Serum neurofilament and glial fibrillary acidic protein in idiopathic and seropositive transverse myelitis.

BACKGROUND: Serum levels of neurofilament light chain (NfL) and glial fibrillary acidic protein (GFAP) reflect the disease activity and disability in central nervous system (CNS) demyelinating diseases. However, the clinical significance of NfL and GFAP in idiopathic transverse myelitis (iTM), an inflammatory spinal cord disease with unknown underlying causes, remains unclear. This study aimed to investigate NfL and GFAP levels in iTM and their association with the clinical parameters compared with those in TM with disease-specific antibodies such as anti-aquaporin 4 or myelin oligodendrocyte glycoprotein antibodies (sTM). METHODS: We collected serum and clinical data of 365 patients with CNS inflammatory diseases from 12 hospitals. The serum NfL and GFAP levels were measured in patients with iTM (n = 37) and sTM (n = 39) using ultrasensitive single-molecule array assays. Regression analysis was performed to investigate the associations between serum levels of NfL and GFAP and the clinical parameters such as higher EDSS scores (EDSS ≥ 4.0). RESULTS: Mean NfL levels were not significantly different between iTM (50.29 pg/ml) and sTM (63.18 pg/ml) (p = 0.824). GFAP levels were significantly lower in iTM (112.34 pg/ml) than in sTM (3814.20 pg/ml) (p = 0.006). NfL levels correlated with expanded disability status scale (EDSS) scores in sTM (p = 0.001) but not in iTM (p = 0.824). Disease duration also correlated with higher EDSS scores in sTM (p = 0.017). CONCLUSION: NfL levels and disease duration correlated with EDSS scores in sTM, and GFAP levels could be a promising biomarker to differentiate iTM from sTM.

Unexpected shunt-dependent hydrocephalus after unruptured aneurysm surgery-a case report.

A chronic hydrocephalus after unruptured aneurysm surgery is an extremely rare condition. Its etiology and pathophysiology are also unclear. We report a case of chronic hydrocephalus in a patient who underwent permanent shunt placement after unruptured aneurysm clipping surgery. A 65-year-old man developed chronic hydrocephalus requiring shunt placement after clipping surgery of left anterior cerebral artery aneurysm and right middle cerebral artery aneurysm. This case shows that chronic hydrocephalus is a possible complication of unruptured aneurysm surgery, which can be resolved with an appropriate shunt operation.

A comprehensive overview of AL amyloidosis disease characteristics accumulated over two decades at a single referral center in Korea.

BACKGROUND: Characteristics of AL amyloidosis across Asia are not well-described in the literature. Thus, we overviewed the incidence and disease characteristics of AL amyloidosis in Korea. METHODS: We collected medical records of 302 AL amyloidosis patients and compared survival outcomes by predominant treatment strategy and at four time points: 1995-2003, 2004-2008, 2009-2013, and 2014-2018. RESULTS: The median age was 62 years (36-83). One hundred forty-one patients were classified as stage III (26.3%) or IV (47.9%). The patients diagnosed between 2014 and 2018 survived longer than those diagnosed at other time points due to the introduction of bortezomib (p < 0.01). In addition, patients who received upfront ASCT survived longer than those who received salvage ASCT or chemotherapy alone (p < 0.01). However, most of the 85 patients who experienced early death within 6 months were older than 75 years, had BMI less than 20, and had a high disease burden. CONCLUSIONS: The incidence of AL amyloid has increased and survival outcomes have improved gradually, most likely due to introduction of novel agents and upfront ASCT. However, not all patients are suitable for these potent treatment modalities, and avoiding early death within 6 months remains a challenge.

Generalised autonomic failure as a prognostic factor in systemic light-chain (AL) amyloidosis.

BACKGROUND: In the present study, it was investigated whether autonomic dysfunction could predict prognosis in light-chain (AL) amyloidosis patients. PATIENTS AND METHODS: Seventy-two patients with biopsy-proven AL amyloidosis were included and underwent an autonomic function test (AFT) between January 2016 and June 2019. Autonomic failure was evaluated using the Composite Autonomic Severity Score (CASS). Survival curves and the three-year overall survival (OS) rate were estimated using the Kaplan-Meier curve, and the Cox proportional hazards regression method was used to evaluate the variables that influenced survival. RESULTS: Autonomic dysfunction was observed in 69 (96%) patients with AL amyloidosis, and the three-year OS rate was 67%. Generalised autonomic failure (GAF) was observed in 31 (43%) patients. In the Kaplan-Meier curve, the three-year OS rates in patients with sudomotor dysfunction or GAF were lower than that in control patients (35 vs. 84%, and 33 vs. 81%, respectively). In Cox proportional hazards regression model, female, bone marrow plasma cell percentage, left ventricular systolic dysfunction, and GAF were significant independent variables associated with survival. CONCLUSION: The results of this study indicate that GAF on the AFT is an independent adverse prognostic factor for survival in AL amyloidosis patients.

