Neurol Case Rep | Volume 5, Issue 1 | Case Report | Open Access

A Rare Cause of Progressive Cerebellar Ataxia and Dementia

Matta C¹, Costa J^1*, Yazbek S² and Abboud H¹

¹Department of Neurology, Faculty of Medicine, Saint Joseph University, Beirut, Lebanon ²Department of Diagnostic Radiology and Nuclear Medicine, University of Maryland School of Medicine, USA

^*Correspondance to: Jad Costa 

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Abstract

Erdheim-Chester Disease (ECD) is a rare histiocytic multisystem disease, affecting bones, heart, aorta, lungs, skin, retroperitoneum and Central Nervous System (CNS), caused by a mutation in the MAPK signaling pathway, often the BRAF proto-oncogene. We report the case of a 42-year-old man presenting with cognitive impairment and progressive cerebellar ataxia due to the CNS involvement of his ECD.

Keywords:

Erdheim-Chester disease; Ataxia; Dementia; Neuroimaging

Citation:

Matta C, Costa J, Yazbek S, Abboud H. A Rare Cause of Progressive Cerebellar Ataxia and Dementia. Neurol Case Rep. 2022; 5(1):1033..