Hyper-Endorphin Syndrome in a Child with Necrotizing Encephalomyelopathy
SUBACUTE necrotizing encephalomyelopathy, or Leigh's syndrome, 1 is a degenerative disorder characterized by progressive deterioration of brain-stem functions, ataxia, convulsions, hypotonia, and failure to thrive. The histopathologic changes are found in the brain stem, basal ganglia, cerebell...
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Veröffentlicht in: | The New England journal of medicine 1980-10, Vol.303 (16), p.914-916 |
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creator | Brandt, Niels Jacob Brandt, Sven Terenius, Lars Jacobsen, Bendt Brock Klinken, Leif Nordius, Åsa Blegvad, Karin Yssing, Minna |
description | SUBACUTE necrotizing encephalomyelopathy, or Leigh's syndrome,
1
is a degenerative disorder characterized by progressive deterioration of brain-stem functions, ataxia, convulsions, hypotonia, and failure to thrive. The histopathologic changes are found in the brain stem, basal ganglia, cerebellar cortex, and dentate nucleus, and they consist of bilateral symmetrical areas of rarefaction, loss of myelin, and cell proliferation in the capillaries and glia. This disorder occurs in infancy and early childhood and is inherited as an autosomal-recessive trait, but the basic biochemical defect is unknown.
We report on a syndrome in a boy suffering from attacks mimicking acute morphine poisoning with prolonged apnea . . . |
doi_str_mv | 10.1056/NEJM198010163031604 |
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1
is a degenerative disorder characterized by progressive deterioration of brain-stem functions, ataxia, convulsions, hypotonia, and failure to thrive. The histopathologic changes are found in the brain stem, basal ganglia, cerebellar cortex, and dentate nucleus, and they consist of bilateral symmetrical areas of rarefaction, loss of myelin, and cell proliferation in the capillaries and glia. This disorder occurs in infancy and early childhood and is inherited as an autosomal-recessive trait, but the basic biochemical defect is unknown.
We report on a syndrome in a boy suffering from attacks mimicking acute morphine poisoning with prolonged apnea . . .</description><identifier>ISSN: 0028-4793</identifier><identifier>EISSN: 1533-4406</identifier><identifier>DOI: 10.1056/NEJM198010163031604</identifier><identifier>PMID: 7412823</identifier><language>eng</language><publisher>United States: Massachusetts Medical Society</publisher><subject>Ataxia ; Ataxia - etiology ; Brain - pathology ; Brain Chemistry ; Brain Diseases - diagnosis ; Brain Diseases - pathology ; Brain Diseases - physiopathology ; Case reports ; Cerebrospinal Fluid - analysis ; Dehydrogenases ; Diagnosis, Differential ; Endorphins ; Endorphins - analysis ; Endorphins - physiology ; Enkephalins - analysis ; Enkephalins - physiology ; Fibroblasts ; Humans ; Infant ; Injections, Intravenous ; Intellectual Disability - etiology ; Male ; Metabolism ; Metabolites ; Morphine - poisoning ; Naloxone - administration & dosage ; Naloxone - therapeutic use ; Narcotics ; Necrosis ; Pediatrics ; Peptides ; Poisoning ; Respiration ; Spinal Cord Diseases - diagnosis ; Spinal Cord Diseases - pathology ; Spinal Cord Diseases - physiopathology ; Syndrome ; Urine</subject><ispartof>The New England journal of medicine, 1980-10, Vol.303 (16), p.914-916</ispartof><rights>Copyright Massachusetts Medical Society Oct 16, 1980</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c364t-bee3692deceace0d6a79aa74cc1b10356ef6326583dab2d2ea91c51a520e0b882</citedby><cites>FETCH-LOGICAL-c364t-bee3692deceace0d6a79aa74cc1b10356ef6326583dab2d2ea91c51a520e0b882</cites></display><links><openurl>$$Topenurl_article</openurl><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>776</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/7412823$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Brandt, Niels Jacob</creatorcontrib><creatorcontrib>Brandt, Sven</creatorcontrib><creatorcontrib>Terenius, Lars</creatorcontrib><creatorcontrib>Jacobsen, Bendt Brock</creatorcontrib><creatorcontrib>Klinken, Leif</creatorcontrib><creatorcontrib>Nordius, Åsa</creatorcontrib><creatorcontrib>Blegvad, Karin</creatorcontrib><creatorcontrib>Yssing, Minna</creatorcontrib><title>Hyper-Endorphin Syndrome in a Child with Necrotizing Encephalomyelopathy</title><title>The New England journal of medicine</title><addtitle>N Engl J Med</addtitle><description>SUBACUTE necrotizing encephalomyelopathy, or Leigh's syndrome,
1
is a degenerative disorder characterized by progressive deterioration of brain-stem functions, ataxia, convulsions, hypotonia, and failure to thrive. The histopathologic changes are found in the brain stem, basal ganglia, cerebellar cortex, and dentate nucleus, and they consist of bilateral symmetrical areas of rarefaction, loss of myelin, and cell proliferation in the capillaries and glia. This disorder occurs in infancy and early childhood and is inherited as an autosomal-recessive trait, but the basic biochemical defect is unknown.
