Clinical features of Guillain\u2013Barr\xe9 syndrome patients with elevated serum creatine kinase levels

Takafumi Hosokawa,Fumiharu Kimura,Eri Sano,Akihiro Tsukahara,Kiichi Unoda,Yoshitsugu Nakamura,Sadaki Sakane,Hideto Nakajima,Taiki Sawai,Shigeki Arawaka,Shimon Ishida

doi:10.1186/s12883-020-01796-z

Abstract

BackgroundIt is not well defined whether Guillain–Barré syndrome (GBS) patients with elevated serum creatine kinase (CK) levels have characteristic clinical features and are related to the subgroups of GBS.MethodsWe retrospectively studied 51 consecutive patients with GBS, who visited our hospital, and compared clinical, laboratory and electrophysiological findings between patients with and without elevated CK levels.ResultsOf 51 patients, 14 patients (27%) showed an elevation of serum CK levels. When compared with patients with the normal CK levels, the ratios of male, antecedent infections, and anti-GM1 antibody positivity were significantly higher in patients with elevated CK levels. The ratios of hypoesthesia, cranial nerve involvement, and urinary retention were significantly less in patients with elevated CK levels. There were no significant differences in disability at peak between two groups. In the electrophysiological examination, sensory nerve abnormalities were not observed. Although some patients with elevated CK levels showed prolongation of distal motor latencies (DMLs) and increase of durations in the initial examination, development of the prolongation of DMLs and increase of durations was not observed in the follow-up examinations. The findings were consistent with acute motor axonal neuropathy (AMAN) with reversible conduction failure (RCF) but not acute inflammatory demyelinating polyneuropathy (AIDP).ConclusionsThe results suggest that the GBS patients with elevated CK levels represent not a group of AIDP but a group of AMAN with axonal degeneration or RCF even though the initial electrophysiological examination shows AIDP pattern.

Highlights

Guillain–Barré syndrome (GBS) is classified into two major subgroups: acute motor axonal neuropathy (AMAN) and acute inflammatory demyelinating polyneuropathy (AIDP) [1,2,3,4,5,6]
Electrodiagnosis based on conventional classification system is reported to be often inconsistent with the true subgroup of GBS. 11– 41% of patients with GBS failed to judge AIDP or AMAN, because electrophysiological findings did not fulfill the criteria for “AIDP pattern” or “AMAN pattern.” [1, 11, 12, 26] AMAN with reversible conduction failure (RCF) is misdiagnosed as AIDP, because electrophysiological examinations at the acute phase indicate the “AIDP pattern,” such as prolongation of Distal motor latency (DML) and increase of durations [11, 26, 27]
We found that AMAN following upper respiratory tract infection (URTI), including with anti-GM1 antibody, may be a specific feature of GBS patients with elevated creatine kinase (CK) levels

Summary

Introduction

Guillain–Barré syndrome (GBS) is classified into two major subgroups: acute motor axonal neuropathy (AMAN) and acute inflammatory demyelinating polyneuropathy (AIDP) [1,2,3,4,5,6]. While cerebrospinal fluid test abnormalities in Guillain–Barré syndrome are well known, serum test abnormalities have been reported One such serum abnormality in GBS is an elevation in creatine kinase. Focusing on the presence or absence of sensory nerve conduction abnormalities, we established new system classifying GBS into AMAN with axonal degeneration, AMAN with RCF, and true AIDP by using only a single electrophysiological examination. It is not well defined whether Guillain–Barré syndrome (GBS) patients with elevated serum creatine kinase (CK) levels have characteristic clinical features and are related to the subgroups of GBS

Methods

Results

Discussion

Conclusion