Heredofamilial Neuritis Of the Brachial Plexus

Poffenbarger, Arthur L.

doi:10.1001/jama.1966.03100040129048

Cited by 2 publications

(3 citation statements)

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“…Individual attacks may be precipitated by infections and also by pregnancy or parturition (Ungley 1933, Taylor 1960, Jacob e l al. 1961, Poffenbarger 1968, Guillozet and Mercer 1973, Warot et al 1973) and perhaps by strenuous exertion, while other relapses may occur without obvious cause.…”

Section: Introductionmentioning

confidence: 99%

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Heredofamilial Brachial Plexus Neuropathy (Hereditary Neuralgic Amyotrophy with Brachial Predilection) in Childhood

Dunn

Daube

Gómez

1978

Develop Med Child Neuro

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SUMMARY Three families with a total of at least 12 members affected by heredo‐familial brachial plexus neuropathy (hereditary neuralgic amyotrophy) are described, and three affected boys (age‐range 4 to 7 years 10 months) are reported in detail. All three children had recurrent episodes of pain in the shoulders and arms, followed by weakness and wasting of affected muscles. Subsequently there was gradual recovery. All three had close‐set eyes, and affected relatives usually had a similar physiognomy. Case 1 had had an inappropriately low birthweight, and his affected mother was significantly small (154cm). The other two boys were of borderline low‐normal stature. Case 2 required plication of his left hemi‐diaphragm at the early age of seven weeks, probably because of left phrenic paralysis. He also had a cleft palate. In all three families the condition appeared to be inherited through an autosomal dominant gene. Electrodiagnostic studies were performed, and Case 3 was tested repeatedly. Conduction velocity in motor fibres of the median, ulnar and lateral popliteal nerves was normal or slightly low, often with low amplitude of evoked muscle‐action potentials. The sensory action potentials in median and radial nerves were of abnormally low amplitude in acutely involved arms. Electromyography showed evidence of denervation in the acutely weak muscles in some patients, and indications of neurogenic atrophy were present in all and extended to leg muscles in two of the three probands, thus demonstrating the widespread distribution of the disorder. The clinical features, differential diagnosis, electrodiagnostic findings, possible location of the neuropathy, and management are discussed. While present data are consistent with the concept of ‘mononeuritis multiplex’ and a genetic predisposition, the exact site of the lesion will have to be determined by proximal‐nerve conduction studies through the brachial plexus in the acute phase of the disease, in addition to the routine investigations reported to date. Meanwhile it is suggested that the more non‐committal term ‘hereditary neuralgic amyotrophy with brachial predilection’ be used for the disease. RESUME Neuropathie hérédo‐familiale du plexus brachial dans l'enfance (amyotrophie névralgique héréditaire à prédilection brachiale) Trois familles regroupant au moins 12 membres atteints d'une neuropathie hérédo‐familiale du plexus brachial (amyotrophie névralgique héréditaire) sont étudiées et trois garçons atteints (âgés de quatre à sept ans dix mois) sont analysés plus en détail. Ces trois enfants avaient présenté des épisodes répétés de douleur dans les épaules et les bras, suivis de faiblesse et atrophie des muscles atteints. Par la suite, il y eut une récupération progressive. Dans les trois cas, on notait des yeux à demi fermés et habituellement les parents atteints avaient la même physionomie. Le cas 1 avait eu un poids de naissance faible pour le terme et sa mère atteinte était significativement petite (154cm). Les deux autres garçons de la fratrie étaient d'une ta...