We report on a syndrome in a boy suffering from attacks mimicking acute morphine poisoning with prolonged apnea . . .</description><subject>Ataxia</subject><subject>Ataxia - etiology</subject><subject>Brain - pathology</subject><subject>Brain Chemistry</subject><subject>Brain Diseases - diagnosis</subject><subject>Brain Diseases - pathology</subject><subject>Brain Diseases - physiopathology</subject><subject>Case reports</subject><subject>Cerebrospinal Fluid - analysis</subject><subject>Dehydrogenases</subject><subject>Diagnosis, Differential</subject><subject>Endorphins</subject><subject>Endorphins - analysis</subject><subject>Endorphins - physiology</subject><subject>Enkephalins - analysis</subject><subject>Enkephalins - physiology</subject><subject>Fibroblasts</subject><subject>Humans</subject><subject>Infant</subject><subject>Injections, Intravenous</subject><subject>Intellectual Disability - etiology</subject><subject>Male</subject><subject>Metabolism</subject><subject>Metabolites</subject><subject>Morphine - poisoning</subject><subject>Naloxone - administration & dosage</subject><subject>Naloxone - therapeutic use</subject><subject>Narcotics</subject><subject>Necrosis</subject><subject>Pediatrics</subject><subject>Peptides</subject><subject>Poisoning</subject><subject>Respiration</subject><subject>Spinal Cord Diseases - diagnosis</subject><subject>Spinal Cord Diseases - pathology</subject><subject>Spinal Cord Diseases - physiopathology</subject><subject>Syndrome</subject><subject>Urine</subject><issn>0028-4793</issn><issn>1533-4406</issn><fulltext>false</fulltext><rsrctype>article</rsrctype><creationdate>1980</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>BEC</sourceid><sourceid>BENPR</sourceid><sourceid>GUQSH</sourceid><sourceid>M2O</sourceid><recordid>eNp9UEtLxDAQDqKs6-ovEKHgUaqZJE2boyzVVdb1oJ5LmszaLn2ZdpH6643s4kmcywx8j5n5CDkHeg00kjer9PEJVEKBguSUg6TigEwh4jwUgspDMqWUJaGIFT8mJ32_ob5AqAmZxAJYwviULBZjhy5MG9u6riib4GVsrGtrDPysg3lRVjb4LIciWKFx7VB-lc17kDYGu0JXbT1i1XZ6KMZTcrTWVY9n-z4jb3fp63wRLp_vH-a3y9BwKYYwR-RSMYsGtUFqpY6V1rEwBnKgPJK4lpzJKOFW58wy1ApMBDpiFGmeJGxGLne-nWs_ttgP2abdusavzCCRKvLPcuVZfMfyN_e9w3XWubLWbsyAZj_hZX-E51UXe-9tXqP91ezT8vjVDq_rPmtwU__r9g3Ii3Y3</recordid><startdate>19801016</startdate><enddate>19801016</enddate><creator>Brandt, Niels Jacob</creator><creator>Brandt, Sven</creator><creator>Terenius, Lars</creator><creator>Jacobsen, Bendt Brock</creator><creator>Klinken, Leif</creator><creator>Nordius, Åsa</creator><creator>Blegvad, Karin</creator><creator>Yssing, Minna</creator><general>Massachusetts Medical Society</general><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>0TZ</scope><scope>7RV</scope><scope>7X7</scope><scope>7XB</scope><scope>8AO</scope><scope>8C1</scope><scope>8FE</scope><scope>8FH</scope><scope>8FI</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AN0</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BEC</scope><scope>BENPR</scope><scope>BHPHI</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>GUQSH</scope><scope>HCIFZ</scope><scope>K0Y</scope><scope>LK8</scope><scope>M0R</scope><scope>M0T</scope><scope>M1P</scope><scope>M2M</scope><scope>M2O</scope><scope>M2P</scope><scope>M7P</scope><scope>MBDVC</scope><scope>NAPCQ</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>PSYQQ</scope><scope>Q9U</scope></search><sort><creationdate>19801016</creationdate><title>Hyper-Endorphin Syndrome in a Child with Necrotizing Encephalomyelopathy</title><author>Brandt, Niels Jacob ; Brandt, Sven ; Terenius, Lars ; Jacobsen, Bendt Brock ; Klinken, Leif ; Nordius, Åsa ; Blegvad, Karin ; Yssing, Minna</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c364t-bee3692deceace0d6a79aa74cc1b10356ef6326583dab2d2ea91c51a520e0b882</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1980</creationdate><topic>Ataxia</topic><topic>Ataxia - 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1
is a degenerative disorder characterized by progressive deterioration of brain-stem functions, ataxia, convulsions, hypotonia, and failure to thrive. The histopathologic changes are found in the brain stem, basal ganglia, cerebellar cortex, and dentate nucleus, and they consist of bilateral symmetrical areas of rarefaction, loss of myelin, and cell proliferation in the capillaries and glia. This disorder occurs in infancy and early childhood and is inherited as an autosomal-recessive trait, but the basic biochemical defect is unknown.
We report on a syndrome in a boy suffering from attacks mimicking acute morphine poisoning with prolonged apnea . . .</abstract><cop>United States</cop><pub>Massachusetts Medical Society</pub><pmid>7412823</pmid><doi>10.1056/NEJM198010163031604</doi><tpages>3</tpages></addata></record> |
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subjects | Ataxia Ataxia - etiology Brain - pathology Brain Chemistry Brain Diseases - diagnosis Brain Diseases - pathology Brain Diseases - physiopathology Case reports Cerebrospinal Fluid - analysis Dehydrogenases Diagnosis, Differential Endorphins Endorphins - analysis Endorphins - physiology Enkephalins - analysis Enkephalins - physiology Fibroblasts Humans Infant Injections, Intravenous Intellectual Disability - etiology Male Metabolism Metabolites Morphine - poisoning Naloxone - administration & dosage Naloxone - therapeutic use Narcotics Necrosis Pediatrics Peptides Poisoning Respiration Spinal Cord Diseases - diagnosis Spinal Cord Diseases - pathology Spinal Cord Diseases - physiopathology Syndrome Urine |
title | Hyper-Endorphin Syndrome in a Child with Necrotizing Encephalomyelopathy |
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