11/18. Diffuse cerebral white matter T2-weighted hyperintensity: a new finding of general paresis.General paresis (parenchymatous neurosyphilis) is a rare disease, and in recent years the number of papers published on the magnetic resonance imaging findings has been limited. The findings are as follows: cerebral atrophy; mesiotemporal T2 hyperintensity; ventriculomegaly; pathological T2 hypointensity of the globus pallidus, putamen, the head of the caudate nucleus and thalamus. We present a new finding, diffuse cerebral white matter T2 hyperintensity, observed in a patient with general paresis with a 5-year history of progressive dementia.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
12/18. Studies on neurotransmitter markers of the basal ganglia in Pick's disease, with special reference to dopamine reduction.gamma-aminobutyric acid (GABA), substance p and dopamine concentrations and choline acetyltransferase (ChAT) activity were measured in post-mortem cerebrocortical and basal ganglial areas of 14 controls and 4 patients with pathologically verified Pick's disease (1 classic case and 3 cases of the generalized form). GABA and substance p levels in the substantia nigra and the globus pallidus were generally decreased, corresponding to the moderate to severe loss of small neurones in the striatum. ChAT activities in the striatum varied from case to case, in proportion to various degrees of loss of large neurones in the striatum. These neurotransmitter abnormalities in Pick's disease were exactly the same as those in Huntington's disease. However, dopamine concentrations were markedly reduced in the striatum in Pick's disease, whereas striatal dopamine in Huntington's disease is reported to be increased. A dopamine reduction in the striatum of Pick's disease was more disproportionately prominent than expected for various degrees of nigral cell loss. This may be one of the important factors which prevents the generation of choreic movements in Pick's disease in spite of definite striatal atrophy similar to Huntington's disease.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
13/18. ataxia, chorea, seizures, and dementia. Pathologic features of a newly defined familial disorder.Five generations of a family exhibit a unique autosomal dominant neurologic disorder characterized by the development (usually between 15 and 30 years of age) of ataxia, seizures, choreiform movements, progressive dementia, and death after 15 to 25 years of illness. Neuropathologic findings in two deceased family members revealed remarkably similar findings, including marked neuronal loss of the dentate nucleus, microcalcification of the globus pallidus, neuroaxonal dystrophy of the nucleus gracilis, and demyelination of the centrum semiovale. The clinical and pathologic findings are closely correlated. ataxia and chorea are related to severe neuronal loss in the dentate nucleus with calcification in the globus pallidus. dementia occurs with progressive demyelination of the centrum semiovale, and loss of posterior column function occurs with neuroaxonal dystrophy of the nucleus gracilis and nucleus cuneatus.- - - - - - - - - - ranking = 2keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
14/18. Pure psychic akinesia with bilateral lesions of basal ganglia.Three patients showed dramatic psychic akinesia after recovery from toxic encephalopathy. They had no or only mild motor disorders. The spontaneous psychic akinesia was reversible when the patient was stimulated, as if there was a loss of self psychic activation. Intellectual capacities were normal. Two patients had stereotyped behaviours resembling compulsions. In all patients CT cans showed bilateral lesions in the basal ganglia, mainly within the globus pallidus.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
15/18. Neuropsychiatric disorders, myoclonus, and dystonia in calcification of basal ganglia pathways.Two cases of basal ganglia calcification involving the globus pallidus are presented. Both patients had cognitive dysfunction, temporal lobe-like symptoms (including amnestic state, perceptual distortions, or complex visual hallucinations), and myoclonus. Patient 1 manifested depression, auditory hallucinations, anxiety, paranoia, and postural tremor; patient 2 manifested multifocal dystonia with dystonic tremor. These cases supplement other reports of psychotic features and dementia associated with pallidal pathology. Additionally, the phenomena encountered in these cases are considered in light of recent advances in our understanding of basal ganglia functional pathways. These cases afford a potential pathophysiological window to the possible role of the globus pallidus in these neuropsychiatric conditions. In concert with other recent findings, these cases suggest specific pathway involvement in hallucinations, paranoia, depression, myoclonus, and dystonia. Further research will indicate if these pathways play a role in schizophrenia, mood disorders, and anxiety disorders.- - - - - - - - - - ranking = 2keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
16/18. Repetitive and compulsive behavior in frontal lobe degenerations.The authors review the relationship of repetitive behaviors to frontal lobe degenerations and report the repetitive and compulsive behaviors, radiologic imaging findings, and neuropathology of 3 patients with dementia secondary to frontal lobe degeneration. These 3 patients and 78% of 46 proven pathologic cases of frontal lobe degeneration described in the literature demonstrate repetitive behaviors ranging from motor stereotypies to complex obsessive-compulsive disorder. This review suggests that combined damage to the frontal lobe, caudate nucleus, and globus pallidus may account for the repetitive behaviors seen in frontal lobe degenerations, idiopathic obsessive-compulsive disorder, and other neuropsychiatric diseases.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
17/18. Obsessive-compulsive disorders in Pick's disease.The authors present a follow-up of a previously described patient with obsessive-compulsive disorder (OCD). Her condition continued to worsen, and she died 5 years after disease onset. Neuropathological findings were consistent with the diagnosis of Pick's disease. They revealed, in addition to the "knife-edge" frontotemporal atrophy, striking atrophy with extensive neuronal loss and gliosis involving the caudate nuclei and, to a lesser degree, the putamens and globus pallidus. neuroimaging data had showed isolated atrophy of the caudate nuclei in the early stages of Pick's disease in this patient when OCD was the leading clinical manifestation. Relevant literature is reviewed, and the role of caudate nuclei atrophy in the development of OCD is discussed.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
18/18. Atypical diffuse lewy body disease with neuritic abnormalities.dementia associated with cortical and subcortical lewy bodies (LB's) is a distinct entity with variable clinico-pathological presentation. We present the case of a 49-year-old male with progressive dementia. At autopsy, the brain showed diffuse cortical atrophy and ubiquitin-positive LB's in the dentate gyrus, deep layers of the neocortex, basal ganglia, nucleus basalis and substantia nigra. Thioflavine S stains of the neocortex and hippocampus were negative for the presence of plaques and tangles. Anti-ubiquitin immunostaining revealed abundant dystrophic neurites, torpedo-like axons and abnormal neuritic processes in the molecular layer of the dentate gyrus, pyramidal cell layer in CA1, subiculum, deep layers of the neocortex, claustrum, caudate, putamen and globus pallidus. Relatively mild neuritic alterations were observed in the nucleus basalis of Meynert (NbM) and locus ceruleus. The presence of this unique type of axonal damage associated with lewy body disease, in the absence of plaques and tangles, might suggest a divergent mechanism of neuritic injury in the wide spectrum of this disorder.- - - - - - - - - - ranking = 1keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
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