Risk factors and outcome of hyponatremia in patients with Guillain-Barré syndrome.

The objective of the present study was to evaluate the risk factors and outcomes associated with hyponatremia in patients with Guillain-Barré syndrome (GBS). We retrospectively studied 80 consecutive patients with GBS who visited our hospital and compared clinical, laboratory, and electrophysiological findings of patients with and without hyponatremia. Disability was evaluated using the Hughes grading system. Of the 80 patients, 18 (23%) had hyponatremia. Hyponatremia was significantly associated with older age (P = 0.003), urinary retention (P < 0.0001), Hughes grade ≥ 4 at admission and nadir (P = 0.003 and P < 0.001, respectively), acute inflammatory demyelinating polyneuropathy subtype (P = 0.017), sepsis (P = 0.001), mechanical ventilator support (P = 0.013), longer hospitalization length of stay (P < 0.0001), and inability to walk independently at 6 months (P < 0.001). Multivariate analysis performed to assess the risk factors of hyponatremia revealed that urinary retention (odds ratio [OR] 30.7, 95% confidence interval [CI] 3.6-264.4; P = 0.002) and mechanical ventilator support (OR 13.8, 95% CI 1.6-118.0; P = 0.017) were significant independent risk factors of hyponatremia. In assessing the outcomes of patients with hyponatremia, multivariate analysis showed that hyponatremia was independently associated with hospitalization length of stay ≥ 60 days and inability to walk independently at 6 month, with the former showing statistical significance but the latter not (OR 9.3, 95% CI 1.8-47.7; P = 0.007 and OR 4.9, 95% CI 0.9-26.3; P = 0.066, respectively). Therefore, we demonstrate that, along with mechanical ventilator support, urinary retention-possibly indicating autonomic dysfunction-is a risk factor of hyponatremia in GBS. Moreover, we confirm that hyponatremia is associated with poor outcome in GBS.

[A pedigree of myotonia congenita with a novel mutation p.F343C of the CLCN1 gene].

A Japanese woman experienced slowness of movement in her early teens and difficulty in opening her hands during pregnancy. On admission to our hospital at 42 years of age, she showed grip myotonia with warm-up phenomenon. However, she had neither muscle weakness, muscle atrophy, cold-induced symptomatic worsening nor episodes of transient weakness of the extremities. Needle electromyography of the first dorsal interosseous and anterior tibial muscles demonstrated myotonic discharges. Whole exome sequencing of the patient revealed a heterozygous single-base substitution in the CLCN1 gene (c.1028T>G, p.F343C). The same substitution was identified in affected members of her family (mother and brother) by Sanger sequencing, but not in healthy family members (father and a different brother). We diagnosed myotonia congenita (Thomsen disease) with a novel CLCN1 mutation in this pedigree. This mutation causes a single amino acid substitution in the I-J extracellular loop region of CLCN1. Amino acid changes in the I-J loop region are rare in an autosomal-dominantly inherited form of myotonia congenita. We think that this pedigree is precious to understand the pathogenesis of myotonia congenita.

Spatial Fluctuation of Central Nervous System Lesions in X-linked Charcot-Marie-Tooth Disease with a Novel GJB1 Mutation.

X-linked Charcot-Marie-Tooth disease type 1 (CMTX1), the most common form of CMTX, is caused by gap-junction beta 1 (GJB1) mutations. We herein report a 25-year-old Japanese man with disorientation, right hemiparesis, and dysarthria. Brain magnetic resonance imaging (MRI) showed high signal intensities in the bilateral cerebral white matter on diffusion-weighted imaging. He had experienced 2 episodes of transient central nervous system symptoms (at 7 and 13 years old). A genetic analysis identified a novel GJB1 mutation, c.169C>T, p.Gln57*. MRI abnormalities shifted from the cerebral white matter to the corpus callosum and had disappeared at the five-month follow-up. Transient changes between these lesions may indicate CMTX1.

[Spinocerebellar ataxia 2 develop lower motor neuron involvement as an initial symptom: a case report].

