Our results show that the presence of MOG-abs strongly depends on the age at disease onset and that high MOG-ab titers were associated with a recurrent non-MS disease course.
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.
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.
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.
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