1/10. Brainstem-type lewy body disease presenting with progressive autonomic failure and lethargy.The authors report an autopsy case characterized by progressive lethargy and autonomic failure with a distinctive pattern of occurrence of lewy bodies. Autonomic dysfunction such as sleep apnea, orthostatic hypotension, dysuria, and hypohidrosis predominated with lethargy, whereas parkinsonism was not apparent. Numerous lewy bodies were widely evident microscopically in brainstem nuclei and the intermediolateral cell columns of the spinal cord, as well as in the sympathetic ganglia, but were rare or absent in the cerebral cortex and other supratentorial structures. Marked neuronal loss was seen in the locus ceruleus, raphe nuclei, dorsal vagal nuclei, and intermediolateral cell columns, but neurons in the substantia nigra, other brain regions, and sympathetic ganglia appeared undiminished. This case represents a specific clinicopathologic form of lewy body disease occurring predominantly in the brainstem, spinal cord, and sympathetic ganglia.- - - - - - - - - - ranking = 1keywords = nigra (Clic here for more details about this article) |
2/10. pure autonomic failure in association with human alpha-synucleinopathy.We studied an autopsy case with pure autonomic failure, using anti-alpha-synuclein antibody. Until now there has been no report about the immunohistochemical properties of alpha-synuclein in pure autonomic failure. In conventional stainings, both pre- and post-ganglionic lesions of the sympathetic and parasympathetic nervous systems were found. lewy bodies and Lewy neurites were abundant especially in the sympathetic nervous system. These inclusions were immunoreactive to anti-alpha-synuclein antibody. The intensity of alpha-synuclein immunoreactivity was stronger in the halos than in the cores of the lewy bodies. The edges of the swollen neurites had strong immunoreactivity. The substantia nigra was well preserved, and no cortical lewy bodies were seen. These findings indicate that pure autonomic failure is one of the Lewy body type alpha-synucleinopathies, such as Parkinson's disease and dementia with lewy bodies, targeting the peripheral autonomic nervous system.- - - - - - - - - - ranking = 1keywords = nigra (Clic here for more details about this article) |
3/10. Familial mydriasis, cardiac arrhythmia, respiratory failure, muscular weakness and hypohidrosis.OBJECTIVES: To describe a family with some sort of progressive autonomic failure in one generation (2 affected of a sibship of 7 sisters). The main features were: mydriasis, cardiac arrhythmia, cardiomegaly, hypohidrosis, respiratory failure, and muscular weakness. methods: Pupillometry, evaporimetry, and isokinetic power measurements were carried out. RESULTS: The autonomic dysfunction pattern (mainly cardiac abnormalities, mydriasis) seems to differ somewhat from that of progressive autonomic failure (shy-drager syndrome). "Lewy body-like" inclusions were present, in particular in substantia nigra, but also in locus ceruleus and raphe nuclei (cell loss only in locus ceruleus). There were no oligodendroglial, cytoplasmatic inclusions, apparently a marker in multiple system atrophy. Proper lewy bodies were also present. Differences seemed to prevail vs the shy-drager syndrome. Various traits: muscular weakness pattern (e.g. preferential peroneal distribution), minor elbow contractures, and arrhythmia were reminiscent of Emery-Dreifuss muscle dystrophy (E-D). Distinguishing features included: hereditary pattern, mydriasis, and hypohidrosis. CONCLUSION: Conceivably, this disorder is close to, but still not identical with E-D.- - - - - - - - - - ranking = 1keywords = nigra (Clic here for more details about this article) |
4/10. Idiopathic Parkinson's disease combined with multiple system atrophy. A clinicopathological report.The clinical and postmortem findings of a patient with Lewy body pathology combined with multiple-system atrophy are described. When alive the patient was diagnosed as having Parkinson's disease. Pathological examination found evidence of striatonigral and olivopontocerebellar degeneration, together with lewy bodies at a number of sites characteristic for idiopathic Parkinson's disease. Taken together, the clinical history and histological findings support the coexistence of two disease processes.- - - - - - - - - - ranking = 1keywords = nigra (Clic here for more details about this article) |
5/10. 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 = nigra (Clic here for more details about this article) |
6/10. shy-drager syndrome: the transitional variant.The clinicopathological features of a patient with the transitional variant of the shy-drager syndrome is described. The only previously reported case of the transitional variant was reexamined and pathological similarities to the present case are reviewed. Both patients exhibited features of Parkinson's disease with lewy bodies in the substantia nigra and locus ceruleus. Striato-nigral degeneration and olivo-ponto-cerebellar atrophy were evident in both cases. They can thus be considered as transitional forms of the shy-drager syndrome with feature of both Parkinson's disease and multiple system atrophy.- - - - - - - - - - ranking = 2keywords = nigra (Clic here for more details about this article) |
