Motor and sensory conduction failure in overlap of Guillain–Barré and Miller Fisher syndrome: Two simultaneous cases
Abstract We report 2 patients diagnosed simultaneously with an overlap of Guillain–Barré syndrome (GBS) and Miller Fisher syndrome (MFS), who had anti-GT1a, anti-GQ1b, anti-GD1a and anti-GD1b antibodies. There was no identifiable specific preceding infection. Both patients presented with upper and l...
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description | Abstract We report 2 patients diagnosed simultaneously with an overlap of Guillain–Barré syndrome (GBS) and Miller Fisher syndrome (MFS), who had anti-GT1a, anti-GQ1b, anti-GD1a and anti-GD1b antibodies. There was no identifiable specific preceding infection. Both patients presented with upper and lower limb paresthesias and severe weakness, bulbar and facial weakness, ophthalmoparesis and areflexia. In one, electrophysiology demonstrated multifocal conduction blocks (CBs) and mild motor conduction velocity slowing in intermediate segments and absent sensory nerve action potentials (SNAPs). The patient improved rapidly and fully recovered within 18 days from onset. CBs resolved, distal compound muscle action potential (CMAP) amplitudes increased and SNAPs normalized on subsequent testing. In the other patient, initial studies showed low/normal CMAPs, with absent SNAPs, without demyelinating features. This patient fully recovered within 21 days from onset. CMAPs markedly increased, SNAPs improved marginally. These 2 patients exhibited features indicative of the pathophysiological mechanism of conduction failure in motor and sensory fibers. This phenomenon relates to rapidly resolving CBs possibly induced by the transitory and limited attack of antiganglioside antibodies at the axolemma of the nodes of Ranvier not progressing to axonal degeneration. These cases widen the range of GBS subtypes in which reversible conduction failure has been described, to include overlap syndromes with MFS. The factors determining the electrophysiology, as well as the rate, degree and quality of recovery in GBS subtypes remain uncertain at the present time. |
doi_str_mv | 10.1016/j.jns.2011.01.019 |
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There was no identifiable specific preceding infection. Both patients presented with upper and lower limb paresthesias and severe weakness, bulbar and facial weakness, ophthalmoparesis and areflexia. In one, electrophysiology demonstrated multifocal conduction blocks (CBs) and mild motor conduction velocity slowing in intermediate segments and absent sensory nerve action potentials (SNAPs). The patient improved rapidly and fully recovered within 18 days from onset. CBs resolved, distal compound muscle action potential (CMAP) amplitudes increased and SNAPs normalized on subsequent testing. In the other patient, initial studies showed low/normal CMAPs, with absent SNAPs, without demyelinating features. This patient fully recovered within 21 days from onset. CMAPs markedly increased, SNAPs improved marginally. These 2 patients exhibited features indicative of the pathophysiological mechanism of conduction failure in motor and sensory fibers. This phenomenon relates to rapidly resolving CBs possibly induced by the transitory and limited attack of antiganglioside antibodies at the axolemma of the nodes of Ranvier not progressing to axonal degeneration. These cases widen the range of GBS subtypes in which reversible conduction failure has been described, to include overlap syndromes with MFS. The factors determining the electrophysiology, as well as the rate, degree and quality of recovery in GBS subtypes remain uncertain at the present time.