Anterior horn cell disease seen in South India

Sayeed, Z A; Velmurugendran, C.U.; Arjundas, G; Masarreen, M.; Valmikinathan, K.

doi:10.1016/0022-510x(75)90049-0

Cited by 10 publications

(4 citation statements)

References 21 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…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 HISTORY This Caucasian girl had always been thin.…”

Section: -14mentioning

confidence: 92%

See 1 more Smart Citation

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

Abarbanel

Ashby

Marquez-Julio

et al. 1991

Can. j. neurol. sci.

View full text Add to dashboard Cite

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...

show abstract

Section: -14mentioning

confidence: 92%

“…19 A sporadic form of anterior horn cell disease has been described in South India. In one series 15 bilateral 8th nerve deficit with dysfunction of 10th, 11th, and 12th nerves occurred in 3 out of 32 patients and bilateral facial palsy occurred in 1 of 32 patients. The male/female ratio in the 7 patients with bulbar symptoms was 4/3.…”

mentioning

confidence: 93%

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

Abarbanel

Ashby

Marquez-Julio

et al. 1991

Can. j. neurol. sci.

View full text Add to dashboard Cite

show abstract

“…Nur in einem Fall in der Literatur war eine schwarze Patientin aus Brasilien betroffen [25]. Ob eine phänotypisch ähnliche, in Süd-Indien (Madras) beschriebene sporadische Form [26,27] einer juvenilen Motoneuronerkrankung dem Brown-Vialetto-Van Laere-Syndrom entspricht, ist unklar. Bei der sog.…”

unclassified

“…Bei der sog. Madrasform wiesen 3 von 32 Patienten eine bilaterale Beteiligung des N. VIII, X, XI und XII auf und ein Patient hatte eine bilaterale periphere Fazialisparese [27]. Unterschiede zum Brown-Vialetto-Van Laere-Syndrom sind die ausgeglichene Geschlechtsrelation, ausschlieûlich sporadisches Auftretens und der benigne Verlauf [28].…”

unclassified

Brown-Vialetto-Van Laere-Syndrom

Urban¹,

Hopf²

2001

Akt Neurol

View full text Add to dashboard Cite

Zusammenfassung: Wir berichten über eine 42-jährige Patientin mit einem Brown-Vialetto-Van Laere-Syndrom, gekennzeichnet durch progressive Bulbärparalyse mit Dysarthrophonie, Zungenatrophie, bilaterale inkomplette periphere Fazialisparese, bilaterale Rekurrensparese, Innenohrschwerhörigkeit, Ateminsuffizienz bei beidseitiger, linksbetonter Zwerchfellparese und atrophen Paresen der distalen Extremitätenmuskulatur.Brown-Vialetto-Van Laere Syndrome: We report on a 42year old woman with Brown-Vialetto-Van Laere syndrome presenting with progressive bulbar paralysis with dysarthria, tongue atrophy, bilateral incomplete facial paresis, bilateral recurrens paresis, and sensorineural hearing loss, respiratory insufficiency due to bilateral diaphragmatic paralysis, and atrophic paresis of distal limb muscles. EinleitungDas Brown-Vialetto-Van Laere-Syndrom ist ein sehr seltenes, bisher in etwa 40 Fällen beschriebenes, distinktes Syndrom, bestehend aus progressiver Bulbärparalyse, Innenohrschwerhörigkeit, respiratorischen Störungen und unterschiedlich stark ausgeprägten atrophen Paresen der Extremitätenmuskulatur. Die Prognose ist sehr unterschiedlich und reicht vom Tod im Kindesalter bis zu einem jahrzehntelangen Verlauf. Die überwiegende Mehrzahl folgt einem autosomal-rezessiven Erbgang mit Auftreten auch sporadischer Fälle. KasuistikDie 42-jährige türkische Patientin stellte sich zunächst in einer pneumologischen Klinik zur Abklärung einer seit mehreren Jahren bestehenden, belastungsabhängig zunehmenden Kurzatmigkeit und Heiserkeit vor. Die Blutgasanalyse ergab eine respiratorische Globalinsuffizienz mit einem pO 2 68 mm Hg, pCO 2 70 mm Hg, pH 7,34, weshalb eine Einstellung auf eine druckkontrollierte, nichtinvasive nächtliche Selbstbeatmung erfolgte. Wir übernahmen die Patientin zur weiteren neurolo-Abb. 4 Atrophe Paresen der Fuûmuskulatur. Abb. 2 Bilaterale Zungenatrophie. Brown-Vialetto-Van Laere-Syndrom Akt Neurol 2001; 28 183 Heruntergeladen von: University of Florida. Urheberrechtlich geschützt.

show abstract