Clinical NeurologyPub Date : 2025-08-28Epub Date: 2025-07-24DOI: 10.5692/clinicalneurol.cn-002123
Tomoki Ishimaru, Kei Oiwa, Sho Seo, Takahiro Kano, Hideki Hozen
{"title":"[A case of Streptococcus suis meningitis in which the patient suffered sensorineural hearing loss and requring long-term antibacterial treatment].","authors":"Tomoki Ishimaru, Kei Oiwa, Sho Seo, Takahiro Kano, Hideki Hozen","doi":"10.5692/clinicalneurol.cn-002123","DOIUrl":"10.5692/clinicalneurol.cn-002123","url":null,"abstract":"<p><p>A man in his 40s was transferred to our hospital due to headache, hearing loss, and altered consciousness, which developed several days after consuming a barbeque meal, including pork. Based on his clinical symptoms and various laboratory findings, he was diagnosed with bacterial meningitis caused by Streptococcus suis, and treatment was initiated with antibacterial agents and dexamethasone. After 3 weeks, his symptoms and laboratory results showed improvement, allowing for the discontinuation of the antibacterial therapy. However, a few days after stopping antibiotics, his symptoms and cerebrospinal fluid (CSF) findings worsened, prompting the resumption of antibacterial treatment. The therapy was ultimately terminated after 5 weeks, following confirmation of clinical and CSF improvement. Despite resolution of the meningitis symptoms, the patient was left with bilateral sensorineural hearing loss, predominantly affecting his right ear. Following systemic steroid administration and hyperbaric oxygen therapy, his left-sided hearing improved, but no improvement was observed in his right ear, and he continued to experience severe hearing loss.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"607-611"},"PeriodicalIF":0.0,"publicationDate":"2025-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144709299","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 : 2025-08-28Epub Date: 2025-07-24DOI: 10.5692/clinicalneurol.cn-002087
Raita Kikuchi, Kenji Ishihara, Jun'ichi Shiota, Mitsuru Kawamura, Mari Yoshida
{"title":"[An autopsy case of spinal bulbar muscular atrophy concomitant with multiple system atrophy pathology].","authors":"Raita Kikuchi, Kenji Ishihara, Jun'ichi Shiota, Mitsuru Kawamura, Mari Yoshida","doi":"10.5692/clinicalneurol.cn-002087","DOIUrl":"10.5692/clinicalneurol.cn-002087","url":null,"abstract":"<p><p>We describe an autopsy case of spinal bulbar muscular atrophy (SBMA) concomitant with multiple system atrophy (MSA). A Japanese male patient developed gait disturbance in his twenties. His brother and niece also presented with similar clinical symptoms. His condition gradually worsened, and he became immobile at the age of 50 years. Genetic analysis revealed the expansion of CAG repeats of the SBMA gene. At 63 years of age, cerebellar ataxia symptoms emerged. Magnetic resonance images of the head showed a \"hot cross bun sign\" at the pontine basis and bilateral atrophy of the middle cerebellar peduncles and cerebellar hemispheres, suggesting MSA. He died of pneumonia at the age of 65 years, with a clinical illness of approximately 40 years. The neuropathological diagnosis was consistent with both SBMA and MSA. Neurons of the spinal anterior horn and brainstem motor nuclei were diminished. 1C2 (polyglutamine) immunoreactive intranuclear and intracytoplasmic inclusions were observed in the neurons in the substantia nigra, brainstem tegmentum, pontine nuclei, spinal anterior horn cells and Onuf's nucleus. These findings were suggestive of SBMA. Meanwhile, neurons of the inferior olivary nuclei, pontine nuclei, and Purkinje cells were nearly completely lost. The cerebellar white matter, pontine basis, and middle cerebellar peduncles showed a prominent loss of fibers. α-synuclein positive glial cytoplasmic inclusions were observed in widespread areas. These findings were suggestive of MSA. To the best of our knowledge, another case of SBMA accompanying MSA, similar to the present case, have been reported to date. Moreover, several cases of pathologically proven amyotrophic lateral sclerosis and MSA have been reported. The development of molecular biological techniques and accumulation of pathologically diagnosed patients may reveal common pathological mechanisms in SBMA and MSA.