Benefit of bilateral pallidotomy in the treatment of generalized dystonia. Case report

This 29-year-old man with cerebral palsy complicated by generalized dystonia was treated by simultaneous bilateral posteroventral pallidotomy. Postoperatively, there was slow, but steady, improvement in the patient's dystonia and disability. However, the improvement in abnormal movements was only prominent for cervical dystonia and oromandibular dyskinesia. The patient's Burke-Fahn-Marsden dystonia scores were 51 preoperatively and 37, 33.5 and 33.5, at 3, 6, and 12 months postoperatively, respectively, demonstrating a maximum improvement of 34%. These results suggest that pallidotomy can be an alternative therapy for those patients suffering from intractable generalized dystonia.     

Inhibitory effect of dimethyl sulfoxide on nuclear factor-kappa B activation and intercellular adhesion molecule 1 gene expression in septic rats

Antioxidants are potent radical scavengers that protect against endotoxemia and septic shock in animal models. Using a rat model of peritonitis sepsis induced by cecal incision we studied the effect of the free radical scavenger dimethyl sulfoxide (DMSO) on hepatic nuclear factor kappa B (NF-kappa B) activation, hepatic intercellular adhesion molecule 1 (ICAM-1) gene expression, serum tumor necrosis factor alpha (TNF) formation, and serum glucose concentration. Five groups of rats (N = 5) were treated as follows: (1) untreated control (Untreated), (2) sham operated with laparotomies (Sham), (3) pretreated with 6 ml/kg DMSO followed by sham operation (DMSO/Sham), (4) cecal incision (Sepsis), and (5) pretreated with DMSO followed by cecal incision (DMSO/Sepsis). In the DMSO/Sham group, DMSO did not affect NF-kappa B activation, ICAM-1 gene expression, and TNF formation. At 3 h postcecal incision, DMSO inhibited sepsis-induced hepatic NF-kappa B activation and hepatic ICAM-1 gene expression to control levels and suppressed serum TNF by 75%. In the late (6 h) septic phase, DMSO inhibited NF-kappa B activation (32%), ICAM-1 gene expression (27%), and TNF formation (71%). These findings suggest that the protective mechanism of antioxidants in septic rats may be partly due to the inhibition of NF-kappa B activation and NF-kappa B-mediated events.     

Regeneration of neuronal nitric oxide synthase (nNOS)-containing nerve fibers in rat corpus cavernosum

Aim: To investigate the effect of cavernous nerve injury on the nNOS-containing nerve fibers in rat corpus cavernosum.Methods: Thirty-three male SD rats were randomized into 3 groups: 5 rats underwent pelvic exploration without tran-section of cavernous nerve as the sham-operated controls, the unilateral injury group (14 rats) had the cavernous nerve cuton one side, and the bilateral injury group (14 rats) had the nerves cut on both sides. Corpora cavernosa were harvestedat the 3rd week and 6th month after surgery, nNOS-positive nerve fibers were examined with strepavidin peroxidase im-munohistochemistry techniques (SP method). Results: After bilateral ablation, the nNOS-positive nerve fibers weresignificantly decreased at both the 3rd week ( 17 ± 4) and the 6th month (16 ± 4). For the unilateral injury group, thenNOS-positive nerve fibers were similarly decreased on the side of the neurotomy at the 3rd week (18 ± 6), but by the 6thmonth, the number increased significantly (61±9) and approximated th     

Reconstruction of longitudinal stability of the forearm after disruption of interosseous ligament and radial head excision (Essex-Lopresti lesion)

We have presented a technique for reconstruction of the central band of the interosseous membrane in conjunction with surgical repair of the distal radial joint and radial head prosthesis. With this technique, we address all three anatomic structures that provide longitudinal stability of the forearm, specifically, (1) radial head replacement, (2) interosseous membrane reconstitution, and (3) TFCC repair.     

No mutation of G209A in the alpha-synuclein gene in sporadic Parkinson's disease among Taiwan Chinese

The role of genetics in Parkinson's disease (PD), previously controversial, is now supported by several studies. A major breakthrough has been the discovery of a single gene defect in familial Parkinson's disease. A single base pair change at position 209 from G to A (G209A) in the fourth exon of the alpha-synuclein gene has been identified in cases of familial PD. We looked for this mutation in 65 cases of sporadic PD in Taiwan Chinese patients but found none of these patients with this mutation. We conclude that mutation of G209A in the alpha-synuclein gene plays no role in sporadic PD among Taiwan Chinese.     