Efficacy and safety of Lenzumestrocel (Neuronata-R® inj.) in patients with amyotrophic lateral sclerosis (ALSUMMIT study): study protocol for a multicentre, randomized, double-blind, parallel-group, sham procedure-controlled, phase III trial.

BACKGROUND: A single cycle (two repeated treatments) with intrathecal autologous bone marrow-derived mesenchymal stem cells (BM-MSCs, 26-day interval) showed safety and provided therapeutic benefit lasting 6 months in patients with ALS but did not demonstrate long-term efficacy. This phase III clinical trial (ALSUMMIT) protocol was developed to evaluate the long-term efficacy and safety of the combined protocol of single-cycle intrathecal therapy and three additional booster injections of BM-MSC (Lenzumestrocel) treatment in patients with ALS. METHODS: ALSUMMIT is a multicentre, randomized, double-blind, parallel-group, sham procedure-controlled, phase III trial for ALS. The 115 subjects will be randomized (1:2:2) into three groups: (1) study Group 1 (single-cycle, two repeated injections with 26-day interval), (2) study Group 2 (single-cycle + three additional booster injections at 4, 7, and 10 months), and (3) the control group. Participants who have an intermediate rate of disease progression will be included in this trial to reduce clinical heterogeneity. The primary endpoint will be evaluated by combined assessment of function and survival (CAFS), also known as joint rank scores (JRS), at 6 months (study Group 1 vs. control) and 12 months (study Group 2 vs. control) after the first Lenzumestrocel or placebo administration. Safety assessment will be performed throughout the study period. Additionally, after the 56-week main study, a long-term follow-up observational study will be conducted to evaluate the long-term efficacy and safety up to 36 months. DISCUSSION: Lenzumestrocel is the orphan cell therapy product for ALS conditionally approved by the South Korea Ministry of Food and Drug Safety (MFDS). This ALSUMMIT protocol was developed for the adoption of enrichment enrolment, add-on design, and consideration of ethical issues for the placebo group. TRIAL REGISTRATION: ClinicalTrials.gov NCT04745299 . Registered on Feb 9, 2021. Clinical Research Information Service (CRIS) KCT0005954 . Registered on Mar 4, 2021.

Two cases of myelin oligodendrocyte glycoprotein antibody-associated disease presenting with Cauda Equina Syndrome without conus myelitis.

Myelin oligodendrocyte glycoprotein antibody-associated disorder (MOGAD) is a central nervous system inflammatory disorder associated with MOG antibodies. Two patients with clinical symptoms of cauda equina syndrome had positive serum MOG antibody tests, and spinal magnetic resonance imaging showed cauda equina enhancement. They were diagnosed with incomplete cauda equina syndrome associated with MOGAD. A few cases of lumbosacral radiculomyelitis associated with MOGAD have been reported; however, this is the first report of isolated lumbosacral radiculitis associated with MOGAD without transverse myelitis. The MOG antibody test should be considered for cauda equina syndrome without compressive lesions.

Increased proportion of CD20+ T cells after rituximab treatment in patient with neuromyelitis optica later diagnosed with lung B-cell lymphoma: A case report.

INTRODUCTION: Neuromyelitis optica (NMO) is a rare inflammatory autoimmune disorder of the CNS. Rituximab is used to treat antibody-mediated autoimmune diseases. CASE PRESENTATION: We report the case a patient with NMO, who was treated with rituximab and presented CD20+ T cells by flow cytometry after treatment, later diagnosed with lung B-cell lymphoma. CONCLUSION: This is the first report of CD20+ T cell detection in an NMO patient. We found that CD20+ T cells recovered faster than B cells after rituximab treatment and that CD20+ T cells seemed to play a role in suppressing tumor growth and memory T cell activity.

Coiled-coil domain containing 50-V2 protein positively regulates neurite outgrowth.

