Differences in caloric utilization in eating disordered adolescents.

Caloric utilization is an important aspect of the clinical management of eating disorders. Caloric intake and body weight of 32 inpatient bulimic and anorectic girls and 30 normal adolescents were measured. Normal weight bulimics ate fewer calories while anorectics ate more calories per kilogram body weight compared with the control group. Anorectics have greater difficulty to eat sufficient calories to maintain their weight. These findings indicate that treatment should be extended beyond the point of time where normal weight is reached.

The original Gerstmann-Sträussler-Scheinker family of Austria: divergent clinicopathological phenotypes but constant PrP genotype.

We present new data on the original Austrian kindred with Gerstmann-Sträussler-Scheinker disease (GSS) which encompasses currently 221 members in 9 generations. The mode of inheritance is autosomal dominant. Predominant clinical features are slowly progressive ataxia and late impairment of higher cerebral functions. In contrast, a recent case with proven P102L mutation of the PRNP gene had rapidly developing dementia and severe cortical damage indistinguishable from the clinicopathological phenotype of Creutzfeldt-Jakob disease (CJD). PRNP codon 129 was homozygous for methionine in both the historic and recent cases. Neuropathology confirms spongiosis of variable degree and numerous protease resistant/prion protein (PrP) amyloid plaques scattered throughout most of the brain as constant features in this family. Some amyloid deposits are surrounded by dystrophic neurites with accumulation of phosphorylated neurofilaments and abnormal organelles, reminiscent of Alzheimer-type plaques. Severe telencephalic damage and a synaptic-type fine granular immunoreactivity in laminar distribution in the cortex with anti-PrP after hydrated autoclaving of sections were seen only in the recent patient. In conclusion, factors in addition to the PRNP genotype at codons 102 and 129 must play a role in determining clinicopathological characteristics of this inherited brain amyloidosis.

MMPI and critical flicker frequency (CFF) analysis in headache patients with and without drug abuse.

Sixty-three headache patients (migraine: n = 28; tension-type headache: n = 35) who fulfilled the IHS criteria of 'drug abuse' were investigated by means of the Minnesota Multiphasic Personality Inventory (MMPI) and the Critical Flicker Frequency (CFF) analysis. The results were compared to those of 63 headache patients without drug abuse (matched-pair case-control study). With respect to the MMPI results, no statistically significant differences between patients with drug abuse and patients without drug abuse were found. However, patients with drug abuse showed significantly decreased CFF values compared to patients without drug abuse. This was true both for patients with migraine and for patients with tension-type headache. Thus, CFF analysis may serve as a useful method to differentiate between headache patients with drug abuse and those without drug abuse.

Gamma-vinyl-GABA (vigabatrin) in the therapy of Lennox-Gastaut syndrome: an open study.

The antiepileptic effect of vigabatrin (gamma-vinyl GABA, VGB) in children has been demonstrated in controlled and open studies. According to the literature, results were good to excellent in partial seizures (with and without becoming secondarily generalized) and promising in infantile spasms (IS). In patients with myoclonic epilepsies of early childhood and especially those with Lennox-Gastaut syndrome (LGS), the effect of VGB has been investigated only to a limited extent and the pattern of response was variable. The present open, add-on, dose-ranging study was initiated to assess the long-term effect and safety of VGB in a cohort of 20 children with LGS who were not responding sufficiently to first-line drug monotherapy with valproate (VPA) instead of adding classical second-line antiepileptic drugs [AEDs: benzodiazepines (BZD), phenobarbital (PB), primidone (PRM)], which usually are associated with rapid diminution of their antiepileptic properties and a high frequency of side effects. Eighty-five percent of children experienced a 50-100% reduction in seizure frequency, even after dose reduction of VPA. No serious side effects occurred except in 1 patient who experienced dyskinesia. Mood changes, sedation, ataxia, and hypersalivation, well-known complications of other AEDs, were not observed.