Perirhinal accumulation of neuronal alpha ‐synuclein in a multiple system atrophy patient with dementia
We report the case of a 79‐year‐old Japanese woman who developed cerebellar ataxia followed by rigidity, dysautonomia and cognitive disorders, and was thus clinically diagnosed as having possible MSA with dementia. Neuropathological findings demonstrated not only olivopontocerebellar and striatonigral degeneration with frequent glial cytoplasmic inclusions (GCIs), but also degenerative changes in the parahippocampal region, accentuated in the anterior portion of perirhinal cortex, where neuronal cytoplasmic inclusions (NCIs) and NFTs were numerous while GCIs were limited. NCIs were frequent in the deep layer, whereas N...
Source: Neuropathology - April 1, 2017 Category: Neurology Authors: Mari Saito, Makoto Hara, Momoko Ebashi, Akihiko Morita, Kyoko Okada, Taku Homma, Masahiko Sugitani, Kentaro Endo, Toshiki Uchihara, Satoshi Kamei Tags: Case Report Source Type: research

MM1 ‐type sporadic Creutzfeldt‐Jakob disease with 1‐month total disease duration and early pathologic indicators
A 62‐year‐old man presented with abnormal behavior and cognitive impairment. Diffusion‐weighted images (DWI) obtained on MRI showed extensive hyperintense regions in the cerebral cortex and striatum. Myoclonus was recognized, and the patient died 1 month after the onset; his condition did not reach the akinetic mutism state. The brain weighed 1300 g and showed no apparent atrophy. Extensive spongiform changes were observed in the cerebral neocortex, striatum, thalamus and cerebellar cortex, but gliosis was mild or absent. Neuropil rarefaction and neuron loss were not apparent. Mild proliferation of anti‐ GFAP‐p...
Source: Neuropathology - April 1, 2017 Category: Neurology Authors: Yasushi Iwasaki, Hiroko Kato, Tetsuo Ando, Maya Mimuro, Tetsuyuki Kitamoto, Mari Yoshida Tags: Case Report Source Type: research

Basal subarachnoid hemorrhage by rupture of arteriovenous malformation at the cerebellopontine angle
A man in his late forties had lived as a recluse for more than ten years. He was found dead in his room. At autopsy, subarachnoid hemorrhage (SAH) was detected at the base of the brain, which weighed 1333 g. The cerebellar tonsil was swollen. The cerebral ventricle was enlarged and filled with blood. A hematoma was observed in the upper part of the left side of the cerebellar hemisphere. The location and size of SAH in this case indicated that the rupture of a cerebral aneurysm (CA) had occurred; however, CA was not detected. A mass of blood vessels buried in the hematoma was observed at the left cerebellopontine angle (C...
Source: Neuropathology - April 1, 2017 Category: Neurology Authors: Mio Takayama, Masayuki Kashiwagi, Kenji Hara, Aya Matsusue, Brian Waters, Natsuki Ikematsu, Shin ‐ichi Kubo Tags: Case Report Source Type: research

Extracellular matrix in the CNS induced by neuropathogenic viral infection
During the early phase of infection with an extremely neurovirulent murine coronavirus, cl‐2, the ER‐TR7 antigen (ERag)‐positive fibers (ERfibs) associated with laminin and collagen III show a rapid increase in expression levels in the meninges, followed by an appearance of the antigens in the ventricle and brain parenchyma. Then, cl‐2 invades the ventricle and ventricular wall along the newly assembled ERfibs after infection, using them as a pathway from the meninges, the initial site of infection. In the lymph nodes and spleen, ERag is mainly produced by fibroblastic reticular cells (FRCs), which play a key role ...
Source: Neuropathology - March 19, 2017 Category: Neurology Authors: Rihito Watanabe, Masatoshi Kakizaki Tags: Original Article Source Type: research

Pigmented intraventricular tumor in a young adult
(Source: Neuropathology)
Source: Neuropathology - March 8, 2017 Category: Neurology Authors: Kirti Gupta, Balan Louis Gaspar, Pravin Salunke, Charan Singh Rayat Tags: Neuropathology Education Source Type: research

Pathology of toxic leucoencephalopathy in drug abuse supports hypoxic ‐ischemic pathophysiology/etiology
The histopathological features of leucoencephalopathy caused by illicit drugs (such as opioids and cocaine) are well documented in acute cases but not in long‐survival cases. There are several hypotheses about the pathogenesis of this disorder, including hypoperfusion, direct drug toxicity resulting from the neurotoxic effects of the drug itself or contaminants in the illicit drug vehicle. We reviewed the post mortem findings in five males (aged 24 to 56 years, with survival intervals ranging from 7 days to 5 months) with a history of illicit drug use and concomitant fatal white matter changes. The histological charac...
Source: Neuropathology - March 8, 2017 Category: Neurology Authors: Murad Alturkustani, Lee ‐Cyn Ang, David Ramsay Tags: Original Article Source Type: research

Intraventricular gliosarcoma with dual sarcomatous differentiation: A unique case
Gliosarcoma, a variant of isocitrate dehydrogenase‐wildtype glioblastoma, is largely a lobar surfacing neoplasm often with dural attachment. In this biphasic neoplasm, the sarcomatous component usually takes the form of fibrosarcoma or malignant fibrous histiocytoma. Heterologous sarcomatous differentiation is a rare phenomenon. Here, we present a case of gliosarcoma with liposarcomatous and myosarcomatous differentiation in a 68‐year‐old man which was purely intraventricular. This is the first report of such a morphologic pattern in this location. Varied histological components with their immunohistochemical profile...
Source: Neuropathology - March 5, 2017 Category: Neurology Authors: Rajalakshmi Poyuran, Nandeesh bn, Yerasi Varun Kumar Reddy, Amey R. Savardekar Tags: Case Report Source Type: research

Comparison of AT8 immunoreactivity in the locus ceruleus and hippocampus of 154 brains from routine autopsies
In this study, we could not confirm that phospho‐tau pathologies begin in the LC. We suspect their simultaneous occurrences in both hippocampal regions and in LC. (Source: Neuropathology)
Source: Neuropathology - February 5, 2017 Category: Neurology Authors: Koichi Okamoto, Masakuni Amari, Toshio Fukuda, Keiji Suzuki, Masamitsu Takatama Tags: Original Article Source Type: research

Intravascular T ‐cell lymphoma: A rare, poorly characterized entity with cytotoxic phenotype
In conclusion, this abnormal population of cytotoxic T‐cells with intravascular localization probably represents a specific type of T‐cell lymphoma with specific clinical, radiologic, molecular and immunophenotypic characteristics. (Source: Neuropathology)
Source: Neuropathology - January 31, 2017 Category: Neurology Authors: Tara L. Sharma, Gabrielle A. Yeaney, Payam Soltanzadeh, Yuebing Li, Claudiu V. Cotta Tags: Case Report Source Type: research

T cell lymphoblastic lymphoma/leukemia within an adrenocorticotropic hormone and thyroid stimulating hormone positive pituitary adenoma: A cytohistological correlation emphasizing importance of intra ‐operative squash smear
We present a rare case of primary pituitary T cell lymphoma/leukemia (T‐LBL) in association with adrenocorticotropic hormone (ACTH) and thyroid stimulating hormone (TSH) expressing pituitary adenoma in a 55‐year‐old woman highlighting the importance of intra‐operative squash smears examination. The patient presented with complaints of headache, diminution of vision and recent onset altered sensorium. MRI revealed a mass lesion in the sellar‐suprasellar region with non‐visualization of pituitary gland separately, extending to involve adjacent structures diagnosed as invasive pituitary macroadenoma. Intra‐opera...
Source: Neuropathology - January 31, 2017 Category: Neurology Authors: Rakesh K. Gupta, Ravindra K. Saran, Arvind K. Srivastava, Anita Jagetia, Lalit Garg, Mehar C. Sharma Tags: Case Report Source Type: research

A case of symptomatic granular cell tumor of the sellar region with large calcification
We report here a rare case of symptomatic granular cell tumor (GCT) of the sellar region with a large calcification. A 70‐year‐old woman presented with a sellar mass, accompanied by bitemporal hemianopia. The patient was diagnosed preoperatively as having a craniopharyngioma or a pituitary adenoma, because of the large calcification. The patient underwent surgical tumor resection via endoscopic trans‐sphenoidal surgery and was diagnosed pathologically as having GCT. The patient's postoperative course was uneventful and her visual field disturbance improved soon after the operation. We briefly discuss the pathological...
Source: Neuropathology - January 31, 2017 Category: Neurology Authors: Shun Yamamuro, Taku Homma, Yuya Hanashima, Sodai Yoshimura, Hideki Oshima, Sumie Ohni, Yoshiaki Kusumi, Atsuo Yoshino Tags: Case Report Source Type: research

SAMP8 mice as a neuropathological model of accelerated brain aging and dementia: Toshio Takeda's legacy and future directions
Senescence accelerated mice P8 (SAMP8) show significant age‐related deteriorations in memory and learning ability in accordance with early onset and rapid advancement of senescence. Brains of SAMP8 mice reveal an age‐associated increase of PAS‐positive granular structures in the hippocampal formation and astrogliosis in the brain stem and hippocampus. A spongy degeneration in the brain stem appears at 1 month of age and reaches a maximum at 4‐8 months. In addition, clusters of activated microglia also appear around the vacuoles in the brain stem. β/A4(Aβ) protein‐like immunoreactive granular structures are obse...
Source: Neuropathology - January 31, 2017 Category: Neurology Authors: Ichiro Akiguchi, Merc è Pallàs, Herbert Budka, Haruhiko Akiyama, Masaki Ueno, Jingxian Han, Hideo Yagi, Tomohumi Nishikawa, Yoichi Chiba, Hiroshi Sugiyama, Ryoya Takahashi, Keiko Unno, Keiichi Higuchi, Masanori Hosokawa Tags: Occasional Review Source Type: research

α‐synuclein astrogliopathy: A possible specific feature in α‐synucleinopathy
(Source: Neuropathology)
Source: Neuropathology - January 31, 2017 Category: Neurology Authors: Shunsuke Koga, Koji Kasanuki, Dennis W. Dickson Tags: Letter to the Editor Source Type: research

A case of high ‐grade astrocytoma with BRAF and ATRX mutations following a long‐standing course over two decades
Pediatric high‐grade gliomas are rare and occasionally hard to classify. These tumors often feature a well‐demarcated histology and are expected to have a better outcome than ordinary diffuse high‐grade gliomas in adults. We herein report a case of circumscribed high‐grade glioma that showed a distinct molecular profile and followed an excellent course for 26 years. The patient, a 3‐year‐old boy at onset, presented with a contrast‐enhancing mass in the right temporal lobe and underwent resection. Histologically, the tumor mainly consisted of compact bundles of GFAP‐positive spindle cells. With its malignan...
Source: Neuropathology - January 31, 2017 Category: Neurology Authors: Satoshi Nakata ( 中田 聡), Keishi Horiguchi, Shogo Ishiuchi, Yuhei Yoshimoto, Seiji Yamada, Sumihito Nobusawa, Hayato Ikota, Junko Hirato, Hideaki Yokoo Tags: Case Report Source Type: research

Intracranial neuromuscular choristoma: Report of a case with literature review
Neuromuscular choristoma (NMC), also called neuromuscular hamartoma or nerve rhabdomyoma, is a rare lesion of the spinal and cranial nerves composed of skeletal muscle intimately associated with nerve fibers. Its origin has not been precisely clarified and a malformative event, resulting from aberrant differentiation or a true neoplastic growth, have been proposed by authors. We hereby present a cerebellopontine angle NMC enlarging the eighth cranial nerve in a 3‐year‐old child, that histologically appeared composed of a large amount of striated muscle mixed with nerve fibers. We also provide a review of the intracrani...
Source: Neuropathology - January 31, 2017 Category: Neurology Authors: Antonella Coli, Mariangela Novello, Gianpiero Tamburrini, Manila Antonelli, Felice Giangaspero, Libero Lauriola Tags: Case Report Source Type: research