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A consecutive series of 47 hospitalized cases of Guillain-Barré syndrome seen over a 10-year period was analysed with respect to: (1) age and sex; (2) antecedent events and seasonal distribution; (3) patterns of clinical presentation; (4) CSF and neurophysiological findings; (5) results of treatment with plasma exchange; and (6) outcome. Twenty-two were children, 20 middle-aged and 5 aged; 37 were male and 10 were female. The most frequent antecedent event was upper respiratory tract infections; a seasonal peak incidence was found in winter. Clinical, CSF and neurophysiological findings concurred with those in the Western literature; 79% of the cases were severe. Plasma exchange performed within the first 2 weeks of onset benefitted in the short-term outcome, i.e. improvement by 1 grade at 4 weeks, but the long-term benefit, i.e. the ability to regain independent locomotion, was questionable. Plasma exchange helped in curtailing the time to walking unaided but had no benefit on the duration of artificial ventilation. Factors associated with an adverse outcome were: age over 15 years, severity of motor electrodiagnostic findings (especially a decreased distal CMAP amplitude and EMG signs of acute denervation), requirement for ventilation and slow progression (>3 weeks) to maximum deficit. After a mean follow-up of 11 months, 55% of the patients regained independent locomotion, which is a comparatively low proportion.  相似文献   
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A six-months-old girl is presented with psychomotor retardation and multiple congenital malformations. The karyotype done on peripheral blood lymphocytes and skin fibroblasts was found to be 46,XX del(5)(411q13). The parents are consanguineous. Their karyotypes were normal.
The genes for Arylsulphatase B and Hexosaminidase B are not located in band 5q12.  相似文献   
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A direct association between aging and drug-induced dyskinesia has been reported by several investigators. Iminiodipropionitrile (IDPN), a prototype nitrile compound produces a motor syndrome in rodents, which resembles neuroleptic drug induced dyskinesia. In this investigation attempt has been made to study the effect of age on IDPN induced vestibular hair cell degeneration and resulting dyskinetic syndrome. Male Wistar rats aged 3, 6 and 12 weeks received IDPN in the doses of 0, 200 and 400 mg/kg, intraperitoneally for 3 consecutive days. IDPN-induced dyskinesia was assessed using a behavioral testing battery on days 3, 4, 5, 6, 7, 14, 21 and 28. The rats were sacrificed on day 28; temporal bones were excised for vestibular histopathology and sera were collected for measuring the indices of oxidative stress (glutathione and conjugated dienes). IDPN in the dose of 200 mg/kg produced dyskinesia in 12 weeks old rats, but failed to do so in 3 and 6 weeks old rats. The high dose of IDPN (400 mg/kg) caused dyskinesia in all age groups, however, its onset and severity were age-dependent. Older rats showed an early onset and significantly high incidence of dyskinesia as compared to younger rats. The susceptibility of rats to IDPN-induced behavioral deficits was proportional to oxidative stress and degeneration of sensory hair cells in the crista ampullaris.  相似文献   
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The significant advances in our understanding of color vision has been due to the convergence of information from behavioral and molecular genetic analyses. The molecular biology of the visual pigments; molecular genetic basis of variation in normal and abnormal color vision, and regulation of the genes at the LWS-MWS pigment gene locus are discussed.  相似文献   
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A review of the lateral radiographs and CT studies of 114 patients with burst fractures, 46 patients with combined injuries in whom bursting was a major component, and 82 patients with simple anterior compression fractures was performed to evaluate the integrity of the posterior vertebral body margin. This structure normally produces a single or bifid vertical line on the lateral radiograph. Disruption, displacement, or rotation of this line was found in all 114 patients with "pure" burst fractures. These abnormalities were also present in 36 of the 46 patients with combined burst injuries. In all patients with simple compression fracture, flexion, distraction or dislocation, and extension injuries, the line was normal. CT studies showed these abnormalities to be the result of retropulsion of one or more bone fragments from the posterior margin of the vertebral body. Disruptive abnormalities of the posterior vertebral body line are reliable plain-film signs that a burst fracture has occurred and that compromise of the vertebral canal and subarachnoid space is present.  相似文献   
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Mega-Liposuction: Analysis of 1520 Patients   总被引:1,自引:0,他引:1  
Fifteen hundred and twenty cases of liposuction/liposculpture were performed at Dr. M. Erfan & Bagedo Hospitals and King Abdulaziz University Hospital in Jeddah from January 1983 to December 1994. These cases were mostly females. The age group was from 16–65 years. Multiple procedures were performed in 11.68% of these cases. The change in hemoglobin and the hematocrit ratio pre- and post-operatively, and the incidence of complications, were studied. The percentage of surface area operated upon rather than the amount of fat removed was the most important relevant factor.  相似文献   
10.
Intractable hiccup induced by brainstem lesion   总被引:2,自引:0,他引:2  
Four patients with brainstem lesions presented with intractable hiccup and mild to moderate neurological signs. Two of the patients had been initially diagnosed as having a psychogenic cause for their hiccup. Magnetic resonance imaging (MRI) demonstrated brainstem infarction in one case, tuberculoma at the junction of the medulla oblongata and the cervical spinal cord in two, and a vermian tuberculoma compressing the brainstem in one. The brainstem infarct and one of the medullary tuberculoma were not detected on high resolution enhanced computed tomography. The 3 patients with CNS tuberculoma were free of hiccup 1-5 months after antituberculous chemotherapy. It is proposed that hiccup is not an abnormal reflex, but a myoclonus generated by repetitive activity of the "inspiratory solitary nucleus" due to release of higher nervous system inhibitory/-regulatory control. The neuroanatomical network and the mechanisms underlying the formation of intractable hiccup are outlined. The value of MRI in the initial diagnosis and follow-up of patients with intractable hiccup due to brainstem lesions is emphasised.  相似文献   
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