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"588-594"},"PeriodicalIF":0.0,"publicationDate":"2025-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144709302","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 treatment-related leukoencephalopathy during combination therapy with daratumumab, lenalidomide, and low-dose dexamethasone for multiple myeloma].","authors":"Keiko Maezono-Kandori, Hiroki Suo, Naoki Tokuda, Atsushi Yamamoto, Shiori Ogura, Yoshinari Nagakane","doi":"10.5692/clinicalneurol.cn-002069","DOIUrl":"10.5692/clinicalneurol.cn-002069","url":null,"abstract":"<p><p>A 75-year-old woman with multiple myeloma was admitted due to an acute disturbance of consciousness and aphasia two months after administration of daratumumab, lenalidomide and dexamethasone combination therapy (DLd therapy). Brain MRI on admission showed no acute ischemic changes. She was treated with antiepileptic drugs, and DLd therapy was discontinued, but her consciousness deteriorated, and follow-up brain MRI showed progressive FLAIR high-signal intensity lesions in the cerebral deep white matter bilaterally. Though methylprednisolone pulse therapy and high-dose intravenous immunoglobulin were ineffective, plasma exchange improved her consciousness, and she began to speak. In addition, her MRI findings improved. Progressive multifocal leukoencephalopathy induced by drugs for multiple myeloma has been reported, but, in this case, leukoencephalopathy associated with daratumumab was suspected because JC virus DNA was not detected in her cerebrospinal fluid.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"573-577"},"PeriodicalIF":0.0,"publicationDate":"2025-08-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144709300","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 : 2025-07-25Epub Date: 2025-06-21DOI: 10.5692/clinicalneurol.cn-002064
Minori Sawada, Gaku Okumura, Ken Takasone, Nagaaki Katoh, Yoshiki Sekijima
{"title":"[A case of POEMS syndrome presenting craniocervical vascular stenosis].","authors":"Minori Sawada, Gaku Okumura, Ken Takasone, Nagaaki Katoh, Yoshiki Sekijima","doi":"10.5692/clinicalneurol.cn-002064","DOIUrl":"10.5692/clinicalneurol.cn-002064","url":null,"abstract":"<p><p>A 54-years-old Japanese man visited local hospital with six-month history of progressive numbness and muscle weakness in his lower limbs. He was diagnosed with POEMS syndrome based on positive serum M-protein, elevated serum VEGF, and splenomegaly. MRI showed multiple cerebral infarcts in the watershed area, and MRA showed complete vascular occlusion of the right internal carotid artery and severe vascular stenosis of the left common carotid artery, suggesting hemodynamic cerebral infarction. He was referred to our department and treated with three courses of Daratumumab-Bortezomib-Dexamethasone (DBd) therapy. After normalization of VEGF was confirmed, right superficial temporal artery-middle cerebral bypass surgery was performed. He has been kept in good condition with improved muscle strength and walking ability, and normalized VEGF level under the maintenance Daratumumab-Lenalidomide-Dexamethasone (DLd) therapy. Because POEMS syndrome-associated vasculopathy can develop even in the very early stage of the disease, intensive evaluation of craniocervical vessels before treatment initiation is very important for treatment selection and risk assessment.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"511-515"},"PeriodicalIF":0.0,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144369324","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 : 2025-07-25Epub Date: 2025-06-21DOI: 10.5692/clinicalneurol.cn-002115
Fujio Umehara
{"title":"[Remarkable improvement of metastatic occipital condyle syndrome in a patient with lung adenocaricinoma treated with osimertinib].","authors":"Fujio Umehara","doi":"10.5692/clinicalneurol.cn-002115","DOIUrl":"10.5692/clinicalneurol.cn-002115","url":null,"abstract":"<p><p>A woman in her 50s. Since October of X-1, she had been suffering from lower back and occipital pain. Despite undergoing medical evaluations at multiple hospitals, no discernible abnormalities were identified. As her symptoms worsened, she presented to our department in January of X, reporting severe pain in the left occipital to posterior neck that increased with both neck flexion and extension. A neurological examination revealed a left-sided deviation of the tongue (left hypoglossal nerve paralysis). A head MRI revealed signal abnormalities in the left occipital condyle and multiple nodules with contrast effects in the brain parenchyma. A computed tomography (CT) scan