A 36-year-old man has developed weakness of left thumb and atrophy of left thenar muscle and left first dorsal interosseous muscle without sensory disturbance for a year. A nerve conduction study revealed decreases in the amplitude of compound muscle action potentials and occurrence of F-waves on left medial nerve. Needle electromyography examination revealed positive sharp waves and later recruited motor units on left abductor pollicis brevis muscle. Brain MRI showed atrophy of bilateral cerebellar hemisphere. His grandmother and his two uncles have been diagnosed as spinocerebellar degeneration. After discharge, he developed bilateral lower limb ataxia. Genetic analysis showed heterozygous CAG repeat expansion (19/39) in ATXN2 gene, being diagnosed as spinocerebellar ataxia 2 (SCA2). A previous report has shown that motor neuron involvement is recognized as part of SCA2 in the same pedigree with full CAG repeat expansions in ATXN2 gene. We here report the patient with lower motor neuron involvement as an initial symptom of SCA2.

Surgical Treatment of Distal Radius Fractures Complicated by Concomitant Flexor Carpi Radialis Brevis: A Case Series and Surgical Techniques.

Introduction: The flexor carpi radialis brevis (FCRB) is a rare anatomical variation, with a reported prevalence ranging from 0.9% to 8.7%. Our previous report showed three cases of FCRB in distal radius fracture (DRF) and found that hypoplastic pronator quadratus (PQ) adjacent to the FCRB muscle made it difficult to cover a volar locking plate (VLP). As we subsequently experienced additional six FCRBs, we report on new findings and surgical tips. Case Report: VLP fixation was performed on DRF with FCRB in nine limbs of eight patients. The prevalence was 2.9% (9 of 310 limbs). Of the seven patients that underwent unilateral surgery, six were muscle type and one was tendon type. One patient who underwent bilateral surgery had a muscle type on the left and a tendon type on the right. In three muscle types, as the FCRB muscle belly was widely attached to the radial side of the radius and the radial side of the PQ was hypoplastic, postoperative covering of the plate by repair of the PQ was impossible. Then, in two of those cases, the PQ and FCRB were sutured and the plate was covered. FCRB muscle could be retracted to the radial side in all cases. One patient with a tendon type had a ruptured tendon, which was left unrepaired. All patients had no postoperative problems. Conclusion: In the muscle-type FCRB, the muscle should be retracted to the radial side for VLP fixation. The muscle belly might occupy the radial side of the radius, and the PQ might be hypoplastic and unrepairable. However, the plate can be covered by suturing the PQ and FCRB.

Post-traumatic Non-haemophilic Haemosiderotic Synovitis of the Elbow: A Case Report.

Haemosiderotic synovitis (HS) is a rare synovial proliferative disease secondary to haemarthrosis, often with articular cartilage destruction. It is most frequently reported in patients with haemophiliacs, and the knee joint is most frequently affected. However, there are no reports on the elbow joint without haemophiliacs. A 60-year-old woman who had undergone osteosynthesis for a left radial head fracture 8 years earlier came to our clinic with left elbow pain. X-rays and CT scans showed osteopenia and osteoarthritic changes throughout the elbow joint. MRI revealed joint effusion and synovial membrane hyperplasia. Surgical synovectomy and screw removal were performed. The pathological diagnosis of the synovial membrane was HS. Postoperatively, the pain was relieved, osteopenia improved and there was no recurrence of symptoms. This is the first report of non-haemophilic HS of the elbow; post-traumatic HS caused elbow arthropathy, which was improved by screw removal and synovectomy. Level of Evidence: Level V (Therapeutic).

[A case of anti-myelin oligodendrocyte glycoprotein antibody-positive multiphasic disseminated encephalomyelitis showing significant recovery after immunoadsorption plasmapheresis].

The patient is an 18-year-old female. She had a history of acute disseminated encephalomyelitis at the age of 6 and 7. She visited our hospital due to acute disturbance of consciousness, quadriplegia, and numbness of left upper and lower extremities. Brain MRI showed multiple DWI/FLAIR high-signal lesions in the bilateral cerebral hemispheres, cerebellum, and brainstem. Qualitative test indicated that serum anti-MOG antibodies was positive, and she was diagnosed with anti-MOG antibody-positive polyphasic disseminated encephalomyelitis. Intravenous mPSL pulse therapy was performed twice, but the symptoms worsened. As a second line treatment, plasma exchange was started. However, she developed transfusion related acute lung injury. Alternatively, she was treated with immunoadsorption plasmapheresis. Her symptoms were significantly improved. This case seems to be valuable because there are few reports showing effectiveness of immunoadsorption therapy on anti-MOG antibody-related diseases, especially for polyphasic disseminated encephalomyelitis.

