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 |
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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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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.</description><subject>Biopsy</subject><subject>Chronic Disease</subject><subject>Demyelinating Diseases - diagnosis</subject><subject>Demyelinating Diseases - pathology</subject><subject>Diagnosis, Differential</subject><subject>Humans</subject><subject>Hypertrophy</subject><subject>Infant</subject><subject>Male</subject><subject>Muscle Hypotonia - diagnosis</subject><subject>Muscular Atrophy - diagnosis</subject><subject>Neuritis - diagnosis</subject><subject>Respiratory Insufficiency - complications</subject><subject>Sural Nerve - pathology</subject><subject>Syndrome</subject><issn>0031-4005</issn><issn>1098-4275</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1979</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNo9kEtLxDAUhYP4GkeX7lxk5a5jcpuk7VIGnwy40XW4bRMn0mljkiL993YYkbM4XPg4cD9CrjlbcSngzps2rpRYzZHVEVlwVpWZgEIekwVjOc8EY_KcXMT4xRgTsoAzcioqKDlfkNf1Ngy9a2hvxjB4TNuJ-mCi6ZPrPylGitR2g_cTdb3FPtEfl7Z0JrwLmIYw0dbFNN_xkpxY7KK5-usl-Xh8eF8_Z5u3p5f1_SZroCxSVre8ZqoBIcvSttCCrJhSDPK6aXImFAAW0ooay0JBDcIiK1sJOaKVQiHmS3J72PVh-B5NTHrnYmO6DnszjFEXooD5yWoGswPYhCHGYKz2we0wTJozvVen9-q0EnqO3PM3f8NjvTPtP31wlf8C4aBrEw</recordid><startdate>197910</startdate><enddate>197910</enddate><creator>Moss, R B</creator><creator>Sriram, S</creator><creator>Kelts, K A</creator><creator>Forno, L S</creator><creator>Lewiston, N J</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>197910</creationdate><title>Chronic neuropathy presenting as a floppy infant with respiratory distress</title><author>Moss, R B ; Sriram, S ; Kelts, K A ; Forno, L S ; Lewiston, N J</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c287t-bd1b06c24588fd2d259066023bcc304622a75f4ba8762b24fa08d523aaf546aa3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1979</creationdate><topic>Biopsy</topic><topic>Chronic Disease</topic><topic>Demyelinating Diseases - diagnosis</topic><topic>Demyelinating Diseases - pathology</topic><topic>Diagnosis, Differential</topic><topic>Humans</topic><topic>Hypertrophy</topic><topic>Infant</topic><topic>Male</topic><topic>Muscle Hypotonia - diagnosis</topic><topic>Muscular Atrophy - diagnosis</topic><topic>Neuritis - diagnosis</topic><topic>Respiratory Insufficiency - complications</topic><topic>Sural Nerve - pathology</topic><topic>Syndrome</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Moss, R B</creatorcontrib><creatorcontrib>Sriram, S</creatorcontrib><creatorcontrib>Kelts, K A</creatorcontrib><creatorcontrib>Forno, L S</creatorcontrib><creatorcontrib>Lewiston, N J</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Pediatrics (Evanston)</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Moss, R B</au><au>Sriram, S</au><au>Kelts, K A</au><au>Forno, L S</au><au>Lewiston, N J</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Chronic neuropathy presenting as a floppy infant with respiratory distress</atitle><jtitle>Pediatrics (Evanston)</jtitle><addtitle>Pediatrics</addtitle><date>1979-10</date><risdate>1979</risdate><volume>64</volume><issue>4</issue><spage>459</spage><epage>464</epage><pages>459-464</pages><issn>0031-4005</issn><eissn>1098-4275</eissn><abstract>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.</abstract><cop>United States</cop><pmid>492811</pmid><doi>10.1542/peds.64.4.459</doi><tpages>6</tpages></addata></record> |
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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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