18 Cases' Clinical Research About Polyneuritis Cranialis Variant Of Guillain-Barré Syndrome

Objective: Numerous variants of Guillain-Barre syndrome(GBS)have been documented during the past decade.These variants have similar symptoms and developmental recovery,and treatment patterns.Cranial nerve palsies are common symptoms of GBS,but polyneuritis cranialis(PNC)as a regional variant of GBS are rare and account for only 5% of patients.PNC is immune-mediated disease triggered by various cases.It is a rare disease which is related to infectious,inflammatory or systemic diseases.Affected patients exhibit bilateral facial nerve palsy followed by bulbar dysfunctions(CN IX and X)with no limb weakness and sensory dysfunctions.For pure nerve involvement but normal motor and sensory limb functions of PNC are more domestic and international case reports,it is the lack of large sample clinical research,and now the subtypes of the diagnostic criteria and treatment policy is controversial.Because many grassroots medical staff to the lack of correct understanding of the disease,easily missed diagnosis and misdiagnosis,therefore,prompt identification of polyneuritis cranialis is crucial,but the clinical characteristics remain poorly defined.The present study was to retrospectively analyze the clinical data of 18 patients diagnosed as “PNC”,and relevant literatures were reviewed.Methods: A retrospective review of medical records of 18 patients diagnosed as PNC admitted to our neurology ward in the XX hospital during January,2002 to December,2015,was performed.To research from the following aspects: the epidemiological characteristics including gender,age,seasonal distribution,and clinical manifestations including preceding events,initial symptoms,neurological findingsduring the course,results of electrophysiological study and cerebrospinal fluid examination,specific treatments including intravenous immunoglobulin(IVIg)and steroids,and outcome.To review the recent related domestic and international literatures to further understand the characteristics of PNC.Results: Ten patients had impairment of CN VII,2 of CN VII?IX?X and CN III?IV?VI?VII and CN III?IV?VI?IX?X,1 of CN IX?X and CN III?IV?VI?VII?IX?X?XII.The main manifestations are bilateral eyes closure weakness and salivation,followed by taste abnormality,dysphagia and diplopia.All patients had areflexia or hyporeflexia.Cerebrospinal fluid(CSF)albuminocytologic dissociation within 5-20 days after onset in 8 of 10 patients.Electrophysiological studies showed motor conduction velocity abnormalities in 14 of 15 patients.Treatments with intravenous immunoglobulin and steroids were effective.All patients recovered completely at the end of 3 month after onset.Conclusions: Adtecedent infectious symptoms in most of patients with polyneuritis cranialis(PNC),disease course was monophasic and progressing to a nadir over up to two weeks.Multiple cranial neuropathy with bilateral facial nerve palsy being the most common,followed by abnormalities of CN IX?X and III?IV?VI?XII.Clinical manifestations,nerve conduction studies abnormalities and cerebrospinal fluid(CSF)albuminocytologic dissociation can provide evidence to support the diagnosis of PNC.Treatment with intravenous immunoglobulin was effective and should be the preferred treatment in patients with multiple CN impairment at the onset of GBS.Patients with cranial nerve variant of GBS usually have good prognosis.The study lacked of relevant imaging and laboratory studies,and all patients had no determine of ganglioside antibodies during hospitalization.