Clinical Neurology最新文献

[Posterior cortical atrophy presenting with agraphia for kanji and statokinetic dissociation (Riddoch phenomenon): a case report]. 后皮质萎缩表现为汉字失写症和静止动力学解离（Riddoch现象）：1例报告。

Clinical Neurology Pub Date : 2025-05-20 DOI: 10.5692/clinicalneurol.cn-002036

Kyoko Maruta, Kazutaka Shiomi

{"title":"[Posterior cortical atrophy presenting with agraphia for kanji and statokinetic dissociation (Riddoch phenomenon): a case report].","authors":"Kyoko Maruta, Kazutaka Shiomi","doi":"10.5692/clinicalneurol.cn-002036","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002036","url":null,"abstract":"We report a patient with posterior cortical atrophy (PCA) who manifested as agraphia for kanji and statokinetic dissociation (Riddoch phenomenon). A 52-year-old, right-handed woman complained that beginning at age 50, she could write only kana (a phonographic script) but not kanji (a morphographic script). She could not write even her own name in kanji. Neuropsychologic examinations disclosed kanji-dominant agraphia, acalculia, right-left disorientation, finger agnosia, constructional apraxia, and simultanagnosia. Many of these, including agraphia, are components of Gerstmann syndrome. She manifested no aphasia or alexia, while not only writing but also copying of kanji was impaired. Speech functions, behavior, and personality were relatively spared. The patient also displayed statokinetic dissociation (Riddoch phenomenon): kinetic Goldmann fields were normal, but static Humphrey visual fields showed an incongruous right homonymous hemianopsia. MRI showed atrophy of the left parietal lobe. 99mTc ethyl cysteinate dimer (ECD) single-photon emission computed tomography (SPECT) showed hypoperfusion , predominantly in the left hemisphere and especially left the parietal lobe . These clinical and neuroradiologic findings are consistent with PCA. In patients with PCA, suspected incomplete homonymous hemianopsia should be confirmed with a Humphrey visual field test. Ishihara pseudoisochromatic plates may not be reliable; color vision should be checked using the panel D-15 test.","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144121047","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}

引用次数: 0

[A case of young woman with intracranial gumma developed within 1 year after Treponema pallidum infection]. 【梅毒螺旋体感染后1年内发生颅内牙龈瘤1例】。

Clinical Neurology Pub Date : 2025-05-20 DOI: 10.5692/clinicalneurol.cn-002073

Takahiro Akamatsu, Yuichi Masuda, Taiki Sawai, Shin Ota, Takafumi Hosokawa, Shigeki Arawaka

引用次数: 0

[Update of clinical management in autoimmune encephalitis-2004]. [自身免疫性脑炎临床治疗进展-2004]。

Clinical Neurology Pub Date : 2025-05-20 DOI: 10.5692/clinicalneurol.cn-002102

Satoshi Kamei

{"title":"[Update of clinical management in autoimmune encephalitis-2004].","authors":"Satoshi Kamei","doi":"10.5692/clinicalneurol.cn-002102","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002102","url":null,"abstract":"Encephalitis is a life-threatening disease with many causes. The continual discovery of newly identified forms of autoimmune encephalitis (AE) associated with antibodies to cell-surface or synaptic proteins has changed the paradigms for diagnosing and treating disorders. AE is one of the most common causes of non-infectious encephalitis. It can be triggered by tumors, infections, or it may be cryptogenic. These disorders can occur in patients with or without cancer. I review here the update of clinical management in AE. Recent clinical trends in AE include 1) the spread of clinical manifestations, 2) pitfalls of misdiagnosed cases and risk factors for misdiagnosis, and 3) treatment trends for refractory cases and symptomatic epilepsy. 1) The spread of clinical manifestations includes the presence of autoimmune psychosis (Pollak TA Lancet Psychiatry 2020), the presence of AE in adult-onset temporal lobe epilepsy (Kuehn JC, PLoS One 2020), and AE cases presenting with progressive dementia (Bastiaansen AEM, Neurol Neuroimmunol Neuroinflamm 2021). 2) Misdiagnosis and inappropriate use of diagnostic criteria for antibody-negative cases have been pointed out (Dalmau J. Lancet Neurol 2023). Misdiagnoses of AE occur for three reasons. First, non-adherence to reported clinical requirements for diagnostic criteria for AE. Second, the evaluation of inflammatory changes in head MRI and cerebrospinal fluid is insufficient. Third, absent or limited use of brain tissue assays along with use of cell-based assays that include only a narrow range of antigens. Red flags suggesting alternative diagnoses included an insidious onset, positive nonspecific serum antibody, and failure to fulfill AE diagnostic criteria. 3) Treatment trends for rituximab-resistant refractory cases include tocilizumab (IL6 receptor monoclonal antibody) and bortezomib (26S proteasome inhibitor). On the other hand, new Na channel inhibitors (lacosamide, etc.) and perampanel may be useful for treating symptomatic epilepsy in AE.","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144121053","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}

