Progressive Bulbar Paralysis Associated With Neural Deafness

Alberca, R; Montero, Claudio; Ibáñez, Agustín; Segura, Dolores; Miranda-Nieves, G

doi:10.1001/archneur.1980.00500530052007

Cited by 27 publications

(23 citation statements)

References 6 publications

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“…The reduced catecholamine levels imply a failure of the peripheral sympathetic efferent system. The ptosis described in several reports and the loss of catecholamine neurons in the brainstem in one autopsied case 10 may be further evidence of autonomic involvement. Treatment with gastrostomy feeding, fludrocortisone acetate and oxygen produced a worthwhile symptomatic improvement.…”

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confidence: 75%

“…In this case 9 there were abnormalities in the substantia nigra, locus coerulus, dorsal column nuclei and degeneration of the lateral leminisci, medial longitudinal fasiculus and trapezoid body and some gliosis of the optic pathways. In the case reported by Alberca et al 10 there was degeneration in spino-cerebellar and pyramidal tracts.…”

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confidence: 84%

“…The syndrome is considered to be hereditary and has been reported in 25 patients. [1][2][3][4][5][6][7][8][9][10][11][12][13][14] Most of the reported cases have come from Europe and North Africa (especially Mediterranean countries such as Portugal, Spain and Tunisia) although a case occurred in a black woman in Brazil. 13 A related syndrome has been described in India and was named the Madras form of anterior horn cell disease.…”

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confidence: 99%

“…1114 A sural nerve biopsy in a 15 year old girl 11 showed only slight depletion of nerve fibers. Autopsies have been carried out on 3 cases: a 25 year old woman, 9 a 27 year old woman 10 and a 2 year old boy. 11 The 8th nerve showed loss of axons and the ventral cochlear nucleus loss of neurons and gliosis.…”

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confidence: 99%

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Bulbo-pontine Paralysis with Deafness: the Vialetto-Van Laere Syndrome

Abarbanel

Ashby

Marquez-Julio

et al. 1991

Can. j. neurol. sci.

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A Caucasian girl developed slowly progressive sensory neural deafness and bulbar and spinal muscle weakness typical of the Vialetto-Van Laere syndrome. As the condition progressed the major disabilities became dysphagia, respiratory muscle weakness and postural hypotension. Treatment with gastrostomy feedings, oxygen and fludrocortisone acetate produced worthwhile functional improvement. RESUME: Paralysie bulboprotuberantielle avec surdite: syndrome de Vialetto-Van Laere. Nous decrivons le cas d'une fille de race caucasienne qui a deVeloppe une surdite neurale lentement progressive et une faiblesse musculaire bulbaire et spinale typique du syndrome de Vialetto-Van Laere. Avec la progression de la maladie, la dysphagie, la faiblesse des muscles respiratoires et l'hypotension posturale ont ete les signes les plus invalidants. L'alimentation par gastrostomie, I'administration d'oxygene et d'acetate de fludrocortisone ont entraine des ameliorations fonctionnelles significatives.Can. J. Neurol. Sci. 1991; 18:349-351 Chronic, progressive bulbo-pontine paralysis associated with sensory neural deafness is a rare disorder also called the Vialetto-Van Laere syndrome. The syndrome is considered to be hereditary and has been reported in 25 patients. [1][2][3][4][5][6][7][8][9][10][11][12][13][14] Most of the reported cases have come from Europe and North Africa (especially Mediterranean countries such as Portugal, Spain and Tunisia) although a case occurred in a black woman in Brazil. 13 A related syndrome has been described in India and was named the Madras form of anterior horn cell disease. 15 ' 16 In this communication we report the clinical and electrophysiological findings of a Canadian patient with VialettoVan Laere syndrome. CASE HISTORYThis Caucasian girl had always been thin. At the age of 12 when riding horses she was aware of weakness of dorsiflexion of the right foot. At the age of 19 she began to have progressive bilateral hearing loss, her face felt "stiff, her voice became nasal and she began choking on liquids. She was admitted to a neurosurgical service and found to have bilateral sensorineural deafness, and weakness of the face and bulbar muscles. Brainstem glioma and multiple sclerosis were considered. MRI scan and 4 vessel angiography were negative. A neurological consultation was requested. Some weakness of the small hand muscles was noted and the diagnosis of Vialetto-Van Laere syndrome was raised. By the age of 21 she had developed drooping of her eyelids and had to tilt her head back in order to see when driving. She now had more obvious weakness of the facial muscles and small muscles of the hand. At age 22 she developed shortness of breath on exertion, postural hypotension, vertigo with sudden head movements and began to spend most of her time in a horizontal posture. There was no family history of neurological disorders and her parents were not related.On examination at the age of 23 she had diffuse loss of muscle bulk (weight 37.8 kg, height 168 cm). The blood pressure supine was 80...

