Atypical Electrophysiological Findings in a Patient with Acute Motor and Sensory Axonal Neuropathy
Guillain–Barré syndrome (GBS) is an immune-mediated polyradiculoneuropathy with acute onset and rapid clinical worsening; early diagnosis and immunomodulating therapy can ameliorate the course of disease. During the first days, however, nerve conduction studies (NCSs) are not always conclusive. Here...
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| Format: | Article |
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Frontiers Media S.A.
2017-11-01
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| Series: | Frontiers in Neurology |
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| Online Access: | http://journal.frontiersin.org/article/10.3389/fneur.2017.00594/full |
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| author | Viviana Versace Viviana Versace Stefania Campostrini Stefania Campostrini Frediano Tezzon Sara Martignago Sara Martignago Markus Kofler Leopold Saltuari Leopold Saltuari Leopold Saltuari Luca Sebastianelli Luca Sebastianelli Raffaele Nardone Raffaele Nardone |
| author_facet | Viviana Versace Viviana Versace Stefania Campostrini Stefania Campostrini Frediano Tezzon Sara Martignago Sara Martignago Markus Kofler Leopold Saltuari Leopold Saltuari Leopold Saltuari Luca Sebastianelli Luca Sebastianelli Raffaele Nardone Raffaele Nardone |
| author_sort | Viviana Versace |
| collection | DOAJ |
| description | Guillain–Barré syndrome (GBS) is an immune-mediated polyradiculoneuropathy with acute onset and rapid clinical worsening; early diagnosis and immunomodulating therapy can ameliorate the course of disease. During the first days, however, nerve conduction studies (NCSs) are not always conclusive. Here, we describe a 73-year-old man presenting with progressive muscular weakness of the lower limbs, ascending to the upper limbs, accompanied by distal sensory disturbances. Neuroimaging of brain and spine and NCSs were unremarkable; cerebrospinal fluid analysis revealed no albuminocytologic dissociation. Based on typical clinical features, and on positivity for serum GD1b-IgM antibodies, GBS with proximal conduction failure at multiple radicular levels was postulated, and a standard regime of intravenous immunoglobulin was administered. Four weeks later, the patient presented with flaccid tetraparesis, areflexia, and reduction of position sense, tingling paresthesias, and initial respiratory distress. Repeat NCS still revealed almost normal findings, except for the disappearance of right ulnar nerve F-waves. A few days thereafter, the patient developed severe respiratory insufficiency requiring mechanical ventilation for 2 weeks. On day 50, NCS revealed for the first time markedly reduced compound muscle action potentials and sensory nerve action potentials in all tested nerves, without signs of demyelination; needle electromyography documented widespread denervation. The diagnosis of acute motor and sensory axonal neuropathy was made. After 3 months of intensive rehabilitation, the patient regained the ability to walk with little assistance and was discharged home. In conclusion, normal NCS findings up to several weeks do not exclude the diagnosis of GBS. Very proximal axonal conduction failure with late distal axonal degeneration should be taken into consideration, and electrodiagnostic follow-up examinations, even employing unusual techniques, are recommended over several weeks after disease onset. |
| first_indexed | 2024-12-21T01:49:38Z |
| format | Article |
| id | doaj.art-68ffe75d5bf9493990b6e35833017ceb |
| institution | Directory Open Access Journal |
| issn | 1664-2295 |
| language | English |
| last_indexed | 2024-12-21T01:49:38Z |
| publishDate | 2017-11-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Neurology |
| spelling | doaj.art-68ffe75d5bf9493990b6e35833017ceb2022-12-21T19:19:55ZengFrontiers Media S.A.Frontiers in Neurology1664-22952017-11-01810.3389/fneur.2017.00594302095Atypical Electrophysiological Findings in a Patient with Acute Motor and Sensory Axonal NeuropathyViviana Versace0Viviana Versace1Stefania Campostrini2Stefania Campostrini3Frediano Tezzon4Sara Martignago5Sara Martignago6Markus Kofler7Leopold Saltuari8Leopold Saltuari9Leopold Saltuari10Luca Sebastianelli11Luca Sebastianelli12Raffaele Nardone13Raffaele Nardone14Department of Neurorehabilitation, Hospital of Vipiteno, Vipiteno, ItalyResearch Unit for Neurorehabilitation South Tyrol, Bolzano, ItalyDepartment of Neurorehabilitation, Hospital of Vipiteno, Vipiteno, ItalyResearch Unit for