Hemiballismus-hemichorea in older diabetic women: a clinical syndrome with MRI correlation

Eight older women from two different continents, all with nonketotic hyperglycemia, presented with hemiballismus-hemichorea (HB-HC) and high signal intensity in the contralateral striatum on T1-weighted MRI scans. Correction of underlying hyperglycemia and supportive care resulted in resolution within days to weeks. This characteristic clinicoradiologic picture suggests a clinical syndrome with benign outcome.     

Absence of G209A and G88C mutations in the alpha-synuclein gene of Parkinson's disease in a Chinese population

A G209A mutation in the alpha-synuclein gene was recently discovered in a large Italian kindred and three unrelated Greek kindreds with autosomal dominant Parkinson's disease (PD). Subsequently, another mutation in the gene (G88C) was also identified in a German family with autosomal PD. These results indicate that the alpha-synuclein gene may have an important role in the pathogenesis of PD. This study was designed to screen the existence of both mutations of the alpha-synuclein gene among 100 Chinese patients with PD, including 80 with sporadic and 20 with familial PD. Results showed that none of our patients, both sporadic and familial PD, had either of the two mutations of this gene. We therefore conclude that although of great interest, these two mutations are not relevant for the pathogenesis of PD in a Han Chinese population.     

Increase in signal intensities on T1-weighted magnetic resonance images in asymptomatic manganese-exposed workers

OBJECTIVES: To clarify the clinical significance of increased signal intensities on T1 weighted magnetic resonance imaging (MRI) we performed a large-scale epidemiological study on asymptomatic manganese (Mn)-exposed workers with its focus on MRI. METHODS: We randomly selected 121 male workers out of a total of 750 workers including Mn-exposed, non-exposed manual, and non-exposed clerical workers in the factories. We studied environmental and biological monitoring, neurological examination, and MRI. RESULTS: The proportion of workers with increased signal intensities among the exposed, the non-exposed manual workers, and the non-exposed clerical workers was 46.1%, 18.8%, and 0%, respectively. Especially, 73.5% of the welders showed increased signal intensities. In no subject, were clinical signs of manganism observed. The pallidal index correlated with blood Mn concentration. CONCLUSION: Increase in signal intensities on the T1-weighted image reflect recent exposure to Mn, but not necessarily manganism. At which increase of signal intensity, the progression of manganism from Mn exposure occurs, remains to be solved.     

Spontaneous oscillations of cerebral blood flow velocity in the middle cerebral arteries of normal subjects and schizophrenic patients

Although many regional cerebral blood flow (rCBF) studies of schizophrenic patients have been carried out, only a few studies have investigated real-time hemodynamic changes in schizophrenic patients. In the present study, we used long-term monitoring of the middle cerebral artery (MCA) by non-invasive transcranial Doppler ultrasonography to obtain real-time CBF data in 55 schizophrenic patients and 20 normal comparison subjects. The mean blood flow velocity and pulsatility index (PI) of the MCA were not constant during long-term monitoring. They showed sinusoidal oscillations similar to those described in previous reports. The amplitude variations of these oscillations in both drug-naive and medicated schizophrenic patients were significantly decreased compared with findings in normal control subjects. The averaged PI values were found to be decreased in patients with illness durations of more than 10 years. After withdrawal of antipsychotic medication, both the amplitude variations of oscillations and the PI values in the drug-withdrawn patients were significantly decreased relative to findings in normal control subjects. Our results show a decreased adjustment ability of cerebral vessel resistance not only in neuroleptic-naive schizophrenic patients but also in patients with longer illness duration. Neuroleptics could affect the adjustment ability of vessel resistance.     

Polymorphisms at - 174 and in the 3' flanking region of interleukin-6 (IL-6) gene in patients with myasthenia gravis

We examined the bi-allelic polymorphism at - 174 in the promoter region and the polymorphism in the 3' flanking AT rich region of the interleukin-6 (IL-6) gene in Swedish patients with myasthenia gravis (MG) and ethnically matched healthy individuals. There was no association between the polymorphisms and the disease. There was no relation of the polymorphisms to the clinical variables, the thymic histopathologies, the level of serum acetylcholine receptor antibodies or the concentrations of IgG and its subclasses. Our data yield no evidence for the IL-6 gene contributing to the disease susceptibility.