7/10. motor neuron disease with multi-system involvement presenting as tetraparesis, ophthalmoplegia and sensori-autonomic dysfunction.We carried out a postmortem examination on two Japanese patients, 64- and 80-year-old men whose survival was prolonged with an artificial respirator. They had no family history of neuropsychiatric disorders and were suspected, clinically, as having a motor neuron disease that differed from amyotrophic lateral sclerosis (ALS). As well as upper and lower motor neuron impairment, they showed a variety of symptoms, such as sensory disturbances, hypohidrosis, impotence, ophthalmoparesis and/or atonic neurogenic bladder, and their protein content in cerebrospinal fluid was elevated markedly. Pathological examination revealed the following extensive nervous system involvement: (1) the upper and lower voluntary motor systems, including the IIIrd, IVth and VIth cranial nerve nuclei: (2) the reticular formation and its major afferent pathways; (3) the vestibulospinal and tectospinal systems; (4) the spinocerebellar system and the exteroceptive somatic afferent pathways; (5) the dentatorubral and pallidoluysian systems; and (6) the substantia nigra, locus ceruleus and intermediolateral and Onufrowicz's nuclei. Neither Bunina bodies, Lewy body-like hyaline inclusions nor ubiquitin immunoreactive skein-like structures were observed. The distribution of the lesions was quite different from that in patients with ALS and the other known related diseases. Recently, seven autopsied cases with clinical and histopathological similarities to our patients have been reported in japan. Our conclusion is that our two and these seven patients should be classified as having a new motor neuron disease entity, which can be is differentiated from ALS.- - - - - - - - - - ranking = 1keywords = nigra (Clic here for more details about this article) |
8/10. Pure progressive autonomic failure: a clinicopathological study.Two cases of pure progressive autonomic failure (PAF) are presented. A postmortem study of one case (case 2) showed a pathology resembling that of Parkinson's disease. Marked cell loss was noted in the substantia nigra, nucleus ceruleus, and intermediolateral column of the spinal cord, while cell loss in the sympathetic ganglion was not remarkable. This case may be an exceptionally rare case of late-onset PAF in which autonomic failure was mainly ascribed to preganglionic (and central) pathology, although autonomic function tests suggested postganglionic sympathetic disorder in both cases.- - - - - - - - - - ranking = 1keywords = nigra (Clic here for more details about this article) |
9/10. Autoimmune autonomic failure in a patient with myeloma-associated shy-drager syndrome.We report here the case of a patient with the shy-drager syndrome and multiple myeloma who had evidence consistent with a central neural autoimmune basis for sympathetic autonomic failure. Autonomic function testing showed no recordable peroneal skeletal muscle sympathoneural traffic, normal arterial norepinephrine (NE) spillover during supine rest and no increment in NE spillover during exposure to lower body negative pressure. The patient's cerebrospinal fluid and serum contained an immunoglobulin g that bound to rat locus ceruleus (LC) in an in vitro test system. The myeloma protein was of the lambda subtype and bound in the rat LC, without binding in the substantia nigra, as demonstrated with anti-lambda antiserum. Since in this case the monoclonal antibody produced by the myeloma bound specifically to LC cells, the results are consistent with the hypothesis that in this patient the shy-drager syndrome may have had an immune-mediated basis.- - - - - - - - - - ranking = 1keywords = nigra (Clic here for more details about this article) |
10/10. The distribution of lewy bodies in pure autonomic failure: autopsy findings and review of the literature.pure autonomic failure (PAF; also known as idiopathic orthostatic hypotension or Bradbury-Eggleston syndrome) is an uncommon sporadic disorder, characterized by autonomic failure without other neurological deficits and histopathologically by cell loss in intermediolateral columns and sympathetic ganglia. Few postmortem studies of patients with PAF have been reported in the literature, and none have demonstrated lewy bodies in distal axons, although this has been described as a feature in Parkinson's disease with autonomic failure. We report a patient with PAF who had orthostatic hypotension and urinary symptoms for 15 years prior to death at the age of 63 years. Postmortem findings included typical and atypical lewy bodies in the substantia nigra, locus ceruleus, substantia innominata, and sympathetic ganglia, as well as in autonomic axons in the epicardial fat, autonomic nerve fascicles in periadrenal adipose tissue, and autonomic nerves in the muscularis of the urinary bladder. Sites of autonomic nerve involvement correlated with clinical symptomatology, and thus were a valuable observation in the complete autopsy. Systemic autopsy results should be reviewed carefully in patients with PAF, as lewy bodies in this disease may be seen in distal axons at a great length from their primary cell bodies.- - - - - - - - - - ranking = 1keywords = nigra (Clic here for more details about this article) |
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