The homozygous c.14576G>A variant in RNF213 could be a good DNA biomarker for predicting the severe type of MMD, for which early medical/surgical intervention is recommended, and may provide a better monitoring and prevention strategy.
Collapsin response mediator protein 1 (CRMP1) and CRMP2 have been known as mediators of extracellular guidance cues such as semaphorin 3A and contribute to cytoskeletal reorganization in the axonal pathfinding process. To date, how CRMP1 and CRMP2 focally regulate axonal pathfinding in the growth cone has not been elucidated. To delineate the local functions of these CRMPs, we carried out microscale-chromophore-assisted light inactivation (micro-CALI), which enables investigation of localized molecular functions with highly spatial and temporal resolutions. Inactivation of either CRMP1 or CRMP2 in the neurite shaft led to arrested neurite outgrowth. Micro-CALI of CRMP2 in the central domain of the growth cones consistently arrested neurite outgrowth, whereas micro-CALI of CRMP1 in the same region caused significant lamellipodial retraction, followed by retardation of neurite outgrowth. Focal inactivation of CRMP1 in its half region of the growth cone resulted in the growth cone turning away from the irradiated site. Conversely, focal inactivation of CRMP2 resulted in the growth cone turning toward the irradiated site. These findings suggest different functions for CRMP1 and CRMP2 in growth cone behavior and neurite outgrowth.
Plasma cell myeloma (PCM) of the skull base is rarely encountered in neurosurgical practice. PCM has a wide spectrum of pathology, including a quite benign, solitary plasmacytoma (SPC), and an extremely malignant, multiple myeloma (MM) at the two ends of the spectrum. We have described two patients with PCM of the skull base, of which one harbored SPC, and the other progressed to MM. In case 1, a 46-year-old man presented with left multiple cranial nerve impairments and had a large skull base tumor. Subtotal removal was performed. The specimen and general examination, including bone marrow aspiration, revealed SPC. Postoperatively 50Gy of external radiotherapy was administered. The patient has no manifestation of MM 24 months after the initial presentation. In case 2, a 53-year-old woman presented with left abducens palsy and had a left petroclival osteolytic mass. Gross total resection was performed. The specimen revealed a plasmablastic tumor, i.e., myeloma. General examination established the diagnosis of MM. She was administrated adjuvant chemotherapy and autologous bone marrow transplantation. She is alive without local recurrence 30 months after the initial presentation.
Haemodynamic changes in cerebral circulation are associated with the natural ageing process and associated pathology, leading to the development of incapacitating neurological and neurovascular diseases. Due to inherent biological limitations, current literatures mostly aimed at studying the correlation descriptively or quantifying the relationship in vitro or using computational models. In this paper, a model of a carotid-jugular fistula in the rat was used to create a haemodynamic insult to the intracranial arterial circulation and subsequent venous drainage. An arterial-venous (AV) fistula was created in 12 rats, 6 of which are normotensive Wistar-Kyoto strain (WKY) and the rest spontaneously hypertensive strain (SHR) with an additional 6 in each strains designed as controls without previous surgery. After 4 weeks of convalescence, all 24 rats were euthanised and their cerebral circulation was examined histomorphologically. We confirmed an intrinsic morphological difference between normotensive WKY and hypertensive SHR and found a modest but significant arterial shrinkage in both strains induced with AV fistula. We also reported that alterations in blood flow are also associated with marked extracellular matrix changes. We concluded that the model was suitable for studying the relative contributions of altering haemodynamic patterns and venous drainage on cerebrovascular changes. We also found that hypertension modulated cerebral vascular changes in addition to disrupted blood flow.
Summary: We report a rare case of dissecting aneurysm of the posterior inferior cerebellar artery (PICA) with vascular variation. A 38-year-old man, who had no medical, familial or traumatic histories, presented with headache, nausea and mild consciousness disturbance.Computed tomography (CT) imaging demonstrated subarachnoid hemorrhage with intraventricular hemorrhage. Angiography disclosed an aneurysm of left PICA, which did not involve the vertebral artery. The PICA was well developed and widely perfused the cerebellum and lower brain stem, which was accompanied by lack of ipsi-lateral anterior inferior cerebellar artery (AICA) and contra-lateral PICA. The aneurysm was successfully treated by trapping and revascularization procedure with occipital artery (OA)-PICA anastomosis.We suggest that congenital vascular variation related to well-developed PICA might lead to dissecting aneurysm, and that direct surgical treatment with bypass would be indicated for such patients to preserve hemodynamic integrity.
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