eMedicine Specialties > Neurology > Pediatric Neurology

Moyamoya Disease

Roy Sucholeiki, MD, Director, Comprehensive Seizure and Epilepsy Program, The Neurosciences Institute at Central DuPage Hospital
Jasvinder Chawla, MBBS, MD, MBA, Associate Professor of Neurology, Director of Neurology Residency Training Program, Director of Clinical Neurophysiology Laboratory, Assistant Director of Neurology Clerkship Program, Department of Neurology, Loyola University Medical Center

Updated: Nov 6, 2008

Introduction

Background

Moyamoya disease is a progressive occlusive disease of the cerebral vasculature with particular involvement of the circle of Willis and the arteries that feed it. Moyamoya (ie, Japanese for "puff of smoke") characterizes the appearance on angiography of abnormal vascular collateral networks that develop adjacent to the stenotic vessels. The steno-occlusive areas are usually bilateral, but unilateral involvement does not exclude the diagnosis.

Pathophysiology

Pathologically, moyamoya disease (MMD) is characterized by intimal thickening in the walls of the terminal portions of the internal carotid vessels bilaterally. The proliferating intima may contain lipid deposits. The anterior, middle, and posterior cerebral arteries that emanate from the circle of Willis may show varying degrees of stenosis or occlusion. This is associated with fibrocellular thickening of the intima, waving of the internal elastic lamina, and thinning of the media. Numerous small vascular channels can be seen around the circle of Willis. These are perforators and anastomotic branches. The pia mater also may have reticular conglomerates of small vessels.

The exact etiology of MMD is unknown. Some genetic predisposition is apparent because it is familial 10% of the time. The disease may be hereditary and multifactorial. A recent Japanese study demonstrated that familial MMD is autosomal dominant with reduced penetrance.

It may occur by itself in a previously healthy individual. However, many disease states have been reported in association with MMD, including the following:

• Immunological -Graves disease/thyrotoxicosis
• Infections -Leptospirosis and tuberculosis
• Hematologic disorders -Aplastic anemia, Fanconi anemia, sickle cell anemia, and lupus anticoagulant
• Congenital syndromes -Apert syndrome, Down syndrome, Marfan syndrome, tuberous sclerosis, Turner syndrome, von Recklinghausen disease, and Hirschsprung disease
• Vascular diseases -Atherosclerotic disease, coarctation of the aorta and fibromuscular dysplasia, cranial trauma, radiation injury, parasellar tumors, and hypertension

These associations may not necessarily be causative, but do warrant consideration due to impact on treatment.

Frequency

United States

The incidence of moyamoya disease is highest in Japan (see International). However, a recent study indicated that the prevalence of MMD in California and Washington was 0.086 case per 100,000 population. In this study, the breakdown based on ethnicity as ratio to whites was 4.6 for Asian Americans, 2.2 for African Americans, and 0.5 for Hispanics.

International

The prevalence and incidence of moyamoya disease in Japan has been reported to be 3.16 cases and 0.35 case per 100,000 people, respectively. With regard to sex, the female-to-male ratio is 1.8:1. A bimodal peak of incidence is noted, with symptoms occurring either in the first decade or in the third and fourth decades of life.

Mortality/Morbidity

Mortality rates of moyamoya disease are approximately 10% in adults and 4.3% in children. Death is usually from hemorrhage. About 50-60% of affected individuals experience a gradual deterioration of cognitive function, presumably from recurrent strokes.

Race

Moyamoya disease occurs primarily in Asians, but it also can occur (with varying degrees of severity) in whites, African Americans, Haitians, and Hispanics.

Sex

The female-to-male ratio of moyamoya disease is 1.8:1.

Age

Ages range from 6 months to 67 years, with the highest peak in the first decade and smaller peaks in the third and fourth decades.

Clinical

History

Children and adults with moyamoya disease may have different clinical presentations. The symptoms and clinical course vary widely from asymptomatic to transient events to severe neurologic deficits. Adults experience hemorrhage more commonly; cerebral ischemic events are more common in children.

• Children may have hemiparesis, monoparesis, sensory impairment, involuntary movements, headaches, dizziness, or seizures. Mental retardation or persistent neurologic deficits may be present.
• Adults may have symptoms and signs similar to those in children, but intraventricular, subarachnoid, or intracerebral hemorrhage of sudden onset is more common in adults.

