Polyneuritis cranialis: oculopharyngeal subtype of Guillain-Barré syndrome

Abstract

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.

Bifacial weakness with paresthesias: Serial nerve conduction studies indicate diffuse demyelinating neuropathy

“…Although weakness was restricted to facial muscles, limb NCS were abnormal and indicated that subclinical peripheral nerve involvement in BFP can be very diffuse. Similar observations have been noted in a significant proportion of patients with MFS and polyneuritis cranialis despite there being no obvious limb weakness, or in some cases, no loss of tendon stretch reflexes . Interestingly, in our patient, NCS indicated that lower limb nerves were affected more and earlier than upper limb nerves, mirroring that which is typically observed in patients with acute inflammatory demyelinating neuropathy (AIDP) who develop an ascending weakness.…”

“…Similar observations have been noted in a significant proportion of patients with MFS and polyneuritis cranialis despite there being no obvious limb weakness, or in some cases, no loss of tendon stretch reflexes. 10,16 Interestingly, in our patient, NCS indicated that lower limb nerves were affected more and earlier than upper limb nerves, mirroring that which is typically observed in patients with acute inflammatory demyelinating neuropathy (AIDP) who develop an ascending weakness. Conversely, serial NCS in a patient with pharyngeal-cervical-brachial (PCB) weakness overlap with MFS showed peripheral nerve involvement to be more prominent and develop earlier in the upper limbs, which paralleled the pattern of limb weakness.…”

“…The presence of antecedent infection, distal paresthesias, and CSF albuminocytological dissociation were highly suggestive of a GBS‐type disorder . The absence of ophthalmoplegia, bulbar weakness, and ataxia indicated that this was not a subtype of Miller Fisher syndrome (MFS) or polyneuritis cranialis . Other important differentials, including Lyme disease, sarcoidosis, and human immunodeficiency virus, were also considered and excluded.…”

“…5 The absence of ophthalmoplegia, bulbar weakness, and ataxia indicated that this was not a subtype of Miller Fisher syndrome (MFS) 9 or polyneuritis cranialis. 10 Other important differentials, including Lyme disease, sarcoidosis, and human immunodeficiency virus, were also considered and excluded. Although bilateral facial palsy has been reported, 11 disease course, rapid recovery, and evidence of neuropathy in our patient were more suggestive of BFP.…”

Polyneuritis cranialis—subtype of Guillain–Barré syndrome?

“…1 In a recent review, we examined the clin ical features of 15 historical cases of poly neuritis cranialis attributed to GBS. 7 In the majority of cases, patients displayed a combi nation of ocular signs (ophthalmo plegia, ptosis or pupillary changes) and bulbar signs (dysarthria or dysphagia), which were often associated with facial weakness. Unlike other GBS subtypes, weakness was frequently asymmetric.…”