引用次数: 0

[A case of bacterial intramedullary spinal cord abscess and cauda equina neuritis that resolved with conservative treatment]. 细菌性脊髓髓内脓肿和马尾神经炎经保守治疗消退1例。

Clinical Neurology Pub Date : 2025-05-20 DOI: 10.5692/clinicalneurol.cn-002099

Fujio Umehara

{"title":"[A case of bacterial intramedullary spinal cord abscess and cauda equina neuritis that resolved with conservative treatment].","authors":"Fujio Umehara","doi":"10.5692/clinicalneurol.cn-002099","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002099","url":null,"abstract":"The patient is a woman in her 70s. Low back pain and left lower limb pain gradually worsened since 1 month ago, urinary retention and bilateral lower limb paralysis appeared and she was admitted to our department. Muscle weakness in both lower limbs, hypoesthesia and pain in both lower limbs predominantly in the right side, and loss of tendon reflexes in both lower limbs were observed. MRI showed severe lumbar deformity as well as swelling of the spinal conus medullaris, ring-shaped contrast effect, and contrast effect of the cauda equina nerve. Diffusion-weighted images of the spinal conus showed multifocal high signal. Cerebrospinal fluid showed 271 cells/mm3 (72% polymorphonuclear cells), 356 ‍mg/dl protein, 15 ‍mg/dl sugar, and negative bacterial culture. Suspecting bacterial intramedullary spinal abscess and cauda equina neuritis, she was started on intravenous Ceftriaxone (CTRX)/ Vancomycin (VCM) and oral MNZ. Thereafter, muscle weakness and sensory disturbance in both lower limbs gradually improved, and the patient was able to walk with a cane one month later. Cerebrospinal fluid and MRI findings gradually normalized. The diagnosis of bacterial intramedullary spinal cord abscess and cauda equina neuritis was made, which improved with conservative treatment.","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144121058","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}

引用次数: 0

[A case of lymphocytic hypophysitis initially diagnosed as aseptic meningitis]. [1例淋巴细胞性垂体炎最初诊断为无菌性脑膜炎]。

Clinical Neurology Pub Date : 2025-05-20 DOI: 10.5692/clinicalneurol.cn-002098

Eito Miura, Uran Komatsubara, Yoshitaka Umeda, Shota Akakabe, Nobuya Fujita, Mutsuo Oyake

引用次数: 0

[An autopsy case of a 76-year-old woman with progressive supranuclear palsy initially presenting with dropped head and clinical features of pure akinesia with gait freezing]. [尸检一例76岁女性进行性核上性麻痹，最初表现为头部下垂，临床表现为纯运动障碍伴步态冻结]。

Clinical Neurology Pub Date : 2025-05-20 DOI: 10.5692/clinicalneurol.cn-002088

Ken Yamamoto, Kenji Ishihara, Yukiko Mori, Yasushi Iwasaki, Mari Yoshida, Hidetomo Murakami

