Moyamoya disease in a patient with schizophrenia

Abstract

We present the case of a 23-year-old Vietnamese male with a 2-year history of a psychotic illness marked by prominent negative symptoms, fatuousness and disturbed behavior. Neuroimaging revealed a prominent vascular flow void affecting the middle and anterior cerebral arteries, with associated increased collateral supply to the frontal cortex, consistent with Moyamoya disease. Neurological examination was unremarkable; however, neuropsychological assessment revealed significant executive dysfunction, including stimulus-driven behavior. Whilst the diagnosis of schizophrenia and Moyamoya disease may be coincidental, an interaction between the 2 diseases may have led to some of the atypical features of this case, including prominent executive dysfunction and marked sensitivity to psychotropic medication. We discuss the nature of possible interactions between the 2 conditions. This case also highlights the importance of re-evaluating patients with atypical or treatment-resistant psychoses for cerebral pathology.

Effect of Moyamoya Disease on Neuropsychological Functioning in Adults

“…Approximately half of the patients in the current study had impairment in executive abilities, such as initiation and mental set-shifting efficiency. In the three previous case studies in which executive functioning was measured (3,14,18), impairment was found on some measures but not others. The consistent impairment of executive abilities in the current study raises the possibility that adult moyamoya disease affects frontal lobe function.…”

“…Three of the patients (and questionably a fourth) had an infarction in the right hemisphere before neuropsychological testing (3,11,14,21). The fifth patient had a diagnosis of schizophrenia and was actively psychotic when evaluated (18). The occurrence of a major cerebral infarction before neuropsychological testing raises a significant conceptual issue when attempting to understand the cognitive effects of moyamoya disease itself; the fundamental pathology may well be that of microvascular ischemia, but some patients do experience one or more major cerebral infarctions or hemorrhages.…”

“…The results of presurgical neuropsychological testing in these cases indicate that impairment in intellect (18), speed of information processing (3), executive functioning (18), and visual spatial ability (11,14,21) can occur. In this small series of cases, the most consistent finding was relatively intact memory functioning (3,18,21). The only exception involved a case of right hemisphere infarction, resulting in impaired nonverbal memory (14).…”

“…The adult literature is composed of five case studies (3,11,14,18,21). The results of presurgical neuropsychological testing in these cases indicate that impairment in intellect (18), speed of information processing (3), executive functioning (18), and visual spatial ability (11,14,21) can occur. In this small series of cases, the most consistent finding was relatively intact memory functioning (3,18,21).…”

Moyamoya syndrome causing stroke in young women with type 1 diabetes

“…This disorder was first described in Asians and has since been reported in American and European populations. In North America, moyamoya tends to affect young women and present as transient ischemic attacks or stroke, with accompanying sensory-motor deficits or cognitive impairment (Bower et al, 2013; Starke et al, 2012); rarer presentations may include personality or behavioral changes that mimic psychiatric illness (Lubman et al, 2003; Scott and Smith, 2009). Moyamoya can develop in association with radiation exposure, certain genetic disorders, or immunological disorders and is inclusively named “moyamoya syndrome,” while the term “moyamoya disease” is reserved for idiopathic cases without associated risk factors (Scott and Smith, 2009).…”