IF 0.5 4区医学

Neurocase Pub Date : 2026-10-01 Epub Date: 2026-09-07 DOI: 10.1080/13554794.2026.2726320

Ali Zare Dehnavi, Jorge Patino, Kendra M Anderson, Ineke Olsthoorn, Natalia Pessoa Rocha, Erin Furr Stimming

引用次数: 0

Pure visuospatial neglect due to cingulum bundle disconnection in right anterior cerebral artery infarction: a case report with diffusion tensor imaging analysis. 右脑前动脉梗死中单纯的视觉空间忽视：弥散张量成像分析1例。

IF 0.5 4区医学

Neurocase Pub Date : 2026-10-01 Epub Date: 2026-08-24 DOI: 10.1080/13554794.2026.2723919

Seung Yoon Choi, Seung Hoon Han, Seong Ho Jang, Jae Hyeon Park, Ji Woo Lee, Yeo Joon Yun

{"title":"Pure visuospatial neglect due to cingulum bundle disconnection in right anterior cerebral artery infarction: a case report with diffusion tensor imaging analysis.","authors":"Seung Yoon Choi, Seung Hoon Han, Seong Ho Jang, Jae Hyeon Park, Ji Woo Lee, Yeo Joon Yun","doi":"10.1080/13554794.2026.2723919","DOIUrl":"10.1080/13554794.2026.2723919","url":null,"abstract":"Visuospatial neglect is a common finding associated with lesions in the middle and posterior cerebral artery territories, with a higher incidence in right hemispheric infarctions. In anterior cerebral artery (ACA) infarction, neglect is typically accompanied by motor deficits or manifests as motor neglect. Pure visuospatial neglect without motor involvement in the setting of ACA infarction therefore represents an uncommon clinical presentation. A 62-year-old woman presented with a cognitive decline, including perseverative behavior and disorientation to time. Brain magnetic resonance imaging (MRI) revealed a right medial frontal acute infarction. Neurological examination revealed intact motor function. However, bedside tests and the Seoul Neuropsychological Screening Battery (SNSB) demonstrated severe left-sided visuospatial neglect with domain-specific dissociation: visuospatial attention was severely impaired while verbal attention remained preserved. Diffusion tensor imaging (DTI) tractography revealed severe and preferential disruption of the right cingulum bundle (78% reduction in tract count; asymmetry ratio 0.22) with relative preservation of the superior longitudinal fasciculus (31.8% reduction in tract count) and intact parietal aslant tract. This case demonstrates that motor-sparing ACA infarction can produce severe pure visuospatial neglect, and highlights the importance of careful neuropsychological evaluation and active rehabilitation even in the absence of motor weakness.","PeriodicalId":49762,"journal":{"name":"Neurocase","volume":" ","pages":"209-214"},"PeriodicalIF":0.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148814754","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Rapid plaque clearance with anti-amyloid therapy for Alzheimer's disease: in consideration of donanemab treatment duration from real-world cases. 抗淀粉样蛋白治疗阿尔茨海默病的快速斑块清除：考虑到现实世界病例的donanemab治疗持续时间

IF 0.5 4区医学

Neurocase Pub Date : 2026-10-01 Epub Date: 2026-09-08 DOI: 10.1080/13554794.2026.2730462

Jeongyeon Hwang, Alison Pietras, Georges Naasan, Fanny M Elahi, Sam Gandy

{"title":"Rapid plaque clearance with anti-amyloid therapy for Alzheimer's disease: in consideration of donanemab treatment duration from real-world cases.","authors":"Jeongyeon Hwang, Alison Pietras, Georges Naasan, Fanny M Elahi, Sam Gandy","doi":"10.1080/13554794.2026.2730462","DOIUrl":"10.1080/13554794.2026.2730462","url":null,"abstract":"To assess the possibility that individualized duration of donanemab treatment might enable early pausing of therapy, and to explore the possible utility of plasma P-tau217 as a biomarker for treatment response monitoring in real-time, two unrelated patients with early-stage Alzheimer's disease were treated with donanemab at the Icahn School of Medicine at Mount Sinai. Clinical progress was monitored via medical chart review, including serial brain MRI, amyloid PET scans, and blood-based biomarker assays (Quanterix Simoa® and Fujirebio Lumipulse®). Both patients achieved complete amyloid clearance as judged by amyloid PET prior to their 6th and 9th infusions, which were significantly earlier than the 12-month mark typically recommended for follow-up amyloid assessment. In agreement with the amyloid PET, concurrently measured plasma P-tau217 levels fell within a low range. Treatment was discontinued for both patients. These patients highlight the possibility of individualized treatment durations for anti-amyloid therapy with donanemab, guided by biomarker response. Early cessation following rapid plaque clearance could reduce healthcare costs and minimize exposure to the risk of amyloid-related imaging abnormalities and the generation of neutralizing antibodies. Plasma P-tau217 warrants further investigation as a reliable, cost-effective surrogate for PET scans to monitor real-time treatment response and guide the timing of discontinuation.","PeriodicalId":49762,"journal":{"name":"Neurocase","volume":" ","pages":"229-232"},"PeriodicalIF":0.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148913906","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Cognitive impairment in adult Alexander disease. 成人亚历山大病的认知障碍。

