Ponto-bulbar palsy with deafness (Brown-Vialetto-Van Laere syndrome): A report on three cases
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Cited by (51)
Brown-Vialetto-van Laere and Fazio-Londe overlap syndromes: A clinical, biochemical and genetic study
2012, Neuromuscular DisordersBrown-Vialetto-Van Laere Syndrome, a Ponto-Bulbar Palsy with Deafness, Is Caused by Mutations in C20orf54
2010, American Journal of Human GeneticsCitation Excerpt :The course is invariably progressive, but the rate of decline is variable within and between families. With disease evolution, long tract signs, lower motor neuron signs, cerebellar ataxia, and lower cranial nerve (III-VI) palsies develop, giving rise to a complex picture resembling amyotrophic lateral sclerosis.5,6,7,8 Diaphragmatic weakness and respiratory compromise are some of the most distressing features, leading to recurrent chest infections and respiratory failure, which are often the cause of patients' demise.5,6
Clinical features and neurophysiological follow-up in a case of Brown-Vialetto-Van Laere syndrome
2005, Neuromuscular DisordersInherited Neuronal Atrophy and Degeneration Predominantly of Lower Motor Neurons
2005, Peripheral Neuropathy: 2-Volume Set with Expert Consult BasicInherited Neuronal Atrophy and Degeneration Predominantly of Lower Motor Neurons
2005, Peripheral Neuropathy
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