1/47. Following the clues to neuropathic pain. Distribution and other leads reveal the cause and the treatment approach.Neuropathic pain can seem enigmatic at first because it can last indefinitely and often a cause is not evident. However, heightened awareness of typical characteristics, such as the following, makes identification fairly easy: The presence of certain accompanying conditions (e.g., diabetes, hiv or herpes zoster infection, multiple sclerosis) pain described as shooting, stabbing, lancinating, burning, or searing pain worse at night pain following anatomic nerve distribution pain in a numb or insensate site The presence of allodynia Neuropathic pain responds poorly to standard pain therapies and usually requires specialized medications (e.g., anticonvulsants, tricyclic antidepressants, opioid analgesics) for optimal control. Successful pain control is enhanced with use of a systematic approach consisting of disease modification, local or regional measures, and systemic therapy.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/47. Nigral degeneration in a case of amyotrophic lateral sclerosis: evidence of Lewy body-like and skein-like inclusions in the pigmented neurons.This report describes a 58-year-old man who exhibited the clinical features of amyotrophic lateral sclerosis (ALS) at autopsy, 1 year after clinical onset of the disease. Neuropathologically, in addition to degeneration of the upper and lower motor neurons, marked degeneration of the substantia nigra with the appearance of Lewy body-like inclusions (LBI) and skein-like inclusions (SLI) were observed in the remaining pigmented neurons. An immunohistochemical study revealed that both LBI and SLI were immunopositive for ubiquitin. Ultrastructurally, LBI in the substantia nigra were composed of randomly arranged 15 nm thick tubular structures associated with ribosome-like granules, similar to those of LBI in the lower motor neurons. SLI in the substantia nigra were composed of curved bundles of 5 nm thick filaments (thinner than those comprising the SLI in the lower motor neurons). We believe that degeneration of the substantia nigra could be one of the degenerative processes involved in ALS.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
3/47. Accumulation of neurofilaments and SOD1-immunoreactive products in a patient with familial amyotrophic lateral sclerosis with I113T SOD1 mutation.OBJECTIVE: To report neuropathologic features of argyrophilic inclusions in the anterior horn cells, motor cortex Betz cells, and neurons of the medullary reticular formation, spinal posterior horn, and Clarke column in a Japanese case of familial amyotrophic lateral sclerosis with I113T substitution in exon 4 of the copper-zinc superoxide dismutase (SOD1) gene. methods AND RESULTS: These inclusions were stained pale pink on the hematoxylin-eosin stain and dark on the Bielschowsky stain. They were positive for antibodies to phosphorylated neurofilaments, ubiquitin, and SOD1. On electron microscopy, they consisted of abundant intermediate filaments of 10 to 20 nm in diameter with disordered array indicating neurofilaments. CONCLUSION: These findings suggest that the I113T mutation induces accumulation of neurofilaments and SOD1 in the central nervous system neurons.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
4/47. Asymmetrical temporal lobe atrophy with massive neuronal inclusions in multiple system atrophy.This report concerns a rare association of asymmetrical temporal lobe atrophy with multiple system atrophy (MSA). A 53-year-old Japanese woman developed cerebellar ataxia and parkinsonism and was diagnosed as olivopontocerebellar atrophy (OPCA). This patient showed forgetfulness and subsequent disorientation even in the early stage of the disease. She fell into a decorticate state at the age of 64, and died a year later. The autopsy showed MSA with asymmetrical atrophy of temporal lobes, intraneuronal globular inclusions mostly confined to the hippocampus, amygdaloid nucleus, and most abundant in the granule cells in the dentate fascia. These inclusions were intensely argyrophilic and expressed marked immunoreactivity to ubiquitin, but not to neurofilament (NF), tau and paired helical filaments (PHF). Ultrastructurally, they were composed of scattered short filamentous structures of 15 to 30 nm in diameter, ribosome-like granules, mitochondria and lipofuscin. The lack of immunoreactivity against tau, NF and PHF suggests that the inclusions are distinct from Pick bodies. To our knowledge, MSA in association with asymmetrical temporal lobe atrophy with the present neuronal inclusions has not been reported. This case is distinct from MSA combined with atypical Pick's disease in the distribution and immunohistochemical properties of neuronal inclusions, and may present a new variant of MSA since the neuronal inclusions are similar, in many respects, to those of neuronal inclusions reported in MSA. Globular inclusions are also discussed in variants of Pick's disease, amyotrophic lateral sclerosis and Alzheimer's disease.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
