Moebius's Syndroma—Congenital Oculofacial Paralysis

Sprofkin, Bertram E.; Hillman, John W.

doi:10.1212/wnl.6.1.50

Cited by 40 publications

(9 citation statements)

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“…Moebius syndrome has been associated with a number of other malformations, including Poland anomaly, Hanhart syndrome, dwarfism, progressive neuropathy, art hr o g r y p o s i s and ag 1 o s s i a-ad act y 1 i a (Sprofkin and Hillman 1956, Jorgenson 1971, Battin 1974, Hall 1975, Rubenstein et al 1975, Herrmann et a/. 1976, Szabo 1976, Grosse and Widermann 1977, Robinow er al.…”

Section: Pathology and Etiologymentioning

confidence: 99%

Speech, Language and Hearing in Moebius Syndrome: a Study of 22 Patients

Meyerson

Foushee²

1978

Develop Med Child Neuro

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SUMMARY A comprehensive profile of communicative disorders in patients with Moebius syndrome was attempted. Seven patients were evaluated by the authors and the findings were added to 15 contributed case‐histories. Most patients demonstrated some degree of dysarthria due to congenital paralysis of the facial nerves. Other problems such as cleft palate, hearing loss, mental retardation and delayed development of language were found in a few of the patients. As most of the older children in the sample and in reports in the literature eventually developed intelligible speech, a program of oral language stimulation and compensatory articulatory adjustments would appear to be the procedure of choice. RÉSUMÉ Parole, langage et audition dans le syndrome de Moebius: Etude de 22 malades Un profil étendu des troubles de la communication chez les sujets atteints de syndrome de Moebius a été essayé. Sept malades ont été examinés par les auteurs et les dossiers recueillis se sont ajoutés aux 15 cas de la littérature. La plupart des malades présentaient un certain degré de dysarthrie lié à la paralysie congénitale des nerfs faciaux. Les autres défauts tels que la fente palatine, la perte de l'audition, le retard mental et le retard de langage n'ont été trouvés que chez quelques sujets. Comme la plupart des enfants plus âgés dans l'échantillon et dans les cas rapportés par la littérature développent ultérieurement un langage intelligible, un programme de stimulations du langage oral et d'adaptations compensatrices d'articulations apparait un moyen de correction de choix. ZUSAMMENFASSUNG Sprechen, Sprache und Hören beim Moebius Syndrom: eine Studie an 22 Patienten Es wurde versucht, eine umfassende Studie über kommunikative Störungen bei Patienten mit Moebius Syndrom zu erstellen. Sieben Patienten wurden von den Autoren untersucht und die Befunde wurden mit 15 anderen Fallbeschreibungen ausgewertet. Die meisten Patienten zeigten eine gewisse Dysarthrie, die durch eine angeborene Lähmung des N. facialis hervorgerufen war. Bei einigen Patienten wurden andere Probleme wie Gaumenspalte, Hörverlust, geistige Retardierung und verzögerte Sprachentwicklung gefunden. Da die meisten älteren Kinder dieser Studie und die in Literaturberichten schliesslich eine verständliche Sprache entwickelten, halten die Auroten ein Programm für Sprach‐ und Artikulationsschulung für sinnvoll. RESUMEN Locución, Lenguaje y audición en el síndrome de Moebius: estudio de 22 pacientes Se intentó un perfil de conjunto de alteraciones de la comunicación en pacientes con sindrome de Moebius. Los autores evaluaron siete pacientes y los hallazgos fueron añadidos a otros 15 casos. La mayoria de los pacientes mostraron un cierto grado de disartria debida a una parálisis congénita de los nervios faciales. En unos pocos pacientes fueron hallados otros problemas tales como paladar hendido, pérdida audición, retraso mental y retraso en el desarrollo del lenguaje. Como la mayoría de los niños mayores de la muestra y en los aportados por la literatura, desarrollaron habitu...

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Section: Pathology and Etiologymentioning

confidence: 99%

Speech, Language and Hearing in Moebius Syndrome: a Study of 22 Patients

Meyerson

Foushee²

1978

Develop Med Child Neuro

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“…Congenital facial diplegia not associated with apparent extraocular palsies does occur in dystrophia myotonica (Parker, 1963), thus demonstrating that at least this portion of the syndrome can have a myopathic basis in certain other disorders. Other clinical arguments that have been advanced for a primary mesodermal defect such as absence of sensory change, the distribution of the facial weakness, and the absence of facial sagging (Richards, 1953) do not appear to be valid, as pointed out by Sprofkin and Hillman (1956). Laboratory evidence supporting this view is limited to altered creatine and creatinine metabolism suggestive of a muscular defect, as reported by Wallis (1960).…”

Section: The Case For the Mesodermal (Peripheral)mentioning

confidence: 99%

Observations on the pathology of the Moebius syndrome

Pitner¹,

Edwards²,

McCormick³

1965

Journal of Neurology, Neurosurgery & Psychiatry

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“…In Case 3 of Reed and Grant (1957) no muscle fibres were seen in fascicles of dense fibrous tissue. Sprofkin and Hillman (1956) record that in a boy with Mobius syndrome and limb evidence of arthrogryposis multiplex, an operation to correct strabismus revealed a few strands of lateral rectus muscle and an inelastic left medial rectus.…”

Section: Nature Of the Eye Signs In The Present Casesmentioning

confidence: 98%