Neuropathy of metachromatic leukodystrophy: improvement with immunomodulation.
Pediatr Neurol
; 15(3): 237-9, 1996 Oct.
Article
em En
| MEDLINE
| ID: mdl-8916163
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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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Azatioprina
/
Imunização Passiva
/
Ciclosporina
/
Imunossupressores
/
Leucodistrofia Metacromática
Tipo de estudo:
Diagnostic_studies
Limite:
Child, preschool
/
Female
/
Humans
Idioma:
En
Revista:
Pediatr Neurol
Assunto da revista:
NEUROLOGIA
/
PEDIATRIA
Ano de publicação:
1996
Tipo de documento:
Article
País de afiliação:
Estados Unidos