Moyamoya-like vasculopathy in neurosarcoidosis

Abstract

A 31-year-old man presented with dull headache and memory disturbance lasting for one week. Computed tomographic scans revealed acute hydrocephalus. The cerebrospinal fluid contained 53 leukocytes/mm(3), with a mononuclear preponderance and no erythrocytes. Magnetic resonance imaging revealed hydrocephalus and leptomeningeal enhancement. Magnetic resonance angiography and digital subtraction angiography showed supraclinoid occlusion of the right internal carotid artery, which resembled unilateral moyamoya disease. Neuroendoscopic biopsy of a lesion in the septum pellucidum revealed noncaseating granulomas, which was consistent with sarcoidosis. The patient was successfully managed with intravenous methylprednisolone and ventriculoperitoneal shunting. To our knowledge, this is the first case of moyamoya-like vasculopathy associated with neurosarcoidosis.

Keywords: Hydrocephalus; Moyamoya disease; Neurosarcoidosis.

Fig. 1

Non-enhanced computed tomogra-phy image showing enlarged ventricles with interstitial edema, consistent with acute hydrocephalus.

Fig. 2

Gadolinium-enhanced axial (A) and sagittal (B) magnetic resonance images show hydrocephalus and thickened enhancing lesions in the septum pellucidum (arrows).

Fig. 3

Magnetic resonance angiography showing supraclinoid occlusion of the right internal carotid artery as well as the absence of the right anterior and middle cerebral arteries.

Fig. 4

Anteroposterior (A) and lateral view (B) of right internal carotid artery angiography demonstrates occlusion distal to the ophthalmic artery origin of the right internal carotid artery (arrows).

Fig. 5

Biopsy specimen shows noncaseating granulomas (H & E, × 400).