Clinical Neurology最新文献

{"title":"[Cryopyrin-associated periodic fever syndrome (CAPS) presenting as early-onset dementia, lacking typical recurrent fever or skin rash: a case report].","authors":"Tomone Taneda, Mizuki Takahashi, Maiko Umeda, Yoshitaka Umeda, Mutsuo Oyake, Ryuta Nishikomori, Nobuya Fujita","doi":"10.5692/clinicalneurol.cn-001956","DOIUrl":"10.5692/clinicalneurol.cn-001956","url":null,"abstract":"<p><p>A 54-year-old man with a university degree was admitted to our hospital because of a two-year history of progressive dementia. He had familial sensorineural hearing loss and had been treated for epilepsy since his 30s. On admission, he showed severe dementia and parkinsonism without fever or skin rash. Systemic inflammation was evident, and the CSF cell count and IL-6 level were elevated to 53/μl and 307 ‍pg/ml, respectively. Brain MRI demonstrated diffuse brain atrophy. More detailed anamnesis revealed a history of rheumatoid arthritis in childhood and aseptic meningitis in his 20s. Genetic examination for autoinflammatory diseases demonstrated compound heterozygotic mutations in the NLRP3 gene, causing cryopyrin-associated periodic fever syndrome (CAPS). This case was atypical CAPS presenting as early-onset progressive dementia, without recurrent fever or urticaria-like eruption which are usually seen in this disease.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"413-416"},"PeriodicalIF":0.0,"publicationDate":"2024-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141155357","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":"[Transient neurological deficits in a patient with chronic subdural hematoma].","authors":"Misa Ishiuchi, Yuichiro Inatomi, Rihito Yamamura, Makoto Nakajima, Toshiro Yonehara","doi":"10.5692/clinicalneurol.cn-001975","DOIUrl":"10.5692/clinicalneurol.cn-001975","url":null,"abstract":"<p><p>A 62-year-old, right-handed man was diagnosed with asymptomatic bilateral chronic subdural hematomas and underwent hematoma removal on the left side only. At 1 month after surgery, he was admitted to our hospital because he began to have one or two attacks/day of apraxia of speech and dysesthesia of the right hand with a duration of approximately 5 ‍min. The left hematoma had not re-expanded, but fluid-attenuated inversion resonance imaging showed hyperintense lesions in the sulci adjacent to the hematoma. Moreover, single-photon emission computed tomography revealed low-uptake lesions in the left cerebrum adjacent to the hematoma. Electroencephalogram showed no abnormalities, and CT angiography showed a slight deviation of the left middle cerebral arteries due to the hematoma. The attacks disappeared within 10 days, although the volume of the hematoma was unchanged. It was suggested that his transient neurological deficits were caused by cerebral ischemia related to chronic subdural hematoma.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"422-426"},"PeriodicalIF":0.0,"publicationDate":"2024-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141175740","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":"[Dynamic activity model of movement disorders: a unified view to understand their pathophysiology].","authors":"Atsushi Nambu, Satomi Chiken, Hiromi Sano, Nobuhiko Hatanaka, José A Obeso","doi":"10.5692/clinicalneurol.cn-001957","DOIUrl":"10.5692/clinicalneurol.cn-001957","url":null,"abstract":"<p><p>Malfunction of the basal ganglia leads to movement disorders such as Parkinson's disease, dystonia, Huntington's disease, dyskinesia, and hemiballism, but their underlying pathophysiology is still subject to debate. To understand their pathophysiology in a unified manner, we propose the \"dynamic activity model\", on the basis of alterations of cortically induced responses in individual nuclei of the basal ganglia. In the normal state, electric stimulation in the motor cortex, mimicking cortical activity during initiation of voluntary movements, evokes a triphasic response consisting of early excitation, inhibition, and late excitation in the output stations of the basal ganglia of monkeys, rodents, and humans. Among three components, cortically induced inhibition, which is mediated by the direct pathway, releases an appropriate movement at an appropriate time by disinhibiting thalamic and cortical activity, whereas early and late excitation, which is mediated by the hyperdirect and indirect pathways, resets on-going cortical activity and stops movements, respectively. Cortically induced triphasic response patterns are systematically altered in various movement disorder models and could well explain the pathophysiology of their motor symptoms. In monkey and mouse models of Parkinson's disease, cortically induced inhibition is reduced and prevents the release of movements, resulting in akinesia/bradykinesia. On the other hand, in a mouse model of dystonia, cortically induced inhibition is enhanced and releases unintended movements, inducing involuntary muscle contractions. Moreover, after blocking the subthalamic nucleus activity in a monkey model of Parkinson's disease, cortically induced inhibition is recovered and enables voluntary movements, explaining the underlying mechanism of stereotactic surgery to ameliorate parkinsonian motor signs. The \"dynamic activity model\" gives us a more comprehensive view of the pathophysiology underlying motor symptoms of movement disorders and clues for their novel therapies.