The coiled-coil domain containing 50 (CCDC50) protein is a phosphotyrosine-dependent signalling protein stimulated by epidermal growth factor. It is highly expressed in neuronal cells in the central nervous system; however, the roles of CCDC50 in neuronal development are largely unknown. In this study, we showed that the depletion of CCDC50-V2 impeded the neuronal development process, including arbor formation, spine density development, and axonal outgrowth, in primary neurons. Mechanistic studies revealed that CCDC50-V2 positively regulated the nerve growth factor receptor, while it downregulated the epidermal growth factor receptor pathway. Importantly, JNK/c-Jun activation was found to be induced by the CCDC50-V2 overexpression, in which the interaction between CCDC50-V2 and JNK2 was also observed. Overall, the present study demonstrates a novel mechanism of CCDC50 function in neuronal development and provides new insight into the link between CCDC50 function and the aetiology of neurological disorders.

Maximum Decompressive Hemicraniectomy for Patients with Malignant Hemispheric Infarction.

OBJECTIVE: The authors applied maximum external decompression for malignant hemispheric infarction and investigated the functional outcome according to the patient age. METHODS: Twenty-five patients with malignant hemispheric infarction were treated using a hemicraniectomy with maximum external decompression, comprising a larger (>14cm) hemicraniectomy, resection of the temporalis muscle and its fascia, spaciously expansive duraplasty, and approximation of the skin flap. The medical and diagnostic imaging records for the patients were reviewed, and 1-year functional outcome data obtained for the younger group (aged ≤ 60 years) and elderly group (aged > 60 years). RESULTS: The patients (n=25) who underwent maximum surgical decompression revealed a minimal mortality rate (n=2, 8.0%). The patients (n=14) in the younger group all survived with mRS scores of 2 (n=1, 7.1%), 3 (n=7, 50.0%), 4 (n=3, 21.4%), or 5 (n=3, 21.4%). A majority of the younger patients (57.1% with mRS ≤3) lived with functional independence. When the 1-year mRS scores were dichotomized between favorable (mRS ≤3) and unfavorable (mRS ≥4) outcomes, the younger group had significantly more patients with a favorable outcome than the elderly group (57.1% versus 9.1%, p=0.033). In contrast, in the elderly group, most patients showed unfavorable outcomes with the mRS scores of 4 (n=5, 45.5%), 5 (n=3, 27.3%), or 6 (n=2, 18.2%), whereas only one patient showed favorable outcome (mRS 3). A majority of the elderly patients (45.5% with mRS 4) survived with moderately severe disability. CONCLUSION: For malignant hemispheric infarction, a hemicraniectomy with maximum external decompression was found to considerably increase survival with a favorable outcome in functional independence (mRS ≤3) for younger patients aged ≤60 years. It can be optimal surgical treatment for younger patients.

Cause of acquired onset of diplopia due to isolated third, fourth, and sixth cranial nerve palsies in patients aged 20 to 50 years in Korea: A high resolution magnetic resonance imaging study.

AIMS: This study aimed to describe the etiologies of acquired onset of diplopia due to isolated third, fourth, and sixth cranial nerve palsies in young adults in Korea. METHODS: This retrospective study included 127 patients aged 20 to 50 years with acquired onset isolated third, fourth, and sixth cranial nerve palsies who received care at the Strabismus and Neuro-ophthalmology Department of Samsung Medical Center from 2013 to 2017. The etiologies of the palsies determined by clinical assessment, high-resolution magnetic resonance imaging (MRI) with three-dimensional constructive interference in steady state, and laboratory testing were analyzed. RESULTS: Fifty-nine patients manifested sixth cranial nerve palsy. Forty-six patients had fourth cranial nerve palsy and 22 patients had third cranial nerve palsy. The most common etiologies of the ocular motor nerve palsies were presumed inflammatory lesions (21.3%), followed by presumed microvascular causes (17.3%), and neoplasms involving the central nervous system (15.7%). Neoplasms were the most common cause of sixth cranial nerve palsy (25.4%). The most common cause of fourth cranial nerve palsy was presumed microvascular ischemia (28.3%), and presumed inflammatory lesions was the most common cause of third cranial nerve palsy (36.4%). Other non-traumatic causes included vascular lesions, ischemic brainstem stroke, intracranial hemorrhage, non-aneurysmal neuro-vascular contact, multiple sclerosis, and infection. CONCLUSION: A substantial proportion of young adult patients with ocular motor nerve palsies manifested pathologies other than presumed microvascular ischemia or idiopathic causes. Neuroimaging and laboratory tests have important roles in the evaluation of patients aged 20-50 years with acquired ocular motor nerve palsies.

