Neuropathy of metachromatic leukodystrophy: improvement with immunomodulation.

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.

• Authors: Y Nevo, A Pestronk, G Lopate, S L Carroll
• Journal: Pediatric neurology (Pediatr Neurol) Vol. 15 Issue 3 Pg. 237-9 (Oct 1996) ISSN: 0887-8994 [Print] United States
• PMID: 8916163 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunosuppressive Agents
• Cyclosporine
• Azathioprine

Topics

• Azathioprine (therapeutic use)
• Child, Preschool
• Cyclosporine (therapeutic use)
• Female
• Humans
• Immunization, Passive
• Immunosuppressive Agents (therapeutic use)
• Leukodystrophy, Metachromatic (diagnosis, therapy)
• Neural Conduction