Physical

Examination findings depend on the location and severity of hemorrhage or ischemic insult.

Causes

The cause of moyamoya disease is not known. The disease is believed to be hereditary. Fukui (1977) reported a family history in 10% of patients with MMD. Moreover, Mineharu suggested that familial MMD is autosomal dominant with incomplete penetrance that depends on age and genomic imprinting factors.¹ Genetically, susceptibility loci have been found on 3p, 6p, 17q, and band 8q23. Recently, Mineharu et al have found a specific gene locus, q25.3, on chromosome 17.²

Differential Diagnoses

Other Problems to Be Considered

Apert syndrome
Aplastic anemia
Brainstem syndromes
Cranial trauma
Coarctation of the aorta
Fanconi anemia
Irradiation injury
Leptospirosis
Marfan syndrome
Mitochondrial cytopathies
Parasellar tumors
Sickle cell disease
Tuberculosis
Turner syndrome
Vasculitis
Carotid disease and stroke

Workup

Laboratory Studies

• In a patient with stroke of unclear etiology, a hypercoagulability profile may be helpful. Significant abnormality in any of these is a risk factor for ischemic stroke.
  • Protein C
  • Protein S
  • Antithrombin III
  • Homocysteine
  • Factor V Leiden
• Erythrocyte sedimentation rate (ESR) can be obtained as part of the initial workup of a possible vasculitis. However, a normal ESR does not rule out vasculitis.

Imaging Studies

• Cerebral angiography is the criterion standard for diagnosis. The following findings support the diagnosis:
  • Stenosis or occlusion at the terminal portion of the internal carotid artery or the proximal portion of the anterior or middle cerebral arteries
  • Abnormal vascular networks in the vicinity of the occlusive or stenotic areas
  • Bilaterality of the described findings (although some patients may present with unilateral involvement and then progress)
• Magnetic resonance angiography (MRA) can be performed. Any of these findings on MRA may preclude the need for conventional angiography.

Histologic Findings

See Pathophysiology.

Treatment

Medical Care

Pharmacologic therapy for moyamoya disease (MMD) is disappointing. Therapy is directed primarily at complications of the disease.

• If intracerebral hemorrhage has occurred, then management of hypertension (if present) is imperative.
• In cases of severe stroke, ICU monitoring is indicated until the patient's condition stabilizes.
• If the patient has had an ischemic stroke, consider anticoagulation or antiplatelet agents.
  • The rationale behind anticoagulation and antiplatelet agents is to prevent further strokes, especially in stenotic vessels where further infarction can occur if occlusion progresses.
  • These medications are not approved by the Food and Drug Administration (FDA) specifically for use in MMD, so the decision to treat with anticoagulants, ie, heparin (and in some cases, warfarin for long-term anticoagulation), or antiplatelet agents (eg, aspirin) rests on the following: angiogram findings, severity of stroke, and risk/benefit analysis by physicians who are experienced in stroke treatment.
• Consultation with an experienced neurologist helps guide appropriate care.
• Angiogram can determine the extent of occlusion or stenosis.

Surgical Care

Patients with moyamoya disease who present for treatment while symptoms are evolving have a better prognosis than those who present with static symptoms (which probably indicate a completed stroke).

• Various surgical procedures have been used: superficial temporal artery–middle cerebral artery (STA-MCA) anastomosis, encephaloduroarteriosynangiosis (EDAS), encephaloduroarteriomyosynangiosis (EDAMS), pial synangiosis, and omental transplantation.
• These procedures can be divided into 2 groups depending on whether they involve direct or indirect anastomosis. Which of these is most effective remains controversial. Sufficient evidence suggests that surgical revascularization procedures result in some symptomatic benefits along with demonstration of improved blood flow. Direct and/or combined procedures provide improved vascularization. However, data proving sustained or improved long-term outcomes is insufficient.
• STA-MCA anastomosis is very difficult in children younger than 2 years because of the small diameter of the STA. In these cases, EDAS may be more suitable. This procedure sometimes has failed because of poor revascularization. Hoffman suggested that this is due to the presence of atrophy and a layer of spinal fluid between the pia and arachnoid tissue.³ Division of this arachnoid membrane and placement of the STA directly on the pial membrane helps to avoid the problem.
• In cases of EDAS failure, EDAMS can be considered.