5/47. Familial amyotrophic lateral sclerosis with onset in bulbar sign, benign clinical course, and Bunina bodies: a clinical, genetic, and pathological study of a Japanese family.We report a Japanese family with autosomal dominant adult-onset amyotrophic lateral sclerosis (FALS) with onset in the bulbar musculature, clinically benign course, absence of the Cu/Zn superoxide dismutase-1 (SOD 1) gene mutation, and many Bunina bodies, in addition to involvement of the upper and lower motor neurons. The proband was a Japanese woman who was 66 years old at the time of death. family history disclosed five patients with FALS over three generations. She developed dysarthria at age 57, followed by dysphagia, muscle weakness of the upper extremities, and difficulty in respiration. She could walk without support until her death. The elder sister of the proband developed dysarthria at age 48 and died at age 58. A genetic study of the nephew of the proband showed the absence of a mutation in the SOD 1 gene. Neuropathological examination of the proband disclosed neuronal loss in the upper and lower motor neurons, and numerous Bunina bodies in the lower motor neurons without Lewy body-like inclusions or ubiquitin-immunoreactive neuronal inclusions. No degeneration of the Clarke's column, middle root zone of the posterior column, or posterior spinocerebellar tract was present. review of the literature revealed that only patients with FALS with a long survival period of over 5 years had pathological findings consistent with FALS with posterior column involvement. This study contributes to the elucidation of the clinicopathological heterogeneity of FALS.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
6/47. Detection of preclinical motor neurone loss in SOD1 mutation carriers using motor unit number estimation.OBJECTIVE: To determine the pattern of motor neurone loss in amyotrophic lateral sclerosis (ALS). In particular, to determine whether there is a gradual life long presymptomatic motor neurone loss or, alternatively, a sudden catastrophic loss just before the onset of symptoms. METHOD: The statistical motor unit number estimation (MUNE) technique was used in a longitudinal study of 19 asymptomatic carriers of the Cu, Zn superoxide dismutase 1 (SOD1) gene. MUNE results were compared with those of 34 age and sex matched SOD1 negative family controls and 23 population controls. Motor neurone loss was also estimated in 12 patients with sporadic ALS. 84 subjects (43 male and 41 female patients) with an age range from 16-73 years were followed up over three years, both clinically and by MUNE, every six months. RESULTS: In 2 of the 19 mutation carriers, there was a sudden reduction in MUNE several months before the onset of weakness. The patients with symptomatic sporadic ALS also had a reduced MUNE, but there was no detectable loss of motor neurones in the remainder of the subjects. CONCLUSION: MUNE can be used to detect preclinical loss of motor units in familial ALS. Normal numbers of motor neurones were maintained in 17 SOD1 mutation carriers over the three year period. There was an abrupt loss of motor neurones just before the onset of symptomatic weakness in two SOD1 mutation carriers. These results suggest that some form of trigger may initiate rapid cell loss and death of motor neurones just before the onset of symptoms.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
7/47. Sporadic amyotrophic lateral sclerosis with circumscribed temporal atrophy: a report of an autopsy case without dementia and with ubiquitinated intraneuronal inclusions.This report concerns an autopsy case of amyotrophic lateral sclerosis (ALS) with circumscribed temporal atrophy. The patient was a Japanese woman without hereditary burden who was 71-year-old at the time of death. She developed dysarthria and gait disturbance at age 69, followed by dysphagia. A neurological examination about 1 year 11 months after the onset of the disease revealed absence of character change and of dementia. Neuroradiological examination disclosed circumscribed atrophy of the anterior part of the right temporal lobe. The patient died of respiratory failure 2 years after the disease onset. No respirator administration was performed throughout the clinical course. Macroscopically, neuropathological examination showed circumscribed atrophy of the right first temporal gyrus. Histologically, there was neuronal loss in the cerebral cortex, including the first temporal gyrus, the parahippocampal gyrus, subiculum, amygdala, substantia nigra, brain stem motor nuclei, and anterior horns of the spinal cord, in addition to loss of Betz cells, obvious degeneration of the pyramidal tracts, and the presence of Bunina bodies. ubiquitin-immunoreactive intraneuronal inclusions were present in the hippocampal dentate granular cells, frontotemporal cortical layer II neurons, and motor neurons in the brain stem and spinal cord. Based on these clinicopathological findings and a review of the literature, we concluded that our case was atypical ALS without dementia, showing temporal lobe atrophy macroscopically, in addition to pathological hallmarks compatible with ALS with dementia. We also note the possibility that there is a forme fruste of ALS with dementia showing no overt dementia clinically.- - - - - - - - - - ranking = 5keywords = sclerosis (Clic here for more details about this article) |
