Arthrogryposis multiplex congenita

From 1975 to 2004 a total of 38 children handicapped by congenital multiple arthrogryposis were cared for. The congenital joint contractures demand a major effort in terms of surgical reconstruction. In the case of distal arthrogryposis the chances that patients will be able to walk without help are good, while those with amyoplasia are likely to be dependent on mobility aids throughout their lives. The ultimate goal of treatment for patients is to develop into self-confident adults who can cope with life despite their handicaps. The hip in arthrogryposis shows variable forms of pathology, ranging from the almost normal hip to hip contractures with dislocation. Its treatment has some limited advantages, but hardly improves mobility. The knee contractures are actively treated to allow patients to sit, stand and walk better. The club foot and the rocker-bottom foot need sophisticated conservative and operative treatments. If conservative manipulation of bilateral extension contractures of the elbow fails operative treatment is carried out on the dominant side. For shoulder, hand and finger contractures conservative manipulation brings about little improvement, and surgical approaches help hardly at all.     

Les stratégies psychothérapeutiques

Psychological support is an essential concern in the management of alcohol misuses. Motivation and readiness to change are key factors of the evolution. They have to be in the centre of the patient-doctor relation. Regular evaluation is a part of a step by step therapeutic strategy.     

Ultrasonographic study on kidneys in patients with acute renal failure]

Ultrasonotomograms of 22 kidneys were obtained in 11 patients with renal-acute renal failure (renal-ARF). The underlying diseases of renal-ARF were acute tubular necrosis in 8 patients and acute on-set chronic glomerulonephritis in 3 patients. They were treated by hemodialysis in 10 patients and intermittent peritoneal dialysis in 1 patient. Ultrasonic measurement of the size of kidneys revealed that the thickness (anterior-posterior diameter) and the ratio of thickness to length (T/L) were greater in patients with ARF than in those with chronic renal failure and normal renal function. The patients with a low value of T/L (under 0.60) had a significantly greater urine volume than those with high a value of T/L (0.60 or more). The sonographic features of renal-ARF kidneys were marked increase in parenchymal echogenicity and appearance of hypoechoic swollen renal pyramids with sharpness of the corticomedullary border. In the course of ARF, these sonographic changes gradually disappeared when the patients had recovered from ARF. However, the prognosis was poor in patients with severer sonographic findings. We believe that repeated ultrasonic examination of the kidneys in patients with renal-ARF is useful for not only differential diagnosis of post-renal urinary obstruction but evaluating the course of ARF.     

Steroids in Duchenne muscular dystrophy--deflazacort trial

We conducted a double blind controlled trial in 28 Duchenne muscular dystrophy (DMD) patients with Deflazacort (DF), an oxazoline derivative of prednisolone which reduces its side-effects. Myometric muscle strength measurements, Scott Score and timed tests showed statistically significant improvement for the treated group (P less than 0.05). Side-effects after 9 months of treatment included mild cushingoid appearance in four patients (28%) and moderate in only one (7%), increased appetite in seven (50%), increased body hair in four (28%), irritability and hyperactivity in three (21%). Increased body weight was not prominent and was controlled with dietary measures. No patient had to be withdrawn from medication. More research and long-term follow-up are needed in order to establish the mechanism of improvement and the consequences of long-term steroid administration in DMD. In this regard DF appears as an alternative to prednisone preserving its benefits but with fewer side-effects.     

Individual psychotherapy with schizophrenic outpatients in the public mental health system.

Forty-two therapists conducting individual psychotherapy with schizophrenic outpatients in a public mental health system responded to a questionnaire that focused on the types of interventions used and the issues and problems encountered in therapy. The therapists spent 59 percent of their time in supportive, problem-solving work and only 32 percent in traditional psychotherapeutic interventions, such as providing insight. The most common issues in therapy were relationship problems, family concerns, depression, losses, and the role of medications in the client's life. The most significant impediments to therapeutic work were the lack of community resources, the client's noncompliance with medications and lack of motivation, and dual diagnoses. Suggestions are offered for improving psychotherapy with schizophrenic outpatients in the public mental health system.     