revealed a mass in the left lung, multiple intrahepatic masses, and bone destruction in the spine and left occipital condyle. In light of these findings, a diagnosis of lung cancer with metastasis to multiple organs and occipital condyle syndrome due to metastasis to the left occipital condyle was suspected. Subsequent cytological analysis of bronchoalveolar lavage fluid and liver biopsy substantiated the diagnosis of adenocaricinoma. The subsequent administration of osimertinib, an epidermal growth factor receptor (EGFR) tyrosine kinase inhibitor, led to a reduction in the size of the tumor, as well as improvements in the hypoglossal nerve palsy and occipital pain. It is imperative to note that occipital condyle syndrome signifies the metastasis of a malignancy to the base of the skull, necessitating meticulous observation and management.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"531-535"},"PeriodicalIF":0.0,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144369328","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 report of leptomeningeal amyloidosis presenting with meningoencephalitis-resembling manifestations in a 54-year-old woman].","authors":"Naomi Suzuki, Takao Fukushima, Ryota Kishi, Masaki Namekawa, Junsuke Shimbo, Kunihiko Wakaki, Kunihiko Makino","doi":"10.5692/clinicalneurol.cn-002066","DOIUrl":"10.5692/clinicalneurol.cn-002066","url":null,"abstract":"<p><p>We report a 54-year-old woman previously healthy who developed acute fever, headache, and impaired consciousness. We administered corticosteroid therapy as autoimmune mediated meningoencephalitis, which improved her symptoms and reduced cerebrospinal IL-6 level, but her cognitive impairment persisted. Contrast-enhanced MRI showed diffuse meningeal enhancement lesion, which led us to the possibility of leptomeningeal amyloidosis (LA). We performed transthyretin gene analysis identified heterozygosity for the c.265T>C, p.Y89H (Y69H) variant, which is known as one of the causative mutations of familial LA. Given the responsiveness to steroid treatment, it is possible that the inflammatory pathology related to amyloid deposition in leptomeninges with encephalitis-like symptoms contributed to the prolonged duration of symptoms lasting from hours to days.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"516-521"},"PeriodicalIF":0.0,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144508767","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 : 2025-07-25Epub Date: 2025-06-26DOI: 10.5692/clinicalneurol.cn-002111
Hiroyuki Ishiura
{"title":"[Molecular genetics of benign adult familial myoclonus epilepsy].","authors":"Hiroyuki Ishiura","doi":"10.5692/clinicalneurol.cn-002111","DOIUrl":"10.5692/clinicalneurol.cn-002111","url":null,"abstract":"<p><p>Benign adult familial myoclonus epilepsy (BAFME) is an autosomal dominantly inherited disease characterized by infrequent seizures and tremorous myoclonus. The disease is also called familial adult myoclonic epilepsy (FAME) or familial cortical myoclonic tremor with epilepsy (FCMTE). Although the causes of BAFME had been unknown for a long, we identified TTTCA and TTTTA repeat expansions in intron 4 of SAMD12 as a cause of BAFME type 1. We also found TTTCA and TTTTA repeat expansions in TNRC6A and RAPGEF2 also cause the disease (BAFME types 6 and 7, respectively), thus proposing a concept of repeat motif-phenotype correlation. After that, TTTCA and TTTTA repeat expansions in STARD7, MARCHF6, YEATS2, and RAI1 have been identified as causes of BAFME types 2, 3, 4, and 8. The findings further supported the concept. The involvement of RNA-mediated toxicity, particularly of UUUCA repeats, is assumed to be the pathomechanism of this disease. The next step will be understanding the molecular pathomechanism of BAFME and identifying molecular targets of more efficient therapeutic approaches.