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Ornithine aminotransferase (Orn-T) activities in Huntington's disease (HD) brains were found to be reduced, when compared to age-matched control brains, by 34-49% in the frontal cortex, parietal cortex, caudate nucleus and putamen. Such changes were not observed in senile dementia of Alzheimer type or schizophrenia. Alterations in choline acetyltransferase(More)
The basal ganglia and substantia nigra, taken from control human brain and from patients dying with a diagnosis of Parkinson's disease or Huntington's chorea, were analysed with histochemical and biochemical techniques. The pigmented neurons of the substantia nigra pars compacta possess tyrosine hydroxylase immunoreactivity and are disposed in three major(More)
A patient with severe Alzheimer's disease (AD) presented with a severe impairment in naming nouns but selective sparing of the naming of verbs. Her impairment in naming nouns was presented across a wide range of categories investigated. To our knowledge, this is the first case documenting the selective preservation of verb naming in a patient with AD. The(More)
Monoamine oxidase activity was higher in the cerebral cortex and basal ganglia of patients dying from Huntington's disease than in controls. Enzyme kinetics and multiple substrate studies indicated that the increased activity was due to elevated concentrations of monoamine oxidase type B. Concentrations of homovanillic acid were increased in the cerebral(More)
Oro-facial dyskinesia and purposeless trunk and limb movements were assessed, using a standard videotape rating technique, in 182 psychiatric patients receiving antipsychotic medication, in a second sample of 43 elderly psychiatric patients also receiving antipsychotic drugs, and 85 normal, drug-naive subjects. In both the first patient sample and the group(More)
Dopamine receptors were studied in post-mortem brains from control and Huntington's disease patients, using the specific binding of [3H]spiperone to dopamine D-2 receptors and [3H]piflutixol to dopamine D-1 receptors. Both [3H]spiperone binding and [3H]piflutixol binding were reduced by 45-50% in Huntington's disease putamen. The loss of [3H]spiperone and(More)
Approximately 20% of patients with the neurodegenerative disorder frontotemporal dementia (FTD) have an autosomal dominant pattern of inheritance. Genetic FTD is caused by mutations in three genes in most cases (progranulin, microtubule-associated protein tau and chromosome 9 open reading frame 72) although a number of other genes are rare causes. Studies(More)
Ten patients with gegenhalten of the upper limb of mixed aetiology were studied, in nine of whom an association with dyspraxia was found. In four of the patients, the rigidity became more pronounced after the instruction to relax, and only one patient showed improvement after this instruction. In these patients, the resistance to movement, evident as(More)