Relationship Between Malunion and Short-Term Outcomes of Nonsurgical Treatment of Distal Radius Fractures in the Elderly: Differences Between Early- and Late-Geriatric Patients.

PURPOSE: Previous studies have suggested little association between radiographic malalignment and long-term functional outcomes of nonsurgical treatment of distal radius fractures in geriatric patients. However, no report has stratified the elderly by age and focused on short-term outcomes. The purpose of this study was to determine how the relationship between malunion and patient outcomes differs between early- and late-geriatric patients in the short and long terms after injury, thereby informing explanations and decision-making on treatment options for geriatric patients with distal radius fractures. METHODS: One hundred patients treated nonsurgically for distal radius fractures were evaluated retrospectively; 52 were defined as early-geriatric patients (aged 60-72 years) and 48 as late-geriatric (aged >77 years). Malunion (dorsal tilt > 10°, ulnar variance > 3 mm, or intra-articular displacement or step-off > 2 mm), range of motion, and grip strength were investigated at 3 months. Multiple regression analysis was performed for each age group using Quick-Disabilities of the Arm, Shoulder, and Hand (QuickDASH) scores at 3 months as the dependent variable. QuickDASH scores over 1 year after injury were analyzed in the same way. RESULTS: The early-geriatric patients included 33 acceptable unions and 19 malunions. The late-geriatric patients included 12 acceptable unions and 26 malunions. The significant predictors of QuickDASH scores at 3 months were malunion for the early-geriatric group and grip strength for the late-geriatric group (standardized coefficient ß, 0.31 and -0.49, respectively). No factor significantly predicted the QuickDASH scores after at least 1 year in either group. CONCLUSIONS: Malunion was associated with worse QuickDASH scores at 3 months after injury in the early-geriatric patients but not in the late-geriatric patients and did not predict the QuickDASH scores at 1 year after injury in either age group. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic IV.

Association between Serum Biomarkers and Peripheral Neuropathy in Microscopic Polyangiitis.

This study aimed to elucidate the pathomechanism of peripheral neuropathy (PN) in microscopic polyangiitis (MPA) and to identify biomarkers useful for diagnosis and severity assessment. Patients with MPA (n = 37) and other non-inflammatory neurological diseases (ONDs; n = 12) were enrolled, and the peripheral nerves of all patients were evaluated using nerve conduction studies. We compared the clinical characteristics and 14 serum biomarker profiles among patients with MPA and PN, MPA without PN, and ONDs. Patients with MPA had a higher prevalence of motor neuropathy than patients with ONDs. Among the patients with MPA, those with motor neuropathy had significantly higher total Birmingham Vasculitis Activity Scores and serum levels of C-reactive protein (CRP), tissue inhibitor of metalloproteinase-1 (TIMP-1), and interleukin-6 than patients without motor neuropathy. Multivariable analyses adjusted for age, serum CRP level, and diabetes mellitus showed that high serum levels of TIMP-1 were independently related to a diagnosis of motor neuropathy in MPA. Additionally, there were significant negative correlations between the serum levels of TIMP-1 and compound muscle action potential amplitudes. Serum levels of TIMP-1 may be associated with the pathomechanism of motor neuropathy in MPA and could be a useful biomarker for diagnosing and evaluating the severity of motor neuropathy in MPA.

Factors Affecting Functional Recovery After Volar Locking Plate Fixation for Distal Radius Fractures.

BACKGROUND: There is still no certainty about factors delaying functional recovery after surgery, although volar locking plate (VLP) fixation is the mainstay of treatment for distal radius fractures (DRFs), and several good postoperative recoveries have been reported. The purpose of this study was to investigate factors affecting functional recovery after VLP fixation for DRF. METHODS: The subjects included 104 patients (84 females, 20 males, mean age: 63.2 ± 13.8 years) treated with VLP fixation for DRF, who could be followed for 1 year. The Quick Disabilities of the Arm, Shoulder, and Hand (QuickDASH) score, grip strength, and range of motion of the wrist joint were measured at 3, 6, and 12 months postoperatively, and the primary outcome was the QuickDASH score. A multiple regression analysis adjusted for age and sex was used to analyze factors affecting functional recovery at 12 months. RESULTS: A multiple regression analysis showed that the factors that significantly influenced the QuickDASH score at 1 year postoperatively were the grip strength ratio to the uninjured side, dominancy of the injured hand, and postoperative ulnar variance, in descending order of involvement. Trauma energy, history of diabetes, fracture type, complications, and range of motion were not included in the model. CONCLUSIONS: Smaller grip strength, dominant-hand injury, and larger postoperative ulnar variance significantly worsened the QuickDASH score at 1 year postoperatively. In order to achieve satisfactory functional recovery at 1 year after surgery, we confirmed that it is important to surgically achieve smaller postoperative ulnar variance and increase grip strength.

