A case of moyamoya disease is presented. The patient is a 15-year-old white Spanish girl who had the onset of the neurological symptoms at 8 months of age with seizures, transient ischemic attacks and residual left hemiparesis. Cerebral arteriography performed at the age of 1 year revealed all the features of moyamoya disease in the territory of both carotids and in the vertebrobasilar arteries. The presence of an embryonic tentorial artery, the Bernasconi-Cassinari artery, originating from the right internal carotid artery, was disclosed at 1 year of age but did not appear in an arteriogram performed at 6 years of age and it was revisualized by magnetic resonance arteriography (MRA) performed at the age of 15 years. This finding seems to indicate a very early intrauterine onset of the disease in this case and demonstrates the superiority of MRA over conventional arteriography to discover anomalies of intracranial vessels. Administration of nicardipine, a calcium channel blocker, added to conventional antiepileptic drugs that the patient had previously taken, improved the epileptic and the neurological disease.

Adolescent↗

PubMedDate not supplied

Endogenous breath ethanol concentrations in abstinent alcohol abusers and normals.

A highly sensitive gas chromatographic breath assay was used to measure the concentration of endogenous ethanol in the breath of fasting volunteers who had consumed no alcoholic beverages. Normal subjects (n = 15) were studied, as well as withdrawn hospitalized alcohol abusers (n = 13) who had abstained from ethanol for at least two weeks. The mean breath ethanol concentrations were 1.88 nmol/l (SD = 1.06) in the normal subjects and 17.84 nmol/l (SD = 8.81) in the abstinent alcohol abusers. The difference between the two groups was statistically significant (p less than 0.001) and could have arisen either from ingestion of exogenous ethanol (from sources such as tobacco smoke) or from metabolic differences between the two groups.

Adult↗

PubMedDate not supplied

Neurotoxicity of valacyclovir in peritoneal dialysis: a pharmacokinetic study.

Valacyclovir is an effective oral agent for the treatment of herpes virus infection, however, the pharmacokinetics of the drug are altered in renal failure. It is increasingly recognized that dose adjustment of oral valacyclovir in renal failure is necessary to avoid neurotoxicity. We studied this drug in a continuous ambulatory peritoneal dialysis (CAPD) and immunocompromised patient. She developed neurotoxicity with an adjustment dosage of valacyclovir for a cutaneous zoster infection. The elimination half-time (15 h) was similar to that reported for end-stage renal disease patients, while the steady-state volume of distribution (85 l) and the area under the curve concentration (127 mg/l.h) were greater. The mean CAPD dialysance was only 5.27 ml/min with less than 1% of an administered dose being recovered in the 24-hour dialysate. 48 h after interrupting treatment, she recovered normal neurological status and 500 mg of valacyclovir every 2 days was effective and well tolerated.

Acyclovir↗

PubMedDate not supplied

Analysis of response to 20 generations of selection for body composition in mice: fit to infinitesimal model assumptions.

Data were analysed from a divergent selection experiment for an indicator of body composition in the mouse, the ratio of gonadal fat pad to body weight (GFPR). Lines were selected for 20 generations for fat (F), lean (L) or were unselected (C), with three replicates of each. Selection was within full-sib families, 16 families per replicate for the first seven generations, eight subsequently. At generation 20, GFPR in the F lines was twice and in the L lines half that of C. A log transformation removed both asymmetry of response and heterogeneity of variance among lines, and so was used throughout. Estimates of genetic variance and heritability (approximately 50% ) obtained using REML with an animal model were very similar, whether estimated from the first few generations of selection, or from all 20 generations, or from late generations having fitted pedigree. The estimates were also similar when estimated from selected or control lines. Estimates from REML also agreed with estimates of realised heritability. The results all accord with expectations under the infinitesimal model, despite the four-fold changes in mean. Relaxed selection lines, derived from generation 20, showed little regression in fatness after 40 generations without selection.

Journal Article↗

PubMedDate not supplied

[Malformations of cortical development and their clinical repercussions in a series of 144 cases].

OBJECTIVE: To show the disorders of the brain cortical development and the possible origin in base to a large series studied in a Pediatric Neurology service. PATIENTS AND METHODS: A series of 144 children with ages ranging between newborn and 12 years was studied from the clinic, image (MR, 3DMR) and evolutive point of views. RESULTS: The diagnosis was: polymicrogyria in 61 cases, lissencephaly in 22, eschizencephaly in 16, heterotopia in 16, cortical dysplasia in 9, hemimegalencephaly in 8, cobblestone in 7, sublobar dysplasia in 3, and 'double cortex' in 2. Mental retardation, motor disorders and epilepsy were the most important anomalies. CONCLUSIONS: Actually, the image is the most important study to make the diagnosis of every type of cerebral malformation. However, to know the specific gene that origin every disorder seems to be the most important thing to make the classification of every malformative type and the possible prevention of this pathology.

Brain Diseases↗

PubMedDate not supplied

[Laryngeal amyloidosis: treatment vs. abstention].

Four cases of laryngeal amyloidosis diagnosed between 1986 and 1992 are reported. Two cases had supraglottic localization and the remaining two involved both glottis and subglottis. CT was performed in all cases. Diagnosis was made in the four cases by biopsy. All of them were classified as localized laryngeal amyloidosis after doing immunoelectrophoretic study in blood and urine, and rectal biopsy in two cases. Surgical excision by carbon dioxide laser was performed in one patient, while in the remaining three patients, a preservative attitude was taken. Results of both attitudes are presented, as well as a literature review on the topic.

Adult↗