高血糖型偏盲:可逆转的非酮性高血糖并发症

Four patients each developed a reversible homonymous hemianopia caused by non-ketotic hyperglycemia. In two patients the homonymous hemianopia was the first manifestation of diabetes mellitus type 2. All four patients had abnormal MRI scans; in the three patients who had late follow-up scans the abnormalities resolved completely. In one patient the progressive visual field defect and unusual MRI findings initially caused concern for a tumor.

特发性震颤患者运动时间的评估

Background: We have observed mild bradykinesia in essential tremor (ET) patients, which do not satisfy the criteria of Parkinson’s disease (PD). Abstract:Objective: To compare the mean movement time for repetitive movements around distal and proximal joints in ET patients with normal controls by using a simple test paradigm. Patients and methods: Seventeen patients with ET and 14 control subjects were instructed to tap with the index finger sequentially on push-button microswitches. Movement times around metacarpophalangeal, wrist, elbow, and shoulder joints of the right side were tested. The data collected were stored on a computer and the time elapsed between sequential taps on two keys (ms) and number of taps on the left key for 15 seconds were evaluated offline. Results: Movement times of the patients with ET were not found to be significantly different from those of the controls at all joints tested despite slight prolongation for movements around the shoulder joint. Conclusion: The simple test paradigm we have used showed that there is no difference in the movement time for repetitive movements around fourjoints of the upper extremity between patients with ET and normal control subjects. The slightly prolonged movement time around the shoulder joint noted in patients with ET may be ascribed to tremor, not bradykinesia. Tremor may cause these patients to pay more attention to the performance of goal-directed finger movements and consequently prolong movement time slightly or it may simply delay the time elapsed to reach the goal in the absence of overt intention tremor.

复发-缓解型多发性硬化症相关的全身和中枢神经系统细胞免疫差异

In order to elucidate the differences between systemic and central nervous system (CNS) immunity that are relevant to exacerbations of multiple sclerosis (MS), paired peripheral blood and cerebrospinal fluid (CSF) samples obtained from 36 nontreated patients with relapsing-remitting MS (RRMS) were simultaneously examined using flow cytometry to determine the percentages of functional lymphocyte subsets, as well as enzyme-linked immunosorbent assays for measu- ring soluble immune mediators.Active RRMS patients (n = 27) were characterized by an increase in CD4+CXCR3+Th1 cells in blood as compared with inactive patients (n = 9), and this parameter was inversely correlated with plasma levels of IL-10 and IL-12p70. In contrast, an increase in the percentage of CD4+CD25+cells and a decrease in the percentage of CD8+CD11ahigh cells were features of CSF samples from those with active RRMS. Further, CSF CD4+CD25+cells had a close association with leukocyte counts as well as albumin and CXCL10 levels in the CSF, and, thus, could be useful as a measure for inflammatory reactions in the CNS. On the other hand, CD8+CD11ahigh cells may function as immunoregulatory cells, as their percentage in the CSF showed a positive correlation with CSF levels of the anti-inflammatory cytokine IL-4. These findings suggest that MS relapses occur in a combination with altered cell-mediated immunity that differs between the peripheral blood and CSF compartments, while measurement of lymphocyte subsets may be helpful for monitoring disease status.

抗体频率导致的B型肉毒素重新治疗颈部肌张力障碍失败

Botulinum toxin induced therapy failure type B antibody (BT-B, BT-B-AB) has so far only been reported after previous formation of antibodies against botulinum toxin type A (BT-A, BTA-AB).We wanted to explore the risk of BT-B-AB-induced therapy failure in patients who were exposed to botulinum toxin for the first time. For this purpose we followed nine patients with cervical dystonia receiving BT-B (NeuroBloc/Myo BlocTM, Elan Pharmaceuticals) in a dose of 11435 ±2977MU during 4.9 ±.3.0 injection series. All patients showed a satisfactory initial therapeutic response as documented by a Toronto Western Spasmodic Torticollis Rating Scale score reduction from 17.7 ±9.4 to 5.3 ±4.8 and an overall subjective improvement of 56.1 ±28.3%. Seven patients experienced systemic anticholinergic side effects. Five patients had stable therapeutic responses over subsequent injection series. Four patients experienced complete therapy failure with BT-B-AB titres in excess of 10mU/ml on the mouse diaphragm assay. Doubling the last effective BT-B dose produced neither therapeutic effects nor side effects. Subsequent applications of botulinum toxin type A produced a continued therapeutic response in one patient and complete therapy failure in the other. Despite the small sample size a frequency of 44 %indicates a high risk for BT-B-AB-in- duced complete therapy failure. The high amount of neurotoxin administered when NeuroBloc/MyoBlocTM is used might be a contributory factor. Further prospective comparative studies are necessary to monitor the frequency and time course of BT-B-AB formation.

特发性常压脑积水患者脑直径与步态障碍的关系

Background and purpose: Although gait disturbance is an important feature of idiopathic normal pressure hydrocephalus (NPH), only tentative theories have been offered to explain its pathophysiology. It has been suggested that the mesencephalic locomotor region is the anatomical substrate for the development of the hypokinetic NPH gait. To investigate this possibility, we evaluated the correlation between gait disturbance and midbrain diameter. Methods: We enrolled 21 patients with NPH and 20 age-matched control subjects for the study. The maximal diameter of the midbrain and pons, and the width of the lateral and third ventricles were measured using midsagittal T1-weighted MRI and axial T2-weighted MRI, respectively. Gait disturbance, cognitive dysfunction, and incontinence were semiquantified. Results: The maximal midbrain diameter was significantly smaller in the NPH group than in the controls (14.8 ±0.9 vs. 17.1 ±0.7mm, p < 0.001). There were inverse correlations between the midbrain diameter and the widths of the two ventricles (r = -0.562, p = 0.008 for the third ventricle, and r = -0.510, p = 0.018 for the lateral ventricle). The severity of gait disturbance was negatively correlated with the midbrain diameter (r = -0.598, p = 0.004), but the degree of cognitive dysfunction and incontinence showed no significant correlation with midbrain diameter or ventricular width. Conclusions: This study suggests that midbrain atrophy is significantly associated with gait disturbance in NPH.

肌萎缩性侧索硬化症患者的功能性运动代偿

The present study investigated the fMRI correlates of functional compensation/neural reorganization of the motor system in patients with amyotrophic lateral sclerosis (ALS). The hypothesis was that ALS patients would recruit additional brain regions compared with controls in a motor task and that activity in these regions would vary as a function of task difficulty. Patients and controls executed a motor task with two sequences (a simple and a more difficult one) of consecutive button presses. Patients and controls both activated brain regions known to be involved in motor execution and control. Activity in ipsilateral motor areas as well as difficulty-related activity in the left cerebellum could only be observed in patients. The behavioral data indicated that the motor task was much more difficult for patients than for controls. At nearly equal difficulty the observed patterns of hemodynamic activity in controls were very similar to those observed in ALS. The findings suggest that functional compensation in ALS relies on existing resources and mechanisms that are not primarily developed as a consequence of the lesion.