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"390-397"},"PeriodicalIF":0.0,"publicationDate":"2024-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141175564","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":"[Peripheral neuropathy associated with severe glial fibrillary acidic protein (GFAP) astrocytopathy: a case report].","authors":"Nobuhiko Ohashi, Shota Kamijima, Rie Watanabe, Ko-Ichi Tazawa","doi":"10.5692/clinicalneurol.cn-001940","DOIUrl":"10.5692/clinicalneurol.cn-001940","url":null,"abstract":"<p><p>A 44-year-old man was admitted due to a fever. He developed unconsciousness and respiratory failure, necessitating mechanical ventilation. After the administration of methylprednisolone and intravenous immunoglobulin for suspected autoimmune encephalitis, his consciousness and respiratory state improved. However, he exhibited pronounced tetraparalysis and impaired sensation below the neck. A spinal MRI revealed swelling of the entire spinal cord, indicating myelitis. Deep tendon reflexes were diminished in all extremities, and a nerve conduction study confirmed motor-dominant axonal polyneuropathy. Subsequently, he developed a fever and headache. Brain MRI demonstrated FLAIR hyperintensities in the basal ganglia and brain stem. CSF analysis for anti-glial fibrillary acidic protein (GFAP) antibody turned out positive, leading to the diagnosis of GFAP astrocytopathy. Although the steroid re-administration improved muscle strength in his upper limbs and reduced the range of diminished sensation, severe hemiparalysis remained. Severe GFAP astrocytopathy can be involved with polyneuropathy. Early detection and therapeutic intervention for this condition may lead to a better prognosis.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"403-407"},"PeriodicalIF":0.0,"publicationDate":"2024-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141155365","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 suspected IgG4-related hypophysitis presented with panhypopituitarism and central diabetes insipidus].","authors":"Hitoshi Kubo, Haruki Ohtsubo, Akira Shiraoka, Masashi Watanabe, Itaru Kyoraku, Kensho Okamoto","doi":"10.5692/clinicalneurol.cn-001934","DOIUrl":"10.5692/clinicalneurol.cn-001934","url":null,"abstract":"<p><p>A 78-year-old man complained of subacute general fatigue and anorexia, following diplopia and gait disturbance. He demonstrated wide-based and small-stepped gait without objectively abnormal ocular movements. Brain ‍MRI showed enlargement of the pituitary stalk and gland with uniform contrast enhancement. PET-CT showed FDG ‍uptake in the pituitary gland, mediastinal lymph nodes, and left hilar lymph nodes. Blood investigations revealed panhypopituitarism and high serum IgG4 levels up to 265 ‍mg/dl. Histopathological examination revealed no IgG4-positive cell infiltration in the biopsied mediastinal lymph nodes. However, we suspected IgG4-associated hypophysitis based on the clinical symptoms and MRI findings, which were markedly resolved with steroid. Central masked diabetes insipidus was manifested, but was improved with oral desmopressin. We should pay close attention to the fact that IgG4-related hypophysitis may present with various symptoms regarded as indefinite complaints related to aging or underlying diseases, especially in elderly patients with multimorbidity.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"398-402"},"PeriodicalIF":0.0,"publicationDate":"2024-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141155355","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":"[Epileptic seizure and migraine attack: A revisit from the \"Borderland of Epilepsy\" to clinical implementation of infraslow activity/DC shifts in scalp EEG].","authors":"Akio Ikeda, Kiyohide Usami, Daisuke Danno, Takao Takeshima, Yoshihisa Tatsuoka","doi":"10.5692/clinicalneurol.cn-001948","DOIUrl":"10.5692/clinicalneurol.cn-001948","url":null,"abstract":"<p><p>Migraine attacks, especially ones with aura, have symptoms similar to epileptic seizures, and the two may sometimes be difficult to differentiate clinically. However, the characteristic minute-by-minute symptom development and progress within 60 ‍min is useful for diagnosis. Although the details of its pathophysiology remain unsolved, cortical spreading depolarization (CSD) is one of the main pathogenetic factors. In epilepsy, clinical data have shown that ictal DC shifts could reflect impaired homeostasis of extracellular potassium by astrocyte dysfunction. Ictal DC shifts were found to be difficult to detect by scalp EEG, but can be clinically recorded from the seizure focus using wide-band EEG method. The similarity between DC shifts and CSD has been gaining attention from the neurophysiology point of view. The clinical implementation of infraslow activity/DC shifts analysis of scalp EEG is expected to elucidate further the pathophysiology of migraine, which may lie in the borderland of epilepsy.