Neurorehabilitation South Tyrol, Bolzano, ItalyDepartment of Neurology, Franz Tappeiner Hospital, Merano, ItalyDepartment of Neurorehabilitation, Hospital of Vipiteno, Vipiteno, ItalyResearch Unit for Neurorehabilitation South Tyrol, Bolzano, ItalyDepartment of Neurology, State Hospital Hochzirl, Zirl, AustriaDepartment of Neurorehabilitation, Hospital of Vipiteno, Vipiteno, ItalyResearch Unit for Neurorehabilitation South Tyrol, Bolzano, ItalyDepartment of Neurology, State Hospital Hochzirl, Zirl, AustriaDepartment of Neurorehabilitation, Hospital of Vipiteno, Vipiteno, ItalyResearch Unit for Neurorehabilitation South Tyrol, Bolzano, ItalyDepartment of Neurology, Franz Tappeiner Hospital, Merano, ItalyDepartment of Neurology, Christian Doppler Medical Center, Paracelsus Private Medical University of Salzburg, Salzburg, AustriaGuillain–Barré syndrome (GBS) is an immune-mediated polyradiculoneuropathy with acute onset and rapid clinical worsening; early diagnosis and immunomodulating therapy can ameliorate the course of disease. During the first days, however, nerve conduction studies (NCSs) are not always conclusive. Here, we describe a 73-year-old man presenting with progressive muscular weakness of the lower limbs, ascending to the upper limbs, accompanied by distal sensory disturbances. Neuroimaging of brain and spine and NCSs were unremarkable; cerebrospinal fluid analysis revealed no albuminocytologic dissociation. Based on typical clinical features, and on positivity for serum GD1b-IgM antibodies, GBS with proximal conduction failure at multiple radicular levels was postulated, and a standard regime of intravenous immunoglobulin was administered. Four weeks later, the patient presented with flaccid tetraparesis, areflexia, and reduction of position sense, tingling paresthesias, and initial respiratory distress. Repeat NCS still revealed almost normal findings, except for the disappearance of right ulnar nerve F-waves. A few days thereafter, the patient developed severe respiratory insufficiency requiring mechanical ventilation for 2 weeks. On day 50, NCS revealed for the first time markedly reduced compound muscle action potentials and sensory nerve action potentials in all tested nerves, without signs of demyelination; needle electromyography documented widespread denervation. The diagnosis of acute motor and sensory axonal neuropathy was made. After 3 months of intensive rehabilitation, the patient regained the ability to walk with little assistance and was discharged home. In conclusion, normal NCS findings up to several weeks do not exclude the diagnosis of GBS. Very proximal axonal conduction failure with late distal axonal degeneration should be taken into consideration, and electrodiagnostic follow-up examinations, even employing unusual techniques, are recommended over several weeks after disease onset.http://journal.frontiersin.org/article/10.3389/fneur.2017.00594/fullGuillain–Barré syndromeacute motor and sensory axonal neuropathyaxonal conduction failurenodo-paranodopathyanti-ganglioside antibodies |
| spellingShingle | Viviana Versace Viviana Versace Stefania Campostrini Stefania Campostrini Frediano Tezzon Sara Martignago Sara Martignago Markus Kofler Leopold Saltuari Leopold Saltuari Leopold Saltuari Luca Sebastianelli Luca Sebastianelli Raffaele Nardone Raffaele Nardone Atypical Electrophysiological Findings in a Patient with Acute Motor and Sensory Axonal Neuropathy Frontiers in Neurology Guillain–Barré syndrome acute motor and sensory axonal neuropathy axonal conduction failure nodo-paranodopathy anti-ganglioside antibodies |
| title | Atypical Electrophysiological Findings in a Patient with Acute Motor and Sensory Axonal Neuropathy |
| title_full | Atypical Electrophysiological Findings in a Patient with Acute Motor and Sensory Axonal Neuropathy |
| title_fullStr | Atypical Electrophysiological Findings in a Patient with Acute Motor and Sensory Axonal Neuropathy |
| title_full_unstemmed | Atypical Electrophysiological Findings in a Patient with Acute Motor and Sensory Axonal Neuropathy |
| title_short | Atypical Electrophysiological Findings in a Patient with Acute Motor and Sensory Axonal Neuropathy |
| title_sort | atypical electrophysiological findings in a patient with acute motor and sensory axonal neuropathy |
| topic | Guillain–Barré syndrome acute motor and sensory axonal neuropathy axonal conduction failure nodo-paranodopathy anti-ganglioside antibodies |
| url | http://journal.frontiersin.org/article/10.3389/fneur.2017.00594/full |
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