1/13. Camptocormia: pathogenesis, classification, and response to therapy.Originally considered a psychogenic disorder, camptocormia, an abnormal posture with marked flexion of thoracolumbar spine that abates in the recumbent position, is becoming an increasingly recognized feature of parkinsonian and dystonic disorders. Prior reports were limited by sample size, short follow-up, and paucity of data on response to therapy. The authors reviewed 16 patients evaluated in their PD Center and movement disorders Clinic diagnosed with camptocormia. In addition to detailed neurologic assessment all patients were videotaped. The mean age was 64.9 /- 17.4 years, mean age at onset of neurologic symptoms was 51.5 /- 19.9 years, duration from onset of neurologic symptoms to development of camptocormia was 6.7 /- 7.6 years, and the mean duration of camptocormia was 4.5 /- 3.9 years. Of the 16 patients, 11 (68.8%) had Parkinson disease (PD); others had dystonia (n = 4) and tourette syndrome (n = 1). Twelve patients received levodopa, with minimal or no improvement in the camptocormia. Nine patients received botulinum toxin type A injections into the rectus abdominus, with notable improvement in their camptocormia in four. One patient underwent bilateral subthalamic nucleus deep brain stimulation for PD, but there was no improvement in camptocormia. Based on this series and a thorough review of the literature of camptocormia, head drop, and bent spine syndrome, the authors propose etiologic classification of camptocormia and conclude that this heterogeneous disorder has multiple etiologies and variable response to systemic and local therapies.- - - - - - - - - - ranking = 1keywords = subthalamic, nucleus (Clic here for more details about this article) |
2/13. dementia of frontal lobe type and motor neuron disease. A Golgi study of the frontal cortex.Neuropathological findings in a 38 year old patient with dementia of frontal lobe type and motor neuron disease included pyramidal tracts, myelin pallor and neuron loss, gliosis and chromatolysis in the hypoglossal nucleus, together with frontal atrophy, neuron loss, gliosis and spongiosis in the upper cortical layers of the frontal (and temporal) lobes. Most remaining pyramidal and non-pyramidal neurons (multipolar, bitufted and bipolar cells) in the upper layers (layers II and III) of the frontal cortex (area B) had reduced dendritic arbors, proximal dendritic varicosities and amputation of dendrites as revealed in optimally stained rapid Golgi sections. pyramidal cells in these layers also showed depletion of dendritic spines. Neurons in the inner layers were preserved. Loss of receptive surfaces in neurons of the upper cortical layers in the frontal cortex are indicative of neuronal disconnection, and are "hidden" contributory morphological substrates for the development of dementia.- - - - - - - - - - ranking = 0.091824654963028keywords = nucleus (Clic here for more details about this article) |
3/13. An autopsy case of atypical motor neuron disease with Bunina bodies in the lower motor and subthalamic neurons.We report a 37-year-old male without any family history of neurological disease who suffered progressive muscular atrophy and sensory impairment of 4 years' duration. autopsy revealed neuronal loss in the anterior horns of the spinal cord and in the hypoglossal and facial nuclei of the brain stem. The corticospinal tracts of the spinal cord showed only mild degeneration. In addition, there were obvious degenerative lesions manifested by loss of neurons, myelin and axons in the spinal posterior columns, Clarke's column, spinocerebellar tracts and dorsal root ganglia as well as in the subthalamic nucleus, globus pallidus, substantia nigra and cerebellar dentate nucleus. Furthermore, we frequently encountered Bunina bodies not only in the lower motor neurons but also in the subthalamic neurons. We consider this case to be an atypical example of motor neuron disease with features of multisystem degeneration. The fact that Bunina bodies were observed in both lower motor and subthalamic neurons in this case suggests a common etiology of neuronal degeneration in these two different systems.- - - - - - - - - - ranking = 6.5408767251849keywords = subthalamic, nucleus (Clic here for more details about this article) |
4/13. Movement disorder following herpes simplex encephalitis.Two children, an eight-year-old girl and a seven-month-old boy, recovered from herpes simplex encephalitis with minimal neurological residua following acyclovir treatment. Subsequently, they experienced marked deterioration, interpreted as either recrudescent infection or a post-infectious phenomenon. Features of the deterioration included encephalopathy and hyperkinetic movement disorder. MRI studies showed extensive neocortical damage, without involvement of the basal ganglia, thalamus or subthalamic nuclei. With aggressive supportive care, both children made a slow, steady recovery over several months. This supportive care is best provided in a closely supervised interdisciplinary setting.- - - - - - - - - - ranking = 0.90817534503697keywords = subthalamic (Clic here for more details about this article) |