IF 0.5 4区医学

Neurocase Pub Date : 2026-10-01 Epub Date: 2026-08-15 DOI: 10.1080/13554794.2026.2718969

Eva Maes, Gert Cypers, Sam Van de Schoot, Jan Van den Stock

{"title":"Cognitive impairment in adult Alexander disease.","authors":"Eva Maes, Gert Cypers, Sam Van de Schoot, Jan Van den Stock","doi":"10.1080/13554794.2026.2718969","DOIUrl":"10.1080/13554794.2026.2718969","url":null,"abstract":"Alexander disease (AxD) is a rare progressive astrogliopathy and leukodystrophy caused by heterozygous pathogenic variants in the gene encoding glial fibrillary acidic protein (GFAP). Cognitive problems in AxD have long been recognized, but domain-specific neuropsychological descriptions of adult-onset Alexander disease (AO-AxD) remain sparse. We report a genetically confirmed 53-year-old man with AO-AxD who underwent comprehensive neuropsychological assessment because of progressive cognitive and behavioral complaints. The profile was characterized by deficient verbal episodic memory retrieval and reduced set-shifting efficiency, with relatively preserved recognition memory, language, visuospatial abilities, and most attentional measures. Structural MRI showed characteristic medullary and upper cervical cord involvement but only limited supratentorial white matter abnormalities. To contextualize the case, we reviewed published AO-AxD reports with domain-specific neuropsychological testing. The available literature remains limited and heterogeneous, but suggests that cognitive manifestations in AO-AxD may include retrieval-based memory deficits, executive inefficiency, and visuospatial difficulties. We discuss these findings in relation to white matter dementia, fronto-subcortical and fronto-hippocampal network dysfunction, and potential astrocyte-mediated mechanisms.","PeriodicalId":49762,"journal":{"name":"Neurocase","volume":" ","pages":"181-189"},"PeriodicalIF":0.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148763403","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Progressive anterior opercular syndrome (Foix-Chavany-Marie syndrome) in neurodegenerative disease: a case series. 神经退行性疾病的进行性前眼窝综合征（Foix-Chavany-Marie综合征）：一个病例系列。

IF 0.5 4区医学

Neurocase Pub Date : 2026-10-01 DOI: 10.1080/13554794.2026.2735595

Hiroyuki Watanabe, Etsuro Mori, Keith A Josephs

{"title":"Progressive anterior opercular syndrome (Foix-Chavany-Marie syndrome) in neurodegenerative disease: a case series.","authors":"Hiroyuki Watanabe, Etsuro Mori, Keith A Josephs","doi":"10.1080/13554794.2026.2735595","DOIUrl":"https://doi.org/10.1080/13554794.2026.2735595","url":null,"abstract":"Anterior opercular syndrome (Foix-Chavany-Marie syndrome) is characterized by severe dysarthria, impaired voluntary orofacial and pharyngolingual movements, and preserved automatic or emotional movements (automatic-voluntary dissociation). Although classically associated with stroke, its progressive neurodegenerative form is rare and poorly understood. We report two patients to characterize its clinical and neuroanatomical features. The cases were identified retrospectively through review of clinical and neuroimaging records from behavioral neurology centers in the United States and Japan. Both had severe dysarthria and oral-phase dysphagia. Voluntary orofacial movements were markedly impaired, whereas reflexive movements, including coughing and yawning, and emotional movements, including smiling in response to humor, were preserved. Coordination, posture, and gait were initially preserved. Both patients showed predominantly upper motor neuron features without lower motor neuron signs; limb and cranial electromyography was normal in the patient tested. Neuroimaging showed bilateral frontal atrophy and hypometabolism or hypoperfusion involving the anterior operculum. These findings suggest that progressive anterior opercular syndrome may emerge when bilateral opercular regions are affected by neurodegeneration. Recognition of this syndrome may facilitate anticipatory management of dysarthria, dysphagia, and nutritional risk and broaden awareness of speech and orofacial motor syndromes in frontotemporal dementia and related phenotypes.","PeriodicalId":49762,"journal":{"name":"Neurocase","volume":" ","pages":"1-7"},"PeriodicalIF":0.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"149117695","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Severe amnestic-cognitive syndrome after right-sided resection in a patient with bilateral trigonal choroid plexus xanthogranulomas: illustrative case. 双侧三角脉络膜丛黄色肉芽肿患者右侧切除后出现严重的遗忘-认知综合征：说明性病例。

