Objectives Neurosarcoidosis is a rare disorder with diverse clinical manifestations and a wide range of radiological features on MRI that make diagnosis challenging. Here we present a case of neurosarcoidosis with perivascular punctate enhancement on brain and spine MRI. Differential diagnosis of this characteristic radiological pattern are discussed.
Case A 43 year old man presented with progressive cognitive decline and severe fatigue. Brain MRI demonstrated confluent white matter changes with punctate, perivascular gadolinium enhancement. Spine MRI showed increased signal intensity with punctate enhancement. A brain biopsy revealed a non-caseating granulomatous process related to blood vessels and the Virchow-Robin space, without necrosis, vascular destruction or any signs of vasculitis. Systemic sarcoidosis was excluded. Cyclophosphamide with steroids was effective.
Conclusions Perivascular punctate enhancement on MRI has been described in a number of neurological conditions including chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS), isolated angiitis of the CNS, lymphoma, multiple sclerosis and natalizumab-associated progressive multifocal leukoencephalopathy. Although rare, it can also be observed in patients with neurosarcoidosis. Recognition of punctuate enhancement on brain or spine MRI should expedite further investigations and include work up for this condition. This case emphasises the inclusion of neurosarcoidosis in the differential diagnosis of perivascular punctate enhancement on brain and spine MRI. Enhancement is caused by perivascular granulomata and although rare, cerebrovascular sarcoidosis is a recognised entity.