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confidence: 84%

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Bulbo-pontine Paralysis with Deafness: the Vialetto-Van Laere Syndrome

Abarbanel

Ashby

Marquez-Julio

et al. 1991

Can. j. neurol. sci.

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“…As alterações anátomo-patológicas são encontradas principalmente no tronco cerebral e medula cervical e são caracterizadas por degeneração neuronal, redução do número de neurônios e gliose , núcleo do abducente 3 e núcleo motor do trigêmeo. O quiasma e trato óptico podem apresentar desmielinização 2 e nos cornos anteriores da medula pode-se observar degeneração e perda neuronal acentuadas 1,2,5,7 .0 estudo da imagem por ressonância magnética às vezes demonstra atrofia do tronco-cerebral e cerebelo 3 ; é importante também para afastar glioma do tronco, siringobulbia e doenças infecciosas e inflamatórias, que também ocorrem na mesma faixa etária.…”

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Síndrome de Brown-Vialleto-Van Laere: relato de dois casos

Oliveira

Moreira

Cardoso

et al. 1995

Arq. Neuro-Psiquiatr.

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RESUMO -A síndrome de Brown-Vialetto-van Laere é entidade degenerativa hereditária ou esporádica, rara, caracterizada por surdez neuro-sensorial seguida ou acompanhada por uma variedade de alterações de nervos cranianos, podendo ainda ocorrer acometimento de corno anterior e vias ópticas. Relatamos dois casos e discutimos o diagnóstico diferencial e a relação da síndrome com as atrofias espinhais e surdez hereditária.PALAVRAS-CHAVE: síndrome de Brown-Vialetto-van Laere, paralisia ponto-bulbar com surdez, atrofia muscular espinhal, surdez hereditária. Brown-Vialetto-van Laere syndrome: report of two casesSUMMARY -Brown-Vialetto-van Laere syndrome is a rare hereditary or sporadic degenerative disorder characterised by progressive sensoryneural deafness, followed or accompanied by cranial nerve palsies. The anterior horn cells and the optic pathways may be involved in some cases. We report two cases, and comment the differential diagnosis and the relationships of this syndrome to the muscle spinal atrophies and the hereditary deafness.KEY-WORDS: Brown-Vialetto-van Laere syndrome, ponto-bulbar palsy with deafness, muscle spinal atrophy, hereditary deafness.A síndrome de Brown-Vialetto-van Laere (SBVL), ou paralisia ponto-bulbar com surdez, é caracterizada por surdez neuro-sensorial seguida ou acompanhada por uma variedade de alterações de nervos cranianos. Geralmente são acometidos os componentes motores do sétimo nervo e dos nervos bulbares. Menos freqüentemente são acometidos o terceiro, o quinto e o sexto nervos. Em 20% dos casos ocorre atrofia óptica 3 -810 . Os cornos anteriores da medula podem também estar comprometidos 4 , estando, portanto, relacionada às atrofias motoras espinhais". Os casos relatados na literatura são hereditários e esporádicos. O padrão hereditário mais comum é autossômico recessivo 3,8 mas há casos de herança autossômica dominante ou uma mutação genética no cromossoma X 7 . Na literatura brasileira há um caso". Relatamos dois casos avaliados no

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Progressive Pontobulbar Palsy With Deafness

Brucher¹

1981

Arch Neurol

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Progressive Bulbar Paralysis Associated With Neural Deafness

Cited by 27 publications

References 6 publications

Bulbo-pontine Paralysis with Deafness: the Vialetto-Van Laere Syndrome

Bulbo-pontine Paralysis with Deafness: the Vialetto-Van Laere Syndrome

Síndrome de Brown-Vialleto-Van Laere: relato de dois casos

Progressive Pontobulbar Palsy With Deafness

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