IF 0.5 4区医学

Neurocase Pub Date : 2026-10-01 Epub Date: 2026-08-30 DOI: 10.1080/13554794.2026.2717152

Amir M Poreh, Lior Ungar, Shachar Zion Shemesh, Paz Kelmer, Moshe Hadanei, Zeev Feldman

{"title":"Severe amnestic-cognitive syndrome after right-sided resection in a patient with bilateral trigonal choroid plexus xanthogranulomas: illustrative case.","authors":"Amir M Poreh, Lior Ungar, Shachar Zion Shemesh, Paz Kelmer, Moshe Hadanei, Zeev Feldman","doi":"10.1080/13554794.2026.2717152","DOIUrl":"10.1080/13554794.2026.2717152","url":null,"abstract":"Choroid plexus xanthogranulomas are rare, typically incidental intraventricular lesions that infrequently become symptomatic. When symptomatic, they most often present with headache or hydrocephalus, whereas cognitive sequelae and postoperative neuropsychological outcomes remain poorly characterized. A 63-year-old right-handed man with bilateral trigonal intraventricular lesions, larger on the right, presented with headaches and a history of subjective progressive memory complaints. He underwent resection of the right-sided lesion through a right temporoparietal approach. Immediately after surgery, he developed a severe and persistent amnestic-cognitive syndrome with profound inability to retain new information, confabulation, reduced insight, and marked remote-memory disturbance. Neuropsychological evaluation 3 months postoperatively demonstrated severe anterograde impairment in verbal learning and delayed visuospatial recall, together with substantial retrograde memory impairment involving autobiographical episodic recollection, personal semantic memory, and public semantic knowledge. This case suggests that bilateral trigonal xanthogranulomas and their surgical treatment may place distributed memory networks at substantial risk. Because formal preoperative neuropsychological testing and direct review of the original preoperative MRI were unavailable, the relative contributions of preexisting bilateral disease burden and postoperative network disruption cannot be fully separated. The case supports broader baseline cognitive assessment, precise laterality-specific surgical description, and structured postoperative neuropsychological follow-up for lesions near posterior medial and limbic white-matter pathways.","PeriodicalId":49762,"journal":{"name":"Neurocase","volume":" ","pages":"202-208"},"PeriodicalIF":0.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148867663","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Cognitive impairment as a manifestation of SPG7: case report. 认知障碍作为SPG7的表现：1例报告。

IF 0.5 4区医学

Neurocase Pub Date : 2026-10-01 Epub Date: 2026-09-06 DOI: 10.1080/13554794.2026.2729249

Fiorella S Guido, Michael R Basso, Ralitza H Gavrilova, W Oliver Tobin

{"title":"Cognitive impairment as a manifestation of SPG7: case report.","authors":"Fiorella S Guido, Michael R Basso, Ralitza H Gavrilova, W Oliver Tobin","doi":"10.1080/13554794.2026.2729249","DOIUrl":"10.1080/13554794.2026.2729249","url":null,"abstract":"Hereditary spastic paraplegia (HSP) is a group of genetic disorders caused by >80 genes that can be described as either pure or complex forms.We report a case of a patient with a complex form of SPG7 with significant cognitive impairment.A 42-year-old man presented with a 10-year history of dysarthria, 5 years of gait difficulties, and 3 years of cognitive symptoms. Neuropsychological analysis showed deficits involving speed of information processing, mental flexibility, ideational fluency, verbal concept formation, visual working memory, and visual-spatial perception. Next-generation sequencing on whole blood revealed 2 heterozygous pathogenic variants in SPG7 (c. 1049_1077del, p. Pro350Glnfs*36 and c. 1529C>T, p. Ala510Val).There are emerging reports of complex forms of SPG7 presenting with impairments in memory, executive dysfunction, language, visuospatial processing, social functioning, and emotional communication. In this report, we describe a complex SPG7 patient who underwent formal neuropsychological testing to assess cognitive status in depth. This revealed deficits in speed of processing, visuospatial working memory and perception, impaired non-verbal intellect, and overall lack of insight, which have not yet been described in the literature.","PeriodicalId":49762,"journal":{"name":"Neurocase","volume":" ","pages":"222-228"},"PeriodicalIF":0.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148901273","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Where does the "ἀφημί" (speechless) in nfvPPA come from?: a case study on phonological retrieval and the potent effect of moraic assistance. nfvPPA中的“φημί ί”（φημί）从何而来？语音检索的个案研究及语音辅助的有效作用。

