Clinical Neuropathology最新文献_第9页

Intracerebral retina-like pigmented tissue in a stillborn fetus with holoprosencephaly. 无前脑畸形死胎的脑内视网膜样色素组织。

IF 1.1 4区医学

Clinical Neuropathology Pub Date : 2022-04-01 DOI: 10.5414/NP301453

Hao Li, Qi Zhang, L. Ang

引用次数: 0

Recurrent cerebellar liponeurocytoma with anaplastic features at initial presentation: A case report. 初次出现时具有间变性特征的复发性小脑脂肪神经细胞瘤：一例报告。

IF 1.1 4区医学

Clinical Neuropathology Pub Date : 2022-03-28 DOI: 10.5414/NP301403

M. Maharaj, S. Tawfik, F. Ziad, T. Muthu

{"title":"Recurrent cerebellar liponeurocytoma with anaplastic features at initial presentation: A case report.","authors":"M. Maharaj, S. Tawfik, F. Ziad, T. Muthu","doi":"10.5414/NP301403","DOIUrl":"https://doi.org/10.5414/NP301403","url":null,"abstract":"BACKGROUND\u0000Cerebellar liponeurocytoma is a rare entity with fewer than 100 reported cases and series in the available literature to date. Although the cerebellum remains the typical primary site, the entity has been shown to demonstrate increased aggressiveness and malignant progression with multiple recurrences.\u0000\u0000\u0000CASE DESCRIPTION\u0000We present a unique case in a 64-year-old gentleman of a cerebellar liponeurocytoma with multiple recurrences and progressive anaplasia. The tumor showed anaplastic features at first presentation and recurred in a more aggressive fashion in a short 2-year period despite surgical debulking and post-operative radiotherapy. It re-recurred within 6 months with subsequent re-debulking without further radiotherapy. At latest follow-up almost 3 years since surgical management of the patient's second recurrence, the patient remains well with minimal neurological impairment and no radiological signs of recurrence.\u0000\u0000\u0000CONCLUSION\u0000Cerebellar liponeurocytoma may present with increasingly atypical histological features that may warrant more aggressive post-operative treatment to prevent disease recurrence and clinical deterioration. This may include a more aggressive surgical resection margin and consideration of adjuvant radiotherapy in all cases.","PeriodicalId":55251,"journal":{"name":"Clinical Neuropathology","volume":" ","pages":""},"PeriodicalIF":1.1,"publicationDate":"2022-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46254024","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

Prolonged central motor conduction time and pyramidal tract degeneration in amyotrophic lateral sclerosis. 肌萎缩性侧索硬化症中枢性运动传导时间延长与锥体束变性。

IF 1.1 4区医学

Clinical Neuropathology Pub Date : 2022-03-28 DOI: 10.5414/NP301438

T. Takeda, M. Iijima, M. Seki, Eiko Higuchi, Yuko Shimizu, N. Shibata, K. Kitagawa

{"title":"Prolonged central motor conduction time and pyramidal tract degeneration in amyotrophic lateral sclerosis.","authors":"T. Takeda, M. Iijima, M. Seki, Eiko Higuchi, Yuko Shimizu, N. Shibata, K. Kitagawa","doi":"10.5414/NP301438","DOIUrl":"https://doi.org/10.5414/NP301438","url":null,"abstract":"Electrophysiological methods to detect the degeneration of the upper motor neuron system have not been fully established in patients with amyotrophic lateral sclerosis (ALS). This may be partly because the parallel demonstration of electrophysiology and a corresponding pathological abnormality is insufficient, and because a substantial number of patients with ALS do not exhibit upper motor neuron degeneration. Recently, we encountered 2 patients with ALS who had been examined for abnormal central motor conduction time (CMCT) using transcranial magnetic stimulation within a 20-day period prior to their death. Autopsy revealed that 1 patient had marked pyramidal degeneration with prolonged CMCT; in contrast, the other patient had no obvious pyramidal degeneration and showed normal CMCT. Both the patients with contrasting clinicopathological differences contributed to the identification that the prolongation of CMCT was possibly linked to the degeneration of the corticospinal tract. This report indicates that CMCT is useful for predicting the severity of upper motor neuron degeneration in patients with ALS.","PeriodicalId":55251,"journal":{"name":"Clinical Neuropathology","volume":"1 1","pages":""},"PeriodicalIF":1.1,"publicationDate":"2022-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"70972760","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

