Chronic neuropathy presenting as a floppy infant with respiratory distress

Respiratory distress was the presenting feature in a 4-month-old male infant suffering from Déjérine-Sottas disease, an inherited sensory-motor polyneuropathy. This unusual but potentially benign disorder can be diagnosed upon peripheral nerve biopsy by noting extensive demyelination with "onio...

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Veröffentlicht in:Pediatrics (Evanston) 1979-10, Vol.64 (4), p.459-464
Hauptverfasser: Moss, R B, Sriram, S, Kelts, K A, Forno, L S, Lewiston, N J
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container_title Pediatrics (Evanston)
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creator Moss, R B
Sriram, S
Kelts, K A
Forno, L S
Lewiston, N J
description Respiratory distress was the presenting feature in a 4-month-old male infant suffering from Déjérine-Sottas disease, an inherited sensory-motor polyneuropathy. This unusual but potentially benign disorder can be diagnosed upon peripheral nerve biopsy by noting extensive demyelination with "onion bulb" formation. Polyneuropathy should be considered in the differential diagnosis of infantile neuromuscular weakness including or solely involving bulbar and respiratory muscles.
doi_str_mv 10.1542/peds.64.4.459
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source MEDLINE; EZB-FREE-00999 freely available EZB journals
subjects Biopsy
Chronic Disease
Demyelinating Diseases - diagnosis
Demyelinating Diseases - pathology
Diagnosis, Differential
Humans
Hypertrophy
Infant
Male
Muscle Hypotonia - diagnosis
Muscular Atrophy - diagnosis
Neuritis - diagnosis
Respiratory Insufficiency - complications
Sural Nerve - pathology
Syndrome
title Chronic neuropathy presenting as a floppy infant with respiratory distress
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