IF 0.5 4区医学

Neurocase Pub Date : 2026-10-01 Epub Date: 2026-09-09 DOI: 10.1080/13554794.2026.2723918

Tomoyuki Kojima

{"title":"Where does the \"ἀφημί\" (speechless) in nfvPPA come from?: a case study on phonological retrieval and the potent effect of moraic assistance.","authors":"Tomoyuki Kojima","doi":"10.1080/13554794.2026.2723918","DOIUrl":"10.1080/13554794.2026.2723918","url":null,"abstract":"Diagnosing non-fluent/agrammatic variant primary progressive aphasia (nfvPPA) relies primarily on apraxia of speech (AOS) and agrammatism. However, differentiating phonetic-level (articulatory) from phonemic-level (phonological) impairments remains clinically challenging, especially in advanced stages. We report a 65-year-old Japanese male with nfvPPA who had become virtually speechless (ἀφημί). Leveraging the Kana writing system's one-to-one correspondence to morae, we designed a cross-modal experiment to isolate his underlying deficit. Speech production was evaluated across four conditions: picture naming, Kanji word reading, Kana word reading, and character-by-character Kana reading. Statistical analysis was performed using Fisher's exact test and adjusted residual analysis. Speech intelligibility significantly improved during character-by-character Kana reading compared to other modalities. Residual analysis confirmed that explicit moraic cues markedly reduced error rates. These findings indicate that our approach effectively unmasked superimposed phonemic-level retrieval deficits contributing to speechlessness in nfvPPA. This study highlights the utility of Japanese orthography in disentangling underlying mechanisms and demonstrates that moraic assistance can serve as a potent facilitation strategy in severe cases.","PeriodicalId":49762,"journal":{"name":"Neurocase","volume":" ","pages":"190-201"},"PeriodicalIF":0.5,"publicationDate":"2026-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148921626","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","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 report of crossed aphasia, grasping, and intermanual conflict with the left hand. 交叉失语、抓握、左手手间冲突1例。

IF 0.5 4区医学

Neurocase Pub Date : 2026-08-01 Epub Date: 2026-05-26 DOI: 10.1080/13554794.2026.2676581

Fuzuki Sakamoto, Yuhei Kodani, Katsuya Nakamura, Shinya Fukunaga

引用次数: 0

A case of Highly Superior Autobiographical Memory (HSAM) accompanied by highly frequent involuntary autobiographical memory. 高水平自传体记忆伴高频率非自愿自传体记忆1例。

IF 0.5 4区医学

Neurocase Pub Date : 2026-08-01 Epub Date: 2026-07-30 DOI: 10.1080/13554794.2026.2701703

Anne M Cleary, Noah Okada, Donald J Bearden, Demy Alfonso, Caitlin Mills, Joseph Neisser, Katherine L McNeely-White, Nigel P Pedersen, Daniel L Drane

{"title":"A case of Highly Superior Autobiographical Memory (HSAM) accompanied by highly frequent involuntary autobiographical memory.","authors":"Anne M Cleary, Noah Okada, Donald J Bearden, Demy Alfonso, Caitlin Mills, Joseph Neisser, Katherine L McNeely-White, Nigel P Pedersen, Daniel L Drane","doi":"10.1080/13554794.2026.2701703","DOIUrl":"10.1080/13554794.2026.2701703","url":null,"abstract":"Highly Superior Autobiographical Memory (HSAM) is the rare ability to recollect nearly all of one's past personal experiences with great detail and accuracy. When given a date, a person with HSAM can recall verifiable details about what they experienced that day. People with HSAM do not tend to stand out on standard neuropsychological assessments, common laboratory tasks, or other measures that assess intentional forms of memory. Some clues suggest that the superior autobiographical remembering of people with HSAM occurs involuntarily. In a newly identified case of a person with HSAM, we found evidence using the Involuntary Autobiographical Memory Inventory (IAMI) that the person's involuntary memory indeed stands out. This person's past-oriented Involuntary Autobiographical Memory (IAM) frequency was two standard deviations above the mean reported in previous research. while, in line with past research, her performance on standard neuropsychological batteries and laboratory tasks was unremarkable. We propose that these intrusive recollections facilitate the consolidation of memory for past events.","PeriodicalId":49762,"journal":{"name":"Neurocase","volume":" ","pages":"152-166"},"PeriodicalIF":0.5,"publicationDate":"2026-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148632479","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

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