Temporal bone findings in trisomy 18 and 21 syndromes

The temporal bones from fetuses with known autosomal trisomy syndromes were examined histopathologically after spontaneous or induced abortions. Fetal ages dated from the 17th to the 24th week of pregnancy. In all, 17 temporal bones from nine fetuses were studied and included seven cases of Down's syndrome and two of Edwards' syndrome. Temporal bones from fetuses with Down's syndrome had more abnormalities of the external and middle ears than of the inner ear. The bones from the fetuses with Edwards' syndrome showed abnormalities of the external or middle ear and retarded development of the inner ear (failed ossification of the otic capsule and unformed organ of Corti).     

The incidence of gout in patients with Dupuytren's disease.

In a retrospective study we found that the incidence of gout in patients with Dupuytren's disease is 3.5%. The mean serum uric acid was 330 mumol/l. (5.5 mg./100 ml.) in men and 240 mumol./l. (4.0 mg./100 ml.) in women. The rate of hyperuricaemia was 7%. These are not significantly higher than the expected figures in Britain.     

Phase mapping from left ventricular radionuclide ventriculograms: interobserver reliability and accuracy of the programme

Phase analysis of data from radionuclide ventriculograms is known to provide space-time information on the mechanical emptying of pixels located in the ventricular regions. It could therefore perhaps be useful for detecting abnormal sequences of contraction of the ventricles as well as to localize sites of premature electromechanical action. The phase programme used in this investigation is designed to trace the electromechanical action of the ventricles from colour-coded phase images (first harmonic Fourier transform), not only visually from a cinematic representation, but particularly from a data output of x, y coordinates and phase angles of sites chosen as they empty in the ventricular regions. Six healthy baboons were subjected to conventional radionuclide ventriculography, performed hourly for the duration of 11 h under anaesthesia. The programme was used by two independent observers to map the progression of emptying for each of the thus acquired 66 left ventricular phase images. The interobserver reliability and the accuracy achieved in mapping normal contraction patterns was found to be good. Abnormalities which appeared during the prolonged anaesthesia and which were confirmed with 12 lead electrocardiography were likewise detected consistently.     

CongenitaI Polycystic Disease of the Brain: Report of an Unusual Case

Multiple congenital supratentorial intracerebral cysts occurred in a newborn infant. The cysts, of varying size, content and lining, occupied the cerebral hemispheres. They formed a mosaic pattern on CT scan and left no trace of a recognisable ventricular system. Multiple surgical procedures were performed during the first year of life, and at one year the child was very well and asymptomatic, with two functioning shunts.     

Osteosarcoma of the nude rat. A model for experimental magnetic resonance imaging studies of bone tumors.

A 144cerium-induced osteosarcoma was transplanted to athymic nude rats (Han:rnu/rnu). Nineteen of the tumor-bearing animals were used to demonstrate the characteristics of the osteosarcoma on magnetic resonance imaging (MRI) in comparison with a group of nine animals that had not received any tumor transplant. The tumor-free animals showed homogeneous results in the measurements of their lower legs (T1, 800-898 mseconds; T2, 33-45 mseconds). The osteosarcomas of the tumor-bearing rats were clearly demonstrated and delineated from adjacent structures by MRI at tumor volumes between 0.3 and 7.5 cm3. A significant increase in T1 and T2 relaxation times was found, which corresponded to the increase in tumor volume. T1 relaxation times decreased slightly when tumor volumes reached greater than 5 cm3, whereas T2 relaxation times remained constant at tumor volumes greater than 2.5 cm3. Histologic changes in the structure of the tumors, occurring naturally during their growth, were proven by immersion-fixed sections embedded in paraffin and stained with hematoxylin-eosin. The water content of small and large tumors was determined by lyophilization and heat drying. Both histologic changes and differences in water content were reflected in the variations of relaxation times.     

Combined Excimer Laser and Topical Tacrolimus for the Treatment of Vitiligo: A Pilot Study

Background. Vitiligo is an acquired skin disorder that is characterized by well-defined, often symmetric white patches. Although current therapeutic modalities are directed toward increasing melanocyte melanin production, few treatment modalities address the immunologic nature of the disease.
Objective. To determine whether excimer laser, a known therapeutic modality, in combination with tacrolimus, a topical immunomodulator, accelerate response time and/or improve the degree of response in patients with this disorder.
Methods. Eight subjects diagnosed with vitiligo were recruited to participate in this institutional review board–approved double-blind, placebo-controlled study. Twenty-four symmetric vitiliginous patches (elbows, knees) from eight subjects received excimer laser treatment three times per week for 24 treatments or 10 weeks. Additionally, topical tacrolimus 0.1% ointment (Protopic) and placebo (Aquaphor) were applied to randomized patches (left or right) twice daily throughout the length of the trial. Vitiliginous patches were monitored with photographs at baseline, every 2 weeks, and 6 months after treatment. Biopsies were performed on subjects with significant results.
Results. Twenty vitiliginous patches from six subjects qualified for evaluation. Fifty percent of patches treated with combination excimer laser and tacrolimus achieved a successful response (75% repigmentation) compared with 20% for the placebo group. Subjects who responded successfully repigmented faster (19%) with combination therapy compared with excimer laser alone. Additionally, three subjects experienced transient hyperpigmentation in lesions treated with combination therapy.
Conclusion. Combining topical immunomodulators with known phototherapeutic modalities may represent a key advancement in the treatment of disease.     

Inhibitory motor control in children with attention-deficit/hyperactivity disorder: event-related potentials in the stop-signal paradigm.

BACKGROUND: The aim of the study was to investigate the inhibitory control of an ongoing motor response and to identify underlying neural deficiencies, manifested in event-related potentials, that cause poorer inhibitory performance in children with attention-deficit/hyperactivity disorder. METHODS: A stop-signal paradigm with a primary visual task and auditory stop signal was used to compare performance in 13 children with attention-deficit/hyperactivity disorder and 13 control children, while event-related potentials were recorded simultaneously. RESULTS: Children with attention-deficit/hyperactivity disorder showed poorer inhibitory performance through a slower inhibitory process. Inhibitory processing of auditory stop signals was marked by a frontal N2 component that was reduced in the attention-deficit/hyperactivity disorder group relative to controls. A central positive component (P3) was associated with the success of inhibiting a response, but there were no group differences in its amplitude or latency. CONCLUSIONS: Findings support the hypothesis of deficient inhibitory control in children with attention-deficit/hyperactivity disorder. Slower inhibitory processing appears to be due to a specific neural deficiency that manifests in the processing of the stop signal as attenuated negativity in the N2 latency range.