Neuron-specific enolase level is a useful biomarker for distinguishing amyotrophic lateral sclerosis from cervical spondylotic myelopathy.

The current study aimed to evaluate whether cerebrospinal fluid (CSF) neuron-specific enolase (NSE) levels are elevated in amyotrophic lateral sclerosis (ALS) and are effective in distinguishing ALS from cervical spondylotic myelopathy (CSM). We retrospectively evaluated 45 patients with ALS, 23 with CSM, 28 controls, and 10 with Parkinson's disease (PD) who underwent analysis of CSF NSE levels. The control group comprised patients aged above 45 years who underwent lumbar puncture because of suspected neurological disorders that were ruled out after extensive investigations. CSF NSE levels were evaluated using the electro-chemiluminescent immunoassay. The ALS group had significantly higher CSF NSE levels than the CSM and control groups (P < 0.001 for both comparisons). The CSM, control, and PD groups did not significantly differ in terms of CSF NSE levels. A receiver-operating characteristic curve analysis was performed to assess the diagnostic value of CSF NSE levels in distinguishing ALS from CSM. The area under the curve for CSF NSE levels was 0.86. The optimal cutoff value was 17.7 ng/mL, with a specificity of 87% and a sensitivity of 80%. Hence, CSF NSE levels are elevated in ALS and are effective in distinguishing ALS from CSM.

Initial serum GM-CSF levels are associated with the severity of cerebral small vessel disease in microscopic polyangiitis patients.

Serum cytokine levels were comprehensively measured, and the association with cerebrovascular lesions on brain magnetic resonance imaging (MRI) in microscopic polyangiitis (MPA) patients was investigated. The initial serum granulocyte-macrophage colony-stimulating factor (GM-CSF) levels were significantly higher in the high-grade white matter hyperintensities (WMH) group than those in the low-grade WMH group. In multivariate analyses, high serum levels of GM-CSF were independently associated with high-grade WMH. The initial serum GM-CSF levels correlated positively with the Birmingham Vasculitis Activity Score and semi-quantitative scales of WMH. The initial serum GM-CSF levels were associated with the severity of WMH in MPA patients.

Assessment of clinical factors affecting outcome of myasthenia gravis.

INTRODUCTION/AIMS: In myasthenia gravis (MG) therapy, achieving Myasthenia Gravis Foundation of America minimal manifestation (MM) or better status is proposed as a desirable target. However, this level of control is often not achieved and clinical factors affecting prognosis remain unclear. METHODS: Participants were 104 consecutive patients with MG who visited Osaka Medical College Hospital. We retrospectively assessed the association of clinical and laboratory features at baseline with prognosis. Eighty patients who achieved MM or better status were classified as the good outcome group and the remaining 24 patients were classified as the poor outcome group. RESULTS: The rate of dysphagia at baseline was significantly higher in the poor outcome group than in the good outcome group (P = .002). The levels of serum total protein and albumin at baseline were both significantly lower in the poor outcome group than in the good outcome group (P = .036 and P = .014, respectively). In addition, Controlling Nutritional Status scores at baseline were significantly higher in the poor outcome group than in the good outcome group (P = .043). Multivariate analysis using a Cox proportional hazards model showed that dysphagia (hazard ratio [HR], 6.92; 95% confidence interval [CI], 1.49-40.31) and hypoalbuminemia (HR, 2.57; 95% CI, 1.04-6.57) at baseline were risk factors that predicted prognosis. DISCUSSION: These findings suggest that dysphagia and hypoalbuminemia at baseline are associated with outcomes and are predictive risk factors for poorer outcomes in patients with MG.

The Quick Disabilities of the Arm, Shoulder, and Hand (QuickDASH) scores in 961 Japanese volunteers.

