We describe a young child with tuberous sclerosis and cardiac rhabdomyoma who developed reversible heart block after being placed on carbamazepine treatment for seizures. Patients of any age with known or suspected intrinsic cardiac disease should be monitored for conduction disturbance if they are treated with carbamazepine.

Term infants and children appear to adapt to large variations in vitamin intakes. This is supported by the finding of similar blood levels of vitamins despite several-fold differences in intake on a body weight basis. By contrast, the finding of marked elevation of some vitamins and low levels of others seen in very-low-birth-weight (VLBW) infants (less than 1500 g) suggest that this group has less adaptive capacity to high- or low-dose intakes. This indicates that their vitamin intakes need to be more closely aligned with actual needs. This paper reviews previously published data on vitamins A, E, B2, and B6 from VLBW infants receiving total parenteral nutrition (TPN). Vitamin A. VLBW infants are relatively deficient in retinol (R) at birth. During TPN large losses of R onto the delivery sets result in a further decline in stores of R as reflected in a progressive decline in plasma R during TPN. Because of the reported lower incidence of bronchopulmonary dysplasia associated with intramuscular vitamin A treatment, alternative methods of vitamin A delivery during TPN have been suggested. First, the vitamins were mixed with Intralipid (IL) and, second, retinyl palmitate (RP) rather than R was used. There was little in vitro loss of R when mixed with IL, and in vivo treatment resulted in higher blood levels after 1 month of retinol administration in IL than seen previously (21.4 +/- 4.2 vs 14.1 +/- 3.7 micrograms/dl).(ABSTRACT TRUNCATED AT 250 WORDS)

The parkinsonian syndrome rests on the clinical tripod: akinesia, rigidity, tremor. Akinesia is the key symptom, broadly defined as a difficulty in initiating and performing movements in proportion to their complexity (sophisticated, simultaneous movements) and their duration (repetitive movements). The most frequent cause of the syndrome is Parkinson's disease. Although this diagnosis needs to be confirmed in pathological terms by the loss of neurons and the presence of Lewy's bodies in the substantia nigra, some clinical data enable it to be envisaged with a minimum of errors; these are pure parkinsonian triad, good response to dopatherapy and asymmetrical symptoms. The other causes of parkinsonian syndrome are usually related to the administration of neuroleptic drugs and to degenerative diseases with lesions that are more diffuse than those of Parkinson's disease. In Steele-Richardson-Olzewski disease a parkinsonian syndrome is associated with supranuclear ophthalmoplegia. Multiple systematized atrophy presents under three different clinical aspects: a parkinsonian syndrome without tremor and resistant to L-dopa, suggesting atrophy of the strionigral tract; a parkinsonian syndrome associated with a cerebellar syndrome, suggesting olivo-cerebellar-pontine atrophy, and Shy-Drager disease which includes primary dysautonomy and other neurological syndromes.

A patient is described who had magnetic resonance imaging (MRI) findings of a left paramedian pontine ischemic stroke. It was associated with right motor sensory and left cerebellar dysfunction and the "one-and-a-half" syndrome of Fisher.

A clinical method for noninvasive measurement of regional cerebral blood flow (rCBF) and blood volume (rCBV) is described, based on Obrist's 10 minute, desaturation method after 1 minute inhalation of 133Xe. Sixteen collimated probes are placed over both hemispheres and brain stem-cerebellar regions. End-tidal 133Xe curves are used for correction of recirculation. KEV discriminators are set to record gamma and x-ray activity separately. Values are printed out automatically by a computer on a brain map. Extracerebral contamination is reduced by 1) computing curves from gamma activity, 2) applying pressure on the scalp beneath the probes, 3) 1 minute inhalation of 133Xe and recording desaturation curves for 10 minutes, thereby minimizing slow clearance from extracranial tissues. Normal values for both fast and slow compartments are reproducible and are in good agreement with the carotid injection method. The speech dominant hemishpere has higher flow than the right under conditions described. Posterior portions of the cranium over the cerebellum and brain stem appear to have higher flow gray values than the cerebral cortex. Gray matter flow decreases with advancing age.