Consultations

• Initial neurologic consultation is imperative. A neurologist can document neurologic deficits, consider the differential diagnosis, conduct testing to validate suspected etiologies, and commence medical management as indicated.
• Neuroradiology consultation is needed to help determine the extent of radiologic testing needed (ie, MRA vs conventional angiography). Based on the results of these tests, a neurosurgeon can decide if surgical intervention would be helpful.

Activity

Rehabilitation with physical therapy, occupational therapy, and speech therapy should be considered depending on the neurologic impairment.

• The extent of therapy can range from bedside to full comprehensive inpatient rehabilitation. The latter would include physical, occupational, speech, and cognitive therapy.
• The condition of the patient including active comorbidities dictate involvement in rehabilitation therapy.

Medication

Drug therapy for moyamoya disease (MMD) depends on the particular manifestations of the disease.

For hemorrhage, therapy revolves around management of hypertension (if present).

For ischemic stroke, anticoagulation with heparin or warfarin may be considered. Safety and efficacy have not been fully established with these drugs, and careful analysis of risk and benefits is needed. These drugs could be useful if thrombosis of vessels is present, but they do not alter the natural history of the disease and they considerably increase the risk of hemorrhage with large strokes.

The same considerations are true for aspirin and other antiplatelet agents.

Treatment with anticoagulation or antiplatelet agents should be pursued only after consultation with a neurologist who is experienced in stroke management.

Anticoagulants

These agents are given for prevention of further thrombosis and potential infarction of the brain. CAUTION: Anticoagulation is of unproven benefit in ischemic stroke associated with MMD. This therapy therefore is considered empirical.

Heparin

Administered IV, and frequently given with initial bolus in cardiac situations. In stroke, bolus not recommended. Target dose aimed at maintaining aPTT 1.5-2 times control.
CT scan of brain must be done prior to any anticoagulant use to rule out preexisting intracranial hemorrhage.

Dosing

Adult

50 U/kg/h IV initially, followed by continuous infusion of 15-25 U/kg/h; increase dose by 5 U/kg/h q4h prn using aPTT results

Pediatric

Not established

Interactions

Digoxin, nicotine, tetracycline, and antihistamines may decrease effects; NSAIDs, aspirin, dextran, dipyridamole, and hydroxychloroquine may increase toxicity

Contraindications

Documented hypersensitivity; conditions that predispose to bleeding; evidence of active bleeding such as severe strokes, presence of intracranial hemorrhage, positive stool blood test findings, or suspected bleeding elsewhere

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

In neonates, preservative-free heparin recommended to avoid possible toxicity (ie, gasping syndrome) of benzyl alcohol, which is used as preservative; caution in severe hypotension and shock; patients should be monitored closely by providers with experience in managing anticoagulation

Warfarin (Coumadin)

Administered orally and used if long-term anticoagulation needed. INR followed with target range of 2-3.
CT scan of brain must be done prior to any anticoagulant use to rule out preexisting intracranial hemorrhage.

Dosing

Adult

5-15 mg/d PO qd for 2-5 d; adjust dose according to desired INR

Pediatric

Not established

Interactions

Drugs that may decrease anticoagulant effects include griseofulvin, carbamazepine, glutethimide, estrogens, nafcillin, phenytoin, rifampin, barbiturates, cholestyramine, colestipol, vitamin K, spironolactone, oral contraceptives, and sucralfate
Medications that may increase anticoagulant effects include oral antibiotics, phenylbutazone, salicylates, sulfonamides, chloral hydrate, clofibrate, diazoxide, anabolic steroids, ketoconazole, ethacrynic acid, miconazole, nalidixic acid, sulfonylureas, allopurinol, chloramphenicol, cimetidine, disulfiram, metronidazole, phenylbutazone, phenytoin, propoxyphene, sulfonamides, gemfibrozil, acetaminophen, and sulindac

Contraindications

Documented hypersensitivity, severe liver or kidney disease, open wounds or GI ulcers, conditions that predispose to bleeding

Precautions

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

Do not switch brands after achieving therapeutic response; caution in active TB or diabetes; patients with protein C or S deficiency are at risk of developing skin necrosis; patients need careful periodic monitoring by personnel who are experienced in using anticoagulants; caution in children whose activity level may be a risk factor for trauma and subsequent hemorrhage

Antiplatelet agents

These agents can be considered to help prevent future ischemic strokes. As with anticoagulation, aspirin is of unproven benefit in MMD; its use is considered empirical.