8/47. A clinical and pathological study of a Japanese case of amyotrophic lateral sclerosis/Parkinsonism-dementia Complex with family history.This report concerns a Japanese family with neuropathological findings consistent with amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) in the Island of guam. The proband was a 68-year-old woman with an 8-year history of parkinsonism which was followed by psychiatric symptoms and neurogenic amyotrophy 5 years after the onset. She had a family history of parkinsonism associated with dementia in all of her three siblings. They grew up in the Hobara village, a focus of amyotrophic lateral sclerosis in the Kii Peninsula of japan in their childhood. Their parents were not consanguineous nor natives of the Kii Peninsula. The brain weight was 1040 g and there were mild frontal lobe atrophy, moderate atrophy of pes hippocampi, decoloration of the substantia nigra and locus coeruleus, and atrophy of the anterior root of the spinal cord. The microscopic examinations revealed degeneration of CA1 portion of the hippocampus to the parahippocampus gyrus, substantia nigra, locus coeruleus and spinal anterior horn with Bunina bodies. The spinal pyramidal tracts also mildly degenerated. neurofibrillary tangles (NFT) were observed in the cerebral cortex, especially in the cortices from hippocampus to lateral occipitotemporal gyri, basal nucleus of Mynert, basal ganglia, thalamus, substantia nigra and widespread regions of the central nervous system through the brainstem to spinal cord including the nucleus of Onufrowitcz. In spite of a small amount of the senile plaques in the cerebral cortex and lewy bodies in the substantia nigra and locus coeruleus, abundant NFT were distributed mainly in the third layer of the cerebral cortex, which is the characteristic feature of ALS/PDC. Thus, this was likely to be an ALS/PDC case outside the guam Island. A tau mutation was not found on dna analysis.- - - - - - - - - - ranking = 2keywords = sclerosis (Clic here for more details about this article) |
9/47. Histopathological observations of presbycusis.temporal bone histopathology of 17 aged patients who had spontaneous and gradually progressive bilateral sensorineural hearing losses associated with aging was studied. Six cases in the present material showed the gradually sloping audiometric curve; nine cases, abrupt high tone hearing loss; and two cases, the flat audiometric curve. The most prominent histopathological change in the inner ear was a decrease in the population of the spiral ganglion cells. However, diffuse senile atrophy was also often seen in the organ of corti and the stria vascularis. A positive correlation between the degree of artheriosclerosis and the degree of sensorineural degeneration in the cochlea was not obtained in the present cases. Also, the correlation was not found to be consistent between the type of the audiometric curve and the localization of lesions in the sensory, the neural or the vascular elements in the cochlea. Our observations show that a certain type of audiometric curve does not necessarily indicate a lesion in a specific cochlear element.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
10/47. Primary lateral sclerosis: a rare upper-motor-predominant form of amyotrophic lateral sclerosis often accompanied by frontotemporal lobar degeneration with ubiquitinated neuronal inclusions? Report of an autopsy case and a review of the literature.We report the autopsy findings of an 82-year-old woman who exhibited slowly progressive upper motor neuron signs (pseudobulbar palsy, muscle weakness and positive Babinski's sign) in the absence of lower motor neuron signs, which were followed by progressive dementia and frontotemporal atrophy, and who died 7 years and 4 months after onset of the disease. In this patient, the upper motor neuron system, including the precentral cortex and descending pyramidal tract, was severely degenerated, but the lower motor neurons and innervated skeletal muscles were well preserved. A few lower motor neurons were found to contain cytoplasmic inclusion bodies characteristic of amyotrophic lateral sclerosis (i.e., Bunina bodies and ubiquitin-positive skeins). However, fragmentation of the golgi apparatus was not evident in the anterior horn cells examined. Therefore, it was considered that the lower motor neurons were also involved, but that the rate of degeneration of these neurons was very slow in the disease process. Marked frontotemporal lobar degeneration characterized by microvacuolation, and ubiquitin-positive neuronal inclusions and dystrophic neurites in cortical layer II were also observed, the precentral cortex being the most severely affected area. Similar ubiquitin-positive structures were also observed in the neostriatum. Finally, a survey of the literature based on this patient's clinical and pathological features led us to conclude that the rare clinical syndrome of primary lateral sclerosis is, in general, a rare upper-motor-predominant form of amyotrophic lateral sclerosis that is often accompanied by frontotemporal lobar degeneration with ubiquitinated neuronal inclusions.- - - - - - - - - - ranking = 11keywords = sclerosis (Clic here for more details about this article) |
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