1/15. MR findings in a patient with kernicterus.We report on a 2.5-year-old boy with severe mental retardation, choreoathetosis, dystonia, muscle rigidity, opisthotonus and severe hearing impairment. He had history of severe hyperbilirubinaemia immediately after birth presumably due to ABO incompatibility. The history and the clinical picture suggested the diagnosis of kernicterus. The MR imaging examination upon admission revealed bilateral signal intensity increase in the globus pallidum on T2-weighted sequences. Additionally, our patient showed signal intensity changes within the subthalamic nuclei, which is known to be another characteristic area of bilirubin deposition in kernicterus.- - - - - - - - - - ranking = 1keywords = globus (Clic here for more details about this article) |
2/15. Bielschowsky bodies (Lafora bodies of Bielschowsky type): report of a case associated with Rosenthal fibers in the brain stem.Bielschowsky bodies are an uncommon type of polyglucosan body. Similar to Lafora bodies, they are characteristically identified within neuronal perikarya and neurites. However, they lack the diffuse distribution of Lafora bodies, and instead are typically restricted to the external pallidum, often in association with status marmoratus or atrophy of the putamen. Fewer numbers of Bielschowsky bodies have also been identified in other areas such as the substantia nigra, putamen and inner globus pallidus. We report an additional case with Bielschowsky bodies in an 18-year old female with cerebral palsy. This case demonstrated multifocal Bielschowsky bodies and abundant Rosenthal fibers in the midbrain and pons. To our knowledge the association of Bielschowsky bodies with this peculiar distribution of Rosenthal fibers has not previously been reported.- - - - - - - - - - ranking = 10.81682301645keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
3/15. A novel grading scale for striatonigral degeneration (multiple system atrophy).striatonigral degeneration (SND) is commonly thought to represent the neuropathological substrate of L-Dopa unresponsive parkinsonism in patients with multiple system atrophy (MSA). Other neuropathological hallmarks of MSA include olivopontocerebellar atrophy (OPCA) and preganglionic sympathetic spinal cord lesions. Clinicopathological evaluation of MSA patients recruited into ongoing natural history studies or neuroprotective intervention trials will require standardized grading of MSA pathology. Based on 25 autopsy cases of MSA, we propose a novel SND grading scale which allows semiquantitative assessment of lesion severity based on neuronal loss, astrogliosis and presence of alpha-synuclein positive glial cytoplasmic inclusions (GCIs) in substantia nigra, putamen, caudate nucleus, and globus pallidus. SND grade I is defined as degeneration of the substantia nigra pars compacta (SNC) with relative preservation of the striatum except for minimal gliosis and GCIs in the posterior putamen ("minimal change MSA"). SND grade II is characterized by neuronal loss, astrogliosis and presence of GCIs in SNC and posterior/dorsolateral putamen. caudate nucleus and external globus pallidus may exhibit slight gliosis. Striatal pathology is severe and extends to anterior ventromedial subregions in SND grade III. There is neuronal loss in caudate nucleus and globus pallidus. GCIs are more abundant in grade II than grade III SNC and putamen. Preliminary clinicopathologic correlation studies suggest milder parkinsonian disability and better initial L-Dopa responsiveness in SND grade I and II cases compared to grade III cases. Prospective clinicopathologic studies are required to validate the proposed SND grading scale and may result in further subdivisions, particularly of SND grade III.- - - - - - - - - - ranking = 32.450469049349keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
4/15. Familial motor neuron disease with Lewy body-like inclusions in the substantia nigra, the subthalamic nucleus, and the globus pallidus.In a familial case of motor neuron disease (MND), 2 unusual features were noted in the necropsy. The first was a pallidoluysonigral degeneration, observed in only 4 other cases of MND and which was here asymptomatic. The second was the presence in degenerated spinal cord anterior horns and in degenerated basal ganglia of neuronal Lewy body-like inclusions stained by antibodies against ubiquitin.- - - - - - - - - - ranking = 43.267292065798keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
5/15. Rapidly progressive autosomal dominant parkinsonism and dementia with pallido-ponto-nigral degeneration.We describe a family with nearly 300 members over 8 generations with 32 affected individuals who have an autosomal dominant neurodegenerative disease characterized by progressive parkinsonism with dystonia unrelated to medications, dementia, ocular motility abnormalities, pyramidal tract dysfunction, frontal lobe release signs, perseverative vocalizations, and urinary incontinence. The course is exceptionally aggressive; symptom onset and death consistently occur in the fifth decade. Positron emission tomographic studies with [18F]6-fluoro-L-dopa (6FD) were performed in 4 patients and 7 individuals at risk for development of the disease. All affected subjects had markedly reduced striatal uptake of 6FD (p less than 0.001). All individuals at risk had normal striatal uptake, but high 6FD uptake rate constants were noted in 3 of the 7 studied. autopsy findings revealed severe neuronal loss with gliosis in substantia nigra, pontine tegmentum, and globus pallidus, with less involvement of the caudate and the putamen. There were no plaques, tangles, lewy bodies, or amyloid bodies. This kindred appears to represent a neurodegenerative disease not heretofore described. We propose the following name for this new genetic disease: autosomal dominant parkinsonism and dementia with pallido-ponto-nigral degeneration.- - - - - - - - - - ranking = 10.81682301645keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
