11/16. serum antibodies to brain proteins in a patient with parkinsonism associated with IgM paraproteinemia.We report a 58 year-old man with various neurological symptoms as parkinsonism, autonomic dysfunction, mental deterioration and lower motor neuron involvement associated with IgM gammopathy whose serum showed antibody activities to human brain proteins. His serum reacted with 156kDa protein from substantia nigra and 130kDa of cerebral cortical grey matter, cerebellar cortex, putamen, thalamus or pallidum by immunoblotting method. The serum of this patient reacted with proteins widely distributed in the central nervous system, and this antibody in his serum might be the cause of his various neurological symptoms.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
12/16. 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 = putamen (Clic here for more details about this article) |
13/16. 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 = putamen (Clic here for more details about this article) |
14/16. Nasu-Hakola disease. A case accompanied by abnormalities in fatty acid composition of serum total lipids and amino acid analysis.A case of Nasu-Hakola disease (membranous lipodystrophy) was reported. The patient is a 33-year-old man who started to have pathological fractures at the age of 24 years. Then, neurological symptoms such as dementia appeared gradually. A biopsy specimen of the bone showed membranocystic changes of the adipose tissue. T2-weighted magnetic resonance images of the head showed low intensity in bilateral putamens, internal capsules, globi pallidi and caudate nuclei. One of his brothers has the same disease. In both patients, fatty acid composition of serum total lipids showed increased nervonic acid and plasma amino acid analysis showed decreased glutamine.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
15/16. Multiple system degeneration and involving thalamus, reticular formation, pallido-nigral, pallido-luysian and dentato-rubral systems. A case report.An autopsy case of multiple system degeneration is characterized by the following; (1) progressive dementia and abnormal sleep patterns, followed by Parkinsonian symptoms with terminal akinetic mutism; (2) severe symmetrical degeneration in the thalamus, particularly nucl. medialis thalami, the reticular formation of the brain stem, also the pallidonigral, pallido-Luysian and dentato-rubral systems. As far as we known, there is no case in the literature, of combined system degeneration, which shows such a wide anatomical range of lesions as the present one. Clinico-pathological correlation between dementia and degeneration of the thalamus, and between abnormal sleep-consciousness mechanism and degeneration of the reticular formation are discussed. The extrapyramidal symptoms are discussed from the clinico-pathological aspect. In addition, peculiar eosinophilic bodies are described, which were most frequently found in the putamen.- - - - - - - - - - ranking = 1keywords = putamen (Clic here for more details about this article) |
16/16. Skein-like inclusions in the neostriatum from a case of amyotrophic lateral sclerosis with dementia.Skeins or skein-like inclusions (SLIs) in motor neurons detected by ubiquitin immunohistochemistry are a characteristic finding of amyotrophic lateral sclerosis (ALS). Here we report ubiquitinated SLIs in the putamen and caudate nucleus from a case of ALS with dementia. A 48-year-old Japanese man developed apathy and amimia. Mental and neurological examinations revealed severe character change, muscle atrophy and fasciculation of the distal upper extremities and the tongue, and an exaggeration of the deep tendon reflex. He subsequently showed dysphagia and dysarthria. He died at the age of 51 years, after a total clinical course of about 2.5 years. By immunohistochemistry, ubiquitin-immunoreactive intraneuronal inclusions were observed in the spinal anterior horn cells, the frontal, temporal and entorhinal cortices, dentate fascia of the hippocampus and the amygdala. In addition, ubiquitinated inclusions were also seen in the putamen and caudate nucleus, which appeared as aggregates of thread-like structures similar to SLIs in the spinal anterior horn neurons. They were not seen on hematoxylin-eosin staining, and they also did not show any argentophilia nor did they react with other antibodies, including antibody against tau protein. To our knowledge, this is the first report of the presence of SLIs in non-motor neurons. Our results thus support the notion that ALS is a multisystem disease, and not simply a disease of the motor neurons.- - - - - - - - - - ranking = 2keywords = putamen (Clic here for more details about this article) |
<- Previous | |