摘要:

This report describes the striking ipsilateral loss of large and medium‐sized neurons in the pedunculopontine tegmental nucleus‐pars compactus (PPTN‐pc) in an 82‐year‐old man with a massive, long‐standing infarct in the left cerebral hemisphere (area of the middle cerebral artery). No pathologic alterations were evident in the remaining neurons. The neurons in the contralateral PPTN‐pc were well preserved. Since the PPTN‐pc receives inputs from the basal ganglia and the cerebral cortex and sends widespread efferent fibers to the ipsilateral sides of these structures, it could be assumed that the observed loss of neurons is a result of anterograde transneuronal degeneration or of retrogra

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00194.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

In this special lecture at the 34th Annual Meeting of the Japanese Society of Neuropathology, the author expressed his respect for and expectation of neuropathology from the field of clinical neurology. For the bases of the conclusion in this study, following details were presented in five parts.1)The author's initial neurological autopsy case of Wallenberg syndrome was described, and the results of further studies on some basic problems arisen from this case were also shown.2)Statistical studies on the autopsy ratio revealed gradual decrease in every field. and reasons for this undeniable fact and plans of the amelioration were discussed.3)The clinical diagnosis and therapeutic effects were reevaluated on 399 autopsy cases in our Hospital, and the clinicopathological discrepancies in diagnosis were classified into three types. The results of further investigations were presented here mainly in disease groups with pathological diagnosis of Parkinson disease, amyotrophic lateral sclerosis, spinocerebellar degenerations, multiple system atrophy, Creutzfeldt‐Jakob disease and Alzheimer disease.4)On the bases of 2,130 cases of muscle biopsy and 415 cases of sural nerve biopsy, the guide manuals was proposed for improved methods, for the study of specimens, and for diagnostic contribution.5)The research project teams for neuropathology have successively been organized in our hospital. The results on various degenerative disease groups were presented.With this presentation, the author emphasized the importance of sustained participation in clinicopathological work and of reciprocal communication between the two discipline

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00195.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

I briefly review spheroids observed in the anterior horns of the spinal cord in amyotrophic lateral sclerosis (ALS). Spheroids are argentophilic bodies more than 20 μm in diameter. Recently, some connections between the proximal axonal swellings including spheroids and the perikarya have been reported in some ALS patients with a short clinical course or mild depletion of anterior horn neurons. Most of the cell bodies directly connected with the axonal swellings appear normal, and spheroids are considered to be one of the hallmarks of the early histological changes in this disorder. Spheroids are strongly positive with anti‐phosphorylated neurofilament antibody, and are also positive with calcitonin gene‐related peptide and anti‐peripherin antibody. Some spheroids are immunostained with anti‐synaptophysin antibody and anti‐ubiquitin antibody. Spheroids are not immunostained with anti‐phosphorylated tau antibody, or high molecular weight microtubule associated proteins. Electron microscopically, spheroids are usually composed of densely packed accumulation of 10 nm neurofilaments with a variety of orientations, plus vesicles, dense bodies and mitochondria. When the swellings of the initial segment is relatively pronounced, the undercoating is obscured and the neurofilaments become interwoven in some parts. In the first internode of the myelinated axons, as the swellings become larger, the neurofilaments lose their parallel orientation and become intermingled. Large accumulation of neurofilaments resembling spheroids in the perikarya of large anterior horn cells suggests that spheroids could be derived not only from the axon including the proximal portion, but also from the perikarya. Structures apparently identical to axonal spheroids are observed at the light and electron microscopic levels in the proximal portion of axons of anterior horn cells in animal models intoxicated with β, β'‐iminodipropionitrile (IDPN), or with aluminum, in hereditary canine spinal muscular atrophy (HCSMA). The pathogenetic mechanism is probably associated with an impairment in slow axonal transport which particularly affects the neurofilaments in IDPN and aluminum intoxication. Impairment of slow axonal transport of neurofilaments also plays an important role in the pathogenesis of ALS. The average diameter of even normalappearing initial segment is larger in ALS than in the controls. The perikarya connected with the swollen proximal axons and their dendrites almost always appear normal. These findings suggest that the slow axonal transport of neurofilaments is probably impaired in this portion of the axon at an early stage in ALS as well as animal models for human ALS. However, techniques to analyze slow axonal transport in humans still remain tobe developed. Recently, overexpression of neurofilament subunits in transgenic mice produces a condition resembling ALS. The transgenic model may offer an interesting perspective not only for testing therapeutic strategies but also for investigating in a systematic way the various genetic and environment factors controlling the onset and progression of the disease and might yield new insights on the

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00196.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Bunina bodies (BBs), small eosinophilic intraneuronal inclusions in the remaining lower motor neurons, are the only specific pathological hallmarks of amyotrophic lateral sclerosis (ALS). This report deals with the study of and provides a review on the morphology and morphogenesis of BBs. Immunohistochemical studies disclosed that BBs express cystatin C, but they failed to react with a variety of other antibodies. Serial electron microscopic investigations documented that BBs consisted of electron‐dense amorphous material that contains tubular and vesicular structures and that in the early stages of BB formation the amorphous material was sometimes present around the tubular and vesicular structures. From immunoelectron microscopic studies with the antiserum to cystatin C, there was evidence for the presence of immunoperoxidase reaction products in the periphery of BBs. Furthermore, cystatin C was mainly localized in the Golgi apparatus of anterior horn cells of an adult cat. It would appear that BBs may represent abnormal accumulations of unknown proteinaceous materials that are associated with the Golgi apparatu

