Adolescent osteoporosis disclosing familial osteopenia.

The observations of familial juvenile osteoporosis, presumably of genetic origin are exceptional. The authors report the observation of a 16-year old adolescent suffering from osteoporosis, confirmed by histomorphometry and decrease in bone density (lumbar vertebrae 0.79 g/cm2 and femoral neck 0.88 g/cm2: LUNAR DPX). We prescribed fluorine and calcium therapy. Lumbar bone density increases by 11% and bone density of the thighbone neck by 7.6%. We cannot rule out growth as a factor in the changes observed, given that the propositus is only 16. A densitometric investigation performed in 4 of his 12 brothers shows a decrease in the lumbar bone mineral content (from 61 to 94% expressed as Z score). A genotypic origin seems to be conceivable, especially since no other cause could be considered (endocrinal, alimentary...). On the other hand, there is no argument in favour of osteogenesis imperfecta disease. The bone densitometry is a useful diagnostic means to detect familial forms of osteoporosis.

[Prospective study of the analgesic action of clomipramine versus placebo in refractory lumbosciatica (68 cases)]. / Etude prospective de l'action antalgique de la clomipramine versus placebo dans les lombosciatalgies rebelles (68 cas).

The authors sought to determine the analgesic activity of clomipramine (CMP) versus placebo, efficacy and side-effects according to the time of administration, long term clinical results and the relationship between plasma levels and analgesic effect. This double-blind randomised trial involved the infusion of 250 ml of glucose solution morning and evening versus placebo. CMP was administered at progressive doses for 8 days (maximum dosage 75 mg), either in the morning at 8 a.m. or in the evening at 6 p.m. Sixty eight patients accepted to be treated, all suffering from low back pain with or without sciatica. Maintenance treatment at the dose of 75 mg/day was then administered. CMP had a statistically significant analgesic action independent (rapidity of action) of its antidepressant activity. CMP was more effective and better tolerated (sedative and tranquilizer effect) in the evening. Long term results were poor (75% of cases). There was no link between plasma levels and clinical response. The authors noted that the analgesic activity of CMP was rapid, but for a duration limited to the short term and that this efficacy compared with the quite good results obtained with the placebo could be explained by a possible methodological bias (patients hospitalised and treated by intravenous infusions). The finding that CMP was better tolerated in the evening, without any loss of efficacy, is a positive feature (chronotherapeutic trial). Poor long term results could be partially explained by side-effects (66%). There was no relationship between analgesic effect and plasma levels.

[The image of intravertebral somatic gaseous dissection. Report of 9 cases one of which has a triple localization and review of the literature]. / L'image de dissection gazeuse somatique intravertébrale. A propos de 9 observations dont une forme à triple localisation et revue de la littérature.

We report a series of 9 cases of vertebral somatic gaseous dissection studied between 1978 and 1989. We find most of the characteristics established from the literature data: advanced age, possible medullar compression contrary to the vertebral compression by common osteoporosis, predilection for the dorso-lumbar junction, frequency of the osteoporosis, possibility of metabolic disorders, of occurrence after radiotherapy. The context of the occurrence and the paraclinical examinations of the whole have enabled us to eliminate, in this series, a malignant pathology. However, when there exists a doubt on a malignant origin, it is preferable to carry out a vertebral biopsy with a trocar. In fact, the image may not be pathognomonic of a benign vertebral osteoporosis. Finally, we give the first description of a triple localization of vertebral gaseous dissection.

Intradiscal injection of triamcinolone hexacetonide for acute, subacute, and chronic sciatica. Results at 3 months an open-prospectus study of 30 cases and review of the literature.

The authors report an open study of 30 cases of intradiscal injection of triamcinolone hexacetonide in the treatment of sciatica. The patients were monitored at months 1 and 3. The results were judged to be good in 36.6% of the cases, moderate in 36.6% and poor in 26.7% of the cases. Two adverse effects were reported: 1 case of reversible urinary retention and 1 case of deficiency of the dorsiflexor muscles of the foot. The good results reported in previous series were only found in this study when the indications were restricted to certain favourable prognostic factors: duration of sciatica less than 6 months and CAT-scan appearance of discal hernia. This technique has the advantage of being simple, economical and nonallergic. On the basis of the encouraging results of the initial series, this technique should be considered as an interesting therapeutic alternative in sciatica. Larger series and double-blind studies, however, are necessary to confirm the initial results.

Ehlers-Danlos syndrome, clotting disorders and muscular dystrophy.

Ehlers-Danlos syndrome includes 11 distinct entities. The diversity of this collagen dysplasia and its combination with other abnormalities make it difficult to understand physiopathologically. A case of Ehlers-Danlos syndrome is reported, which is novel owing to its combination with clotting abnormalities and especially with muscular dystrophy. To our knowledge this has not previously been reported. The patient was a young man aged 16 years who presented with Ehlers-Danlos syndrome satisfying Perelman's diagnostic criteria. His father and two brothers had comparable clinical symptoms, but his mother and sister were healthy. The four male subjects had an increased cephalin-kaolin time, reduced levels of factor VIII and Willebrand's factor (but without haemophilia A or Willebrand's disease), and, especially, an abnormal platelet ATP secretion. The proband alone had muscular disease with bilateral quadriceps fatigability and amyotrophy. The muscle enzyme levels were greatly increased, the electromyographic trace was myogenic, and the biopsy showed severe muscular dystrophy. This new observation poses the problem of the relation between clotting abnormalities and collagen abnormalities in the Ehlers-Danlos syndrome. It is difficult to classify this case within any of the 11 known types because of its muscular manifestations. It may perhaps be a fortuitous combination or an extension of the nosological framework of this syndrome.

Two familial cases of malignant Reiter's syndrome.

A Cambodian man and his son concomitantly developed malignant Reiter's syndrome soon after their arrival in France. In both cases generalized skin lesions of pustular psoriasis and systemic features were present. The son died after 2 years of unresponsive continuously progressive disease. The father received pulses of high dose immunosuppressants that worked rapidly and prevented a life threatening course. These 2 cases illustrate the pathophysiologic hypothesis of Reiter's syndrome, emphasizing the role of environmental triggering factors and the relationship between spondylarthropathies in B27 positive patients. Fatal cases of Reiter's syndrome are very rare in the review of the literature.