CP ON MOYAMOYA
CP ON MOYAMOYA
INDEX
- Male Patient
- Female Patient
- Marriage
- Male death
- Female death
PAST MEDICAL HISTORY
Mr. Madhurjya Kishor Das is a known case of Moyamoya disease and had undergone
craniotomy in 2017 and 2018 in Narayana Hospital, Bengaluru.
There is no other illness like DM, HTN, Tuberculosis etc.
PHYSICAL EXAMINATION
SYSTEMS FINDINGS
General Assessment The patient is conscious and alert at the time
of examination.
GCS – 14/15. Pupil reaction – 2(+) B/L
Height 175 cm
Weight 50 kg
BMI 16.33 kg/m2
Integumentary System
Colour is normal.
Capillary refill time is normal < 3
secs
No Icterus present.
BIO-PHYSICAL MEASUREMENT
Blood Pressure – 130/70 mmHg
Heart rate - 105 b/m
Respiratory Rate – 20 b/m
Temperature – 98 F
RBS – 89 mg/dl
GCS Scale – E4 V5 M5 (14/15)
INVESTIGATION
Date Name of Investigation Findings Normal Value Remarks
30/01/25 TLC
(Total Leucocyte Count) 10800/cumm 4000-10000 High
WBC
MEDICATIONS
DEFINITION
Moyamoya disease is a rare, progressive cerebrovascular disorder caused by blocked arteries at the base of
the brain in an area called the basal ganglia. Moyamoya means “puff of smoke” in Japanese and is used to
describe the tangled appearance of tiny vessels compensating for the blockage.
Moyamoya disease is an uncommon blood vessel ailment that results in constriction or obstruction of
the carotid artery in the head. The carotid artery is a significant blood vessel that provides blood to the brain.
When the brain's blood flow is blocked, it is reduced. Then, tiny blood vessels start to grow at the base of the
brain, trying to supply it with blood.
RELATED ANATOMY AND PHYSIOLOGY
Parietal lobe
Temporal lobe
INCIDENCE
The incidence of Moyamoya disease exhibits significant regional differences, with a high
incidence in East Asia and a low incidence in other regions. According to previous studies,
the prevalence of Moyamoya disease is 10.5/100,000 individuals and the incidence rate is
0.94/100,000 individuals in Japan; in South Korea, the prevalence rate is 16.1/100,000 and
the incidence rate is 2.3/100,000 individuals. The incidence of Moyamoya disease was as low
as 0.09/100,000 individuals in other regions, including North America, but it has exhibited an
upward trend in the US. In Nanjing (China), the prevalence of MMD in the time frame of
2000–2007 was 3.92/100,000. According to the most recent study, 2,430 cases of MMD have
been reported in China since 1976.
Worldwide, the age of onset of MMD is significantly bimodal in distribution, with a bimodal
peak consisting of a major peak in the first decade of life and a moderate peak in the late 20
to 30s. Of note, geographic differences in sex distribution have been observed. In foreign
populations, the incidence of MMD in females was reported to be higher than that in males
with the male-to-female ratio ranging from 1:1.8 to 1:2.2; however, the sex ratio is 1:1 in
China.
Moyamoya disease is more common in people of Asian origin, including those in India. In
India, Moyamoya disease has a bimodal age distribution, with peaks at 3–8 years and 41–47
years.
ETIOLOGY AND RISK FACTORS:
BOOK PICTURE PATIENT’S PICTURE
RISK FACTORS
Asian origin: Although the disease is Present. Patient is from Assam
found all throughout the world, East
Asian countries like China, Japan,
and Korea have higher rates of
moyamoya infection. This may be
the result of some genetic factors
present in those populations. It has
been discovered that Asians living in
Western countries exhibit the same
elevated frequency.
Health problems: Sometimes, a Absent
number of diseases,
including neurofibromatosis type 1,
sickle cell disease, and Down
syndrome, coexist with moyamoya
syndrome.
Sex: Moyamoya is a condition that Absent
affects women significantly more
frequently.
Age: Although it can occasionally Present. Patient was diagnosed at the
affect adults, age of 12.
moyamoya sickness primarily
affects children under the age of
fifteen. Absent
Smoking: causes constant
inflammation and weaken the
immune system.
Alcohol use: affects a wide range of Absent
structures and processes in the
central nervous system.
