Early Diagnosis and Monitoring of Neurodegenerative Langerhans Cell Histiocytosis.

ABSTRACT:

Background

Neurodegenerative Langerhans Cell Histiocytosis (ND-LCH) is a rare, unpredictable consequence that may devastate the quality of life of patients cured from LCH. We prospectively applied a multidisciplinary diagnostic work-up to early identify and follow-up patients with ND-LCH, with the ultimate goal of better determining the appropriate time for starting therapy.

Methods

We studied 27 children and young adults with either ND-LCH verified by structural magnetic resonance imaging (MRI) (group 1) or specific risk factors for (diabetes insipidus, craniofacial bone lesions), but no evidence of, neurodegenerative MRI changes (group 2). All patients underwent clinical, neurophysiological and MRI studies.

Results

Seventeen patients had MRI alterations typical for

SUBMITTER: Sieni E 

PROVIDER: S-EPMC4503531 | biostudies-literature | 2015

REPOSITORIES: biostudies-literature