Aspirin (Anacin, Ascriptin, Bayer Aspirin)

Efficacy in preventing stroke relies on its inhibitory effect on platelet function. This presumably would help prevent thrombus formation and propagation.

Dosing

Adult

81-650 mg PO qd

Pediatric

Not established; 2-5 mg/kg/d PO typically used

Interactions

Effects may decrease with antacids and urinary alkalinizers; corticosteroids decrease salicylate serum levels; additive hypoprothrombinemic effects and increased bleeding time may occur with coadministration of anticoagulants; may antagonize uricosuric effects of probenecid and increase toxicity of phenytoin and valproic acid; doses > 2 g/d may potentiate glucose-lowering effect of sulfonylurea drugs

Contraindications

Documented hypersensitivity; liver damage; hypoprothrombinemia; vitamin K deficiency; other coagulation or bleeding disorders; concurrent anticoagulants; severe anemia; asthma; preexisting gastric ulcer disease; positive test for stool blood; due to possible association with Reye syndrome, do not use in children (<16 y) with flu

Precautions

Pregnancy

D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus

Precautions

May cause transient decrease in renal function and aggravate chronic kidney disease

Follow-up

Prognosis

Outcome of moyamoya disease depends on severity and nature of hemorrhage; prognosis depends on recurrent attacks.

Miscellaneous

Medicolegal Pitfalls

• Misdiagnosis and delayed diagnosis of moyamoya disease (MMD) are particular pitfalls. Misdiagnosis can occur easily if the physician does not incorporate MMD in the differential diagnosis of any patient presenting with stroke. How high MMD is ranked in the differential will depend on presence of atypical features such as young age and absence of obvious risk factors for stroke. If MMD is not considered seriously, then appropriate diagnostic tests may not be performed and a delay in diagnosis could result. Since definitive treatment may be surgery, any delay could allow unnecessary progression of disease.
• If an ischemic stroke that is being treated with antiplatelet agents or anticoagulants does not respond to therapy, then MMD should be considered as a possible etiology. This is especially true if results of a hypercoagulability profile are unremarkable.
• Physicians practicing in the community who encounter atypical stroke presentations should not hesitate to seek consultation with a stroke specialist or even to transfer a patient to a facility equipped to care for complex cases.

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Keywords

moyamoya disease, MMD, arterial occlusive disease, primary cerebral, puff of smoke, Graves disease, thyrotoxicosis, leptospirosis, tuberculosis, aplastic anemia, Fanconi anemia, sickle cell anemia, lupus anticoagulant, Apert syndrome, Down syndrome, Marfan syndrome, tuberous sclerosis, Turner syndrome, von Recklinghausen disease, Hirschsprung disease, atherosclerotic disease, coarctation of the aorta and fibromuscular dysplasia, cranial trauma, radiation injury, parasellar tumors, hypertension

Contributor Information and Disclosures

Author

Roy Sucholeiki, MD, Director, Comprehensive Seizure and Epilepsy Program, The Neurosciences Institute at Central DuPage Hospital
Roy Sucholeiki, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, and American Neuropsychiatric Association
Disclosure: UCB Pharma Honoraria Speaking and teaching

Coauthor(s)

Jasvinder Chawla, MBBS, MD, MBA, Associate Professor of Neurology, Director of Neurology Residency Training Program, Director of Clinical Neurophysiology Laboratory, Assistant Director of Neurology Clerkship Program, Department of Neurology, Loyola University Medical Center
Jasvinder Chawla, MBBS, MD, MBA is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, and American Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Robert Stanley Rust Jr, MD, MA, Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, Director, Child Neurology, University of Virginia; Chair-Elect, Child Neurology Section, American Academy of Neurology
Robert Stanley Rust Jr, MD, MA is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, American Headache Society, American Neurological Association, Child Neurology Society, International Child Neurology Association, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Kenneth J Mack, MD, PhD, Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic
Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, and Society for Neuroscience
Disclosure: Nothing to disclose.

CME Editor

Matthew J Baker, MD, Consulting Staff, Collier Neurologic Specialists, Naples Community Hospital
Matthew J Baker, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD, Assistant Professor, Department of Neurology, Division of Pediatrics, Department of Pediatrics, Oregon Health and Science University; Consulting Staff, Shriners Hospital for Children
Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, and Child Neurology Society
Disclosure: Nothing to disclose.

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