</description><identifier>ISSN: 0022-510X</identifier><identifier>EISSN: 1878-5883</identifier><identifier>DOI: 10.1016/j.jns.2011.01.019</identifier><identifier>PMID: 21316711</identifier><identifier>CODEN: JNSCAG</identifier><language>eng</language><publisher>Amsterdam: Elsevier B.V</publisher><subject>Action Potentials - physiology ; Adult ; Autoantibodies - immunology ; Axonal ; Axons - pathology ; Biological and medical sciences ; Conduction block ; Conduction failure ; Electric Stimulation ; Electrophysiological Phenomena ; Female ; Gangliosides - immunology ; Guillain-Barre Syndrome - complications ; Guillain-Barre Syndrome - physiopathology ; Guillain–Barré syndrome ; Humans ; Medical sciences ; Miller Fisher syndrome ; Miller Fisher Syndrome - complications ; Miller Fisher Syndrome - physiopathology ; Motor Neurons - physiology ; Muscle Weakness - etiology ; Neural Conduction - physiology ; Neurologic Examination ; Neurology ; Paresthesia - etiology ; Pharyngeal–cervical–brachial variant ; Respiratory Tract Infections - complications ; Sensory Receptor Cells - physiology ; Speech Disorders - etiology ; Tumors of the nervous system. Phacomatoses</subject><ispartof>Journal of the neurological sciences, 2011-04, Vol.303 (1), p.35-38</ispartof><rights>Elsevier B.V.</rights><rights>2011 Elsevier B.V.</rights><rights>2015 INIST-CNRS</rights><rights>Copyright © 2011 Elsevier B.V. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c469t-7e1830f5da7e17ec9ca6caa563f52bc05ef6400b4b459e7e69f4bf33afcd7c503</citedby><cites>FETCH-LOGICAL-c469t-7e1830f5da7e17ec9ca6caa563f52bc05ef6400b4b459e7e69f4bf33afcd7c503</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.jns.2011.01.019$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,777,781,3537,27905,27906,45976</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=23977330$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/21316711$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Rajabally, Yusuf A</creatorcontrib><creatorcontrib>Hassan-Smith, Ghaniah</creatorcontrib><creatorcontrib>Notturno, Francesca</creatorcontrib><creatorcontrib>Eames, Penelope J</creatorcontrib><creatorcontrib>Hayton, Thomas</creatorcontrib><creatorcontrib>Capasso, Margherita</creatorcontrib><creatorcontrib>Uncini, Antonino</creatorcontrib><title>Motor and sensory conduction failure in overlap of Guillain–Barré and Miller Fisher syndrome: Two simultaneous cases</title><title>Journal of the neurological sciences</title><addtitle>J Neurol Sci</addtitle><description>Abstract We report 2 patients diagnosed simultaneously with an overlap of Guillain–Barré syndrome (GBS) and Miller Fisher syndrome (MFS), who had anti-GT1a, anti-GQ1b, anti-GD1a and anti-GD1b antibodies. There was no identifiable specific preceding infection. Both patients presented with upper and lower limb paresthesias and severe weakness, bulbar and facial weakness, ophthalmoparesis and areflexia. In one, electrophysiology demonstrated multifocal conduction blocks (CBs) and mild motor conduction velocity slowing in intermediate segments and absent sensory nerve action potentials (SNAPs). The patient improved rapidly and fully recovered within 18 days from onset. CBs resolved, distal compound muscle action potential (CMAP) amplitudes increased and SNAPs normalized on subsequent testing. In the other patient, initial studies showed low/normal CMAPs, with absent SNAPs, without demyelinating features. This patient fully recovered within 21 days from onset. CMAPs markedly increased, SNAPs improved marginally. These 2 patients exhibited features indicative of the pathophysiological mechanism of conduction failure in motor and sensory fibers. This phenomenon relates to rapidly resolving CBs possibly induced by the transitory and limited attack of antiganglioside antibodies at the axolemma of the nodes of Ranvier not progressing to axonal degeneration. These cases widen the range of GBS subtypes in which reversible conduction failure has been described, to include overlap syndromes with MFS. The factors determining the electrophysiology, as well as the rate, degree and quality of recovery in GBS subtypes remain uncertain at the present time.