Neurologic presentation of Erdheim-Chester disease

Abstract

Erdheim-Chester disease is a rare, systemic histiocytosis that involves multiple organ systems and causes symmetric sclerosis of the metaphysis and diaphysis of the long bones. We present 2 cases and reviewed 108 patients reported in the literature who had neurologic manifestations of Erdheim-Chester disease. After eye involvement or diabetes insipidus, cerebellar symptoms were most frequently encountered, followed by tumor, headaches, cord compression, mental status change, seizures, and change in libido. A wide range of neurological symptoms can be seen in ECD. Therefore we hope the review brings more awareness about this disorder.