Neurological manifestations of Erdheim-Chester Disease.

ABSTRACT:

Objective

To characterize the spectrum of neurologic involvement in Erdheim-Chester Disease (ECD), a treatable inflammatory neoplasm of histiocytes.

Methods

Sixty-two patients with ECD were prospectively enrolled in a natural history study that facilitated collection of clinical, imaging, laboratory, neurophysiologic, and pathologic data.

Results

Ninety-four percent of the patients had neurologic abnormalities on examination or imaging, and 22% had neurologic symptoms as the initial presentation of ECD. The most common neurologic findings were cognitive impairment, peripheral neuropathy, pyramidal tract signs, cranial nerve involvement, and cerebellar ataxia. Imaging revealed atrophy and demyelination along with focal lesions that were located throughout the nervous s

SUBMITTER: Boyd LC 

PROVIDER: S-EPMC7187721 | biostudies-literature | 2020 Apr

REPOSITORIES: biostudies-literature