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 No patient had to be withdrawn from medication.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.     

Basicranial diastematomyelia: a case report

Diastematomyelia is a congenital dysraphism of the spinal cord in which the affected segment is longitudinally divided by a band of fibrous tissue, cartilage, or bone. Diastematomyelia has been well described in the cervical, thoracic, lumbar, and sacral spinal cord; this paper presents a case involving the basicranium. Based on the early embryologic development of the basicranium and brain, this case demonstrates that the same mechanisms proposed as the origin of spinal diastematomyelia may also operate at a more cephalad level.     

Altered excitatory and inhibitory amino acid receptor binding in hippocampus of patients with temporal lobe epilepsy.

We examined binding to excitatory amino acid and inhibitory amino acid receptors in frozen hippocampal sections prepared from surgical specimens resected from 8 individuals with medically refractory temporal lobe epilepsy. The excitatory receptors studied included N-methyl-D-aspartate (NMDA), strychnine-insensitive glycine, phencyclidine, and quisqualate. The inhibitory receptors studied were gamma-aminobutyric acid type A (GABAA) and benzodiazepine. Excitatory and inhibitory amino acid receptor binding were differentially altered in the patients with temporal lobe epilepsy in comparison to 8 age-comparable autopsy control subjects, and changes in receptor binding were regionally selective in four areas. Binding to phencyclidine receptors associated with the NMDA channel was reduced by 35 to 70% in all regions in the hippocampi of the patients. In contrast, binding to the NMDA recognition site and its associated glycine modulatory site was elevated by 20 to 110% in the cornu ammonis (CA) 1 area and dentate gyrus of the hippocampus of the patients. Binding to these sites was unaffected in area CA4. Binding to the quisqualate-type excitatory amino acid receptor was unchanged in all regions except the stratum lacunosum moleculare CA1, where it was increased by 63%. GABAA and benzodiazepine receptor binding was reduced by 20 to 60% in CA1 and CA4, but unchanged in dentate gyrus. The data indicate that excitatory and inhibitory amino acid receptors are altered in the hippocampus of patients with temporal lobe epilepsy.     

Increased lymphocyte beta-adrenergic receptor density in progressive multiple sclerosis is specific for the CD8+, CD28- suppressor cell.

Beta-adrenergic receptor density on T cells from healthy humans is greatest on suppressor cells (CD8+, CD28-) and the effect of catecholamines, secreted by the sympathetic nervous system, predominates on this subset. The sympathetic skin response, a measure of sympathetic nervous system function, is absent in most patients with chronic progressive multiple sclerosis (MS). We measured beta-adrenergic receptor density on suppressor cells, cytotoxic cells, and monocytes from patients with chronic progressive MS and healthy control subjects. Control receptor density on suppressor cells was 2.8 +/- 0.3 fmol/10(6) cells versus a density of 5.1 +/- 0.7 fmol/10(6) cells for patients. Cytotoxic cell (CD8+, CD28+) receptor density was 1.4 +/- 0.4 fmol/10(6) cells in control subjects and 0.9 +/- 0.3 fmol/10(6) cells in the patients. Monocytes displayed beta-adrenergic receptor densities of 2.6 +/- 0.4 fmol/10(6) cells in normal individuals and 2.7 +/- 0.4 fmol/10(6) cells in the patient group. CD8 lymphocyte beta-adrenergic receptor densities in patients with relapsing-remitting and those with stable MS were not different from control values, yet were significantly less than the values for patients with chronic progressive MS. We find that mononuclear cells from healthy control subjects and patients with chronic progressive MS proliferate in response to 200 units/ml of recombinant human interleukin-2 (IL-2) similarly. However, IL-2 treatment increased beta-adrenergic receptor density on normal mononuclear cells, but failed to increase it on mononuclear cells from patients with chronic progressive MS.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pleuropulmonary disease associated with dopamine agonist therapy.

Artificial dopamine agonists are widely employed for the treatment of idiopathic parkinsonism. Pleuropulmonary disease has previously been reported to occur with the use of bromocriptine and mesulergine. We report similar adverse effects induced by the newer agonists lisuride and cabergoline. All these agents are tetracyclic ergot derivatives. This suggests a causal link between ergot-derived dopamine agonists and pleuropulmonary disease.     

Postencephalitic acquired Tourette-like syndrome in a child.

We report a 6-year-old girl who developed a Tourette-like syndrome following presumed herpes encephalitis. This case suggests that tics can be acquired in childhood and may be associated with lesions involving basal ganglia or limbic circuitry.     

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.     

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.