NEUROPATHY OF METACHROMATIC LEUKODYSTROPHY - IMPROVEMENT WITH IMMUNOMODULATION

A 4-year-old child with metachromatic leukodystrophy was initially dia gnosed with chronic immune demyelinating polyneuropathy and treated wi th immunosuppressive therapy, Physical examination revealed diffuse, d istal >proximal weakness and areflexia, Electrodiagnostic studies reve aled nerve conduction velocities that were slowed to variable degrees in different nerves. In the 18 months after institution of immunomodul ating therapy, she had functionally significant improvement and a quan titative increase in her strength. Treatment was discontinued at age 6 years when the patient developed urinary incontinence, followed by lo ss of motor and cognitive skills. We conclude that immunomodulation ea rly in the course of metachromatic leukodystrophy presenting as a neur opathy may result in temporary functional improvement, Whether the imm unomodulation altered the disease progression or had direct effects on the function of the dysmyelinated axons is not known. (C) 1996 by Els evier Science Inc. All rights reserved.