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Section: Introductionmentioning

confidence: 99%

“…Since then a number of family studies have been presented (Taylor 1960, Jacob et a/. 1961, Roger et al 1965, Gardner and Maloney 1968, Poffenbarger 1968 01. 1971, Warot et a/.…”

Section: Introductionmentioning

confidence: 99%

Heredofamilial Brachial Plexus Neuropathy (Hereditary Neuralgic Amyotrophy with Brachial Predilection) in Childhood

Dunn

Daube

Gómez

1978

Develop Med Child Neuro

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“…(1) In one group there is painful amyotrophic affection of the area of the brachial plexus or of a nerve branch (Dreschfeld 1887, Ross and Bury 1893, Ungley 1933, Taylor 1960, Jacob et al, 1961, Roger et al 1965, Gardner and Maloney 1968, Poffenbarger 1968, De Graaf 1972, Guillozet and Mercer 1973, Geiger et al 1974, Wiederholt 1974. The clinical picture closely resembles that seen in the Parsonage-Turner syndrome (Parsonage and Turner 1948).…”

Section: Discussionmentioning

confidence: 99%

Otologic manifestations of histiocytosis X

Hudson

Kenan

1969

The Laryngoscope

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Este diagndstico debe ser tomado en consideracidn en cualquier paciente con hipertension endocraneana, supuracidn de oidos y evidencia radiobgica de una enfermedad mastoidea. Cuando se reconoce pronto y se trata enseguida, el prondstico de una recuperacidn completa parece bueno. REFERENCES Altman, J., Winkelmann, R. K. (1963) 'Xanthomatous cutaneous lesions of histiocytosis X.' Archives of Dermatology, 87, 164. Boddie, H. G., Banna, M., Bradley, W. B. (1974) "'Benign" intracranial hypertension-a survey of the clinical and radiologic features and long-term prognosis.' Brain, 97, 31 3. Buchheit, W. A., Burton, C., Haag, B., Shaw, D. (1969) 'Papilledema and idiopathic intracranial hypertension.' New England Journal of Medicine, 280, 938. Cohn, A. M., Sataloff, J., Lindsay, J. R. (1970) 'Histiocytosis X (Letterer-Siwe disease) with involvement of the inner ear.' Archives of Otolaryngology, 91, 24. Feinberg, S. B., Langer, L. 0. (1965) 'Roentgen findings of increased intracranial pressure and communicating hydrocephalus as insidious manifestations of chronic histiocytosis X.' American Journal of Roentgenology, 95, 41. Foley, J. (1955) 'Benign forms of intracranial hypertension, "toxic" and "otitic" hydrocephalus.' Brain, 78, 1. Glatt, M. A. (1946) 'Xanthoma or lipoid granuloma of the temporal bone (Hand-Christian-Schiler syndrome).' Archives of Otolaryngology, 43, 110. Greer, M. (1962) 'Benign intracranial hypertension. I. Mastoiditis and lateral sinus obstruction.' Neurology, 12,472. -(1967) 'Benign intracranial hypertension (pseudo-tumor cerebri).'Eosinophilic granuloma of skin and mucous mem-McGavran, M. H., Spady, H. A. (1960) 'Eosinophilic granuloma of bone: A study of twenty-eight cases.' Martin, T. H. (1969) 'Solitary eosinophilic granuloma of the temporal bone-a case report.' Laryngoscope, Rosenwasser, H. (1940) 'Lipoid granulomatosis (Hand-Schiiller-Christian disease) involving the middle Schuknecht, H. F., Perlman, H. B. (1948) 'Hand-Schiiller-Christian disease and eosinophilic granuloma brane: association with diabetes insipidus.' Archives of Dermatology, 75,45. Journal of Bone and Joint Surgery, 42A, 979. 70,2165. ear and temporal bone.' Archives of Otolaryngology, 32, 1045. of the skull.' Annals of Otology, Rhinology and Luryngology, 57, 643.

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Heredofamilial Neuritis Of the Brachial Plexus

Cited by 2 publications

References 5 publications

Heredofamilial Brachial Plexus Neuropathy (Hereditary Neuralgic Amyotrophy with Brachial Predilection) in Childhood

Heredofamilial Brachial Plexus Neuropathy (Hereditary Neuralgic Amyotrophy with Brachial Predilection) in Childhood

Otologic manifestations of histiocytosis X

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