Pituitary atypical teratoid rhabdoid tumor in a patient with prolactinoma: A unique description
Atypical teratoid rhabdoid tumor (ATRT) is an aggressive tumor of the CNS and characteristically occurs in the pediatric age. In adulthood, ATRT is rare and it is mainly localized in the cerebral hemispheres. Only 16 cases of ATRT have been described in the sellar region up to now. Interestingly, all sellar ATRTs occurred in adult female patients. Herein we report a novel case of sellar ATRT in a patient with previous history of lactotroph adenoma. Similar to other sellar ATRTs, this case occurred in a female adult patient. At histological examination, it was characterized by a small number of rhabdoid cells. In addition, ...
Source: Neuropathology - November 6, 2017 Category: Neurology Authors: Valeria Barresi, Simona Lionti, Alessandro Raso, Felice Esposito, Salvatore Cannav ò, Filippo F. Angileri Tags: Case Report Source Type: research

Primary central nervous system extranodal nasal ‐type natural killer/T‐cell lymphoma with CD20 expression
Neuropathology, EarlyView. (Source: Neuropathology)
Source: Neuropathology - October 24, 2017 Category: Neurology Source Type: research

The perivascular microenvironment in Epstein –Barr virus positive primary central nervous system lymphoma: The role of programmed cell death 1 and programmed cell death ligand 1
Neuropathology, EarlyView. (Source: Neuropathology)
Source: Neuropathology - October 24, 2017 Category: Neurology Source Type: research

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Neuropathology, Ahead of Print. (Source: Neuropathology)
Source: Neuropathology - October 24, 2017 Category: Neurology Source Type: research

Amyotrophic lateral sclerosis and parkinsonism ‐dementia complex of the Hohara focus of the Kii Peninsula: A multiple proteinopathy?
Neuropathology,Volume 38, Issue 1, Page 98-107, February 2018. (Source: Neuropathology)
Source: Neuropathology - October 23, 2017 Category: Neurology Source Type: research

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Neuropathology,Volume 38, Issue 1, Page 98-107, February 2018. (Source: Neuropathology)
Source: Neuropathology - October 23, 2017 Category: Neurology Source Type: research

Amyotrophic lateral sclerosis and parkinsonism ‐dementia complex of the Hohara focus of the Kii Peninsula: A multiple proteinopathy?
The high incidence of amyotrophic lateral sclerosis (ALS) and parkinsonism‐dementia complex (PDC) has been previously known in the Kii Peninsula of Japan and in Guam. Recently, the accumulation of various proteins, such as tau, trans‐activation response DNA binding protein 43 kDa (TDP‐43), and alpha‐synuclein (αSyn), was reported in the brains of patients with ALS/PDC in Guam. To confirm whether similar findings are present in Kii ALS/PDC, we neuropathologically examined the brains and spinal cords of 18 patients with ALS/PDC (clinical diagnoses: eight ALS and 10 PDC) in Hohara Village, which is the eastern focu...
Source: Neuropathology - October 23, 2017 Category: Neurology Authors: Maya Mimuro, Mari Yoshida, Shigeki Kuzuhara, Yasumasa Kokubo Tags: Symposium: Comorbid pathologies of neurodegenerative diseases Source Type: research

Combined morphological, immunohistochemical and genetic analyses of medulloepithelioma in the posterior cranial fossa
Neuropathology, EarlyView. (Source: Neuropathology)
Source: Neuropathology - October 3, 2017 Category: Neurology Source Type: research

Phenotype variability and histopathological findings in patients with a novel DNM2 mutation
Neuropathology,Volume 38, Issue 1, Page 34-40, February 2018. (Source: Neuropathology)
Source: Neuropathology - October 3, 2017 Category: Neurology Source Type: research

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Neuropathology, Ahead of Print. (Source: Neuropathology)
Source: Neuropathology - October 3, 2017 Category: Neurology Source Type: research

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Neuropathology,Volume 38, Issue 1, Page 34-40, February 2018. (Source: Neuropathology)
Source: Neuropathology - October 3, 2017 Category: Neurology Source Type: research

Phenotype variability and histopathological findings in patients with a novel DNM2 mutation
We describe a young man manifesting as length‐dependent sensorimotor neuropathy with hypertrophic cardiomyopathy, but his mother only had very mild symptoms of peripheral neuropathy. The electrophysiological data meet the criteria of intermediate CMT. The main pathological findings of sural nerve biopsy reveal a severe loss of large myelinating fibers and some clusters of regenerative fibers in fascicles, which are consistent with an axonal neuropathy. However, myopathological changes show a chronic myopathy‐like pattern characterized by great variations of fiber size, increased connective tissue, rimmed vacuoles and p...
Source: Neuropathology - October 3, 2017 Category: Neurology Authors: Shuyun Chen, Ping Huang, Yusen Qiu, Qian Zhou, Xiaobing Li, Min Zhu, Daojun Hong Tags: Original Article Source Type: research

Seronegative granulomatosis with polyangiitis presenting with multiple cranial nerve palsies
Granulomatosis with polyangiitis (GPA), previously known as Wegener’s granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti‐neutrophil cytoplasmic antibody (ANCA)‐negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. This patient was a 50‐year‐old man whose first manifestation was multiple neurologic defects without respiratory or renal...
Source: Neuropathology - October 1, 2017 Category: Neurology Authors: Eunhye Lee, Jynkyun Park, Seung Hong Choi, Sung ‐Hye Park Tags: Case Report Source Type: research