Oral contraceptives in adult female: Absent
can increase the risk of blood clot
forming.
ETIOLOGY
PATHOPHYSIOLOGY
Progressive stenosis of the internal carotid artery (ICA) at the base of the
brainabsorbed.
The narrowing of the ICA leads to decreased blood supply to the brain regions
The inner lining of the ICA develops abnormal thickening causing narrowing of the
vessel lumen.
In response to ischemia, the brain attempts to develop new, abnormal blood vessels
(collateral vessels) around the stenotic area, appearing as a "puff of smoke"
There is growing evidence that MMD is primarily a proliferative disease of the intima. The
smooth muscle proliferation that is associated with an ACTA2 mutation has been postulated to
be the key mechanism of the vascular occlusion in familial MMD. The histopathological
findings in the distal ICA have shown a proliferation of the smooth muscle cells or
endothelium and a stenosis or occlusion associated with the fibrocellular thickening of the
intima. An enhancement of the stenotic segments may represent either a neo-vascularization
or an intimal hyperplasia.
The moyamoya vessels are the dilated perforating arteries that have various histopathological
changes, including fibrin deposits in the wall, fragmented elastic laminae, attenuated media,
and the formation of microaneurysms. In addition to the moyamoya vessels, cortical
microvascularization, which is characterized by a substantially increased microvascular
density and diameter, is suggested as a specific finding in MMD. These basal and cortical
vessels may represent compensatory mechanisms for the reduced cerebral blood flow or the
aberrant active neo-vascularization before the vascular occlusion
CLINICAL MANIFESTATIONS
BOOK PICTURE PATIENT’S PICTURE
Stroke: Ischemic strokes Absent
(blockages) or hemorrhagic strokes
(bleeding) as it causes a narrowing
of the major arteries supplying blood
to the brain, significantly reducing
blood flow and oxygen delivery to
the brain tissue
Hemiparesis: Weakness or paralysis Present
on one side of the body as it causes a
narrowing of the major arteries
supplying blood to the brain, which
results in reduced blood flow to one
side of the brain, leading to
weakness or paralysis on the
opposite side of the body
Headaches: Severe headaches, or Absent
migraine-like headaches due to the
narrowed blood vessels in the brain
causing reduced blood flow, which
triggers the activation of pain
receptors
Vision changes: Blurred vision or Absent
vision loss as it causes a narrowing
of the major blood vessels supplying
the brain, including those that feed
the retina, resulting in reduced blood
flow to the eyes
Cognitive problems: Difficulty Present
learning, focusing, or remembering
as it causes chronic cerebral
hypoperfusion, meaning the brain
doesn't receive enough blood flow
due to narrowed arteries at the base
of the brain, which results in
impaired brain function
Seizures: Epilepsy or seizures as it Absent
causes a significant reduction in
blood flow to the brain due to the
narrowing of the major arteries
supplying it, leading to areas of
brain tissue becoming ischemic
Involuntary movements: Jerky Absent
movements due to the reduced blood
flow to the brain caused by the
narrowing of major arteries
Sensory problems: Loss of hearing, Absent
sight, smell, taste, or touch as it
affects the blood supply to the brain,
specifically the areas responsible for
auditory processing
Slurred speech: Difficulty speaking Present
or understanding words as it causes
a reduced blood flow to the brain
Facial asymmetry: Asymmetry of Present
the face.
Dizziness as the narrowed blood Present
vessels in the brain, known as
"moyamoya vessels," significantly
reduce blood flow to the brain,
causing oxygen deprivation which
can manifest as dizziness
Vomiting is when the blood flow to Present
the brain is significantly reduced due
to narrowed arteries
DIAGNOSTIC EVALUATION
BOOK PICTURE PATIENT’S PICTURE
History and Physical Examination. Done.
Magnetic resonance imaging
(MRI). An MRI uses powerful Not done.
magnets and radio waves to create
detailed images of the brain. A
health care provider may inject a dye
into a blood vessel to view the
arteries and veins and highlight
blood circulation. This type of test is
called a magnetic resonance
angiogram.
Computerized tomography (CT) Done
scan. A CT scan uses a series of X- Result shows significant luminal
rays to create a detailed image of narrowing with multiple stenotic
your brain. A health care provider segments in bilateral supraclinoid ICAS
may inject a dye into a blood vessel and proximal MACS and ACAS with
to highlight blood flow in your multiple surrounding collaterals
arteries and veins. This is called a formation-Suggestive of residual
(CT angiogram) disease.
Cerebral angiogram. In a cerebral
angiogram, a health care provider Done
inserts a long, thin tube called a Present DSA features are consistent
catheter into a blood vessel in the with Grade 3 Moyamoya disease
groin. The provider then guides it to (Extensive involvement of the left
the brain using X-ray imaging. The side noted). Bilateral distal MCA
provider injects dye through the branches seen filling from collaterals
catheter into the blood vessels of the and STA-MCA bypass.
brain.
Positron emission tomography Not done
(PET) scan or single-photon
emission computerized
tomography (SPECT): In these
tests, you're injected with a small
amount of a safe radioactive
material. PET provides visual
images of brain
activity. SPECT measures blood
flow to regions of the brain.
Electroencephalogram Not done
(EEG). An EEG monitors the
electrical activity in your brain using
small metal discs called electrodes
attached to your scalp.
Transcranial Doppler Not done
ultrasound. In surgical transcranial
Doppler ultrasound, sound waves are
used to obtain images of your head
and sometimes your neck.
Specialists may use this test to
evaluate blood flow in blood vessels
in your neck.
MANAGEMENT
BOOK PICTURE PATIENT’S PICTURE
Medical Management
Surgical Management
Revascularization surgery:
surgeons bypass blocked arteries.
They do this by connecting blood
vessels on the outside and inside of
the skull to help restore blood flow
to your brain. This may include
direct or indirect revascularization
procedures. Or it may include a
combination of both.
a) Direct revascularization
procedures. In direct
revascularization surgery, surgeons
stitch the scalp artery directly to a Not done.
brain artery. This is also known as
superficial temporal artery to middle
cerebral artery bypass surgery. This
procedure increases blood flow to
your brain immediately.
Direct bypass surgery may be
difficult to perform in children due
to the size of the blood vessels to be
attached. But it's the preferred option
in adults. This intervention can be
performed safely and effectively by
an experienced surgical team that
treat moyamoya patient
on a daily basis.
COMPLICATIONS
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Ischemic stroke Absent.
Can be life-threatening, and
symptoms include weakness,
numbness, balance problems, and
headaches
Hemorrhagic stroke Absent.
More common in adults, and
symptoms include severe bleeding
and brain damage
Paralysis. Absent.
Vision problems. Absent.
Speech problems.
Movement disorders. Present.
Present.
developmental delays
Seizure Absent.
Absent.
DIETARY ASSESSMENT
Good nutrition is a vital part of staying healthy. Diet plan for Moyamoya disease include low
potassium diet, moderate calorie, low sodium diet, high protein diet, high vitamin and
mineral diet.
1.Patient clinical history taken: Yes
2.Anthropometric measurement:
-Weight: 50 kg
-Height: 175 cm
BMI: 16.33 kg/m2
3.Dietary Habit: On hospitalization, patient is advised to take low sodium diet, low potassium
diet, moderate calorie, high protein diet. Patient is both vegetarian and non-vegetarian.
4.Food allergies: No significant of food allergies.
5.Nutritional requirement: Diet for patient with Moyamoya disease should include the
following: -
Veg curry (mixed potato+ beans) 1 serve 90.87 16.67 1.94 2.0 30.5 0.6
Lentil dal (cooked) 1 bowl 170.5 20.8 6.8 10.1 29.6 3.2
Veg Steamed (mixed potato+ 1 serve 85.5 12.4 0.8 0.8 1.6 0.1
bean+ cabbage + carrot)
Fish curry/Chicken/Egg white 1 medium size 89 0.8 1.6 1.6 0.32 5.2
fish
4:30 pm Evening Tea:
Red Tea 1cup 15 2 0 1 38.7 0
(100 ml)
Biscuit 2 pcs 30 2.2 0.1 2 0.2 0.01
6:30 pm Dal/Chicken soup/Oat/Suji 1 bowl 172.3 22.2 7.4 12.2 32.7 3.5
9:00 pm Dinner:
2Rice (cooked) 1bowl 287.3 63.1 0.42 6.4 6.4 0.52
Dal (lentil) 1 bowl 170.5 20.1 6.8 10.1 29.6 3.2
Veg curry (potatoes+ beans) 1 serve 124 8.4 1.32 5.37 50.72 0.82
Self-Care
-Grooming
-Food
-Exercise
-Medications
-Sleep pattern
-Ambulation and
support was
provided to meet
his self-care needs
Orem (1991) has identified five areas of activity for nursing practices as given below:
Entering into and maintaining nurse patient relationship with individuals, families or
groups until patients can legitimately be discharged from nursing.
Determining if and how patients can be helped through nursing.
Responding to patient’s requests, desires and needs for nurse contacts and assistance.
Prescribing, providing and regulating direct help to patients in the form of nursing.
Coordinating and integrating nursing with the patients daily living, other health care
needed or being received, and social and educational services needed or being
received.
Metaparadigm is the concepts which define the nursing practice. Person, health,
nursing, and environment are considered as the metaparadigm of nursing theories by
many nursing theorists.
4) Nursing: Orem have distinct approaches towards the concept of nursing. Orem
presented nursing as a unique field of knowledge and an action system, which
is professional practice. According to her nursing is the intervention to meet
the required need for self-care and need for medical care of patients.
NURSING PROCESS
NURSING ASSESSMENT
To obtain detail history and physical examination.
Obtain a thorough medical history to identify potential risk factors and current
symptoms like headaches, dizziness, seizures, or transient ischemic attacks (TIAs)
Assess for other illness like diabetes, hypertension, sleeping disorder and drug
therapy.
Obtain lifestyle history related to dietary and habits, type of food he usually consume.
To obtain history related to allergic reaction regarding to food and drugs.
Obtain family history related to Moyamoya disease, heart problem and diabetes,
hypertension, Tuberculosis or any other chronic illness.
Assess patient and family knowledge about the diseases.
To perform head to toe examination and note any abnormalities.
To focus on detailed neurological evaluation, including mental status, motor function,
sensory perception, speech, and cranial nerve function.
To obtain investigation and identify any abnormalities.
NURSING DIAGNOSIS
1) Ineffective cerebral perfusion related to decreased cerebral blood flow secondary to
occlusion in the cerebral artery as evidenced by dizziness and weakness on the left
side of the body.
2) Disturbed sensory perception related to motor disturbances as evidenced by patient is
having weakness on the left side if the body and deviation of the mouth.
3) Decreased activity intolerance related to motor disturbances as evidenced by patient is
having weakness on the left side of the body.
4) Imbalanced nutrition less than body requirements as evidenced by patient is having
loss of appetite and patient’s BMI is 16.33 kg/m2.
5) Self-care deficit related to weakness as evidenced by patient needs help in meeting his
daily activities.
6) Risk for ineffective airway clearance related to neurological complications that can
affect their ability to cough effectively, including facial weakness secondary to
Moyamoya as evidenced by patient is having deviation of mouth.
7) Risk for injury related to seizure activity secondary to cerebral irritation.
BIBLIOGRAPHY
BOOK REFERENCES
1. Sharma S, “Lippincott Manual of Medical Surgical Nursing”, 2nd edition, published
by Wolter Kluwer (India) Pvt Ltd, pages number: 840-841.
2. Harding, Kwong, Robert, Hagler, Reinish, “Lewis Medical Surgical Nursing
4Assessment and Management of Clinical Problems”, Fourth South Asia edition,
published by Elsevier, pages number: 573-574.
3. Black J, Hawks J, “Black’s Medical Surgical Nursing Clinical Management for
Positive Outcomes”, Fourth South Asia edition, published by Elsevier, pages number:
789-781.
4. Mosby’s Nursing Drug Reference 2021, 26th edition, published by Elsevier, India Pvt
Ltd.
INTERNET REFERENCES
1) Thermer A, Available from Mayo clinic, 2023
https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-
treatment/drc-20355591
2) Rushiter S, Available from Cleveland Clinic, 2024
https://my.clevelandclinic.org/health/diseases/17244-moyamoya-disease
3) Sam P, Available from Indian Journal of Continuing Nursing Education, 2016
https://journals.lww.com/ijcn/fulltext/2015/16020/
caring_for_a_patient_with_moyamoya_disease__a_case.3.aspx