Esclerose lateral amiotrófica com surdez: relato de um caso e revisão da literatura

Amorim, Annibal Coelho de; Mattos, James Pitágoras de; Lima, J.A. De; Filho, C. A. Cavalcante; Lima, Ana Luisa Xavier de

doi:10.1590/s0004-282x1982000200012

Arq. Neuro-Psiquiatr.

1982

DOI: 10.1590/s0004-282x1982000200012

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Esclerose lateral amiotrófica com surdez: relato de um caso e revisão da literatura

Annibal Coelho de Amorim¹,

James Pitágoras de Mattos²,

J.A. De Lima³

et al.

Abstract: É relatado um caso de paralisia bulbo-pontina crônica progressiva com surdez, de início precoce, evoluindo de maneira protraída há 9 anos. Pelos achados histopatológicos e eletromiográficos concluem tratar-se de variante da forma juvenil de esclerose lateral amiotrófica, forma de Van Laere.

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Cited by 3 publications

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Brown-Vialetto-Van Laere syndrome in a large inbred Lebanese family: Confirmation of autosomal recessive inheritance?

Mégarbané¹,

Desguerres

Rizkallah

et al. 2000

Am. J. Med. Genet.

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Brown-Vialetto-Van Laere syndrome or pontobulbar palsy with deafness is a rare disorder characterized by bilateral nerve deafness, a variety of cranial nerve disorders usually involving the motor components of the 7th and 9th to 12th cranial nerves, and less commonly an involvement of spinal motor nerves and upper motor neurons. Familial and sporadic cases have been reported. Based on particular evidence, autosomal recessive, autosomal dominant, and X-linked inheritance, as well as autoimmune origin have been considered. We report on a large inbred Lebanese family with four patients of both sexes, strongly suggesting autosomal recessive inheritance.

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Brown-Vialetto-Van Laere syndrome in a large inbred Lebanese family: Confirmation of autosomal recessive inheritance?

Mégarbané¹,

Desguerres

Rizkallah

et al. 2000

Am. J. Med. Genet.

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show abstract

Juvenile-onset bulbospinal muscular atrophy with deafness: Vialetta-van laere syndrome or madras-type motor neuron disease?

et al. 1987

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A girl with rapid-onset, bulbospinal muscular atrophy and deafness is described. The patient's mother and brother showed EMG features consistent with subclinical involvement. T is bulbospinal form of spinal muscular atrophy associated with deafness described by Vialetto and van Laere closely resembles the Madras type of motor neuron disease, also associated with deafness, described by Jagganathan and colleagues.

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The Brown‐Vialetto‐van Laere syndrome: a case report and literature review

Davenport

Mumford

1994

Euro J of Neurology

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We describe a female presenting with a progressive bulbar palsy, deafness and respiratory failure necessitating long-term ventilation. These clinical features are consistent with the Brown-Vialetto-van Laere syndrome, a rare, sometimes inherited progressive ponto-bulbar neuronopathy with associated deafness. This case provides further evidence of the genetic heterogeneity of the syndrome and we describe a previously unreported form of treatment which had negative results. We suggest that improved recognition of this rare syndrome is essential to provide aetiological clues and we describe the worthwhile symptomatic improvement which can be achieved with simple supportive measures.

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Esclerose lateral amiotrófica com surdez: relato de um caso e revisão da literatura

Cited by 3 publications

References 6 publications

Brown-Vialetto-Van Laere syndrome in a large inbred Lebanese family: Confirmation of autosomal recessive inheritance?

Brown-Vialetto-Van Laere syndrome in a large inbred Lebanese family: Confirmation of autosomal recessive inheritance?

Juvenile-onset bulbospinal muscular atrophy with deafness: Vialetta-van laere syndrome or madras-type motor neuron disease?

The Brown‐Vialetto‐van Laere syndrome: a case report and literature review

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