Mirroring with Indocyanine Green Angiography in Aneurysm Surgery: Technical Note and Case Presentations.

OBJECTIVE: The authors used a micromirror under a microscope with an indocyanine green (ICG) imaging system to assess clipped aneurysms and the blood flow in hidden regions during aneurysm surgery. This study then investigated the usefulness of such mirroring with ICG angiography (MICGA). METHODS: A micromirror was used during aneurysm surgery on 25 patients, and MICGA was performed on 10 of these 25 patients to inspect the hidden region after clipping. The mirrored aneurysms were located at the posterior communicating artery (n = 4), anterior choroidal artery (n = 4), proximal A1 segment (n = 1), and middle cerebral artery (n = 1). RESULTS: In all 10 cases, MICGA was successful in assessing the state of the clipped aneurysm and blood flow of the vessels in the hidden region after clipping. This led to clip repositioning in 3 patients (30.0%) because of incomplete clipping of a hidden aneurysm or occlusion of a hidden perforator. Complete occlusion of the aneurysm was achieved in 8 patients, and the other 2 patients showed near complete occlusion because of an intentional residual aneurysm to avoid a small vessel adherent to the posterior wall of the aneurysm base. CONCLUSIONS: MICGA can provide useful and reliable information on the state of a clipped aneurysm and the blood flow of associated vessels and perforators in a hidden region after aneurysm clipping.

Idiopathic third and sixth cranial nerve neuritis.

PURPOSE: To present cases with idiopathic third and sixth cranial nerve neuritis. STUDY DESIGN: Retrospective observational study METHODS: The results of high resolution pre- and post- cranial nerve magnetic resonance images (MRI) with three-dimensional sequences for visualizing cranial nerves in patients with third, fourth, and sixth cranial nerve palsies who were treated at the Neuro-ophthalmology Department of Samsung Medical Center were reviewed. Patients with cranial nerve enhancement confirmed by experienced radiologists were identified. The medical records of these patients were reviewed, and their demographics, clinical presentations, laboratory results, and clinical outcomes were analyzed. RESULTS: Of 265 patients with third, fourth, and sixth cranial nerve palsy, 60 were identified by high resolution MRI as having enhancement of the corresponding cranial nerve. Among these, 17 patients with infiltrative, granulomatous, or tumorous lesions were excluded. In addition, 28 patients with identifiable causes of cranial nerve palsy, such as Miller-fisher syndrome, virus infection, or radiation-induced neuropathy, as well as patients with vasculopathic risk factors, were also excluded. Ultimately, a total of 15 patients with idiopathic third and sixth cranial nerve neuritis were included in this study. The mean age of these patients was 43 ± 15 years. Eight patients had sixth cranial nerve palsy, six third cranial nerve palsy (two partial and four complete), and one patient with complete third and sixth cranial nerve palsy. Nine patients received steroid treatment. Eleven patients recovered fully within a period ranging from a few days to one year. Two patients were much improved up to 1 month after initial presentation, but were then ultimately lost to follow-up. Another patient was lost to follow-up after the initial work-up. The other patient lost to follow-up had partially recovered during the first 6 months. CONCLUSIONS: We present patients with idiopathic third and sixth cranial nerve neuritis. They tended to respond well to steroid treatment and to have good prognoses. In order to better understand the long-term prognosis of cranial nerve neuritis and possible association with other neurologic disorders, a larger scale and longer-term study is needed.

Acquired onset of third, fourth, and sixth cranial nerve palsies in children and adolescents.