INTRODUCTION: Patient-reported outcomes recently have been used to assess treatment outcomes. The Quick Disabilities of the Arm, Shoulder, and Hand (QuickDASH) is a particularly convenient and useful tools. However, data on the normative values of Japanese population are lacking, so the present study was conducted to gather this information. METHODS: We assessed 1098 volunteers over 18 years of age (363 men and 735 women, average 50 years old) who had not received upper limb treatment in a medical facility. These participants included our institution's staff, their family members, and the participants in the group meetings held by institution's staff. Their occupations were also examined. We divided occupations into nonmanual and manual labor. These factors of the participants were then analyzed to clarify which (if any) influenced the QuickDASH. RESULTS: Valid answers were obtained from 961 subjects (87.5%). The median score was 2 (mean: 4.8) in the overall population, 0 (mean: 2.6) in men, and 2.5 (mean: 6.0) in women. The scores increased with age and were higher in women than in men. There were no significant differences by manual labor. Female sex and older age were identified as factors that influenced the QuickDASH score in the multiple regression analysis. There were high correlations among QuickDASH, work and sports/music scores. CONCLUSIONS: The present study provided QuickDASH scores for Japanese volunteers who had not received upper limb treatment in a medical facility. The scores were associated with older age and female sex. This study helps us to know the degree of potential upper limb impairment in the general population, and will help in populational strategies as primary and secondary preventive medicine for upper limb-related diseases.

Effect of circularly polarized light on germination, hypocotyl elongation and biomass production of arabidopsis and lettuce: Involvement of phytochrome B.

Circular dichroism (CD), defined as the differential absorption of left- and right-handed circularly polarized light (CPL), is a useful spectroscopic technique for structural studies of biological systems composed of chiral molecules. The present study evaluated the effects of CPL on germination, hypocotyl elongation and biomass production of Arabidopsis and lettuce. Higher germination rates were observed when Arabidopsis and lettuce seedlings were irradiated with red right-handed CPL (R-CPL) than with red left-handed CPL (L-CPL). Hypocotyl elongation was effectively inhibited when Arabidopsis and lettuce seedlings were irradiated with red R-CPL than with red L-CPL. This difference was not observed when a phytochrome B (phyB) deficient mutant of Arabidopsis was irradiated, suggesting that inhibition of elongation by red R-CPL was mediated by phyB. White R-CPL induced greater biomass production by adult Arabidopsis plants, as determined by their fresh shoot weight, than white L-CPL. To determine the molecular basis of these CPL effects, CD spectra and the effect of CPL on the photoreaction of a sensory module of Arabidopsis phyB were measured. The red light-absorbing form of phyB showed a negative CD in the red light-absorbing region, consistent with the results of germination, inhibition of hypocotyl elongation and biomass production. L-CPL and R-CPL, however, did not differ in their ability to induce the interconversion of the red light-absorbing and far-red light-absorbing forms of phyB. These findings suggest that these CPL effects involve phyB, along with other photoreceptors and the photosynthetic process.

Clinical features of Guillain-Barré syndrome patients with elevated serum creatine kinase levels.

BACKGROUND: 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: We 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. RESULTS: Of 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). CONCLUSIONS: The 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.

Relationship Between Hand Dominance and Treatment Outcomes for Distal Radius Fractures in the Elderly in the Short-Term.

Purpose: Many studies have found no notable long-term differences in functional outcomes between operative and conservative treatments for distal radius fractures (DRFs) in elderly patients. However, those studies have not considered hand dominance. The current study compared outcomes between both treatments in a dominant wrist-injured group (dominant group) and nondominant wrist-injured group (nondominant group). Methods: A total of 101 patients aged 65 years and older who experienced displaced DRF requiring reduction and who were managed for over 3 months with either operative or conservative treatment were examined. The dominant group included 46 subjects (operative, n = 26; conservative, n = 20), and the nondominant group included 55 subjects (operative, n = 28; conservative, n = 27). All operative treatments were performed with volar locking plate fixation, and all conservative treatments were immobilized with a sugar-tong orthosis or forearm cast. Functional outcomes and radiographic assessments were compared 3 months after treatment. The primary outcome measure was the Quick-Disabilities of the Arm, Shoulder, and Hand (QuickDASH) score; secondary outcomes were grip strength, range of motion, and Mayo wrist score. We also examined QuickDASH scores after at least 1 year. Results: The QuickDASH score showed no significant differences between treatments by dominance at 3 months or more than a year. In the dominant group, operative treatment resulted in significant 7-kg greater grip strength at 3 months, whereas the nondominant group showed no significant differences in functional outcomes between treatments. Conclusions: Although QuickDASH scores were similar at 3 months and 1 year between treatments regardless of hand dominance, surgery for dominant-side DRF resulted in better grip strength than conservative treatment at 3 months. Clinical relevance: This study will help clarify potential outcomes differences between operative and conservative DRF treatment in patients aged over age 65 years.

