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Neuropathy of metachromatic leukodystrophy: improvement with immunomodulation.

Authors
  • Nevo, Y
  • Pestronk, A
  • Lopate, G
  • Carroll, S L
Type
Published Article
Journal
Pediatric Neurology
Publisher
Elsevier
Publication Date
Oct 01, 1996
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.

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