Impact of Possible Sarcopenia and Nutritional Status on Postoperative Quick Disabilities of the Arm, Shoulder, and Hand Score in Geriatric Women With Distal Radius Fracture.

PURPOSE: Distal radius fracture (DRF), sarcopenia, and malnutrition have been reported to be interrelated. However, there are few reports on the effects of sarcopenia and malnutrition on DRF patients' postoperative outcomes. This study examined the healthy-side grip strength and preoperative blood tests to determine the presence of possible sarcopenia (PS) and malnutrition in geriatric women with DRF and their impact on postoperative functional outcomes. METHODS: Fifty-five woman older than 60 years treated with volar-locking plate fixation for low-energy DRF from standing-level falls were retrospectively studied. Based on the criteria of The Asian Working Group for Sarcopenia 2019, patients with a healthy-side grip strength <18 kg were defined as PS. Nutritional assessment was performed using Onodera's Prognostic Nutritional Index (PNI) before surgery, with a value <50 defined as malnutrition. The Quick Disabilities of the Arm, Shoulder, and Hand (QuickDASH) was used for functional assessment at 1 year after surgery. Patients were divided into two groups according to PS, and patient demographic data and postoperative outcomes were compared. Multiple regression analysis was performed to estimate the regression coefficient and 95% confidence intervals for 1-year QuickDASH after surgery with adjustment for age, PS, and malnutrition. RESULTS: Possible sarcopenia was present in 10 patients (18.2%), and malnutrition in 24 patients (43.6%). Possible sarcopenia patients were older, had lower PNI, serum albumin, and both sides grip strength, and worse QuickDASH compared with non-PS patients. In multiple regression analysis, age, PS, and malnutrition were significant predictors of QuickDASH (standardized coefficient ß, 0.35, 0.34, and 0.24; 95% confidence interval, 0.22-1.02, 3.52-16.49, and 0.50-10.78). CONCLUSIONS: Possible sarcopenia with a healthy-side grip strength <18 kg and malnutrition with a PNI <50 were associated with worse 1-year QuickDASH after surgery in women DRF patients over 60 years. TYPE OF STUDY/LEVEL OF EVIDENCE: Prognostic Ⅳ.

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.

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.

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.

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.

Anti-MOG antibody-positive ADEM following infectious mononucleosis due to a primary EBV infection: a case report.

BACKGROUND: Anti-Myelin oligodendrocyte glycoprotein (MOG) antibodies are detected in various demyelinating diseases, such as pediatric acute disseminated encephalomyelitis (ADEM), recurrent optic neuritis, and aquaporin-4 antibody-seronegative neuromyelitis optica spectrum disorder. We present a patient who developed anti-MOG antibody-positive ADEM following infectious mononucleosis (IM) due to Epstein-Barr virus (EBV) infection. CASE PRESENTATION: A 36-year-old healthy man developed paresthesia of bilateral lower extremities and urinary retention 8 days after the onset of IM due to primary EBV infection. The MRI revealed the lesions in the cervical spinal cord, the conus medullaris, and the internal capsule. An examination of the cerebrospinal fluid revealed pleocytosis. Cell-based immunoassays revealed positivity for anti-MOG antibody with a titer of 1:1024 and negativity for anti-aquaporin-4 antibody. His symptoms quickly improved after steroid pulse therapy followed by oral betamethasone. Anti-MOG antibody titer at the 6-month follow-up was negative. CONCLUSIONS: This case suggests that primary EBV infection would trigger anti-MOG antibody-positive ADEM. Adult ADEM patients can be positive for anti-MOG antibody, the titers of which correlate well with the neurological symptoms.

[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.

[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.

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.

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).

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.

[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.

Interferon-ß1b increases Th2 response in neuromyelitis optica.

A Japanese randomized controlled study showed that Interferon â (IFN-â1b) therapy is clinically effective in decreasing the frequency of attacks in multiple sclerosis (MS), even in optico-spinal MS (OSMS). However, recent studies have shown that IFN-â (IFN-â1a/IFN-â1b) treatment was not effective in neuromyelitis optica (NMO) patients and that the diminished benefit of IFN-â treatment in NMO may be due to different immune responses to IFN-â. We determined longitudinally the expression of CCR5, CXCR3 and CCR4 on CD4+ T and CD8+ T cells in the blood from patients with NMO and MS treated with IFN-â1b. During a 12-month period of IFN-â1b therapy, the annualized relapse rate decreased in MS patients but not in NMO patients. There was no significant difference in the expression of the chemokine receptors between NMO and MS at baseline. The percentages of CD4+CCR5+ and CD4+CXCR3+ T cells, representative of the Th1 response, were decreased in both NMO and MS after treatment. The percentage of CD4+CCR4+ T cells, representative of the Th2 response, was decreased in MS, but those for NMO was significantly increased compared with the pretreatment levels. Our results indicate that IFN-â1b-induced up-modulation of the Th2 response in NMO patients may be the source of differences in the therapeutic response to IFN-â1b therapy. In the present study, Th2 predominance is involved in the pathogenesis of NMO.

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.

Onset and spreading patterns of lower motor neuron involvements predict survival in sporadic amyotrophic lateral sclerosis.

OBJECTIVE: To define patterns of spread through the order of lower motor neuron involvement (first, second or third order), relationships between interval or sites of affected areas from onset to involvement of a second region, and prognosis, including 5 year survival, normal preservation of motor function at onset of respiratory symptoms and cumulative occurrence of each region and direction of spread. METHOD: 150 patients with sporadic amyotrophic lateral sclerosis (ALS) underwent follow-up at 3 month intervals until the appearance of respiratory symptoms. Symptom appearances were determined using the revised version of the ALS Functional Rating Scale. RESULT: Median survival with combined type onset (two regions simultaneously) was shorter (18 months) than with bulbar onset (26 months, p=0.01). The interval from onset to involvement of the second region correlated significantly with survival, independent of particular combinations. 5 year survival rate was 21% for lower limb onset, 18% for upper limb onset and 16% for bulbar onset. No patient with a rapid spread pattern (two regions within 3 months from onset) survived >5 years. Early manifestations of bulbar symptoms within 1 year were associated with worse survival (p<0.001) although no significant difference in survival was seen between groups with and without bulbar symptoms (p=0.51). In terms of cumulative occurrence, symptoms spread longitudinally to adjacent regions. Bulbar function remained preserved in 27%, lower limb function in 10% and upper limb function in 2.7%. CONCLUSION: The interval between onset and involvement of the second region is an important predictor of survival. The data support the contiguous anatomical propagation of lower motor neuron involvement in sporadic ALS.

Solitary osteochondroma of the trapezium: case report.

Osteochondroma of the carpal is rare. We found only 1 case of osteochondroma of the trapezium in the literature. We present a case of a 52-year-old woman with an osteochondroma of the left trapezium and trapeziometacarpal arthritis.

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.