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"383-389"},"PeriodicalIF":0.0,"publicationDate":"2024-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141175565","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":"[Primary central nervous system lymphoma presenting as a unilateral internal auditory canal lesion: a case report].","authors":"Rie Tohge, Masaaki Iwase, Takayuki Kondo","doi":"10.5692/clinicalneurol.cn-001939","DOIUrl":"10.5692/clinicalneurol.cn-001939","url":null,"abstract":"<p><p>A 61-year-old man with right hearing loss and staggering for seven months was diagnosed with sudden deafness although previous evaluation with MRI indicated minor abnormal findings. During follow-up, he developed hypogeusia, right facial nerve palsy, pain in right mandible, right-sided temporal pain, and cerebellar ataxia. Cerebrospinal fluid examination at admission revealed reduced glucose concentration and elevated soluble interleukin-2 receptor (sIL-2R) level, whereas serum sIL-2R level was within the normal range. Brain MRI showed a swollen contrast-enhanced lesion extending from the right internal auditory canal to the middle cerebellar peduncle. Gallium-67 (⁶⁷Ga) single-photon emission-computed tomography-computed tomography (SPECT-CT) revealed abnormal accumulation at the lesion site. Pathologic analysis of the tumor after resection led to the diagnosis of primary central nervous system lymphoma. In the present case, the MRI and ⁶⁷Ga SPECT-CT characteristics were distinct from those of vestibular schwannoma. In addition, elevation of sIL-2R in the cerebrospinal fluid but not in serum was useful for differential diagnosis.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"349-355"},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140872726","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 primary central nervous system lymphoma of the sellar region presented with panhypopituitarism].","authors":"Ayaka Seki, Fumiaki Henmi, Shinji Ito, Hironori Uruga, Kei Arisawa, Yoshikazu Uesaka","doi":"10.5692/clinicalneurol.cn-001941","DOIUrl":"10.5692/clinicalneurol.cn-001941","url":null,"abstract":"<p><p>The patient is a 41-year-old woman. She presented with vomiting and lightheadedness, and blood tests showed a generalized decrease in pituitary hormones and hyperprolactinemia. A head MRI showed increased signal intensity lesions on FLAIR image in the pituitary stalk, corpus callosum, periventricular area of the fourth ventricle, and superior cerebellar peduncle. The lesions were homogeneously enhanced, and a brain biopsy confirmed the diagnosis of primary diffuse large B-cell lymphoma of the central nervous system, and chemotherapy was started. Although the suprasellar region is a rare site for primary central nervous system lymphoma (PCNSL), it should be diagnosed early by biopsy.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"356-360"},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140869708","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":"[Analysis of factors leading to brain MRI lesions in Wernicke's encephalopathy].","authors":"Daisuke Kuzume, Yuko Morimoto, Satoshi Tsutsumi, Masahiro Yamasaki, Naohisa Hosomi","doi":"10.5692/clinicalneurol.cn-001919","DOIUrl":"10.5692/clinicalneurol.cn-001919","url":null,"abstract":"<p><p>[Objective] To investigate association between Wernicke encephalopathy (WE) and brain MRI. [Subjects] 26 patients (7 females, mean age 63.9 ± 12.7 years) with WE admitted to our department between May 2008 and September 2022. [Methods] Wernicke's encephalopathy in patients with MRI lesions was defined as \"MRI-positive group\" (MPG), and those without MRI lesions as \"MRI-negative group\" (MNG). The following parameters were assessed between the two groups: age, sex, alcoholism, neurological symptoms, vitamin B₁, lymphocyte, total cholesterol, albumin, and outcome at discharge. [Results] There were 17 patients in MPG. Compared to MNG, MPG had lower rates of alcohol abuse (10.0% vs 77.8%, P = 0.025), lower vitamin B₁ (median 10.0 ‍ng/ml vs 29.0 ‍ng/ml, P < 0.001), and more vitamin B₁ treatment dose (median 1900 ‍mg vs 600 ‍mg, P = 0.016). [Conclusion] Alcoholic WE may be overlooked if the focus is solely on brain MRI findings.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"361-363"},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140861217","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 neurosarcoidosis initially diagnosed as cervical spondylotic myelopathy, leading to diagnosis by gadolinium contrast-enhanced MRI].","authors":"Ayano Matsuyoshi, Daiji Uchiyama, Toshinori Kawanami, Yukiko Inamori, Wataru Shiraishi","doi":"10.5692/clinicalneurol.cn-001921","DOIUrl":"10.5692/clinicalneurol.cn-001921","url":null,"abstract":"<p><p>A 70-year-old female presented with bilateral numbness in her upper limbs. She was diagnosed with cervical spondylotic myelopathy and underwent cervical laminoplasty. However, there was no significant improvement in sensory disturbance, and at 6 months after surgery, she developed subacute motor and gait disturbance in four extremities. Spinal MRI revealed a long lesion of the spinal cord with edema, and a part of the lesion showed gadolinium contrast enhancement. Bronchoscopy revealed an elevated CD4/8 ratio, and gallium scintigraphy demonstrated an accumulation in the hilar lymph nodes, leading to a diagnosis of neurosarcoidosis. In case of rapid deterioration during the course of cervical spondylotic myelopathy, neurosarcoidosis should be considered as a differential diagnosis, which can be assessed by contrast-enhanced MRI.</p>","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":"339-343"},"PeriodicalIF":0.0,"publicationDate":"2024-05-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140860829","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}