A tube is passed from the lateral ventricle through an occipital burr hole
subcutaneously through the posterior aspect of neck and para spinal region
to the peritoneal cavity through a small incision in the right lower quadrant.
2. Ventriculo atrial shunt (V-A shunt):
 - A tube is passed from the dilated lateral ventricle through a burr hole in the
parietal region of the skull.
- It then is passed under the skin behind the ear and into a vein down to a
point where it discharges into the right atrium or superior venacava.
- A one way pressure sensitive valve will close to prevent reflex of blood into
the ventricle and open as ventricular pressure rises, allowing fluid to pass
from the ventricle into the blood stream.
3. Ventriculo pleural shunt:

- Diverts CSF to the pleural cavity

- Indicated when the V-P or V-A route cannot be used.
4. Ventricle-gall bladder shunt:

- Diverts CSF to the common bile duct

- Used when all other routes are unavailable

Shunt complications:

1. Need for shunt revision frequently occurs because of occlusion, infection or

malfunction.
2. Shunt revision may be necessary because of growth of the child, newer
models, however include coiled tubing to allow the shunt to grow with the
child.
3. Shunt dependency frequently occurs, the child rapidly manifests symptoms
of increased intracranial pressure of the shunt does not function optimally.
4. Children with V-A shunt may experience endocardial contusions and
clotting, leading to bacterial endocarditis, bacteremia and ventriculitis or
thromboembolism and corpulmonale.
Nursing Management:

Nursing Assessment – Infants:

1. Assess head circumference – point out largest circumference

2. Palpate fontanelle for bulging and tenderness
3. Assess pupillary response
4. Assess level of consciousness
5. Evaluate breathing patterns and effectiveness
6. Assess feeding patterns and patterns of emesis
7. Assess motor activity
8. Determine attainment of developmental milestones.
Older child:

1. Measure vital signs for signs of increased ICP

2. Assess patterns of headache, emesis
3. Determine pupillary response
4. Evaluate the level of consciousness
5. Assess motor function
6. Evaluate attainment of mile stones, school performance
7. Obtain parents report of recent behaviour.

Nursing Diagnosis:

1. Impaired cerebral tissue perfusion related to increased ICP.

2. Impaired nutritional status less than body requirement related to reduced oral
intake and vomiting
 3. Risk for impaired skin integrity related to enlarged head.
4. Anxiety related to abnormal condition and surgical interventions.
5. Risk for infection related to introduction of infecting organism through the
shunt.
6. Risk for fluid volume deficit related to CSF drainage.
7. Ineffective family coping related to life threatening problem of infant.

Pre-operative Nursing Care:

1. Observe for the signs of increased ICP i.e. for altered mental state, vomiting,
strabismus, slowed respiration, decreased pulse or increased pulse.
2. Measure the occipital frontal circumference daily
3. Daily palpate the fontanelles for bulging, size and tension.
4. Record vital signs
5. Small and frequent feeds are given to child to prevent vomiting.
6. Give the feeds only when the child is relaxed and calm.
7. After every feed burp the baby and place him on his right side with head
elevated to prevent aspiration.
8. While holding the baby, avoid pressure on the neck by supporting the head.
9. Position of the baby is changed every 2 hourly to prevent the occurance of
pressure sores on the scalp and to prevent hypostatic pneumonia.
10.a pad of cotton can be placed under the head.
11.The infant must be kept clean and dry especially around the creases of the
neck.
12.While moving the baby proper should be given to the head.
13.Give psychological support and detailed information to parents regarding
each investigations and about the condition of the child.
 14.Involve the parents in daily care of the child.

Post-operative Nursing Care:

1. The vital signs are checked every 15-30 mins.

2. Observe for increased ICP and if present it should be reported.
3. If shunt is done, the catheter tract is checked for swelling and tenderness.
4. The shunt is tested for patency.
- .