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"495-502"},"PeriodicalIF":0.0,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144508768","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 severe COVID-19 infection with multiple brain microbleeds spreading to the subcortical white matter, internal capsule and splenium of the corpus callosum].","authors":"Shoko Aburatani, Eito Miura, Yoshitaka Umeda, Shimon Aoki, Nobuya Fujita, Mutsuo Oyake","doi":"10.5692/clinicalneurol.cn-002072","DOIUrl":"10.5692/clinicalneurol.cn-002072","url":null,"abstract":"<p><p>A 23-year-old female student was admitted to a local hospital because of consciousness disturbance caused by diabetic ketoacidosis on the 4th day after COVID-19 infection. She was subsequently transferred to our hospital, having developed respiratory failure, acute renal failure and DIC. Although intensive treatment improved her condition, she remained hypoactive. Brain MRI revealed multiple microbleeds (MBs) spreading to the subcortical white matter, internal capsule, splenium of the corpus callosum and brainstem. The WAIS-IV score suggested general attention deficit disorder and mild impairment of working memory and processing speed. These symptoms disappeared with time, and she was able to return to her studies without any after-effects. COVID-19 tends to cause thrombosis and MBs in the brain due to vascular endothelial damage. Although the reason for the specific localization of these MBs remains unclear, differences in regional vulnerability to cytokines may have been partly responsible.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"522-525"},"PeriodicalIF":0.0,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144369326","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":"[Questionnaire survey regarding pediatric to adult transitional medical care targeting members of Japanese Society of Neurology].","authors":"Toshio Saito, Yoshio Sakiyama, Katsuhisa Ogata, Yoko Mochizuki, Ichiro Yabe, Hideki Mochizuki","doi":"10.5692/clinicalneurol.cn-002055","DOIUrl":"10.5692/clinicalneurol.cn-002055","url":null,"abstract":"<p><p>The Special Committee on Measures for Transition from Pediatric to Adult Health Care surveyed approximately 9,000 members of the Japanese Society of Neurology regarding transitional care. Only 744 responses were returned, less than 10% of the total number of members contacted. More than half answered that they generally provide treatment for adult patients with a childhood-onset disease, with many noting as reasons that the related diseases and ages in such cases are targeted by neurology specialists, and that other adult medical departments are not equipped to treat them. As for reasons given for not treating such patients, lack of knowledge related to developmental disorders, lack of support system, and difficulties with communication were noted. There were no noticeable differences in the responses of providing treatment for adult patients with a childhood-onset disease in association with the affiliated regional association or branch. These results indicate that resolution of issues related to neurologist unfamiliarity with pediatric neurological disorders and lack of relevant information are important issues to be addressed for establishment of a smooth medical care transition.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"503-510"},"PeriodicalIF":0.0,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144369327","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 : 2025-07-25Epub Date: 2025-06-26DOI: 10.5692/clinicalneurol.cn-002108
Toshiyuki Mizukura, Yu Sugiyama
{"title":"[A case of recurrence after more than 20 years from transverse myelitis suspected to be neuromyelitis optica spectrum disorder].","authors":"Toshiyuki Mizukura, Yu Sugiyama","doi":"10.5692/clinicalneurol.cn-002108","DOIUrl":"10.5692/clinicalneurol.cn-002108","url":null,"abstract":"<p><p>This patient was a 78-year-old woman. In year X-23, she presented with gait disturbance and sensory impairment below the chest level and was diagnosed with and treated for acute transverse myelitis. The neurological symptoms recurred in year X-22, and she was treated under suspicion of opticospinal multiple sclerosis (OSMS). Subsequently, she did not experience a relapse of neurological symptoms; however, she developed left facial paralysis, abnormal sensations in both upper limbs, and muscle weakness in the left lower limb in year X. MRI revealed lesions extending from the medulla oblongata to the C6 level, and serum testing was positive for anti-AQP4 antibodies, leading to a diagnosis of neuromyelitis optica spectrum disorder. This case was considered clinically significant as an example of neuromyelitis optica spectrum disorder recurrence after a long period.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"536-539"},"PeriodicalIF":0.0,"publicationDate":"2025-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144508766","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}