10.4066/AMJ.2014.2123

Dosi, Rupal; Ambaliya, Annirudh; Patel, Niyati; Shah, Monisha; Patell, Rushad

doi:10.4066/amj.2014.2123

amj

2014

DOI: 10.4066/amj.2014.2123

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10.4066/AMJ.2014.2123

Rupal Dosi¹,

Annirudh Ambaliya²,

Niyati Patel³

et al.

Abstract: We report the case of a 20-year-old male who presented to us with acute bilateral multiple cranial neuropathy in the form of bilateral total ophthalmoplegia and bulbar dysfunction. The patient had normal haematological and biochemical investigations, however, cerebrospinal fluid (CSF) analysis showed raised protein (96mg/dl) in the second week of illness. Peripheral nerve conduction studies and an MRI of the brain were normal. The patient showed gradual improvement after three weeks of supportive treatment. Co… Show more

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Cited by 3 publications

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Polyneuritis cranialis: oculopharyngeal subtype of Guillain-Barré syndrome

Wakerley

Yuki

2015

J Neurol

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Guillain-Barré syndrome (GBS) constitutes a spectrum of related post-infectious neuropathies, which are characterized by their anatomical patterns of weakness and neurological involvement. Historically, the term polyneuritis cranialis has been used to describe some patients with GBS presenting with multiple cranial neuropathies in the absence of limb weakness. We examine previous reports of polyneuritis cranialis to determine disease characteristics and define new diagnostic criteria. Disease characteristics were determined from 15 historical case reports of patients presenting with isolated 'polyneuritis cranialis', 'cranial polyneuropathy', 'multiple cranial neuropathy' and 'multiple cranial neuropathies' due to GBS. Median age was 40 years. 80% displayed antecedent infectious symptoms. In all cases, disease course was monophasic with clinical improvement within weeks or months. Initial symptoms were ocular (73%) or bulbar (33%). Mean number of cranial nerves involved was 6 (range, 3-10). 93% displayed ocular signs, 73% facial weakness or numbness and 87% bulbar signs. In 3 patients (20%), there was significant asymmetry. Cerebrospinal fluid albuminocytological dissociation was present in 67% of cases. Serum anti-ganglioside antibodies were tested for in 8 of 15 patients and anti-GQ1b antibodies were found in 3 patients, whilst anti-GT1a antibodies were found in 1 patient. Polycranial neuritis (the oculopharyngeal subtype of GBS) can be defined in patients with disease characteristics of GBS who display ocular and pharyngeal weakness in the absence of limb weakness or ataxia. In half of cases tested for, anti-ganglioside antibodies were present and most frequently against GQ1b.

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Polyneuritis cranialis: oculopharyngeal subtype of Guillain-Barré syndrome

Wakerley

Yuki

2015

J Neurol

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Co-occurrence of polyneuritis crainials and visual impairment: a case report and literature review

Huang

et al. 2022

Neurol Sci

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Background Polyneuritis cranialis (PNC) with the disease characteristics of Guillain-Barré syndrome (GBS) in addition to both ocular and bulbar weakness in the absence of limb paralysis or ataxia is defined as an unusual variant of GBS. As evidence of central nervous system (CNS) involvement, visual impairment is an unusual finding complicating with GBS spectrum disorders and has never been reported in patients with PNC. Methods We describe a very rare case who clinically presented with progressive multiple cranial nerve palsy and visual impairment. Furthermore, a literature search of concurrent GBS and optic neuritis (ON) as well as PNC attributed to GBS was conducted. Results A diagnosis of PNC was considered due to the typical clinical characteristics as well as the presence of cerebrospinal fluid cytoalbumin dissociation and serum antibodies against gangliosides. The clinical manifestations and the bilateral optic nerve involvement in brain magnetic resonance imaging further suggested possible optic neuritis (ON). The patient received treatment with intravenous immunoglobulin followed by short-term use of corticosteroids and finally achieved a full recovery. Thirty-two previously reported cases (17 women, mean age 40) of concurrent GBS and ON and 20 cases of PNC (5 women, mean age 40) were analyzed. We further provided a comprehensive discussion on the potential etiologies, clinical features, therapeutic strategies, and prognosis. Conclusions This rare case with the co-occurrence of PNC and visual impairment and the related literature review may help clinicians advance the understanding of GBS spectrum disorders and make appropriate diagnoses and treatment decisions for the rare variants and CNS complications of GBS. Supplementary Information The online version contains supplementary material available at 10.1007/s10072-022-06580-0.

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An Oculopharyngeal Subtype of Guillain-Barré Syndrome Sparing the Trochlear and Abducens Nerves

et al. 2020

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Guillain-Barré syndrome (GBS) comprises a group of polyneuropathies characterized by rapid progression of limb paralysis. Various subtypes of GBS have been reported. The oculopharyngeal subtype of GBS is currently understood to be primarily a cranial polyneuropathy without limb weakness or cerebellar ataxia. In our case of 62-year-old man, gastrointestinal infection was followed by paranesthesia of the hands. He had bilateral ptosis, pharyngeal disorder, and tongue and bifacial weakness. We diagnosed oculopharyngeal subtype of GBS. It responded to intravenous immunoglobulin. This case highlights the need for further characterization of unusual GBS subtypes.

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10.4066/AMJ.2014.2123

Cited by 3 publications

References 5 publications

Polyneuritis cranialis: oculopharyngeal subtype of Guillain-Barré syndrome

Polyneuritis cranialis: oculopharyngeal subtype of Guillain-Barré syndrome

Co-occurrence of polyneuritis crainials and visual impairment: a case report and literature review

An Oculopharyngeal Subtype of Guillain-Barré Syndrome Sparing the Trochlear and Abducens Nerves

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