A Rare Cause of Seizure; Lipoid Proteinozis (Urbach-Wiethe disease): A Case Report

Lipoid Proteinozis (LP) known as Urbach-Wiethe disease is a rare autosomal recessive inherited genodermatozis. Patients usually present with hoarseness and skin-mucosa abnormalities. Lipoid Proteinozis involves the central nervous system (CNS) rarely. The essential imaging finding in LP is appearance of atypical intracranial calcifications, mostly occurring in the medial temporal lobes.  Herein we report a rare case presenting with seizure accompanied computed tomography (CT), magnetic resonance imaging (MRI) findings and also with pathological confirmation.