5/13. An autopsy case of atypical infantile motor neuron disease with hyaline intraneuronal inclusions.We describe a 5-year-old boy who had had a progressive motor weakness with bulbar palsy and spasticity of the lower extremities since age 3 years and who died of bronchopneumonia after about two and a half years of the illness. Neuropathologic examination revealed combined degenerative processes in the upper and lower motor neurons, the spinocerebellar and olivocerebellar systems, and the ventral thalamic nuclei. Lewy body-like intraneuronal hyaline inclusions, which ultrastructurally showed irregular accumulations of trilaminar membranous profiles, were detected in the spinal anterior horn, Clarke's dorsal nucleus, facial nerve nucleus, inferior olivary nucleus, and substantia nigra. This case could be considered as a unique form in the group of the infantile motor neuron diseases associated with multi-systemic degenerations in the central nervous system.- - - - - - - - - - ranking = 0.27547396488908keywords = nucleus (Clic here for more details about this article) |
6/13. Sensory neuron degeneration in familial Kugelberg-Welander disease.A 53 year old man developed symptoms of motor neuron disease in childhood. There was a family history of a similar disorder and it was felt to represent a form of Kugelberg-Welander disease. In addition to the motor deficits, sensory abnormalities in his legs were documented during life. autopsy revealed anterior horn cell loss throughout the length of the spinal cord, with preservation of the phrenic nucleus. The lumbar dorsal root ganglia showed active degeneration of sensory neurons, with nuclear changes exceeding cytoplasmic ones. The fasciculus gracilis showed wallerian degeneration. The findings provide direct evidence that sensory neurons can degenerate in some forms of motor neuron disease, and that the "demyelination" or "degeneration" of posterior columns sometimes seen in the various forms of motor neuron disease may actually be secondary to cell body disease in the dorsal root ganglia.- - - - - - - - - - ranking = 0.091824654963028keywords = nucleus (Clic here for more details about this article) |
7/13. Juvenile multisystem degeneration with motor neuron involvement and eosinophilic intracytoplasmic inclusions.A case of juvenile multisystem degeneration with motor neuron involvement, possibly of familial type, showing many unusual clinical and pathologic features is reported. Eosinophilic intracytoplasmic inclusions were present in some remaining anterior horn cells and motor nerve nuclei of the brain stem as well as in a few neurons of the reticular activating system, the dorsal vagus nuclei, and the intermediolateral cell column. Smaller eosinophilic inclusions were seen in large neurons of the caudate nucleus and putamen, substantia nigra, and subthalamic nucleus.- - - - - - - - - - ranking = 1.091824654963keywords = subthalamic, nucleus (Clic here for more details about this article) |
8/13. Fatal reducing body myopathy. Ultrastructural and immunohistochemical observations.Two female infants who developed normally during infancy began to have progressive muscle hypotonia and weakness from 2 years 10 months and 2 years 3 months of ages, respectively. Both patients had rapidly progressive muscle weakness with death from respiratory failure at 4 years 11 months and 3 years 9 months, respectively. In addition to mild inflammation in their muscle biopsies, the most striking finding was the presence of numerous reducing bodies (RB) in almost all degenerating fibers. By electron microscopy, these bodies consisted of fine granular material, usually located around the degenerating nucleus. These bodies showed no immunohistochemical reaction to antibodies against structural, cytoskeletal and membrane proteins and a histone-specific antibody against nuclei and chromosomes. They were occasionally positively stained with a ubiquitin antibody. Although the origin of these bodies remains unknown, they appeared to be related to active myofibrillar degeneration, probably resulting from primary nuclear degeneration.- - - - - - - - - - ranking = 0.091824654963028keywords = nucleus (Clic here for more details about this article) |
9/13. Paraballism associated with anterior opercular syndrome: a case report.We describe the combination of acute bilateral ballism and anterior opercular syndrome in a 75-year-old female hypertensive patient with bilateral multiple small infarcts involving the basal ganglia, the subcortical white matter and the area of the subthalamic nucleus, detected by CT scan and MRI. These clinical manifestations were accompanied by apathy, somnolence and cognitive changes usually observed in cortical involvement. Pathogenetic mechanisms for this unusual clinical picture are discussed on the basis of CT, MRI, and SPECT findings.- - - - - - - - - - ranking = 1keywords = subthalamic, nucleus (Clic here for more details about this article) |
10/13. Atypical McLeod syndrome manifested as X-linked chorea-acanthocytosis, neuromyopathy and dilated cardiomyopathy: report of a family.We report a family with three members affected by a typically X-linked McLeod syndrome. In the proband a very weak positivity for antigens of the Kell group was detected. His sister showed a normal antigenic pattern. We emphasize the prominent neurological picture characterized by a choreic syndrome with atrophy of the caudate nucleus on MRI, psychiatric disturbances, peripheral nerve and muscle biopsy findings indicating slight neuromuscular involvement, and cardiac abnormalities. The differential diagnosis is discussed.- - - - - - - - - - ranking = 0.091824654963028keywords = nucleus (Clic here for more details about this article) |
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