Properties of fibres,endplates and acetylcholine receptors in the diaphragm,masseter, laryngeal,abdominal and limb muscles in the goat

Purpose

Although differences in fibre composition, fibre size or acetylcholine receptor (AChR) density between muscles have often been proposed to explain the unequal sensitivities of muscles to muscle relaxant drugs, it is not clear whether or how these parameters differ among muscles or are related to one another. In this study, several muscles were examined to determine the composition and cross-sectional area (CSA) of types I and II fibres, the surface area of their motor endplates (ESA), and their AChR density.

Methods

Biopsies from the thyroarytenoideus, cricoarytenoideus dorsalis, masseter, diaphragm, transversus abdominis, rectus abdominis, gastrocnemius and soleus muscles of goats were processed by muscle histochemistry and morphometry and the ESA:CSA ratio was computed. The number and density of AChRs per endplate were estimated by¹²⁵I-α-bungarotoxin binding studies.

Results

The mean type 1 fibre composition (range: 0–100%), fibre diameter (28–50 μm) and the ESA:CSA ratio (0.27–1.01) differed among muscles (P = 0.0001), but there were no significant differences (P > 0.05) in the mean endplate size (577–725 μm²), AChR number (6.6?14.5 × 10⁶) or AChR density (8,900–22,300 μm^?2) probably because of marked individual variations. Fibre size increased and the ESA:CSA ratio decreased in the order laryngeal, diaphragm, jaw, limb and abdominal muscles.

Conclusion

It is concluded that between muscles fibre size varies more than endplate size or AChR number.     

Diffusion of information about genetic risk within families

Recent developments in genetics are likely to exacerbate the ethical issues in clinical practice, especially with regard to privacy and disclosure of genetic information. To evaluate the behaviour of patients with respect to transmitting carrier information, we undertook a survey of 283 families with a balanced chromosomal rearrangement as a model. In these families, 1816 relatives were considered at risk and 806 of them were karyotyped (44.4%). The percentage of karyotypes performed is significantly related to the number of living children of the index couple, the reason for referral, the nature of the anomaly, the training of the counsellor and the age of the index case. This study shows the limits of the screening of at risk individuals within families, based on the willingness of the patients, and addresses practical and ethical issues around family disclosure in medical genetics.     

Leucoéncephalopathie multifocale progressive

Summary A young woman presented a mixed congenital and familial immunodeficiency syndrome consisting in an absence of IgA and lowered levels of IgG and IgM, with a defect in cellular immunity. She had a mild malabsorption syndrome with slight alterations of the jejunal mucosa. Non-caseating tuberculoid granulomata were found in skin lesions, in lymph nodes and in the spleen. At age 27 the patient died of a neurological disease of 4 months duration. Autopsy revealed a very widespread demyelinating process involving mainly the right cerebellar hemisphere but also most of the pons and left cerebellum, with the typical morphologic characters of PML. In the hemispheres lesions were limited to microscopical microglial nodules with discrete demyelination. A review of 86 published cases of PML revealed 9 other cases in which lesions showed a strong predilection for the subtentorial territories. This sampling allows for the assumption that some 11% of the cases of PML have this particular lesion distribution. Other pertinent features of this case are briefly discussed.

     

THIP inhibits feeding behavior in fasted rats

The effects of 4,5,6,7-tetrahydroisoxazolo-[5,4-c]pyridin-3-ol (THIP) were compared with those of d-amphetamine and GABA in fasted rats. Intravenously-administered THIP produced a dose-dependent decrease in food consumption (ED₅₀1.5 mg/kg) by an action that was not reversed by prior subcutaneous or simultaneous intravenous (IV) injection of bicuculline. d-Amphetamine-SO₄ also produced a decrease in food consumption in this model (ED₅₀0.2 mg/kg, IV). Unlike THIP, GABA (in doses up to 100 mg/kg, IV) did not produce a marked anorexigenic effect. These results provide further evidence that THIP can penetrate the blood-brain barrier, and that central GABA-ergic systems are involved in controlling food intake.     

Ultrasonographic study of the effect of different miotics on the eye components.

The effect of topical instillations of carbachol 3%, pilocarpine 2%, aceclidine 2%, aceclidine 2%-adrenaline 1%, and of the Ocusert delivery system was determined and compared in 151 eyes. The depth of the anterior chamber, the thickness and the position of the lens, the length of the vitreous and the refraction were studied. Aceclidine has negligible side effects on the ocular components (maximal change of the anterior chamber 0.20 mm; maximal change of the lens thickness 0.14 mm; maximal myopisation: -1.5 delta. Carbachol has the strongest side effects (maximal change of the anterior chamber 0.80 mm; maximal change of the lens thickness 0.80 mm; maximal myopisation -11.50 delta). Carbachol and pilocarpine may cause an important forward displacement of the lens with the risk of an angle-closure glaucoma in an eye with shallow anterior chamber.     

Localized pelvic neuroblastoma: excellent survival and low morbidity with tailored therapy--the 10-year experience of the French Society of Pediatric Oncology.

PURPOSE: To assess the results and morbidity of treatment of children with localized pelvic neuroblastoma (NB). PATIENTS AND METHODS: All consecutive cases of localized pelvic NB registered in the French multicenter prospective studies NBL90 and NBL94 between 1990 and 1999 were reviewed. Resectability was decided on the basis of clinical and radiologic evaluation. In unresectable tumors, primary chemotherapy (combinations of carboplatin-etoposide and vincristine-cyclophosphamide-doxorubicine) was administered before surgery. RESULTS: Forty-seven children (with 26 resectable tumors and 21 unresectable) were included in this study. At the end of treatment, 31 children were in complete remission (66%). Long-term neurologic sequelae were observed in seven patients (15%), directly attributable to surgery in three cases. After a median follow-up of 48 months (range, 13 to 129 months), 44 patients are alive. Six children experienced local relapse; four of these children achieved subsequent remission. The projected overall survival and event-free survival (EFS) rates at 5 years are, respectively, 93% +/- 4% and 84% +/- 5%. Survival of children treated with preoperative chemotherapy are similar to those treated by primary surgery (80% and 88% respectively). The extent of surgical resection seemed to have no influence on the outcome (EFS rates 76% and 89% in case of gross residue and complete resection or microscopic residue, respectively). CONCLUSION: Our data confirm the excellent survival of localized pelvic NBs. Considering the efficacy of preoperative chemotherapy, patients with pelvic NB should be carefully screened for primary surgery. The risk of neurologic impairment during radical excision should be balanced with the good survival of children with minimal residual disease.