Affordable Access

Neuropathy of metachromatic leukodystrophy: improvement with immunomodulation.

Authors
Type
Published Article
Journal
Pediatric Neurology
0887-8994
Publisher
Elsevier
Publication Date
Volume
15
Issue
3
Pages
237–239
Identifiers
PMID: 8916163
Source
Medline
License
Unknown

Abstract

A 4-year-old child with metachromatic leukodystrophy was initially diagnosed with chronic immune demyelinating polyneuropathy and treated with immunosuppressive therapy. Physical examination revealed diffuse, distal > proximal weakness and areflexia. Electro-diagnostic studies revealed nerve conduction velocities that were slowed to variable degrees in different nerves. In the 18 months after institution of immunomodulating therapy, she had functionally significant improvement and a quantitative increase in her strength. Treatment was discontinued at age 6 years when the patient developed urinary incontinence, followed by loss of motor and cognitive skills. We conclude that immunomodulation early in the course of metachromatic leukodystrophy presenting as a neuropathy may result in temporary functional improvement. Whether the immunomodulation altered the disease progression or had direct effects on the function of the dysmyelinated axons is not known.

Statistics

Seen <100 times