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00197.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Hyaline (HI) and skein‐like inclusions (SLI) are characteristic of familial and sporadic amyotrophic lateral sclerosis (ALS). The HIs are usually round eosinophilic structures, some of which consist of cores and halos and are called Lewy body‐like HIs. The HIs, composed of various amounts of granule‐associated thick filaments and neurofilaments, have varied appearances and may be heterogeneous. The SLI is basically a thready structure of a bundle of thick filaments. The threads may be present singly or in the form of networks or aggregates with each other. Occasionally, aggregates of threads may form single larger inclusions that resemble HIs. These features indicate that the HI and the SLI may be closely related. Other than their intense ubiquitination, the molecular nature of HI and SLI is still unknown. The investigation of the genesis of these inclusions may provide important clues for elucidating the pathomechanism o

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00198.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects the motor neuron system. However dose only the motor neuron system degenerate selectively in this disease? On the basis of histologic and morphometric studies on Clarke's column and intermediolateral nucleus (IML) neurons, which belong to the non‐motor neuron system in the spinal cord, we report in this Symposium presentation that in sporadic ALS, they are also primarily involved in the disease process. However, the rate of degeneration of these neurons is much slower than that of lower motor neurons. Our results indicate that Clarke's column neurons and IML neurons in the lower thoracic segments may start to disappear after the patients have begun to require respiratory support, which is the final stage of the natural hintory of the disease. In addition, it should also be empasized that Bunina bodies and spheroids are neuropathological features of Clarke's column in ALS patients, but not of the IM

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00199.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

A neuropathological study of cases of amyotrophic lateral sclerosis with dementia [ALS‐D (+)] revealed, in addition to changes of the motor neuron system, temporal lobe (TL) lesions that had a characteristic distribution. The alterations include neuronal loss emphasized in the medial cortex of the temporal tip and focal depletion of pyramidal neurons in the pes hippocampi. These observations indicate that these regions may be the first non‐motor cerebral cortex structures to be involved in this condition, and that the neuronal loss in these TL areas may play a role in the mental symptoms observed in ALS‐D(+). Moreover, the TL of approxiamtely 10% of ALS cases without dementia [ALS‐D (‐)]had similar alterations. This poses the question of whether these cases may represent an intermediate subgroup between ALS‐D (‐) and ALS‐D (+), or whether the ALS‐D (‐) patients with TL lesions were indeed demented, but in whom the proper the proper diagnoses were overlooked. Answers to these points can only be provided by detailed clinicopathological studies on appropriately

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00200.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

We performed a neuropathological study on 13 patients with amyotrophic lateral sclerosis (ALS) who had been on respirator support. Twelve patients had developed upper and lower motor neuron signs, exclusively, before being placed on respirators. One patient was diagnosed as having the pseudoneuritic type of ALS. The main pathological findings in the 13 cases were the simultaneous involvement of both motor neurons and the presence of Bunina bodies and ubiquitinated filaments in the anterior horn cells. While on the respirator, two rapidly progressing patients developed supranuclear ophthalmoplegia and ended in totally locked‐in state. These two cases showed widespread systemic degenerative changes in the central nervous system (CNS) that involved the frontoparietal cortices adjacent to the precentral gryrus, the pallido‐luysio‐nigral system, thalamus, hypothalamus, amygdala, brain stem tegmentum, dentate‐rubral system, Clarke's spinocerebellar‐middle root zone and Onuf's nucleus. Eight normally or rapidly progressing patients developed supranuclear ophthalmoplegia, with or without emotional disinhibition and blood pressure lability. Some of the CNS structures affected in the two patients described above, were also altered in these eight cases. We attribute the ophthalmoplegia to the involvement of the brain stem tegmentum and frontal cortices, which are concerned with eye movement. In addition, the emotional and autonomic labilities of some of the patients may be related to alterations of the limbic system. By contrast, the clinicopathological features of the remaining three, slowly progressing patients were confined to both motor neurons. On the basis of these findings we address the intrinsic uniformity of ALS and some of its d

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00201.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

This report concerns the evaluation of the clinicopathological features of amyotrophic lateral sclerosis (ALS) in two male patients who survived ten years with the assistance of artificial respirators. The patients were 54 and 52 years old at disease onset and they were placed on respirators 32 and 27 months later, respectively. The patients did not show impairments in occulomotor, sensory and rectovesical functions nor develop bedsores, with the tetrad of ALS being negative. The main histopathological changes observed include neuron loss of the upper and lower motor systems and degeneration of the pyramidal tract. By contrast, other systems such as the posterior column, spinocerebellar tracts, Clarke's nucleus, oculomotor nucleus and Onuf's nucleus remained intact. These findings on our two patients suggests that there may be cases in which the selective features of ALS are maintained throughout prolonged respiratory management.

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00202.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY