Moyamoya Disease
Description
● General Background
•A rare, progressive steno-occlusive cerebral vascular disease with an unclear aetiology is called Moyamoya disease.
•Most common: Asians, particularly those from Japan.•Male-to-female ratio: 1:1.8.
•First ten years of life is the most common diagnosis.
•Involvement is typically bilateral, but it can be unilateral.
● Case Presentation:
➣Patient: 12-year-old boy, 37 kg.
➣Symptoms:
7 years of recurrent right-sided weakness.
➣Episodes:
Abrupt onset, approximately five minutes, complete recovery, and no convulsions or loss of consciousness.
10–12 attacks every two to three months over years.reated empirically with anticonvulsants → no improvement.
4 months ago new right-sided weakness, persistent, no full recovery. No history of headache, trauma, vision problems. Birth history preterm (30 weeks), normal delivery, no consanguinity.
Exam: Right-sided muscle power = 4/5; no systemic abnormalities.
● Investigations
➣Standard laboratory tests: normal (CBC, ESR, thyroid, coagulation, renal, metabolic/lipid profile, ANA, ANCA, and homocysteine).
➣Imaging:
•MRI → old infarct (right capsule-ganglionic), recent infarct (left paraventricular/centrum semiovale).
•EEG → discharges of interictal epileptiform.
•MRA :results in constricted MCA, a lack of MCA territory vessels, and significant stenosis at the terminal internal carotid arteries.
•MRV : normal.
•Collaterals found in the thalamo-capsuloganglionic area and deep white matter.
● Discussion
➣Pathology: Media thinning, intimal thickening, and alterations in the elastic lamina all contribute to progressive stenosis or occlusion.
➣Can be idiopathic or associated with:
•Immunologic (Graves’ disease),
•Infectious (TB, leptospirosis),
•Hematologic (sickle cell, aplastic anemia, Fanconi anemia),
•Vascular (atherosclerosis, trauma, radiation),
•Congenital syndromes (Down, Marfan, NF1, Turner, Hirschsprung).
➣Clinical features:
•Hemiparesis, sensory loss, seizures, and cognitive deterioration are examples of ischaemic events in children.
•Hemorrhagic episodes are more likely to occur in adults.
➣Diagnosis:
DSA = gold standard (high-resolution, evaluates stenosis & collaterals).
●Prognosis
➣Mortality:
•4.3% of children, 10% of adults.
•Recurrent strokes cause gradual cognitive deterioration in more than 50% of patients.
•Early treatment before a stroke occurs will improve the prognosis.
•In this instance, there was a delayed presentation with ongoing hemiparesis but no cognitive impairment.
Protocol
●Management:
•Family opted for medical management due to financial constraints.
•Given anti-platelet therapy + regular follow-up.
•No surgical revascularization performed.
● Options for treatment:
•When possible, surgery should be the mainstay.
•Direct revascularisation: anastomosis between STA and MCA.
•Using vascularised tissue (dura, temporalis, and STA) to promote angiogenesis is known as indirect revascularisation.
•Medical treatment includes risk factor adjustment, symptom control, and anti-platelets.
● Problems Faced by Cancer Patients:
➣Toxicities associated with treatment: Fatigue, cytopenias, mucositis, vomiting, skin toxicity, and organ malfunction
➣High load of symptoms: Anorexia, weariness, pain, and cachexia
➣Distress on a psychological level: Depression, anxiety, and fear of the prognosis
Inadequate compliance and therapeutic disruptions: Because to adverse effects, regimen complexity, and logistical and budgetary challenges
➣Insufficient cooperation among providers: Surgeons, radiologists, oncologists, and palliative care providers are not always in agreement.
➣Informational gaps and communication breakdowns: Patients who don't know when to seek help, red flag symptoms, or how to take care of themselves
➣Symptoms not being reported enough: Patients either avoid reporting adverse effects or normalise them.
➣Inadequate supervision of outpatients: Lack of surveillance in between treatment visits results in issues being discovered later.
➣Cachexia and nutritional decline: From anorexia, gastrointestinal adverse effects, and metabolic alterations
➣Palliative and end-of-life needs: Psychosocial, familial, and symptom management are necessary
● Management & Nursing Interventions:
➣ Case / Care Management
•Comprehensive assessment & care planning
•Assess medical, psychosocial, nutritional, and financial needs
•Create individualized care plans covering treatment, supportive care, palliative care
•Care coordination
•Act as liaison between multiple providers
•Ensure timely appointments and results sharing
•Regular monitoring & follow-up
•Track labs, adherence, side effects
•Use electronic reminders and checklists
•Patient education & empowerment
•Teach about regimen, side effects, red-flag symptoms
•Provide written materials and confirm understanding (teach-back method)
•Risk stratification
•Identify high-risk patients (elderly, comorbidities, poor support) for closer monitoring
•Telephone / remote nurse monitoring
•Schedule weekly calls to assess symptoms, toxicities, adherence
•Escalate to oncologist if red-flags appear
•Psychosocial & financial support
•Link to counseling, social workers, financial/transport assistance
•Outcome evaluation
•Measure hospitalizations, toxicity severity, adherence, quality of life
➣Nursing Interventions (Problem → Intervention)
•Vomiting and nausea
•Give antiemetics prior to chemotherapy.
•Promote a bland diet and modest, regular meals.
•Teach non-pharmacological techniques (acupressure, ginger).
•Keep an eye on electrolytes and hydration.
•Mucositis / oral care
•Soft diet, baking soda/saline rinses
•Steer clear of mouthwashes with alcohol.
•Apply topical pain relievers.
•Keep your mouth clean.
•Weariness
•Promote the timing of rest and exercise.
•Instruct students on energy-saving techniques.
•Encourage mild physical activity
•Work together with the physiotherapist.
•Pain
•Use scales (0–10, PQRST) to evaluate
•Give analgesics (opioids, adjuvants) in accordance with protocol.
•Provide non-drug techniques (relaxation, heat/cold packs)
•Watch for adverse effects of analgesics.
•Risk of infection and cytopenias
•Keep an eye on CBCs
•Implement neutropenic measures (mask usage, avoiding raw foods, and hand cleanliness).
•Patients should be taught to report fevers right away.
•If an infection is detected, start antibiotics right away.
•Cachexia and nutritional deterioration
•Evaluate weight trend, albumin, and BMI.
•Offer high-protein, high-calorie supplements.
•Work together with a dietician
•If severe, think about enteral or parenteral assistance.
Notes
For more information visit
10.7759/cureus.39209
