Atypical Mood Symptoms as the Presenting Feature of Moyamoya Disease: A Case Report

Dear Sir,

Moyamoya disease is a rare vaso-occlusive disease that results in transient ischemic attacks or strokes. Neuropsychiatric manifestations have been uncommonly reported to be associated with moyamoya disease. We report the case of Mrs S, a 35-year-old, right-handed female who presented to Regional Mental Hospital, Nagpur, India. She was brought by her husband, with complaints of pervasive irritable behavior, sleep disturbances, and intermittent headaches for the last one year. She complained of difficulties in concentration and in remembering recent events and of not being able to perform her daily chores as earlier. She had a history of a similar episode at the age of 24 years. At the age of 30 years, she had had a transient right-sided hemiparesis, which resolved over a week. On mental status examination, she expressed persecutory delusions, her mood was predominantly irritable, and she had increased psychomotor activity. Her physical examination findings were unremarkable. Neurological examination did not reveal any residual motor paresis or other signs of upper motor neuron lesions. Routine lab investigations inclusive of hemogram, fasting glucose, serum electrolytes, and kidney, liver, and thyroid function tests were normal. Her 2D echocardiogram revealed mild mitral valve prolapse with normal biventricular function.

In view of the atypical nature of her mood symptoms and the history of hemiparesis, a magnetic resonance imaging of the brain was performed. It revealed a focal area of gliosis in the left frontal lobe. Subsequently, magnetic resonance (MR) angiography revealed gross irregular narrowing of the distal internal carotid artery and proximal middle cerebral artery and non-visualization of the distal anterior and middle cerebral arteries. There was extensive bilateral collateralization, and multiple chronic infarcts were noted. Digital subtraction angiography revealed complete occlusion of bilateral middle and anterior cerebral arteries, with collaterals. The above findings were supportive of a diagnosis of moyamoya disease,¹ which was also confirmed by a neurologist. She was managed conservatively with antiplatelet drugs and folate supplementation.

Her mood syndrome fulfilled the criteria for an episode of mania with psychotic symptoms. She was started on T. lithium carbonate 600 mg/day, T. divalproex sodium 1,000 mg/day, T. olanzapine 10 mg/day, and T. trihexyphenidyl 4 mg/day. The mood symptoms responded well to the regime. The psychotic symptoms too resolved, and olanzapine was stopped within two weeks. As her primary diagnosis was a mood disorder, lithium and divalproex sodium were continued in the same dose. The patient is on regular follow-up till date, and she has not had a recurrence of manic symptoms, though cognitive deficits and an impairment in socio-occupational functioning persists. The patient’s husband consented for reporting this case.

Moyamoya disease is a rare inherited vaso-occlusive disease of uncertain etiology that typically presents in the first or fourth decades of life. The genetics of moyamoya disease is not well understood. Adults with the disorder usually present with intracranial hemorrhage or cerebral infarction, whereas pediatric cases present with recurrent episodes of cerebral ischemia.² The disease is characterized by unilateral or bilateral stenosis or occlusion of the intracranial segment of the internal carotid artery and the proximal parts of anterior and middle cerebral arteries. Collateral vessels develop subsequently, giving the characteristic “puff of smoke” appearance on cerebral angiogram.³

Psychotic disorders and mood disorders have been reported in moyamoya disease.⁴ Behere⁵ reported the case of a 16-year-old boy who presented with similar mood symptoms such as pervasively irritable mood and increased psychomotor activity. Lai et al.⁶ reported the case of a 34-year-old Chinese female with depression and catatonic symptoms in association with moyamoya disease. Singh et al.⁷ reported the case of a 22-year-old female from Delhi, where a focus on the treatment of behavioral symptoms led to a delay in the diagnosis of moyamoya disease.

In the current case, moyamoya disease was identified as an incidental finding in a 35-year-old female who presented with a mood disorder. Adding a mood stabilizer with antiepileptic properties such as valproate or carbamazepine may be preferred in the treatment of organic mood disorders.⁸ Nagata et al.⁹ reported the efficacy of carbamazepine in controlling impulsive aggression secondary to frontal infarcts in a patient with moyamoya disease.

There are certain similarities in cases reporting neuropsychiatric manifestations in moyamoya disease: (a) Cases came to clinical attention for their mood symptoms and behavioral disturbances; (b) atypical presentation of mood symptoms and past history of neurological deficit raised suspicion towards underlying organicity, prompting investigations; and (c) diagnosis of moyamoya disease was “incidental” and supported by classical findings on MR angiography. These observations point towards an emerging consistent pattern in the atypical clinical presentation of mood disorder associated with moyamoya disease. In the evaluation of possible organic causes for mood disorders, clinicians need to consider moyamoya disease as a possible differential diagnosis.