6/15. Brain magnetic resonance imaging in tyrosinemia.A 3.5-year-old girl with tyrosinemia is reported. A computed tomography scan of the abdomen revealed multiple hepatic nodules. Brain magnetic resonance imaging revealed bilateral high-signal changes confined to the globus pallidus on T2-weighted images. globus pallidus lesions likely represented neuropathologic changes such as astocytosis, delayed myelination, and status spongiosus (myelin splitting and vacuolation).- - - - - - - - - - ranking = 12.990781531097keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
7/15. Parkinsonism and extraocular motor abnormalities with unusual neuropathological findings.Parkinsonian patients with ocular motility abnormalities are usually considered to have progressive supranuclear palsy. However, a number of other conditions have been noted to have the combination of parkinsonism and ocular problems. We report a case of rigid akinetic parkinsonism, oculomotor palsy, and eyelid apraxia with postmortem examination. Our findings are unusual in that there was marked gliosis of the substantia nigra with a large amount of free extracellular neuromelanin despite a 3-year clinical course. Only rare hyaline inclusion bodies and no neurofibrillary tangles were seen in the brainstem. Excessive calcification of the vessels of the globus pallidus were also noted. This case represents another example of the diversity of conditions producing parkinsonism with extraocular motor abnormalities.- - - - - - - - - - ranking = 10.81682301645keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
8/15. Long duration Jakob-Creutzfeldt disease.The first patient was a woman hospitalized at the age of 43 years; she received the diagnosis of presenile dementia because of a progressive deterioration of her intellectual functions over an interval of 3 years. On admission there were no localizing neurological findings. During the ensuing years she became unable to speak and was no longer able to respond to commands. death occurred at the age of 46 years from bronchopneumonia. The brain showed marked atrophy of the frontal lobes, moderate in the temporal lobes. The anterior horns of the lateral ventricles were greatly dilated. Microscopically cortical atrophy was severe, with marked depletion of neurons in involved regions. Lamination and polarity were not preserved. There was considerable increase of astrocytes and microglia, and focal sponginess was prominent. The hippocampus showed atrophy of Sommer's sector and subiculum. There were no neurofibrillary tangles and no argyrophilic plaques. The occipital sections showed little neuronal loss and no increased astrocytes or sponginess. The insulae showed neuronal loss and gliosis. There was bilateral atrophy of the caudate nuclei and globus pallidus adjacent to the dystrophic anterior limbs of the internal capsule. In the brain stem the frontopontine tracts were partially demyelinated and showed reactive gliosis. Ventral horns were atrophic with moderate glial reaction. In the second patient the microscopic changes were quite similar to those in Case 1, but there was more severe degeneration of the corticospinal tracts and the ventral horns of the spinal cord which showed considerable loss of neurons and degenerative changes in the remaining nerve cells and nerve fibers. There were many instances of axonal degeneration.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 10.81682301645keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
9/15. A case of progressive supranuclear palsy with fibrillary gliosis of the midbrain and pontine reticular formation.An autopsy case of progressive supranuclear palsy with onset at age 53 and a clinical course of 7 years is reported. The clinico-pathological characteristics of the case were as follows: Fibrillary gliosis of the midbrain and pontine reticular formation corresponded clinically to personality changes: The patient had formerly been irascible and became extremely mild-mannered. He showed lack of spontaneity and initiative and slowing of thought processes, taking him longer than normal to find words and ideas. gliosis of the nucleus ventralis lateralis of the thalamus was secondary to the degeneration of the cerebellar dentate nucleus and the globus pallidus from which they receive fibers. neurofibrillary tangles were found in the cerebral cortex.- - - - - - - - - - ranking = 10.81682301645keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
10/15. multiple system atrophy with neuronal intranuclear hyaline inclusions. Report of a new case with light and electron microscopic studies.An 18-year-old girl died following a slowly progressive neurodegenerative disease of nine years duration. At 9 years of age, she developed intellectual deterioration associated with speech difficulty, pseudobulbar palsy and ataxia. The progression included spastic quadriplegia, anarthria, severe dysphagia, ophthalmoplegia, and pes cavus. There was no family history. The brain was uniformly small and the substantia nigra was not pigmented. Neuronal loss and gliosis involving globus pallidus, subthalamic nucleus, thalamic nuclei, brain stem, cerebellum, and spinal cord gave the picture of multisystem atrophy. Intranuclear hyaline inclusions were observed in numerous neurons of the central and peripheral nervous system. These were auto-fluorescent and were made up of intermingled straight filaments (8-9 nm in diameter). Only two previously reported cases showing these same inclusions are known. They are reviewed, compared, and discussed in relation to primary neuronal degenerations.- - - - - - - - - - ranking = 10.81682301645keywords = globus pallidus, pallidus, globus (Clic here for more details about this article) |
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