Spinocerebellar ataxia types 2 and 3 segregating simultaneously in a single family.

Spinocerebellar ataxia (SCA) types 2 and 3 are autosomal-dominant neurodegenerative disorders caused by mutations in two different genes. We identified mutations for SCA2 and SCA3 segregating simultaneously in a single Brazilian family. The index patient had SCA2, whereas her two second-degree cousins had SCA3. Disease was more rapidly progressive in the SCA2 patient, who presented severe brainstem and pancerebellar atrophy, as opposed to the two SCA3 patients, who had only mild cerebellar vermian atrophy. In such situations, molecular confirmation of all patients may avoid misdiagnosis of SCA subtypes and eventual errors in predictive testing of unaffected family members.     

Evaluation of Tc-99m(V) DMSA for imaging inflammatory lesions: An experimental study

The present study evaluated^99mTc(V) DMSA as an agent for the visualization of inflammatory lesions in comparison to^99mTc(HI) DMSA and^99mTc-HIG. All three radiopharmaceuticals were prepared with commercial kits.^99mTc(V) DMSA was prepared at neutral pH by the addition of first bicarbonate and then pertechnetate to the kit contents. The labeling efficiency was 99% as determined by ITLC. Abscesses were induced by i.m. injection of 50 μl turpentine into the right thighs of 36 Swiss albino mice. Six days later 3.7 MBq of each radiopharmaceutical was i.v. administered to 12 mice. The mice were sacrificed at 1,3,6 and 24 h later. Scintigrams were obtained with a gamma camera. The abscesses were better visualized on scintigrams with^99mTc(V) DMSA compared to^99mTc(III) DMSA, starting at 1 h. The animals were dissected and the organs were removed, weighed and the radioactivity determined with a gamma counter. The abscess to other tissue ratios were higher with^99mTc(V) DMSA than the other radiopharmaceuticals. The max. abscess/muscle ratios were 9.46 ± 3.20 (24 h), 4.19 ± 1.39 (6 h) and 5.98 ± 1.17 (24 h) and max. abscess/blood ratios were 6.22 ± 1.41, 4.09 ± 0.84 and 0.914 ± 0.351 all at 24 h for^99mTc(V) DMSA,^99mTc(III) DMSA and^99mTc-HIG, respectively. Experimental arthritis was produced in 6 New Zealand white rabbits by intra-articular injection of ovalbumin. Four days later 37 MBq of^99mTc(V) DMSA and^99mTc-HIG were each i.v. administered to 3 rabbits. Scintigrams obtained at 1, 3, 6, and 24 h clearly demonstrated arthritic joints. ROFs over arthritic joints were compared to contralateral normal joints (A/C). The max. A/C ratios were 2.10 ± 0.31 (3 h) and 2.92 ± 0.99 (24 h) for^99mTc(V) DMSA and^99mTc-HIG, respectively. Our results indicated the feasibility of imaging inflammatory lesions with^99mTc(V) DMSA.     

Dorsal and ventral dopaminergic innervation of the spinal cord: Functional implications

Several studies have demonstrated that a descending dopaminergic pathway innervates the dorsal and the intermediate gray matter of the spinal cord and have suggested that this pathway is involved in pain modulation and in the control of autonomie functions. Other studies have also demonstrated the presence of dopamine (DA) and DA metabolites as well as of DA receptors in the ventral cord. There is also evidence for the implication of DA in the control of motor functions at the spinal level. The occurrence of a dopaminergic innervation in the ventral horn has been, however, disputed until recently. But recent work has demonstrated that the motoneural cell groups in the ventral horn (lamina IX) are a target for descending dopaminergic fibers. In addition, the possibility that DA is a mediator of primary afferent fibers has also been postulated. Finally, the occurrence of dopaminergic cell bodies has been suggested in the spinal cord. This indicates that DA is probably implicated in a complex manner in spinal functions. In the present paper the possible involvement of DA in sensory and in motor functions at spinal level will be discussed in view of neurochemical observations made in polyarthritic rats, in which pain-related behavior and reduction of locomotor activity associated with a marked decrease in mobility, are observed.     

Massive hemolysis and acute mitral insufficiency one year following mitral valve repair. Apropos of a case

The authors report a case of massive haemolytic anaemia with acute mitral valve regurgitation and left cardiac failure, which occurred one year after surgical reconstruction of the mitral valve for rupture of smaller leaflet chordae. Anaemia, mitral regurgitation and cardiac failure disappeared after mitral valve replacement, using a Carpentier Edwards No. 29 valve. Haemolytic anaemia following mitral valve reconstruction is exceptional. It seems to be due to the suture material lying in a turbulent regurgitation stream when mitral incompetence develops again.     

Comparison of local and general anesthesia in tension-free (Lichtenstein) hernioplasty: a prospective randomized trial

To compare pulmonary effects, postoperative pain and fatigue, morbidity, patient satisfaction, and cost of different anesthetic techniques for inguinal hernia repair, 50 patients were randomized to local and general anesthesia groups (LA and GA). All patients received the same premedications and the same postoperative analgesic regimen. The standardized postoperative analgesic, intramuscular pyroxicam 20 mg, was given to all patients in the recovery room and an additional 20 mg on the same day was given as requested by each patient. Pulmonary function studies and arterial blood gas analysis were performed 1 h prior to the operation and at the postoperative 8th and 24th hours. All patients underwent Lichtenstein's tension-free hernioplasty. Postoperative pain and fatigue were registered 8 h and 24 h after the operation. A questionnaire was filled out by the patients, and they were asked to give grades for the general comfort of the anesthesia and the surgical procedure (1=worst, 10=best). Postoperative pulmonary function tests were significantly poorer in the GA group both on 8th- and 24th-hour measurements (P<0.05). Patients who underwent LA had significantly lower PCO₂ and higher PO₂ at the postoperative 8th hour (P<0.05). Mean postoperative pain and fatigue scores revealed a significant difference in favor of local anesthesia at only the 8th hour (P<0.05). There were two complications, one in each group (a hematoma in LA and a urinary retention in GA). Patient satisfaction grades were not different in the two groups. We conclude that LA in inguinal hernia repair does not adversely affect pulmonary functions, patients feel less pain, and patient satisfaction is comparable to that with GA. Electronic Publication