Acute Motor and Sensory Axonal Neuropathy in LEOPARD Syndrome

Richard J. Beukers, Annemarie C. M. van Bellegem, Mariken Gruppen, Wouterina C. G. Overweg-Plandsoen, Marinus Vermeulen

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Abstract

A case of acute predominantly axonal motor and sensory neuropathy (AMSAN) is reported in a 16-year-old boy with LEOPARD syndrome (the acronym represents lentigines, ECG conduction abnormalities, ocular hypertelorism, pulmonic stenosis, abnormal genitalia, retardation of growth, and sensorineural deafness). The presentation was atypical for acute motor and sensory axonal neuropathy, in that this patient had progression of symptoms of more than 4 weeks and there were signs of reinnervation in the acute phase. Treatment response to intravenous immunoglobulins was excellent. In patients with LEOPARD syndrome and acute neuropathies, treatment with intravenous immunoglobulins should be considered. (C) 2010 by Elsevier Inc. All rights reserved
Original languageEnglish
Pages (from-to)301-303
JournalPediatric neurology
Volume42
Issue number4
DOIs
Publication statusPublished - 2010

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