Non-demyelinating, reversible conduction failure in a case of pharyngeal–cervical–brachial weakness overlapped by Fisher syndrome

Chan, Yee Cheun; Ahmad, Aftab; Paliwal, Prakash; Yuki, Nobuhiro

doi:10.1016/j.jns.2012.07.037

Cited by 19 publications

(14 citation statements)

References 20 publications

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“…Follow-up testing in these patients showed rapid recovery in the absence of temporal dispersion, indicating no evidence of remyelination. A similar pattern was observed in a patient diagnosed with PCB overlap with FS, who displayed antibodies against GT1a and GQ1b 5. Together, these studies provide serological and neurophysiological evidence that PCB and AMAN form a continuum.…”

Section: Pathophysiologysupporting

confidence: 74%

“…Autoantibody binding could result in complement activation followed by the disappearance of voltage-gated sodium channel clusters and the disruption of axo-glial or neuromuscular junctions, as demonstrated in animal models of AMAN,27 28 and cause PCB. Immune mediated attack to the axolemma at the nodes of Ranvier may also explain reversible conduction slowing or block in some patients with PCB 5 14…”

Section: Pathophysiologymentioning

confidence: 99%

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Pharyngeal-cervical-brachial variant of Guillain-Barre syndrome

Wakerley

Yuki

2013

Journal of Neurology, Neurosurgery & Psychiatry

131

135

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The pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré syndrome is defined by rapidly progressive oropharyngeal and cervicobrachial weakness associated with areflexia in the upper limbs. Serial nerve conduction studies suggest that PCB represents a localised subtype of Guillain-Barré syndrome characterised by axonal rather than demyelinating neuropathy. Many neurologists are unfamiliar with PCB, which is often misdiagnosed as brainstem stroke, myasthenia gravis or botulism. The presence of additional ophthalmoplegia and ataxia indicates overlap with Fisher syndrome. Half of patients with PCB carry IgG anti-GT1a antibodies which often cross-react with GQ1b, whereas most patients with Fisher syndrome carry IgG anti-GQ1b antibodies which always cross-react with GT1a. Significant overlap between the clinical and serological profiles of these patients supports the view that PCB and Fisher syndrome form a continuous spectrum. In this review, we highlight the clinical features of PCB and outline new diagnostic criteria.

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Section: Pathophysiologysupporting

confidence: 74%

Section: Pathophysiologymentioning

confidence: 99%

Pharyngeal-cervical-brachial variant of Guillain-Barre syndrome

Wakerley

Yuki

2013

Journal of Neurology, Neurosurgery & Psychiatry

131

135

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“…RCB has also been reported in AMSAN, Miller-fissure syndrome, and pharyngeal-cervical-brachial GBS as well as AMAN. 2,9 The present case exhibited a rapid progression and rapid recovery with minor residual symptoms. Serial NCS exhibited RCBs in motor nerves.…”

Section: Discussionmentioning

confidence: 53%

A case of acute motor sensory axonal neuropathy presenting reversible conduction block

Lee

Kim

Park

et al. 2018

Ann Clin Neurophysiol

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Acute motor axonal neuropathy (AMAN) is a subtype of Guillain-Barre syndrome (GBS), which is relatively common in East Asia including Japan, China, and Korea.1,2 According to some reports, AMAN is more prevalent than typical GBS known as acute inflammatory demyelinating polyradiculoneuropathy (AIDP). [3][4][5] On the other hand, acute motor sensory axonal neuropathy (AMSAN) is a rare disease compared with AMAN, but the pathophysiology and clinical course of AMSAN are reported to be similar to those of AMAN except sensory nerve involvement. Reversible conduction block (RCB) which was known to be associated with a sodium-channel dysfunction in node of Ranvier has been reported in some AMAN patients. AMAN with RCB is known to show relative rapid recovery and good prognosis.1,2 However, RCB was rarely reported in patients with AMSAN and the clinical course and outcome in patients with AMSAN with RCB were not well-known. Recently, we experienced a case of AMSAN with RCB in serial electrophysiologic studies. Reversible conduction block (RCB) was rare in patients with acute motor sensory axonal neuropathy (AMSAN). A-46-year-old man presented with paresthesia, weakness, diplopia, and dysarthria. Nerve conduction study (NCS) exhibited axonal changes with conduction block in motor and sensory nerves. His symptoms were rapidly progressed and recovered. Conduction block was disappeared in the follow-up NCS performed after 2 weeks. The AMSAN case with RCB showed rapid progress and rapid recovery of clinical symptoms as acute motor axonal neuropathy patients with RCB.

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“…Studies have shown that antiganglioside antibodies induce a complementmediated attack at the nodes of Ranvier, which disrupts the sodium channel, resulting in conduction failure in motor axonal neuropathies. [3][4][5] This pathology can theoretically arrest at this stage with a functional improvement or, as in our case, can progress to axonal degeneration. 5,6 We tested serum anti-GT1a antibodies as prior studies have described patients with anti-GT1a antibody presenting clinically with PCB/MFS.…”

Section: Sectionmentioning

confidence: 50%

“…[3][4][5] This pathology can theoretically arrest at this stage with a functional improvement or, as in our case, can progress to axonal degeneration. 5,6 We tested serum anti-GT1a antibodies as prior studies have described patients with anti-GT1a antibody presenting clinically with PCB/MFS. 7 However, the test was negative.…”

Section: Sectionmentioning

confidence: 50%

Clinical Reasoning: Oculobulbar dysfunction

Karwa

Nozaki

2017

Neurology

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A 45-year-old man presented a few days after an upper respiratory tract infection with complaints of diplopia, dizziness, and difficulty walking that progressed within a few hours to complete ophthalmoplegia and facial diplegia. Brain MRI showed nonspecific T2 hyperintensities prior to transfer to our facility. On arrival, he was alert and oriented to person, place, and time. Pupils were dilated nonreactive and corneal reflexes were absent bilaterally. He had complete ophthalmoplegia, facial diplegia, and areflexia. His strength in the extremities per Medical Research Council (MRC) scale on initial presentation was preserved at 5/5. His clinical status declined rapidly, necessitating intubation for respiratory failure. Routine blood laboratory workup for infections, metabolic derangements including vitamin B 1 , and thyroid function tests were within normal limits. On day 1, CSF testing showed mildly elevated protein of 47 mg/dL with a normal cell count. CSF cultures and herpes simplex virus (HSV) testing were negative. He also had an EMG/nerve conduction study (NCS), repetitive nerve stimulation (RNS), and single fiber EMG on day 1, all of which were within normal limits. Serum acetylcholine receptor antibody testing was obtained on admission, which was negative.Questions for consideration:

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Non-demyelinating, reversible conduction failure in a case of pharyngeal–cervical–brachial weakness overlapped by Fisher syndrome

Cited by 19 publications

References 20 publications

Pharyngeal-cervical-brachial variant of Guillain-Barre syndrome

Pharyngeal-cervical-brachial variant of Guillain-Barre syndrome

A case of acute motor sensory axonal neuropathy presenting reversible conduction block

Clinical Reasoning: Oculobulbar dysfunction

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