Polyneuritis cranialis: oculopharyngeal subtype of Guillain-Barré syndrome

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.