Volar Locking Plate Fixation of Distal Radius Fracture with a Flexor Carpi Radialis Brevis and a Hypoplastic Pronator Quadratus.

INTRODUCTION: Recently, distal radius fracture (DRF) has been treated with internal fixation using volar locking plates and several reports describing patients with a flexor carpi radialis brevis (FCRB) appeared. However, no studies have so far investigated the location of the FCRB relative to the volar locking plate. CASE REPORT: We herein report three cases of DRF with an FCRB. Two patients had a bilateral FCRB, and an FCRB was detected in 5 of 174 limbs (2.9%). In all cases, the FCRB had a muscle belly and was retracted to the radial side and volar plate fixation was performed without difficulty. The pronator quadratus (PQ) under the FCRB was thin. In one case, the PQ was hypoplastic and restoration was impossible. The distance from the plate to the FCRB and that from the plate to the flexor pollicis longus (FPL) tendon were examined postoperatively using ultrasound. In the case in which the PQ could not be restored, the FPL tendon was located close to the plate and the FCRB was in contact with the plate. CONCLUSION: As volar locking plate fixation of a DRF with an FCRB and a hypoplastic PQ may cause the restoration of the PQ impossible, the operation should be performed more carefully and follow-up is necessary to avoid post-operative FPL tendon injury and FCRB tendinopathy due to friction with the plate.

Fulminant Guillain-Barré syndrome showing severe pharyngeal-cervical-brachial weakness in the recovery phase: a case report.

BACKGROUND: Fulminant Guillain-Barré syndrome (GBS) is characterized clinically by rapid progression of severe symptoms, such as the absence of brainstem reflexes, complete tetraplegia and respiratory arrest. The clinical course of fulminant GBS remains unclear. Here, we report a patient with fulminant GBS, who showed severe weakness of the pharyngeal-cervical-branchial (PCB) area in the recovery phase. CASE PRESENTATION: A 38-year-old man rapidly developed fulminant GBS. In blood examination, he was positive for a broad range of anti-ganglioside antibodies, including anti-GQ1b, GT1a, GT1b, GD1a, GD1b and GD3 IgG antibodies. We performed immunosuppressive therapies using intravenous immunoglobulin and intravenous methylprednisolone. Although disturbance of consciousness and weakness of the distal upper and lower limbs improved gradually, weakness of the oropharynx, neck, and proximal upper limbs were resistant to these therapies. Anti-GT1a IgG antibodies remained persistently positive. Consequently, mechanical ventilation and tube feeding were required for 7 and 10 months, respectively. Two years later, weakness of the proximal upper limbs and mild respiratory dysfunction remained as sequelae. CONCLUSION: Anti-GT1a IgG antibodies are known to be detected in patients with the PCB variant of GBS. In fulminant GBS, the persistent presence of anti-GT1a IgG antibodies may be associated with occurrence of severe PCB-like weakness in the recovery phase.

[An autopsy case of nivolumab-induced myasthenia gravis and myositis].

An 84-year-old woman developed blepharoptosis, diplopia, weakness of extremities, and dysphagia with elevation of serum CK levels after treatment with nivolumab against renal cell carcinoma. 3 Hz repetitive stimulation showed waning in the trapezius muscle, leading to the diagnosis of myasthenia gravis. Laboratory examination showed that anti-acetylcholine receptor antibody was negative. We performed IVIg and steroid therapy. However, her symptoms did not improve, and she died of respiratory failure, although serum CK levels ameliorated to the normal range. The results of autopsy showed atrophy of muscle fibers and massive infiltration of inflammatory cells in the endomysium of the iliopsoas muscle and diaphragm, indicating occurrence of myositis. Immunohistochemical analysis showed that CD8-positive T cells mainly infiltrates in the endomysium with a small number of CD4-potive T cells. Here, we report an autopsy case of nivolumab-induced myasthenia gravis and myositis.