{"title":"[An autopsy case of a 76-year-old woman with progressive supranuclear palsy initially presenting with dropped head and clinical features of pure akinesia with gait freezing].","authors":"Ken Yamamoto, Kenji Ishihara, Yukiko Mori, Yasushi Iwasaki, Mari Yoshida, Hidetomo Murakami","doi":"10.5692/clinicalneurol.cn-002088","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002088","url":null,"abstract":"We present a case of a 76-year-old woman diagnosed with pathologically confirmed progressive supranuclear palsy (PSP) with pallido-nigral-luysial atrophy, who initially presented with a dropped head. Upon her first visit, neurophysiological and neuroradiological examinations provided no definitive cause, and the tactile trick was effective, leading to a diagnosis of cervical dystonia. Trihexyphenidyl treatment had no effect, but her condition gradually improved over 3 years. By age 74, she developed gait freezing without muscle rigidity or tremor. Dopamine-transporter scintigraphy revealed reduced tracer uptake in the bilateral corpus striata, prompting the diagnosis of pure akinesia with gait freezing (PAGF). At age 76, the patient developed retrocollis, muscle rigidity in all extremities, and recurrent temporomandibular dislocation. She eventually died from aspiration pneumonia after several years of illness. At autopsy, the brain weighed 1,370 ‍g. Macroscopic examination showed atrophy of the pallidum and subthalamic nucleus and depigmentation of the substantia nigra. Histopathological analysis revealed degeneration with 4-repeat tau pathology in the substantia nigra, globus pallidus, and subthalamic nucleus, along with tufted astrocytes in the globus pallidus and putamen, confirming a pathological diagnosis of pallido-nigral-luysial atrophy-type PSP. We suggest that the clinical presentation of PAGF correlates well with the pathological findings of pallido-nigral-luysial atrophy. While dystonia in PSP is typically observed in the limbs, blepharospasm, or retrocollis, only two other cases of PSP with a dropped head have been reported. The pathophysiological mechanism remains unclear, but we hypothesize that 4-repeat tau pathology in the globus pallidus may contribute to the development of cervical dystonia. Further neuropathological studies are needed to confirm this hypothesis.","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144121081","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}

引用次数: 0

[Anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis diagnosed from T₁ signal intensity changes in basal ganglia: a case report and literature review]. 【基底节T1信号强度变化诊断抗富亮氨酸胶质瘤失活1 （LGI1）脑炎1例报告及文献复习】。

Clinical Neurology Pub Date : 2025-05-20 DOI: 10.5692/clinicalneurol.cn-002084

Takanobu Kita, Hiroaki Okada, Yoshiyuki Nakai, Masahiro Kanai, Keiji Yamaguchi

引用次数: 0

[Summarising a case you experienced: how to create understandable and effective slides]. [总结你经历过的一个案例：如何制作容易理解和有效的幻灯片]。

Clinical Neurology Pub Date : 2025-05-20 DOI: 10.5692/clinicalneurol.cn-002104

Akiyuki Hiraga

引用次数: 0

Syphilitic gummas affecting brain and spinal cord: a case report. 影响脑和脊髓的梅毒牙龈1例。

Clinical Neurology Pub Date : 2025-05-20 DOI: 10.5692/clinicalneurol.cn-002105

Hikari Kondo, Toru Watanabe, Kazuyoshi Kobayashi, Amane Araki, Kazuhiro Hara, Keizo Yasui

{"title":"Syphilitic gummas affecting brain and spinal cord: a case report.","authors":"Hikari Kondo, Toru Watanabe, Kazuyoshi Kobayashi, Amane Araki, Kazuhiro Hara, Keizo Yasui","doi":"10.5692/clinicalneurol.cn-002105","DOIUrl":"https://doi.org/10.5692/clinicalneurol.cn-002105","url":null,"abstract":"Syphilitic gumma is a rare manifestation of neurosyphilis that can cause mass lesions in the central nervous system. We present an atypical case of a 53-year-old man presenting with syphilitic gummas affecting both the brain and spinal cord. The patient presented with right facial numbness, worsening back pain, gait disturbances, and lower-limb weakness. Serological tests were positive for syphilis, and cerebrospinal fluid analysis showed elevated cell count, protein concentration, and positive syphilis tests. Brain and spinal cord MRI revealed dural-based enhancing mass lesions in the right middle cerebellar peduncle and conus medullaris. The patient underwent posterior decompression and biopsy of the conus medullaris. Histopathological findings excluded malignancy and were consistent with syphilitic gumma. The patient received intravenous benzylpenicillin, followed by oral amoxicillin, resulting in partial improvement of neurological symptoms and gradual regression of the lesions on follow-up MRI. This case highlights the importance of considering syphilitic gumma in the differential diagnosis of intracranial and spinal cord lesions in patients with syphilis. Prompt antibiotic treatment and serial MRI imaging are crucial for managing this condition.","PeriodicalId":39292,"journal":{"name":"Clinical Neurology","volume":" ","pages":""},"PeriodicalIF":0.0,"publicationDate":"2025-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144121098","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}

引用次数: 0

[Current trends in multiple sclerosis/neuromyelitis optica spectrum disorder therapy: considerations for domestic administration and personalized therapy]. [多发性硬化症/视神经脊髓炎谱系障碍治疗的当前趋势：对国内管理和个性化治疗的考虑]。

Clinical Neurology Pub Date : 2025-04-30 DOI: 10.5692/clinicalneurol.cn-002095

Masami Tanaka

引用次数: 0