Primary Neurosarcoidosis

Abstract

Neurosarcoidosis is primarily a disease of the leptomeninges although parenchymal involvement is less common. We describe a patient presenting with symptoms and signs of intracranial hypertension and diabetes insipidus. Brain CT scan showed periventricular and hypothalamic areas of increased density that enhanced after contrast administration. After steroid treatment, neurological symptoms resolved but diabetes insipidus persisted.

MRI study performed 10 days later revealed a significant reduction of the previously demonstrated lesions, that were slightly hyperintense in T2-weighted images, while the hypothalamic lesions enhanced after Gd-DTPA administration. These lesions, due probably to infallamatory infiltration and/or oedema, were not present in a neuroradiological follow-up performed 2 and 3 months later. Two months after neurological onset the patient developed a systemic sarcoidosis. Primary neurosarcoidosis should be suspected in the presence of multiple intracranial lesions which improve after steroid therapy. MRI is the neuroradiological study of choice in the diagnosis and follow-up of this rare disease.