MOYAMOYA DISEASE IS an inherited vaso-occlusive disease in which dilated collateral vessels appear hazy on angiography.1 Neuropsychiatric manifestations associated with moyamoya disease are uncommon.2 We describe a case of a 16-year-old boy with atypical presentation of mania, prompting investigations and a subsequent diagnosis of moyamoya disease. Consent for publishing this letter was obtained from the boy's father.

A 16-year-old boy presented with a 4-month history of a pervasively irritable mood, increased psychomotor activity, and unremarkable family, developmental and substance use histories. He met the ICD-10 criteria for mania without psychotic symptoms, and he scored 29 on Young's Mania Rating Scale. Neurological examination revealed right upper motor neuron facial palsy, right extensor plantar response, and grade 5 power in all limbs, with other systems being normal. On treatment with risperidone (tablet) 4 mg/day and trihexyphenidyl (tablet) 4 mg/day, he developed extrapyramidal side effects with no improvement in his irritability.

Atypical clinical presentation with focal neurological signs prompted a CT scan of the brain that showed left hemiatrophy, left parietal post-central calcification with small collaterals in sylvian fissure, and non-visualization of the left middle cerebral artery. MRI and magnetic resonance angiography showed narrowing of the left supraglenoid internal carotid artery with watershed infarcts, suggestive of moyamoya disease.

Further investigations including a complete hemogram, liver and renal function tests, fasting blood glucose, lipid profile, serum ceruloplasmin, homocysteine, urine copper and abnormal metabolites, echocardiography and electroencephalogram were normal. Serum antiphospholipid antibody, Venereal Disease Research Laboratory and HIV tests were negative.

With combination of lithium carbonate and olanzapine, the patient again developed extrapyramidal side effects. His manic symptoms eventually improved with sodium valproate (tablet) 1000 mg/day. The neurosurgical team advised conservative management along with folate supplementation. Over next 6 months, the patient had no relapse of manic symptoms or further neurological deficits, but he did not continue with follow-ups after that time.

Neuropsychiatric manifestations of moyamoya disease are uncommon. Previous case reports have described psychotic disorders in association with moyamoya disease.2,3 However, mania in association with this disease is rare. The atypical presentation of mood disorder in this case prompted neuroimaging, which revealed findings consistent with moyamoya disease.

The patient was observed to be susceptible to the side effects of conventional psychotropics. As such, valproate appears to be a safer choice in treating comorbid mood disorders. Nagata et al. reported the efficacy of carbamazapine in controlling impulsive aggression secondary to frontal infarcts in a patient with moyamoya disease.4 Our case report supports previously reported observations that anticonvulsants in general and divalproex sodium in particular are effective in secondary mania.5

Recent genetic studies have implicated chromosome 17 in bipolar disorder and moyamoya disease,6 which suggests a common neurobiological basis. This case highlights the importance of evaluating patients with atypical presentation of psychiatric disorders for possible underlying neurological conditions and the challenges in treating them.


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