Steroid-responsive multifocal demyelinating neuropathy with central involvement
β Scribed by Rocco Liguori; Romana Rizzi; Roberto Vetrugno; Fabrizio Salvi; Alessandra Lugaresi; Sabina Cevoli; Pasquale Montagna
- Publisher
- John Wiley and Sons
- Year
- 1999
- Tongue
- English
- Weight
- 108 KB
- Volume
- 22
- Category
- Article
- ISSN
- 0148-639X
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β¦ Synopsis
We describe 2 patients with associated central and peripheral demyelination. Electrophysiological studies revealed a demyelinating polyneuropathy with sensory and motor conduction blocks. Visual evoked potentials were abnormal. Motor evoked potentials showed abnormal central conduction time in 1 patient. Magnetic resonance imaging revealed regions of abnormal high signal in the spinal cord and brain; sural nerve biopsy disclosed a demyelinating neuropathy. Both patients showed clinical and electrophysiological improvement after steroid therapy.
π SIMILAR VOLUMES
Hereditary neuropathy with liability to pressure palsies (HNPP) is usually caused by a 1.5-Mb deletion in chromosome 17~11.2, the inverse mutation to the duplication seen in the majority of Charcot-Marie-Tooth type 1A (CMT IA) patients. Although most patients with HNPP present with pressure palsies