{"title":"[Symptomatic narcolepsy type 2 in a patient with AQP4-positive neuromyelitis optica spectrum disorder].","authors":"Takashi Yoshimura, Masakazu Wakai, Takashi Kanbayashi, Masahisa Katsuno","doi":"10.5692/clinicalneurol.cn-002048","DOIUrl":"10.5692/clinicalneurol.cn-002048","url":null,"abstract":"<p><p>A 63-year-old woman, previously diagnosed with multiple sclerosis (MS) and treated with IFNβ-1b, developed asymptomatic lesion expansion surrounding the thalamus and basal ganglia. However, an AQP4 autoantibody ELISA was negative. The patient was subsequently hospitalized due to drowsiness, lethargy, and difficulty in managing housework. During lumbar puncture, the patient lost consciousness. Electroencephalography (EEG) revealed rapid eye movements, and a multiple sleep latency test (MSLT) indicated narcolepsy. Cerebrospinal fluid (CSF) orexin levels were normal, and no cataplexy was observed. The patient was thus diagnosed with narcolepsy type 2. The patient tested positive for AQP4 antibody, and was diagnosed with neuromyelitis optica spectrum disorder (NMOSD). Sleep physiology testing is crucial because some patients with NMOSD do not exhibit hypothalamic lesions or decreased CSF orexin levels.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"278-283"},"PeriodicalIF":0.0,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143732207","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Clinical NeurologyPub Date : 2025-04-25Epub Date: 2025-04-11DOI: 10.5692/clinicalneurol.cn-002030
Mari Fukuda, Kazutoshi Ikeda, Kentaro Yamakawa, Yohei Takenobu, Akihiko Ozaki
{"title":"[Rapidly progressive muscle weakness in anti-myelin associated glycoprotein (MAG) neuropathy successfully treated with intravenous immunoglobulin].","authors":"Mari Fukuda, Kazutoshi Ikeda, Kentaro Yamakawa, Yohei Takenobu, Akihiko Ozaki","doi":"10.5692/clinicalneurol.cn-002030","DOIUrl":"10.5692/clinicalneurol.cn-002030","url":null,"abstract":"<p><p>Anti-myelin associated glycoprotein (MAG) neuropathy typically progresses slowly, but rare cases exhibit rapid deterioration. We report an 83-year-old man with a two-year history of paresthesia in both feet and recent-onset gait ataxia who developed rapidly progressive muscle weakness in all four limbs over several days. Nerve conduction studies and positive anti-MAG antibodies confirmed the diagnosis of anti-MAG neuropathy. The patient's muscle weakness improved with intravenous immunoglobulin (IVIg) therapy. This case highlights the existence of atypical anti-MAG neuropathy mimicking chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with a rapid deterioration course and suggests the potential efficacy of IVIg in such presentations.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"267-272"},"PeriodicalIF":0.0,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143732203","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[Is neurofilament light chain useful as a disease progression marker for ATTRv amyloidosis? A literature review].","authors":"Yoshiki Sekijima, Mitsuharu Ueda, Kentaro Takahashi, Hiroaki Kitaoka","doi":"10.5692/clinicalneurol.cn-002063","DOIUrl":"10.5692/clinicalneurol.cn-002063","url":null,"abstract":"<p><p>ATTRv amyloidosis is an autosomal-dominant disorder characterized by mutations in the transthyretin (TTR) gene, systemic deposition of transthyretin amyloid fibrils, and progressive polyneuropathy. Current scoring systems developed for ATTRv amyloidosis to measure the severity of polyneuropathy are not sufficiently sensitive or are difficult to implement in daily practice. Results of phase 3 trials for oligonucleotide therapeutics and real-world evidence have shown that neurofilament light chain (NfL), a key structural component of axons, is a reliable blood biomarker for assessing disease progression and treatment response in patients with ATTRv amyloidosis with polyneuropathy. Because blood NfL levels can be affected by factors such as age, body mass index (BMI), and renal function, its significance in patient monitoring needs to be assessed carefully while considering the clinical characteristics of each patient.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"251-259"},"PeriodicalIF":0.0,"publicationDate":"2025-04-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143701831","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Clinical NeurologyPub Date : 2025-03-27Epub Date: 2025-02-22DOI: 10.5692/clinicalneurol.cn-002047
Yasuko Mori, Yoya Ono, Takayoshi Shimohata
