摘要:

This report concerns the presence and distribution of Lewy bodies in the peripheral nervous system of three patients with Parkinson's disease and ten age‐matched nonparkinsonian individuals. We examined the paravertebral and celiac ganglia as representatives of the sympathetic system; the submandibular ganglion as representative of the parasympathetic system; the esophagus, stomach, duodenum, jejunum, ileum and colon as representatives of the enteric system, and the trigeminal and dorsal spinal ganglia as representatives of the somatic sensory system. Lewy bodies were observed in the peripheral sympathetic and enteric systems of two of the three patients with Parkinson's disease. These two patients had numerous Lewy bodies in the submandibular ganglia. The third patient did not have Lewy bodies in the peripheral autonomic nervous system, nor did the ten control individuals studied. Lewy bodies were not detected in the somatic sensory system of any of the three parkinsonian patients. Our findings indicate that the peripheral autonomic system, including the parasympathetic system is affected in Parkinson's diseas

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1994.tb00236.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

This report presents the results of a quantitative investigation on incidence and distribution of senile plaques (SPs) and neurofibrillary tangles (NFTs) in ten cases of late‐onset diffuse Lewy body disease (DLBD). The methenamine‐silver method was used. SP were distributed throughout the cerebral cortices of all cases. The SP stages in seven of them were compatible with those of Alzheimer‐type dementia (ATD). NFTs occurred most frequently in the transentorhinal and entorhinal pre α regions and the hippocampal CA‐1 area. The occurrence of NFTs was generally restricted to the hippocampus and parahippocampal cortex. NFTs were rarely encountered in the neocortex. The NFT stages of all ten cases were compatible with those borderline between ATD and physiological aging. When both, the SP and NFT stages are considered concurrently, the majority of our late‐onset DLBD cases had levels that corresponded to those in the very mild of ATD or the borderline between ATD and physiological aging. The occurrence of certain DLBD cases with senile changes that are insufficient for a diagnosis of ATD indicates that DLBD should not be regarded as representing part of an AT

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1994.tb00237.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

This report concerns a clinicopathological study of six Japanese patients with Machado‐Joseph disease. All were unrelated females; their ages at disease onset ranged from 17 to 36, and the length of the clinical course from 12 to 50 years. The results of neurological examinations indicated that lower motor neuron signs such as ophthalmoparesis, dysphagia, atrophy of the tongue, and muscular weakness of the extremities appeared in the intermediate stages of the disease, and that from these to the end stages, involvement of the lower motor neurons became cardinal signs of the disease. Urinary and/or fecal incontinence was evident in four patients.Post‐mortem investigations revealed that, in comparison with European and American cases, the involvement of the globus pallidus (particularly the medial segment) and grumose degeneration of the dentate nucleus were salient pathological features of Japanese Machado‐Joseph disease patients. We also noticed involvement of the Onufs nucleus of the two cases in which this structure was examined.The application of a semiquantitative method revealed that neuronal loss in the pontine nucleus, hypoglossal nucleus, and anterior horn of the cervical cord increased in relation to the duration of the disease. By contrast, the severe loss of neurons in the dentate nucleus and Clarke's colum was not related to the length of the clinical course.We suggest that in the initial stages of Machado‐ Joseph disease the cerebellar ataxia is probably due to the involvement of the dentate nucleus and Clarke's column, and that in the advanced stages it is caused by alterations of the pontine nucleus as well. We believe that bulbar signs and muscular weakness of the extremities that appear in the middle stages of the disease are due to progressive neuronal loss in the hypoglossal nucleus and anterior horn. The issue regarding the neuropathological similarities of Machado‐Joseph disease in Japanese patients, “Marie's ataxia” and spinopontine degeneration is addressed in t

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1994.tb00238.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

This report deals with the study of aging gracile axonal dystrophy in an old (28 years), but healthy Japanese monkey. Light and electron microscopy was used throughout. The animal was systemically perfused, under deep anesthesia, with a mixture of glutaraldehyde and paraformaldehyde. For light microscopy, epoxy resin‐embedded tissue of the gracile nucleus was cut into one μm sections, and for electron microscopy (accelerating voltage 300 KV), into 0.2 μm sections. The fine structure of over 20 different spheroids was investigated. Several ultrastructural features not previously observed and/or documented in lower mammals were found in the spheroids of the monky studied. These features are: 1) a unique pattern of smooth endoplasmic reticulum proliferation, characterized by dense parallel alignment of non‐branching tubules; 2) the accumulation of dense homogeneous osmiophilic substance; 3) presence of screw‐like paracrystalline needles, and 4) absence of such characteristic secondary structures as layered loops of membranes and coiled tubular rings. The dense homogeneous osmiophilic substance and the screw‐like paracrystalline needles were previously described or claimed to be present in aging axonal dystrophy of humans. The author addresses and emphasizes the value and importance of a comparative approach in neuropathology and concludes that the study of the brains of other primates is indispensable for the elucidation of human axonal dystrophy, brain aging in humans, and human brain diseases i

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1994.tb00239.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

This report describes a case of unclassifiable presenile dementia. A 38‐year‐old man, blind since shortly after birth, started to exhibit personality changes, such as irritability and withdrawal. The following year he began to experience disorientation, auditory hallucinations, delusions of persecution, memory disturbances, and generalized convulsions. Two years later, the patient exhibited snout reflex, grasp reflex, and small steppage gait. He became apallic and died of acute heart failure at the age of 41, three years after the onset of neurologic symptoms. The neuropathological examination revealed numerous ring‐, crescent‐, or horseshoe‐shaped neurofibrillary tangles around the nuclei of neurons, particularly those in the external granular layer of the cerebral cortex and the dentate gyrus of the hippocampus. However, no senile plaques were found. Neuropil threads and glial cytoplasmic inclusions of the oligodendroglia were detected in the cerebral white matter, hippocampus, basal ganglia, diencephalon, and brainstem. To our knowledge, no similar case of presenile dementia has been described h

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1994.tb00240.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Shy‐Drager syndrome (SDS) is a clinical disease entity that, because of its pathological features, is included in the category of multiple‐system atrophy (MSA). However, we describe in this report a case of SDS that differed clinicopathologically from MSA. A 65‐year‐old man gradually developed impotence, orthostatic hypotension, parkinsonism, dysarthria, and bulbar signs in the terminal stage, all compatible with SDS. In addition to these signs, involuntary movements of the mouth and toes were noted in the course of the disease, and severe myoclonus‐like movements were observed during the terminal stage. The postmortem examination revealed degenerative neuron changes with astrocytosis in the substantia nigra, putamen, caudate nucleus and nucleus accumbens that were accompanied by the presence of Rosenthal fibers. Loss of myelin sheaths which were replaced by fiber gliosis with Rosenthal fibers, was evident in the hilus of the dentate nucleus. By comparison, the neurons of the autonomic spinal centers were intact. The overall features of the present case were different from those hitherto described for SDS, suggesting that they may reflect presentation of a new disease category for this

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1994.tb00241.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY