There is an unusual vascular network at the base of the brain in patients with moyamoya disease. We detected various histologic lesions in the perforating arteries of 22 patients. Vessels showing rupture ranged from 50 to 530 microns in diameter; they were dilated, some had fibrin deposits in the wall, fragmented elastic laminae and attenuated media. Non-ruptured perforating arteries (diameter 200 to 550 microns) revealed microaneurysm formation, focal fibrin deposits and marked attenuation of the wall thickness with diminution of the elastic lamina. These changes seem to predispose to rupture of perforating arteries. Stenotic changes such as fibrous intimal thickening, collapse of the lumen and thrombosis were detected in 14 out of 22 cases. Morphometric analysis of perforating arteries indicated that arteries showing extreme degrees of stenosis or dilatation were more frequent in the patients with moyamoya disease than in the control cases. Dilative arteries were more frequent in the young patients and stenotic vessels were, in contrast, less frequent in the young patients.

In the past 10 years, the authors performed microsurgical evacuation of hypertensive intracerebral hematoma in 100 cases during the ultra-early stage (within 7 hours) after the apoplectic attack. Operative indications were the presence of obvious hemiplegia and disturbed consciousness (from stupor to semicoma). Functional outcomes at 6 months postoperatively were as follows: 15 patients had returned to a full social life, 35 were capable of self-care at home, 33 required partial care at home, two were bedridden and in a vegetative state, and seven had died.

A portal vein anomaly associated with a rightward-deviated ligamentum teres is an important clinical entity that is not as rare as indicated in the literature. This anomaly should be kept in mind during preoperative examination of the liver.

Gait disturbance in idiopathic normal pressure hydrocephalus (iNPH) is reminiscent of parkinsonism. Our recent PET study showed reduction in postsynaptic D 2 receptor binding concomitant with a normality of presynaptic dopamine transporter binding. Here, we investigated the plasticity of D 2 receptor in treating iNPH patients with ventriculoperitoneal (VP) shunting using PET with 11 C-raclopride and discuss the contribution of D 2 receptor to the pathophysiology of iNPH. Methods: Eight iNPH patients participated in this study. After evaluation of their neuropsychologic abilities, all patients underwent 3-dimensional MRI and quantitative PET measurements twice before and 1 mo after VP shunting. MRI-based morphometric analyses were performed to examine postoperative variations of the ventricles. Estimation of binding potential (BP) for 11 C-raclopride was based on Logan plot analysis. Region-of-interest analysis was used to examine changes in 11 C-raclopride BP in the striatum. A 2-tailed paired t test was used for evaluating changes in PET and MRI parameters between conditions, and correlation analysis was used to investigate clinicopathophysiologic relevance (clinical vs. in vivo findings). Results: Clinical evaluation revealed significant recovery in a 5-m back-and-forth navigation test and an affect test and a mild increase in Mini-Mental State Examination scores after VP shunting. Significant postoperative increases in 11 Craclopride BP were found in the nucleus accumbens and dorsal putamen, and the increases were significantly associated with emotional (Spearman rank r 5 0.66, P , 0.05) and navigational improvement (r 5 0.72, P , 0.05), respectively. The 11 C-raclopride BP increase in the striaum as a whole correlated significantly with improvement in general cognitive ability. There was a mild ventricular shrinkage after surgery, albeit there was no correlation of its size with clinical and PET parameters. Conclusion: Striatal upregulation of D 2 receptor after VP shunting is associated with amelioration of hypokinetic gait disturbance and anhedonic mentation in iNPH patients, indicating that the effect of VP shunting may reside in noninhibition of functionally suppressed D 2 receptor in the striatum. D 2 receptor responsiveness may indicate a mechanism for iNPH pathophysiology. The clinical triad for idiopathic normal pressure hydrocephalus (iNPH) consists of gait disturbance, progressive dementia, and urinary incontinence (1), which develop insidiously without causative disorders (2). In the clinical setting, gait disturbance is likely the first sign and important symptom in NPH (3). However, this hypokinetic type of gait disturbance is not unique in other neurologic diseases such as Parkinson's disease (PD) and dementia with extrapyramidal symptoms. To diagnose iNPH, a spinal tap is considered prerequisite, and empirically its effect on gait improvement is the most remarkable (4). Our previous study highlighted a close relationship of gait impairment with putaminal D 2 receptor downregulation in iNPH (5)...

Thirty-seven small thalamic hemorrhages (less than 2 cm) were classified into four types depending on topographic location. Patients with posterolateral lesions had severe sensory and motor disability as well as the worst prognosis. Anterolateral lesions resulted in mild prefrontal signs with milder sensory and motor impairment. Medial hematomas disturbed consciousness in the acute stage, followed by impaired prefrontal signs of long duration. Dorsal hematomas were associated with ipsilateral parieto-occipital signs (aphasia on the left and topographic memory disturbance on the right).

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We here show that the epitope-tagged human TSH receptor (TSHRmyc) is covalently modified with palmitic acid by thioesterification. Side-directed mutagenesis identified Cys699 in the C-terminal cytoplasmic tail of the receptor as the putative palmitoylation site. Mutation of Cys699 to Ala results in the nonpalmitoylated receptor (TSHRmycC699A) in which high affinity TSH binding, Gs coupling, homologous desensitization and TSH-induced internalization are unaffected. In contrast, abolition of palmitoylation appears to decrease the rate of the intracellular trafficking of the receptor. However, since most of TSHRmycC699A seems to be fully processed finally and the receptor number of TSHRmycC699A on the cell surface is comparable to that of TSHRmyc, our results suggest that abolition of palmitoylation delays the cell surface expression of TSHR, but does not trap the receptor intracellularly, although another possibility for proteolytic degradation of either the 95 kDa or the 100 kDa mutant receptor can not be excluded. Thus, post-translational modification of TSHR by palmitoylation may provide a novel mechanism of enhancing the rate of intracellular trafficking of the receptor.

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