PMID: 2110121Feb 1, 1990Paper

Late onset globoid leukodystrophy: unusual clinical and CSF findings

Italian Journal of Neurological Sciences
S RolandoA Leonardi

Abstract

During a febrile upper respiratory tract illness this 4 year old boy developed left hemiparesis, which progressed to loss of walking and even of sitting finally to tetraplegia. The cerebrospinal fluid protein pattern showed blood-brain barrier damage with additional intrathecal IgG synthesis. The symptoms responded to steroid therapy but resumed and worsened on withdrawal. Only late, when visual evoked potentials and nerve conduction velocity proved to be impaired, was Krabbe disease diagnosed on the assay of cultured fibroblasts for galactocerebroside-beta-galactosidase. We discuss the significance of possible endogenous production of IgG in the CNS.

Citations

May 1, 1993·Italian Journal of Neurological Sciences·A R Giovagnoli
Feb 21, 2008·Zeitschrift für Gerontologie und Geriatrie·A SchmidtkeC Löhr

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