{"title":"[Varicella zoster infection as a risk factor for dementia: a scoping review].","authors":"Yasuko Mori, Yoya Ono, Takayoshi Shimohata","doi":"10.5692/clinicalneurol.cn-002047","DOIUrl":"10.5692/clinicalneurol.cn-002047","url":null,"abstract":"<p><p>A scoping review analyzing 21 selected publications was conducted to determine \"whether the varicella zoster virus (VZV) is a risk factor for dementia\". One meta-analysis concluded that VZV infection increases dementia risk, while another meta-analysis contradicts this finding. Several reports have shown an increased risk of dementia associated with VZV infections of the eyes and central nervous system. Additionally, a third meta-analysis reported that VZV vaccination reduces dementia. Therefore, VZV infection may be a risk factor for dementia. However, most of the reviewed articles were retrospective cohort studies, which limits the strength of conclusions that can be drawn. To provide more robust evidence, prospective cohort studies and clinical trials are needed to evaluate the impact of VZV itself, as well as the effectiveness of vaccines and antiviral therapies.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"191-196"},"PeriodicalIF":0.0,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143494039","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[A case of possible multifocal motor neuropathy with suspected posterior interosseous nerve palsy].","authors":"Koichi Tanaka, Akihiro Kitamura, Ryutaro Nakamura, Isamu Yamakawa, Tomoya Terashima, Makoto Urushitani","doi":"10.5692/clinicalneurol.cn-002053","DOIUrl":"10.5692/clinicalneurol.cn-002053","url":null,"abstract":"<p><p>We present the case of a 33-year-old man with progressive weakness in the left wrist and finger extensors. Initially, posterior interosseous nerve (PIN) palsy was suspected. However, nerve conduction studies detected conduction block and ultrasonography demonstrated swelling between the elbow and spiral groove in the left radial nerve. Anti-GM1 IgM antibodies were positive, leading to a diagnosis of possible multifocal motor neuropathy (MMN). Although MMN usually represents multifocal pure motor neuropathies, the involvement of a single nerve allows for diagnosis of possible MMN. PIN palsy of indeterminate etiology should prompt consideration, underscoring the utility of ultrasonography in the diagnostic process.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"230-235"},"PeriodicalIF":0.0,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143494028","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Undiagnosed headaches in the emergency department: clinical characteristics and outcomes.","authors":"Ryo Sasaki, Yoshiaki Takahashi, Mizuki Morimoto, Kazuhiro Sasaki, Nobutoshi Morimoto","doi":"10.5692/clinicalneurol.cn-002052","DOIUrl":"10.5692/clinicalneurol.cn-002052","url":null,"abstract":"<p><p>Headaches are a common complaint in the emergency department (ED). Understanding the characteristics and outcomes of headaches, especially in undiagnosed patients, is important for improving headache care in the ED. We conducted a retrospective study of 171 headache patients at the ED of Kagawa Prefectural Central Hospital, with a follow-up for the primary and undiagnosed headache groups via telephone to assess long-term outcomes. Primary, secondary, and undiagnosed headaches accounted for 15.2%, 58.4%, and 26.3% of cases, respectively. All life-threatening secondary headaches were successfully excluded with imaging tests performed on 73.7% of cases, despite the low rate of treatment in the ED (18.1%). Among the undiagnosed headache cases, the recurrence of severe headaches was low (11.9%), though awareness of chronic headaches was high (47.6%). Emergency physicians should be aware of the possibility that patients at an ED presenting an undiagnosed headache may include chronic headache patients. Seamless collaboration between the ED and headache specialists is needed to manage undiagnosed headaches in the ED.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"197-202"},"PeriodicalIF":0.0,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143494042","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"[A case of anti-synthetase syndrome: negative ELISA/immunoblot, but positive RNA immunoprecipitation with multiple antibodies].","authors":"Saya Ikeda, Takahiro Shimizu, Tadashi Adachi, Shigeakai Suzuki, Ichizo Nishino, Ritsuko Hanajima","doi":"10.5692/clinicalneurol.cn-002009","DOIUrl":"10.5692/clinicalneurol.cn-002009","url":null,"abstract":"<p><p>A 68-year-old woman presented with a one-month history of polyarthritis and fever, followed by myalgia and muscle weakness involving the proximal limbs, neck, trunk, and distal upper extremities. Myositis was suspected based on an elevated serum CK level. Chest CT revealed interstitial pneumonia. Commercial laboratory tests, including ELISA and immunoblot, were positive for anti-Ro-52 antibodies but negative for anti-aminoacyl transfer RNA synthetase (ARS) antibodies. However, muscle pathology revealed perifascicular necrosis and perimysial pathology, strongly suggesting anti-synthetase syndrome (ASS). Further investigation using RNA immunoprecipitation (RIP) assay identified positive anti-EJ antibodies, leading to the diagnosis of ASS. In addition, anti-Ku and anti-U1 RNP antibodies were co-positive. These findings highlight that, even when ELISA and immunoblot were negative for specific antibodies, RIP assay is necessary when ASS is suspected based on myopathologic findings.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"211-217"},"PeriodicalIF":0.0,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143516932","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Clinical NeurologyPub Date : 2025-03-27Epub Date: 2025-02-22DOI: 10.5692/clinicalneurol.cn-002058