</description><subject>Action Potentials - physiology</subject><subject>Adult</subject><subject>Autoantibodies - immunology</subject><subject>Axonal</subject><subject>Axons - pathology</subject><subject>Biological and medical sciences</subject><subject>Conduction block</subject><subject>Conduction failure</subject><subject>Electric Stimulation</subject><subject>Electrophysiological Phenomena</subject><subject>Female</subject><subject>Gangliosides - immunology</subject><subject>Guillain-Barre Syndrome - complications</subject><subject>Guillain-Barre Syndrome - physiopathology</subject><subject>Guillain–Barré syndrome</subject><subject>Humans</subject><subject>Medical sciences</subject><subject>Miller Fisher syndrome</subject><subject>Miller Fisher Syndrome - complications</subject><subject>Miller Fisher Syndrome - physiopathology</subject><subject>Motor Neurons - physiology</subject><subject>Muscle Weakness - etiology</subject><subject>Neural Conduction - physiology</subject><subject>Neurologic Examination</subject><subject>Neurology</subject><subject>Paresthesia - etiology</subject><subject>Pharyngeal–cervical–brachial variant</subject><subject>Respiratory Tract Infections - complications</subject><subject>Sensory Receptor Cells - physiology</subject><subject>Speech Disorders - etiology</subject><subject>Tumors of the nervous system. Phacomatoses</subject><issn>0022-510X</issn><issn>1878-5883</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2011</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkt1qFTEQxxdR7Gn1AbyR3IhXe8zHfmQVBC22Ci1eWMG7kM1OMOtucprZbTl3fQefwufwTXwSsz1HBS8UBiaE339mkv9k2SNG14yy6lm_7j2uOWVsTZdo7mQrJmuZl1KKu9mKUs7zktFPB9khYk8praRs7mcHnAlW1YytsuvzMIVItO8IgscQt8QE381mcsETq90wRyDOk3AFcdAbEiw5nd0waOd_3Hx9rWP8_u1Wfp4uIZITh59Twq3vYhjhObm4DgTdOA-T9hBmJEYj4IPsntUDwsN9Pso-nry5OH6bn70_fXf86iw3RdVMeQ1MCmrLTqdTDaYxujJal5WwJW8NLcFWBaVt0RZlAzVUjS1aK4S2pqtNScVR9nRXdxPD5Qw4qdGhgTT_7TBKVryWiZP_J0vJZcklTyTbkSYGxAhWbaIbddwqRtVijOpVMkYtxii6RJM0j_fV53aE7rfilxMJeLIHNBo92Ki9cfiHE01dC7E86MWOg_RrVw6iQuPAG-hcBDOpLrh_jvHyL7UZnHep4RfYAvZhjj7ZoZhCrqj6sGzQskCMpeXhqcBP2unDuQ</recordid><startdate>20110415</startdate><enddate>20110415</enddate><creator>Rajabally, Yusuf A</creator><creator>Hassan-Smith, Ghaniah</creator><creator>Notturno, Francesca</creator><creator>Eames, Penelope J</creator><creator>Hayton, Thomas</creator><creator>Capasso, Margherita</creator><creator>Uncini, Antonino</creator><general>Elsevier B.V</general><general>Elsevier</general><scope>IQODW</scope><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><scope>7TK</scope><scope>F1W</scope><scope>H95</scope><scope>L.G</scope></search><sort><creationdate>20110415</creationdate><title>Motor and sensory conduction failure in overlap of Guillain–Barré and Miller Fisher syndrome: Two simultaneous cases</title><author>Rajabally, Yusuf A ; Hassan-Smith, Ghaniah ; Notturno, Francesca ; Eames, Penelope J ; Hayton, Thomas ; Capasso, Margherita ; Uncini, Antonino</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c469t-7e1830f5da7e17ec9ca6caa563f52bc05ef6400b4b459e7e69f4bf33afcd7c503</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2011</creationdate><topic>Action Potentials - physiology</topic><topic>Adult</topic><topic>Autoantibodies - immunology</topic><topic>Axonal</topic><topic>Axons - pathology</topic><topic>Biological and medical sciences</topic><topic>Conduction block</topic><topic>Conduction failure</topic><topic>Electric Stimulation</topic><topic>Electrophysiological Phenomena</topic><topic>Female</topic><topic>Gangliosides - immunology</topic><topic>Guillain-Barre Syndrome - complications</topic><topic>Guillain-Barre Syndrome - physiopathology</topic><topic>Guillain–Barré syndrome</topic><topic>Humans</topic><topic>Medical sciences</topic><topic>Miller Fisher syndrome</topic><topic>Miller Fisher Syndrome - complications</topic><topic>Miller Fisher Syndrome - physiopathology</topic><topic>Motor Neurons - physiology</topic><topic>Muscle Weakness - etiology</topic><topic>Neural Conduction - physiology</topic><topic>Neurologic Examination</topic><topic>Neurology</topic><topic>Paresthesia - etiology</topic><topic>Pharyngeal–cervical–brachial variant</topic><topic>Respiratory Tract Infections - complications</topic><topic>Sensory Receptor Cells - physiology</topic><topic>Speech Disorders - etiology</topic><topic>Tumors of the nervous system. Phacomatoses</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Rajabally, Yusuf A</creatorcontrib><creatorcontrib>Hassan-Smith, Ghaniah</creatorcontrib><creatorcontrib>Notturno, Francesca</creatorcontrib><creatorcontrib>Eames, Penelope