PURPOSE: To describe the causes of third, fourth, and sixth cranial nerve palsies in children and adolescents. METHODS: In this retrospective case series, a total of 66 patients aged 0-19 years with third, fourth, and sixth cranial nerve palsies seen in strabismus and neuro-ophthalmic practice from 2010 to 2017 were included. Causes of palsies were determined based on clinical assessment, high-resolution magnetic resonance imaging (MRI), and laboratory work-up. RESULTS: Thirty-five patients had sixth cranial nerve palsy, 14 patients had third cranial nerve palsy (7 partial, 7 complete), 13 patients had fourth cranial nerve palsy, and 4 patients had combined cranial nerve palsies in this study. Neoplasia involving central nervous system was one of the most common causes of third, fourth, and sixth cranial nerve palsies both in children (age: 0-14 years) and adolescents (age: 15-19 years) (20% and 31%, respectively). Overall, neoplasia (23%) was the most common cause of acute third, fourth, and sixth cranial nerve palsies, followed by idiopathic cause (14%), inflammation (11%), and non-aneurysmal vascular contact (11%). Neoplasia was also the most common cause of sixth and third cranial nerve palsies (25% and 29%, respectively). The most common cause of fourth cranial nerve palsy was late decompensation in congenital fourth cranial nerve palsy (46%). CONCLUSIONS: A substantial proportion of pediatric and juvenile patients had serious pathologies for third, fourth, and sixth cranial nerve palsies. If nerve palsies are indicated, prompt diagnosis of etiologies using high-resolution MRI with contrast and laboratory work-up are important for this disease population.

Characteristics of South Korean Patients with Hereditary Transthyretin Amyloidosis.

BACKGROUND AND PURPOSE: This retrospective cross-sectional study included 18 patients from unrelated families harboring mutations of the transthyretin gene (TTR), and analyzed their characteristics and geographical distribution in South Korea. METHODS: The included patients had a diagnosis of systemic amyloidosis, clinical symptoms, such as amyloid neuropathy or cardiomyopathy, and confirmation of a TTR gene mutation using genetic analysis recorded between April 1995 and November 2014. RESULTS: The mean age at disease onset was 49.6 years, and the mean disease duration from symptom onset to diagnosis was 3.67 years. Fifteen of the 18 patients were classified as mixed phenotype, 2 as the neurological phenotype, and only 1 patient as the cardiac phenotype. The most-common mutation pattern in South Korea was Asp38Ala, which was detected in eight patients. Thirteen patients reported their family hometowns, and five of the eight harboring the Asp38Ala mutation were from the Gyeongsang province in southeast Korea. The other eight patients exhibited a widespread geographical distribution. A particularly noteworthy finding was that the valine at position 30 (Val30Met) mutation, which was previously reported as the most-common TTR mutation worldwide and also the most common in the Japanese population, was not detected in the present South Korean patients. CONCLUSIONS: South Korean patients with hereditary TTR amyloidosis exhibited heterogeneous TTR genotypes and clinical phenotypes. The findings of this study suggest that the distribution of TTR amyloidosis in South Korea is due to de novo mutations and/or related to the other countries in East Asia.

Missense mutation of SPAST protein (I344K) results in loss of ATPase activity and prolonged the half-life, implicated in autosomal dominant hereditary spastic paraplegia.

The spastin protein (SPAST) contains an ATPase with diverse cellular activities (AAA) domain and regulates microtubule dynamics. Missense mutations of the SPAST gene are frequently detected in patients with hereditary spastic paraplegias (HSPs) and represent the main reason of loss of SPAST function; however, the pathogenicity of mutant SPAST is heterogeneous. Here, SPAST variant with an I344K mutation (I344K-SPAST) was identified in a Korean family with autosomal dominant-type HSP. We investigated the role of the I344K-SPAST in HSP to provide a therapeutic mechanism. The I344K-SPAST mutation prolonged the half-life of the protein compared to wild-type SPAST (WT-SPAST) in cells by modulating post-translational modifications for proteasomal degradation. I344K-SPAST was localized in microtubule but defective in microtubule severing and ATPase activity compared to WT-SPAST in vitro and in cells. Mutant M87 isoform harboring the same mutation with I344K-M1 SPAST also increased protein stability and loss of MT severing activity, but the pathogenicity was not stronger than I344K-M1 SPAST in neurite outgrowth. Overexpression of I344K-SPAST resulted in microtubule accumulation following inhibited neurite growth in neuroblastoma, neural progenitor cells and mouse primary cortical neurons. Conversely, these pathogenic effects of I344K-SPAST were reduced by overexpression of WT-M1 SPAST in a dose dependent manner since WT-SPAST could interact with I344K-SPAST. Our data therefore provide proof-of-concept that gene transfer of WT-M1 SPAST may serve as a valid therapeutic option for HSPs.

Age-Dependent Attitudes of Ischemic Patients Toward Disability After Decompressive Hemicraniectomy for Malignant Middle Cerebral Artery Infarction.

