Controlled study of haloperidol, pimozide and placebo for the treatment of Gilles de la Tourette's syndrome

The results of this controlled study of the treatment of 57 patients with Gilles de la Tourette's syndrome suggested that both haloperidol and pimozide were more effective than placebo, but that haloperidol was slightly more effective than pimozide. Adverse effects occurred more frequently with haloperidol vs placebo than with pimozide vs placebo, but the frequency was not significantly different for haloperidol compared with pimozide. Clinically significant cardiac effects did not occur at a maximum dosage of 0.3 mg/kg or 20 mg/d for pimozide and 10 mg/d for haloperidol. However, the QTc interval was prolonged during pimozide treatment compared with that during haloperidol treatment, although the values for both medications were not in an abnormal range.     

Chronic myopathy with a partial deficiency of the carnitine palmityltransferase enzyme

To date, chronic myopathy has not been reported (to our knowledge) to occur in carnitine palmityltransferase (CPT) deficiency, a disorder of muscle lipid metabolism. We describe two patients with CPT deficiency: a mother, who had a partial CPT deficiency associated with fixed proximal weakness but without rhabdomyolysis, and her son, who had a complete CPT deficiency (95% reduction in enzyme activity) and who suffered from classic attacks of exercise-induced rhabdomyolysis but had normal strength on recovery. Careful examination of family members of patients with complete CPT deficiency is suggested in order to identify clinically affected heterozygotes.     

Medullary paracoccidioidomycosis: report of a case

Paracoccidioidomycosis of the central nervous system presents either as meningeal or pseudotumoral lesions. Although occurring more frequently in the brain and meninges, they can occasionally involve the spinal cord. A case of paracoccidioidomycosis in the cervical spinal cord is reported in this paper. Difficulties in establishing the etiologic diagnosis, the importance of radiologic examination of the thorax, and the treatment of the patient are commented. In an extensive review of the literature on the subject, only three other cases have been found, which are also discussed.     

Are ADDH and ADHD the same or different?

To assess the relationship between the DSM-III criteria for attention deficit disorder with hyperactivity (ADDH) and the DSM-III-R criteria for attention-deficit hyperactivity disorder (ADHD), children from an inner city parochial school were evaluated using a 30-item teacher questionnaire consisting of the DMS-III and DSM-III-R criteria for these disorders, the revised Conners Parent and Teacher Questionnaires, and a continuous performance test. Diagnostic groups were established based on teacher ratings of the DSM items and evaluated in relation to the rating scale data and continuous performance test. While children who were identified by teachers as having ADDH almost always satisfied the criteria for ADHD, a new group of children who were hyperactive and impulsive but less clearly inattentive also met the criteria for ADHD. Implications of the change in diagnostic criteria are discussed.     

Nuclear medicine diagnosis of the thyroid

The progress made in ultrasonography and improved in vitro tests have changed the field of application for scintigraphy of the thyroid. Thyroid scanning itself has been improved by the use of isotopes with better imaging properties and less radiation burden (99mTc, 123I) and by gamma cameras for imaging. Scintigraphy yields real topographic and functional information on the gland, in addition to which ultrasonography gives morphological data only. This holds true especially for autonomous nodular goiter with iodine deficiency. The goal of thyroid imaging is always to match the appropriate diagnostic procedure with all the clinical data available. When optimized techniques (gamma camera, on-line processor) are used thyroid scintigraphy is useful for the diagnosis, adequate therapy and follow-up of various thyroid disorders.     

Results of long-term leukocyte interferon (alpha IFN) therapy using an individually determined dose schedule in recurrent laryngeal papillomatosis

Out of 24 patients with laryngeal papillomatosis 6 (5 female, 1 male) suffered from repeated relapses and underwent long-term treatment with alpha-IFN-therapy. Age at onset of the disease: 1 5/12-16 2/12 years. Duration of illness: 1-7 years, with several relapses were treated surgically and with laser-coagulation. Three out of 6 patients had a tracheal cannula and were cauterized by podophylline at 2-4 week intervals. IFN was given in dosages of 5-20 X 10(4) U/kg 2 or 3 times a week. IFN-dosage for each patient was determined using the induction kinetics of (2'-5')-oligo(A)synthetase (OAS) in the mononuclear cells of the circulating blood of patients with laryngeal papillomatosis. A continuous effect could be achieved by the dose of IFN determined in the described way always before OAS activity decreased to its initial level. All 6 patients responded favorably to the alpha-IFN-therapy. Two patients treated only with IFN showed remission without relapses. In 2 cases IFN was successfully used to prevent relapses after surgical treatment and laser-coagulation. In 2 patients with papillomatosis extending into the main bronchi the disease could only be brought to a standstill, i.e. it was not necessary to remove the papillomas. Two out of 3 patients with laryngeal papillomatosis could be decannulated. Long-term therapy following the above described principles is efficient and without significant side-effects. Three patients are in treatment for more than 3 10/12 years.     

PILOT PROJECTS TO STIMULATE ADVERSE DRUG REACTION REPORTING

Schemes for stimulating adverse drug reporting are described with particular emphasis on a recently introduced Mississippi Adverse Drug Reaction Reporting Programme.     

Mapping the active-site tyrosine of vaccinia virus DNA topoisomerase I.

Site-directed mutagenesis of the vaccinia virus gene encoding a type I DNA topoisomerase implicates Tyr-274 as the active-site residue that forms a covalent adduct with DNA during cycles of DNA-strand breakage and reunion. Replacement of Tyr-274 by phenylalanine results in loss of the ability of the enzyme to relax negatively supercoiled DNA as well as to form the covalent DNA-protein intermediate. Substitution of phenylalanine for tyrosine at nine other sites in the protein has no apparent effect on enzyme activity. Amino acid sequence alignment reveals Tyr-274 to be homologous to Tyr-727 and Tyr-771, respectively, of the type I topoisomerases from Saccharomyces cerevisiae and Saccharomyces pombe; Tyr-727 and Tyr-771 have been shown to represent the active-site tyrosines of those enzymes. Sequence comparison of the active-site regions defines a motif Ser-Lys-Xaa-Xaa-Tyr common to the viral and cellular type I topoisomerases, including the human enzyme.     

A comparison of sensory loss tests and histopathology in the diagnosis of leprosy

Three different sensory loss tests, for anaesthesia to light touch, for diminished pain sensation and for loss of thermosensation, were compared with histopathological examination results in the diagnosis of suspected tuberculoid leprosy in 120 individuals with 126 lesions. Though none of the 3 tests used in this study was found to be strikingly superior to any of the others, the results indicate potentially important differences in their usefulness in different subgroups of suspected patients. The methodological problems inherent in such studies are discussed.