J</creatorcontrib><creatorcontrib>Hayton, Thomas</creatorcontrib><creatorcontrib>Capasso, Margherita</creatorcontrib><creatorcontrib>Uncini, Antonino</creatorcontrib><collection>Pascal-Francis</collection><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><collection>Neurosciences Abstracts</collection><collection>ASFA: Aquatic Sciences and Fisheries Abstracts</collection><collection>Aquatic Science & Fisheries Abstracts (ASFA) 1: Biological Sciences & Living Resources</collection><collection>Aquatic Science & Fisheries Abstracts (ASFA) Professional</collection><jtitle>Journal of the neurological sciences</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Rajabally, Yusuf A</au><au>Hassan-Smith, Ghaniah</au><au>Notturno, Francesca</au><au>Eames, Penelope J</au><au>Hayton, Thomas</au><au>Capasso, Margherita</au><au>Uncini, Antonino</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Motor and sensory conduction failure in overlap of Guillain–Barré and Miller Fisher syndrome: Two simultaneous cases</atitle><jtitle>Journal of the neurological sciences</jtitle><addtitle>J Neurol Sci</addtitle><date>2011-04-15</date><risdate>2011</risdate><volume>303</volume><issue>1</issue><spage>35</spage><epage>38</epage><pages>35-38</pages><issn>0022-510X</issn><eissn>1878-5883</eissn><coden>JNSCAG</coden><abstract>Abstract We report 2 patients diagnosed simultaneously with an overlap of Guillain–Barré syndrome (GBS) and Miller Fisher syndrome (MFS), who had anti-GT1a, anti-GQ1b, anti-GD1a and anti-GD1b antibodies. There was no identifiable specific preceding infection. Both patients presented with upper and lower limb paresthesias and severe weakness, bulbar and facial weakness, ophthalmoparesis and areflexia. In one, electrophysiology demonstrated multifocal conduction blocks (CBs) and mild motor conduction velocity slowing in intermediate segments and absent sensory nerve action potentials (SNAPs). The patient improved rapidly and fully recovered within 18 days from onset. CBs resolved, distal compound muscle action potential (CMAP) amplitudes increased and SNAPs normalized on subsequent testing. In the other patient, initial studies showed low/normal CMAPs, with absent SNAPs, without demyelinating features. This patient fully recovered within 21 days from onset. CMAPs markedly increased, SNAPs improved marginally. These 2 patients exhibited features indicative of the pathophysiological mechanism of conduction failure in motor and sensory fibers. This phenomenon relates to rapidly resolving CBs possibly induced by the transitory and limited attack of antiganglioside antibodies at the axolemma of the nodes of Ranvier not progressing to axonal degeneration. These cases widen the range of GBS subtypes in which reversible conduction failure has been described, to include overlap syndromes with MFS. The factors determining the electrophysiology, as well as the rate, degree and quality of recovery in GBS subtypes remain uncertain at the present time.</abstract><cop>Amsterdam</cop><pub>Elsevier B.V</pub><pmid>21316711</pmid><doi>10.1016/j.jns.2011.01.019</doi><tpages>4</tpages></addata></record> |
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subjects | Action Potentials - physiology Adult Autoantibodies - immunology Axonal Axons - pathology Biological and medical sciences Conduction block Conduction failure Electric Stimulation Electrophysiological Phenomena Female Gangliosides - immunology Guillain-Barre Syndrome - complications Guillain-Barre Syndrome - physiopathology Guillain–Barré syndrome Humans Medical sciences Miller Fisher syndrome Miller Fisher Syndrome - complications Miller Fisher Syndrome - physiopathology Motor Neurons - physiology Muscle Weakness - etiology Neural Conduction - physiology Neurologic Examination Neurology Paresthesia - etiology Pharyngeal–cervical–brachial variant Respiratory Tract Infections - complications Sensory Receptor Cells - physiology Speech Disorders - etiology Tumors of the nervous system. Phacomatoses |
title | Motor and sensory conduction failure in overlap of Guillain–Barré and Miller Fisher syndrome: Two simultaneous cases |
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