Primary angiitis of the central nervous system

Case contributed by Dr Mohammad A. ElBeialy

Presentation

Young boy with headache, mental status change and right sided weakness.

Patient Data

Age: 12 years
Gender: Male

Left basal ganglia few (about three) foci of abnormal signal intensity are seen demonstrating hypointense T1 / FLAIR and hyperintense T2 signal with perifocal gliosis.

MR angiography shows attenuated cervical petrous, cavernous and supraclinoid portions of the left ICA with mural irregularity. Attenuated A1 segment of the left ACA as well as M1 and proximal M2 segments of the left MCA with mural irregularity and beaded appearance. Normal caliber and signal intensity of the right ICA as well as the right ACA and MCA.  Attenuated left vertebral artery otherwise, normal vertebro-basilar system as well as normal both posterior cerebral arteries. No evidence of aneurysmal dilatation or AVM.

MR venography shows no evidence of venous sinus thrombosis; normal deep and cortical veins.

Case Discussion

The above described findings are suggestive for CNS vasculitis, mostly primary CNS angiitis, as well as left basal ganglia lacunar infarction with developing encephalomalacia.

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Case information

rID: 23877
Case created: 15th Jul 2013
Last edited: 17th Dec 2015
Inclusion in quiz mode: Included

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