Histologically heterogeneous pediatric glioneuronal tumor with FGFR1::TACC1 fusion. FGFR1:：TACC1融合的组织学异质性儿童胶质神经元肿瘤。

IF 1.1 4区医学

Clinical Neuropathology Pub Date : 2022-03-28 DOI: 10.5414/NP301461

F. Canan, Elena V Daoud, J. Weon, J. Raisanen, D. Burns, K. Hatanpaa, Jason Y. Park, S. Kelley, V. Rajaram

引用次数: 0

Hypothyroidism combined with anti-signal recognition particle immune-mediated necrotizing myopathy: A case report and review of the literature. 甲状腺功能减退合并抗信号识别粒子免疫介导的坏死性肌病1例报告及文献复习。

IF 1.1 4区医学

Clinical Neuropathology Pub Date : 2022-03-01 DOI: 10.5414/NP301434

Jiaying Shi, Huaxing Meng, Wei Zhang, Junhong Guo

{"title":"Hypothyroidism combined with anti-signal recognition particle immune-mediated necrotizing myopathy: A case report and review of the literature.","authors":"Jiaying Shi, Huaxing Meng, Wei Zhang, Junhong Guo","doi":"10.5414/NP301434","DOIUrl":"https://doi.org/10.5414/NP301434","url":null,"abstract":"Immune-mediated necrotizing myopathy (IMNM) is characterized by proximal limb weakness, elevated creatine kinase (CK) levels, and myofiber necrosis without or with only a small amount of inflammatory cell infiltrate. There is only 1 report of hypothyroidism combined with antibody-negative IMNM to date. We aimed to describe a rare case of hypothyroidism combined with anti-signal recognition particle (SRP) IMNM for the first time and review the previous literature. A 50-year-old male, who had a 4-year history of hypothyroidism treated with levothyroxine replacement therapy, presented with progressive symmetrical proximal muscle weakness. Laboratory testing showed an elevated CK level of 6,106 U/L. Electrophysiological examination elicited carpal tunnel syndrome and myogenic damage. Muscle MRI revealed diffuse abnormal signals in both lower limbs. Given that muscle symptoms are widely recognized among hypothyroid patients, hypothyroid myopathy was initially suspected, and thyroid hormone tablets were added for a week. However, muscle weakness persisted along with an even higher CK (7,020 U/L). Quadriceps muscle biopsy was performed and indicated inflammatory myopathy. Myositis specific antibodies (MSAs) detection revealed that anti-SRP was positive. A diagnosis of hypothyroidism combined with anti-SRP IMNM was finally made. Treatment of corticosteroid and immunosuppressive agents achieved a positive clinical and biochemical response. This case indicates that hypothyroidism combined with anti-SRP IMNM is a rare clinical entity, possibly caused by a general immunologic dysregulation.","PeriodicalId":55251,"journal":{"name":"Clinical Neuropathology","volume":"41 2","pages":"83-88"},"PeriodicalIF":1.1,"publicationDate":"2022-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39686635","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: 1

Cerebral amyloidomas: Perspective on unusual morphological features. 脑淀粉样瘤:异常形态学特征的透视。

IF 1.1 4区医学

Clinical Neuropathology Pub Date : 2022-03-01 DOI: 10.5414/NP301440

Bette K Kleinschmidt-DeMasters

{"title":"Cerebral amyloidomas: Perspective on unusual morphological features.","authors":"Bette K Kleinschmidt-DeMasters","doi":"10.5414/NP301440","DOIUrl":"https://doi.org/10.5414/NP301440","url":null,"abstract":"Aims: Cerebral amyloidomas (CAs) are mass-producing congophilic lesions most commonly due to λ light chain deposits, contrasting them with light chain deposition disease (LCDD) which has non-polarizable, often κ light chain deposition.Materials and methods: Although usual histological features are well known, we detail 3 recent CAs with unusual morphological findings and review the literature specifically for these features.Results: Two women, aged 56 and 58 years, had right cerebral white matter CAs. The biopsy of case 1 disclosed congophilic polarizable deposits with prominent dystrophic mineralization as well as scant plasma cells. Case 2 had a CA with significant multinucleated giant cell reaction to the amyloid and additionally contained an area suspicious for marginal zone B-cell lymphoma. Case 3 was a clinically unsuspected CA identified at autopsy in a 75-year-old woman that manifested as several contiguous left frontal lobe white matter erythematous, hyperemic lesions; microscopy showed nodular and concentric amyloid deposits and thick perivascular cuffs of plasma cells. Mass spectrometry proved λ light and α heavy chain amyloid deposits in all 3 cases.Conclusion: These 3 CA cases illustrate several unusual gross and microscopic features that are discussed in context with the literature.","PeriodicalId":55251,"journal":{"name":"Clinical Neuropathology","volume":"41 2","pages":"46-52"},"PeriodicalIF":1.1,"publicationDate":"2022-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39766178","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