Dissection of the extracranial vertebral artery: report of four cases and review of the literature.

Four cases of cervical vertebral artery (VA) dissection are reported. In three patients VA dissection was associated with neck trauma. All patients were young or middle-aged (range 27 to 49 years). In two there was a history of migraine. Pain preceded neurological symptoms from hours to six weeks. Three patients had neurological deficits including elements of the lateral medullary syndrome, and one experienced recurrent transient ischaemic attacks in the vertebrobasilar territory. Angiographic findings included irregular stenosis, occlusion and pseudoaneurysm; in two patients VA abnormalities were bilateral. All patients were treated with anticoagulants and improved. In a review of 28 cases with traumatic dissection and 29 cases with spontaneous dissection of the VA reported in the literature, distinct clinical and angiographic features emerge. Aetiology remains obscure in most cases of spontaneous dissection and management is still controversial.     

Alternatives to lithium in the treatment of bipolar disorder.

Bipolar disorder is a serious mental illness affecting a large number of Americans. While lithium, the treatment of choice for this disorder, is usually effective, a substantial number of individuals with bipolar disorder are not helped by lithium or are intolerant to its side effects. Consequently, investigators are searching for alternative drugs in order to treat lithium-resistant patients. Although a number of alternative drug treatments have been discussed in the literature, only the anticonvulsants carbamazepine and valproate appear to warrant serious consideration at this time. Future research will further define their roles as antimanic agents.     

Test-retest reliability in EEG frequency analysis.

This study was performed to gain a better understanding of EEG frequency analysis test-retest reliability in normal healthy adults, and to evaluate factors which could influence the measured inter-record differences. Nineteen subjects underwent serial EEG recordings at 5 min and 12-16 week intervals. Records were visually edited using a standardized protocol, and FFT frequency analysis performed on segments of 60, 40, or 20 sec total length. Correlation coefficients for broad band features averaged 0.92 over the 5 min retest interval and 0.84 over the 12-16 week interval. There was essentially no difference between correlation coefficients of absolute and relative power features. Coefficients based on 60 sec records were marginally higher than those of 40 or 20 sec records. On the other hand, test-retest percent differences were typically lower for relative as opposed to absolute power features, and 60 sec records showed consistently lower percent differences than did 40 or particularly 20 sec records. Peak alpha frequency and mean frequency were the most stable EEG features at either interval. Montage had significant effects on test-retest differences at the 12-16 week interval. A significant association between intra-record and inter-record variability could not be demonstrated.     

Effects of crude oil and ultraviolet radiation on immunity within mouse skin

Previous studies indicate that crude oil leads to increased pigmentation and erythema (sunburn) in response to sunlight in exposed individuals. However, no information is currently available concerning whether crude oil exposure might enhance the immunosuppressive effects of solar ultraviolet radiation (UVR) on the skin. In order to address this question, the back skin of shaved, female C3H/HeN mice was exposed to crude oil with or without subsequent treatment with medium-wavelength (UVB) (200 J/m2) or long-wavelength (UVA) (20,000 J/m2) UVR. Immune function was assessed in treated mice by measuring their ability to mount contact hypersensitivity responses to a hapten (2,4-dinitro-1-flyorobenzene, DNGFB) applied to the site of crude oil and UVR treatment as determined by ear swelling upon subsequent challenge. Since Langerhans cells represent an important component of immunity within the skin and because suppression of contact hypersensitivity following UVR treatment is often accompanied by disappearance of Langerhans cells from the epidermis, the impact of these agents on epidermal Langerhans cell density was also analyzed. This was accomplished by enumerating IA-positive cells within the epidermis of treated skin. In these studies, crude oil alone induced inhibition of contact hypersensitivity but had no effect on epidermal Langerhans cells. In contrast, combined treatment with crude oil and UVA led to suppression of contact hypersensitivity, which was accompanied by depletion of epidermal Langerhans cells.