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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Veröffentlicht in:Journal of the neurological sciences 2011-04, Vol.303 (1), p.35-38
Hauptverfasser: Rajabally, Yusuf A, Hassan-Smith, Ghaniah, Notturno, Francesca, Eames, Penelope J, Hayton, Thomas, Capasso, Margherita, Uncini, Antonino
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container_end_page 38
container_issue 1
container_start_page 35
container_title Journal of the neurological sciences
container_volume 303
creator Rajabally, Yusuf A
Hassan-Smith, Ghaniah
Notturno, Francesca
Eames, Penelope J
Hayton, Thomas
Capasso, Margherita
Uncini, Antonino
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. 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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. 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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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