Dados do Trabalho

Título

HIV associated Moyamoya syndrome manifestation as an acute hemichorea

RESUMO

Case report: A 24 year-old presented to our hospital clinic complaining about spontaneous movements in the left side of her body which began approximately 6 months earlier. Her medical history was notable for congenital HIV infection (undetectable viral load and CD4 count of 506), well controlled epilepsy and migraine. She has been on antiretroviral therapy (ART), amitriptyline, valproate and low dose chlorpromazine. She denied any other neurological or psychiatric symptoms, use of other drugs or seizures recurrence in the past ten years. Her level of consciousness was normal. There were random involuntary movements in the left side of her face, arm and leg in a choreoathetoid phenomenology and incorporated to voluntary movements. Distraction had no influence on the pattern of her movements. Her left limbs were hypotonic. CSF profile, bacterial and fungi cultures, erythrocyte sedimentation rate, thyroid function tests, glycosylated hemoglobin A1c were unremarkable. Brain magnetic resonance imaging (MRI) showed no acute or chronic stroke lesions, nor evidence of neuroinfection. The time-of-flight angiogram was notable for absence of flow in both middle cerebral arteries, suggesting Moyamoya syndrome (MMS). Arterial angiography located the occlusion on both supraclinoid segments of internal carotid arteries with blood flow redirected to a wide collateral circulation and posterior communicating arteries, confirming MMS pattern. Reviewing older exams a contrast CT scan done 4 years before had no abnormalities, including arterial circulation. We found no other etiology to justify her chorea besides the MMS. The patient received daily risperidone 2mg with complete resolution of the symptoms. Discussion: MMS is a rare cause of chorea with a frequency of 3 to 6%, as well incidence of MMD in HIV patients. The dilated collateral vessels compress the basal ganglia or can cause local ischemia, which can lead to a disruption of normal striatal activity. HIV infection has been reported as one of many conditions implicated in the etiopathogenesis of MMS. HIV can cause endothelial damage and also induce proinflammatory states that later manifest as stenosis or dilation of the vessel wall that can occur despite effective ART and stable CD4 counts. Final conclusion: Since HIV infection has a well recognised relation to cerebrovascular disease as well as to movement disorders, we believe that it was a major factor to the development of this secondary form of chorea.

Palavras Chave

Coreia, HIV, Moyamoya

Área

Doença Cerebrovascular