Yoko Tsuboyama, Akihiko Mitsutake, Yuto Sakai, Nobue K Iwata
{"title":"[A case of meningitis due to anti-centromere antibody-positive Sjögren syndrome].","authors":"Yoko Tsuboyama, Akihiko Mitsutake, Yuto Sakai, Nobue K Iwata","doi":"10.5692/clinicalneurol.cn-002058","DOIUrl":"10.5692/clinicalneurol.cn-002058","url":null,"abstract":"<p><p>A 79-year-old woman was diagnosed with Sjögren's syndrome (SjS) at the age of 73 years by lip biopsy, gum test, and salivary gland scintigraphy with positive antinuclear (ANA), positive anti-centromere (ACA), and negative anti-Ro/SS-A antibodies. Seven days before admission, the patient developed gait disturbance, which progressed to difficulty in walking two days before admission. She was hospitalized because of subacute gait disturbance. Neurological examination showed gait disturbances characterized by small steps, wide-based gait, left upper limb clumsiness, and frontal lobe dysfunction. Laboratory tests confirmed positive ANA and ACA and negative anti-Ro/SS-A and anti-La/SS-B antibodies. Cerebrospinal fluid analysis showed mildly elevated protein levels and increased monocyte count. Brain MRI showed hyperintensity in the bilateral frontal regions on fluid-attenuated inversion recovery (FLAIR)/contrast-enhanced T<sub>1</sub>-weighted imaging, and N-isopropyl-p-<sup>123</sup>I-iodoamphetamine single-photon emission computed tomography (<sup>123</sup>I-IMP SPECT) showed decreased accumulation in the frontal region. After excluding cancerous, infectious, and immune-mediated causes, ACA-single-positive SjS-related meningitis was diagnosed. The patient responded well to steroid therapy. The patient was able to walk short distances and showed improvement in frontal lobe function. Follow-up MRI and <sup>123</sup>I-IMP SPECT demonstrated the resolution of previous abnormalities. Knowingly, there have been no reported cases of SjS-related meningitis with positive ACA. Considering that anti-Ro/SS-A antibodies are involved in the pathogenesis of meningitis, this case is extremely rare. We propose that ACA-positive SjS be considered in the differential diagnosis of aseptic meningitis.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"236-239"},"PeriodicalIF":0.0,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143494023","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Clinical NeurologyPub Date : 2025-03-27Epub Date: 2025-02-27DOI: 10.5692/clinicalneurol.cn-002054
Yasumasa Yamamoto, Yoshinari Nagakane
{"title":"[Identification of potential embolic source and consideration for ischemic lesion formation using the consecutive patient registry of acute embolic stroke].","authors":"Yasumasa Yamamoto, Yoshinari Nagakane","doi":"10.5692/clinicalneurol.cn-002054","DOIUrl":"10.5692/clinicalneurol.cn-002054","url":null,"abstract":"<p><p>We investigated the 992 consecutive patients with acute embolic stroke and identified potential embolic sources (PES) for 88.3% applying transesophageal echocardiography (TEE). We classified patients into 3 groups, the Group A (n = 560): PES were identified after general examinations, the Group B (n = 366): PES were identified after TEE or long-term EEG monitoring and the Group C (n = 66): TEE could not be performed. In Group A, continuous atrial fibrillation (cAf) was most prevalent (n = 464, 82.8%), succeeded by myocardial infarction (n = 39, 6.9%) and Trousseau syndrome (n = 27, 4.8%), etc. In Group B, paroxysmal atrial fibrillation (pAf) was most prevalent (n = 168, 45.9%), succeeded by paradoxical embolism (n = 77, 21.0%) and aorto-embolism (n = 77, 19.3%). We investigated the association of topographic diffusion-weighted imaging patterns (DWI) with PES. DWI was determined based on the arterial supply. Middle cerebral arteries were particularly divided into 4 segments, i.e., M1~M4. Moreover, M2 segments were subdivided into superior and inferior branches. The infarcts of larger caliber arteries such as internal carotid artery, M1 and M2, and multiple cortical branches were mostly associated with atrial fibrillation including cAf and pAf. Striatocapsular infarction were significantly associated with paradoxical embolism. Multiple small scattered infarcts were predominant in aorto-embolism as well as paradoxical embolism, Trousseau syndrome and thrombocytosis. The associations of DWI with different PES have their distinctive characteristics and may help predict PES in patients with embolic stroke of undetermined source. DWI pattern may be determined by the composition of thrombi and hydrodynamics of cerebral vasculature.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"203-210"},"PeriodicalIF":0.0,"publicationDate":"2025-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143516937","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}