A case with unusual stroke and fulminant outcome in a Hispanic male

Abstract

Patient: Male, 42 FINAL DIAGNOSIS: Moyamoya disease (MMD) Symptoms: Aphasia • concentration difficulty • dysarthria • personality change

Medication: - Clinical Procedure: - Specialty: Radiology.

Objective: Rare disease.

Background: Moyamoya disease (MMD) was first described in 1957 as "hypoplasia of the bilateral internal carotid arteries." The characteristic appearance of the associated network of abnormally dilated collateral vessels on angiography was later likened to "something hazy, like a puff of cigarette smoke," which, in Japanese, is Moyamoya. This paper describes the fulminant course of the disease in a Hispanic male involving the corpus callosum.

Case report: A 42-year-old Hispanic male with progressive aphasia, slow mentation, and sudden onset of sensorimotor symptoms with gait disturbance was found to have multiple intracranial supratentorial infarcts of variable stages of evolution involving, but not limited to, the anterior corpus callosum, followed by rapid development of further infarcts. Angiography demonstrated right ACA occlusion, left supraclinoid ICA occlusion with a Moyamoya pattern of collateralization, and diffuse arteriopathy. A fulminant course ensued and the patient did not survive the acute phase of ischemic disease.

Conclusions: Moyamoya disease may rarely present in North American Hispanic males, with advanced atypical clinical and imaging features involving the anterior corpus callosum and having a fulminant course.

Keywords: corpus callosum; infarction; moya moya disease.

Figure 1

Brain MRI. (A, B) diffusion weighted sequence and apparent diffusion coefficient map, respectively, demonstrate a focal area of restriction of diffusion involving the right rostrum, genu and body of the Corpus Callosum with mild enhancement on post-gadolinium T1W sequence (C) and moderate edema and local mass effect on T2W sequence (D). A second lesion involving the right basal ganglia and genu of the right internal capsule demonstrates incomplete peripheral enhancement with central necrosis, without mass effect or restriction of diffusion.

Figure 2

Cerebral angiography. Biplanar cerebral angiography of the left internal carotid artery ((A, B) lateral and AP respectively) demonstrated complete occlusion of the left supraclinoid internal carotid artery with a patent left anterior choroidal artery supplying lenticulostriatal vessels giving the angiographic appearance of a “puff of smoke,” suggestive of Moyamoya angiographic pattern. Collateral flow from the right posterior choroidal artery supplying the pericollosal, frontopolar and callosmarginal arteries demonstrated on lateral view on selective angiogram of the left vertebral artery (C). Intracranial view of the right internal carotid artery in the AP projection (D) demonstrates an irregular M1 segment and complete occlusion of the mid A1 segment of anterior cerebral artery. Cortical branches also appeared irregularity consistent with a proliferative intracranial vasculopathy.