OBJECTIVE: To investigate patient attitudes toward acceptable levels of disability after decompressive surgery for malignant middle cerebral artery infarction, including impact of patient age, to improve process of surgical informed consent. METHODS: Patients who had experienced a recent minor stroke were asked to complete a questionnaire containing 2 parts: demographic information, including patient age, sex, years of education, working status, religion, and economic status, and acceptable level of disability based on modified Rankin Scale (mRS) with corresponding illustrations to explain mRS levels. RESULTS: Patient age was identified as an independent determinant of the worst acceptable mRS score with a negative correlation. For nondominant hemispheric malignant infarction, the worst acceptable mRS score was significantly lower (mean ± SD 2.0 ± 1.3) for the oldest patients (>70 years old) compared with patients <60 years old (mean ± SD 3.0 ± 0.6) and 60-70 years old (mean ± SD 3.0 ± 0.8). For dominant hemispheric malignant infarction with language impairment, all age groups showed a significantly lower value for worst acceptable mRS score (mean ± SD 1.8 ± 1.1 for patients <60 years old, 1.8 ± 1.2 for patients 60-70 years old, and 1.0 ± 1.4 for patients >70 years old). CONCLUSIONS: Patients showed different attitudes toward disability according to their age. Patients >70 years old showed the lowest worst acceptable mRS score after surgical treatment of malignant infarction. Language impairment with dominant hemispheric infarction further decreased the worst acceptable mRS score.

The IL-10-producing regulatory B cells (B10 cells) and regulatory T cell subsets in neuromyelitis optica spectrum disorder.

B cells contribute to the pathogenesis of neuromyelitis optica (NMO) by producing Aquaporin 4-specific autoantibodies (AQP4-ab); on the other hand, there are certain B cells that suppress immune responses by producing regulatory cytokines, such as IL-10. In this study, we investigated the presence of IL-10-producing Breg cells among lymphocyte subsets. Twenty-two seropositive NMO spectrum disorder (NMOSD) patients (29 samples) and 13 healthy controls (HCs) (14 samples) were enrolled. All NMOSD patients have received one or more immunosuppressive drugs. The phenotype and frequency of B cell and T cell subsets in the peripheral blood were measured by flow cytometry. We defined Breg cells as IL-10-producing B (B10) cells, which are CD19+CD39+CD1d+IL-10+. The potential relations were evaluated between specific lymphocyte subsets and AQP4-ab intensity measured by the cell-based indirect immunofluorescence assay. The frequency of B10 cells was higher in patients with NMOSD regardless of the disease status than that in HCs (attack samples; p = 0.009 and remission samples; p < 0.001, respectively). In addition, the frequency of IL-17+ Treg cells among Treg cells was higher during remission than during an attack (uncorrected p = 0.032). Among the lymphocyte subsets, B10 cells alone showed a positive correlation with the intensity of AQP4-ab positivity (ρ [rho] = 0.402 and p = 0.031). It was suggested that the suppressive subsets including B10 and IL-17+ Treg cells might have important roles in controlling disease status in NMOSD. Further functional studies may help to elucidate the immunological role of B10 and IL-17+ Treg cells in NMOSD.

Physician- and self-assessed myasthenia gravis activities of daily living score.

INTRODUCTION: The Myasthenia Gravis-Activities of Daily Living (MG-ADL) profile scale is a simple-to-use instrument. We aimed to validate this scale in the Korean language and compare physician- and self-assessed MG-ADL scores (pMG-ADL-K and sMG-ADL-K). METHODS: pMG-ADL-K and sMG-ADL-K and MG Composite (MGC) scores were obtained from patients. The correlation between pMG-ADL-K and MGC and the relationship between the pMG-ADL-K and sMG-ADL-K were assessed using the Cronbach α and the Spearman coefficient. By intraclass correlation coefficient (ICC), the reliability of each sub-item of pMG-ADL-K and sMG-ADL-K was evaluated. RESULTS: We included data from 40 patients. The pMG-ADL-K score showed a strong correlation with the MGC score (rho = 0.80, P < 0.01). The Cronbach α was 0.98 between pMG-ADL-K and sMG-ADL-K, and sub-items showed good consistency (ICC 0.684-0.985, P < 0.001). DISCUSSION: The MG-ADL-K is a valid tool and the sMG-ADL-K shows excellent correlation with pMG-ADL-K. Both the pMG-ADL-K and sMG-ADL-K can be used to measure MG severity. Muscle Nerve 57: 419-422, 2018.