CNS paraganglioma: 15-year experience in a tertiary care hospital with literature review. 中枢神经系统副神经节瘤:在三级护理医院15年的经验与文献综述。

IF 1.1 4区医学

Clinical Neuropathology Pub Date : 2022-03-01 DOI: 10.5414/NP301414

Nandita Ghosal, Parul Jain, Sumit Thakar, Koustav Ghosal

引用次数: 0

Primary histiocytic sarcoma of the clivus with focal extension into central nervous system and neurologic manifestations: First description at an unusual site with an overwhelming and rapid progression. 坡地原发性组织细胞肉瘤伴局灶性延伸至中枢神经系统及神经系统表现:首次描述为不寻常部位，进展迅速且势如挡。

IF 1.1 4区医学

Clinical Neuropathology Pub Date : 2022-03-01 DOI: 10.5414/NP301400

Ivette Perez, Murat Gokden, John D Day, Hadi Yaziji, Sergio Pina-Oviedo

{"title":"Primary histiocytic sarcoma of the clivus with focal extension into central nervous system and neurologic manifestations: First description at an unusual site with an overwhelming and rapid progression.","authors":"Ivette Perez, Murat Gokden, John D Day, Hadi Yaziji, Sergio Pina-Oviedo","doi":"10.5414/NP301400","DOIUrl":"https://doi.org/10.5414/NP301400","url":null,"abstract":"Histiocytic sarcoma (HS) is a rare malignant neoplasm of macrophage-dendritic cell lineage that can occur at any site. Primary base of skull involvement is exceedingly rare. We present the case of a previously healthy 56-year-old man who complained of headaches and showed localized neurologic symptoms. Magnetic resonance imaging demonstrated a hyperintense and enhancing mass involving the sphenoid bone and the clivus with an extradural component that compressed the distal pons. The differential diagnosis included chordoma or chondrosarcoma. An endoscopic trans-sphenoidal resection was performed. Microscopically, the tumor showed epithelioid and spindle morphology with atypia, mitoses, and necrosis. No osteoid, cartilaginous, or myxoid matrix was identified. By immunohistochemistry, the tumor was positive for CD68 (KP-1) and lysozyme, variably positive for CD4, CD11c, CD14, CD68 (PGM-1), CD45, and CD163, and negative for markers of epithelial, melanocytic, lymphoid, myeloid, muscle, and dendritic cell origin. Expression of PD-L1 by immunohistochemistry and BRAF V600E mutation analysis by PCR were negative. Tumor recurrence developed after radiation treatment with overwhelming progression into a largely infiltrating mass within 2 weeks with clinical deterioration, and the patient died 3 months later. To our knowledge, this represents the first case of primary HS of the clivus reported to date in the English literature, further expanding the spectrum of neoplasms seen at this site as well as the sites where HS can be seen. The overall prognosis of HS in the skull base is poor, with no standard treatment. Further research is warranted to develop effective treatment approaches, which in the future may rely on the expression of checkpoint inhibitors and/or specific molecular markers.","PeriodicalId":55251,"journal":{"name":"Clinical Neuropathology","volume":"41 2","pages":"74-82"},"PeriodicalIF":1.1,"publicationDate":"2022-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39765769","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: 1

Purulent meningitis as complication of acute sinusitis in a patient with attenuated lamina cribrosa. 化脓性脑膜炎作为急性鼻窦炎的并发症，减薄薄层患者。

IF 1.1 4区医学

Clinical Neuropathology Pub Date : 2022-03-01 DOI: 10.5414/NP301416

Pavol Janega, Kristina Mikus Kuracinova, Barbora Kleinova, Jarmila Zelezkovova, Andrea Janegova

引用次数: 0

Clinical Neuropathology 2-2022. 临床神经病理学2-2022。

IF 1.1 4区医学

Clinical Neuropathology Pub Date : 2022-03-01 DOI: 10.5414/NPP41043

Ingeborg Fischer, Johannes A Hainfellner

引用次数: 0