Apolipoprotein E and clusterin inhibit the early phase of amyloid-β aggregation in an in vitro model of cerebral amyloid angiopathy

Sporadic cerebral amyloid angiopathy (CAA) is characterized by cerebrovascular amyloid-β (Aβ) deposition, which leads to lobar hemorrhage and dementia. Biological molecules affecting the development of CAA have not been fully characterized. In this study, we performed proteome analysis of biopsied leptomeningeal and cortical vessels obtained from 6 CAA patients and 5 non-CAA patients who underwent surgery for large lobar hemorrhages. We found that 6 proteins, including Aβ, apolipoprotein E (apoE), clusterin (CLU), albumin, complement C4 and vitronectin were significantly upregulated in the vessels of CAA patients as compared to non-CAA patients. ApoE and CLU were found in all CAA patients. We next examined the effects of apoE and CLU on the early phase of Aβ aggregation, using a simple yet powerful in vitro model of CAA, which recapitulates the intramural periarterial drainage pathway model. We found that physiological concentrations of apoE and CLU delayed the initiation time of amyloid growth kinetics in a concentration-dependent manner. These data indicate that apoE and CLU may act as extracellular chaperones to inhibit Aβ amyloid deposition in CAA

Effect of the Intrathecal Baclofen Screening Test on the Spatiotemporal Gait Motion Parameters of Patients with Cervical Spinal Cord Injuries Who Exhibited Diffuse Spasticity: A Report of Three Cases

We examine the quantitative changes in the gait motion of patients with cervical spinal cord injuries (CSCIs) before and after the intrathecal baclofen (ITB) screening test. The subjects were three patients with CSCI, who exhibited spasticity in the lower extremities. They could all walk 10 or more meters with/without aids. All patients were subjected to the ITB screening test, in which they had gabalon (50 μg) injected into their spinal column via paramedian puncture at the L3–4 level. The subjects had their ankle clonus; patellar tendon reflex; and modified Ashworth scale, Berg balance scale, Spinal Cord Independence Measure, and 10-meter walk test (10MWT) assessed before and 5 hours after the ITB screening test. At 5 hours after the ITB screening test, all of the patients exhibited decreased spasticity in static position, and improved balance. There were no differences in the abilities of any of the patients to perform ADL. One patient did not change the spatiotemporal gait motion parameters (walking time, step count, and step length in the 10MWT). Therefore, the pomp implantation for ITB therapy was not performed. Two patients who had suffered CSCI more than 20 years ago exhibited a reduced walking time, increased step count, and step length. Out of the two patients one received the pomp of implantation after ITB screening test, and the other was planned to operate. The spatiotemporal gait motion parameters might be one of the useful tests to decide the pomp implantation for CSCI patients who hope improvement of gait ability

Usefulness of endoscopic third ventriculostomy for hydrocephalus from pineal lesion meningioma in an elderly patient: A case report

Endoscopic third ventriculostomy (ETV) is a major treatment procedure for obstructive hydrocephalus in children and adults. However, previous studies to predict the outcome of ETV, such as ETV success score, were primarily based on pediatric populations; hence, the effectiveness of ETV in elderly populations remains unclear. Here, we report the case of an 80-year-old woman with cognitive impairment and gait disturbance due to obstructive hydrocephalus caused by a pineal region meningioma. Considering that the radical resection was risky for this patient, simultaneous endoscopic tumor biopsy and ETV was performed. Based on the histopathological findings, the patient was diagnosed with meningioma. Cognition improved and gait disturbances diminished gradually 2 months after the operation. Surgical treatments for the elderly patients should not compromise activity of daily living (ADL). Our findings might help the management of elderly patients with obstructive hydrocephalus, especially in aging societies

Astroblastoma: Immunohistochemical and ultrastructural study of distinctive epithelial and probable tanycytic differentiation

We report the clinicopathological findings of astroblastoma found in an 8-year-old girl who was subsequently treated for 11 years. The primary superficially circumscribed tumor was located in the frontoparietal lobe, while the recurrent and the second recurrent tumor were restricted to the same region 11 years later. The tumors obtained on these three occasions showed fundamentally the same histological, immunohistochemical and fine structural features. They exhibited astrocytic as well as ependymal tanycytic features with apparent epithelial cell lineage. The tumor cells showed typical features of astroblastoma comprising prominent perivascular pseudorosettes with remarkable vascular sclerosis. The immunohistochemical study revealed intensive positivity of GFAP, vimentin, epithelial membrane antigen (EMA), cytokeratin, connexin 26 and 32, desmocollin 1 and neuronal cadherin. The fine structure revealed divergent types of junctional complexes, some of which were connected with tonofilament bundles. Numerous microvilli protruded and basal lamina abutted on the tumor cell surface. We report these unique histological features, and stress that astroblastoma should be categorized as a specific type of neuroepithelial tumor