1/84. foramen magnum meningioma presenting as amyotrophic lateral sclerosis.amyotrophic lateral sclerosis (ALS) is a progressive, degenerative disease of the nervous system. It affects adults in all age groups and leads to death few years after the diagnosis. The etiology is unknown, and there no convincing medical or surgical therapy Some diseases can mimic ALS. If diagnosed early these may be treatable, but if misdiagnosed as ALS and left untreated they may be fatal. We present a case of foramen magnum meningioma presenting clinically as ALS. After surgery, the symptoms disappeared and the neurological deficits improved.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/84. amyotrophic lateral sclerosis with dementia. Case report.A patient is described in whom a profound and rapidly progressive dementia occurred in association with clinical features of amyotrophic lateral sclerosis. A magnetic resonance imaging showed signs of frontal and especially left temporal atrophy. The pattern of dementia indicated impaired frontotemporal lobe functions, evidenced by reduced tracer uptake in the frontotemporal lobes on brain single photon emission computed tomography. Neuropathological examination in this patient revealed mild frontotemporal atrophy with spongiform changes and neuronal loss affecting mainly layers II and III of the frontotemporal cortices. There was atrophy of the hypoglossal nuclei. The spinal cord changes were consistent with motor neuron disease. The patient showed an irreversible and progressive course. A review of the relevant literature was made.- - - - - - - - - - ranking = 2.1374667044419keywords = brain (Clic here for more details about this article) |
3/84. reflex sympathetic dystrophy associated with amyotrophic lateral sclerosis.reflex sympathetic dystrophy (RSD) is a syndrome characterised by severe distal pain and vasomotor changes. It is believed to be caused by sympathetic nervous system overactivity. Trauma is the most frequent precipitant event. An association with amyotrophic lateral sclerosis (ALS) has been reported only once. We report three patients with ALS in whom the occurrence of RSD, in one of them at a very early clinical stage, seemed to have precipitated a more rapid clinical evolution. New sprouting re-innervating fibres have abnormal ion channels which might increase the risk of RSD. On the other hand, motor changes have been described in RSD, as well as motor strength improvement after RSD treatment. The complex relation of ALS with RSD is discussed. In all ALS patients pain followed by further loss of function should prompt a search for RSD.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
4/84. 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 = 2.631535104031keywords = central nervous system, nervous system (Clic here for more details about this article) |
5/84. motor neuron disease with predominantly upper extremity involvement: a clinicopathological study.We report two autopsy cases of motor neuron disease (MND) patients with an unusual type of muscular atrophy predominantly affecting the shoulder girdle and the upper extremities with proximal dominance. Both patients are considered to be clinically categorized into the El Escorial suspected form of amyotrophic lateral sclerosis (ALS). At autopsy, they showed marked loss of spinal anterior horn cells accompanied by astrogliosis positively immunostained with anti-glial fibrillary acidic protein antibody at the cervical level. At the lumbosacral level, anterior horn neurons were relatively well preserved and Bunina bodies, ubiquitin-positive skein-like inclusions and Lewy body-like inclusions were observed in the remaining neurons. In one patient, brain stem motor neurons (nerves V, VII, XII) and motor cortex, including Betz cells, were also affected and the corticospinal tracts were degenerated at the level of the thoracic and lumbar spinal cord. Pathological findings of this patient are consistent with those of ALS. In the other patient, the motor cortex, brain stem motor nuclei and the corticospinal tracts were well preserved, which is pathologically compatible with progressive spinal muscular atrophy. These patients with such a peculiar pattern of progressive muscular atrophy should be placed in a subgroup of ALS.- - - - - - - - - - ranking = 4.2749334088838keywords = brain (Clic here for more details about this article) |
6/84. amyotrophic lateral sclerosis with a 'pseudo-infarction' pattern on the electrocardiograph. A case report.amyotrophic lateral sclerosis (ALS) is a motor neuron disease involving both the upper and lower motor neurons in the brain stem and spinal cord. Although it is well known that various central nervous system disorders can produce a 'pseudo-infarction' pattern on the electrocardiogram, there have been no reports of this particular pattern in ALS patients. This report concerns an ALS patient who presented with an ECG pattern of S-T elevation followed by biphasic T and inverted T without any detectable myocardial abnormality. Data from the present case suggest that this pattern may be an inherent characteristic of ALS.- - - - - - - - - - ranking = 4.7690018084729keywords = brain, central nervous system, nervous system (Clic here for more details about this article) |
7/84. Basophilic cytoplasmic inclusions in a case of sporadic juvenile amyotrophic lateral sclerosis.A 24-year-old woman presented with progressive muscle atrophy and weakness of the right upper extremity. Subsequently her weakness rapidly extended to the left upper extremity, neck and lower extremities. Neurological examination disclosed involvement of the lower motor neuron system. She died 7 months after the onset. There was neuronal loss and reactive gliosis in the anterior horns of the spinal cord and much less frequently in the motor cortex. Basophilic cytoplasmic inclusions were observed in the thalamus and brain stem as well as the upper and lower motor neurons. Ultrastructurally, the inclusions lacked a limiting membrane and consisted of a meshwork of filamentous structures associated with granules. The inclusions failed to react with antibodies against phosphorylated neurofilament or cystatin c. Most of the inclusions show no reaction with anti-ubiquitin antibody, however, a few inclusions show granular reaction product deposits with this antibody. The inclusions were not immunostained with antibodies against TGN46 and MG-160, markers of the trans-golgi network and the medial cisternae of the golgi apparatus, respectively, suggesting that they were not derived from the golgi apparatus which was fragmented.- - - - - - - - - - ranking = 2.1374667044419keywords = brain (Clic here for more details about this article) |
8/84. ALS-Plus syndrome. A clinical and neuropathological case study.ALS-Plus syndrome occurs rarely and usually presents typical ALS phenotype associated with dementia, Parkinsonism or both. We reported a case of sporadic, definite ALS with pseudobulbar palsy, emotional lability and selective cognitive deficit in the presence of frontal lobe dementia. neuropsychological tests predominantly demonstrated perserveration and dynamic apraxia, CT and MRI scans showed widened subarachnoideal spaces in the frontal and temporal regions. The neuropathological findings confirmed ALS processes i.e. atrophy of motor nuclei in brainstem and anterior horns of cervical spinal cord and showed mild atrophy and status spongiosus in the frontal lobes. These findings suggest the co-occurrence of sporadic ALS and frontal lobe dementia: ALS-Plus syndrome.- - - - - - - - - - ranking = 2.1374667044419keywords = brain (Clic here for more details about this article) |
9/84. amyotrophic lateral sclerosis in a battery-factory worker exposed to cadmium.A 44-year-old patient died from amyotrophic lateral sclerosis (ALS) after nine years of heavy exposure to cadmium (Cd) in a nickel cadmium (Ni-Cd) battery factory. Two years after starting work he and co-workers had experienced pruritus, loss of smell, nasal congestion, nosebleeds, cough, shortness of breath, severe headaches, bone pain, and proteinuria. Upper back pain and muscle weakness progressed to flaccid paralysis. EMG findings were consistent with motor neuron disease. Cd impairs the blood-brain barrier, reduces levels of brain copper-zinc (Cu-Zn) superoxide dismutase (SOD), and enhances excitoxicity of glutamate via up-regulation of glutamate dehydrogenase and down-regulation of glutamate uptake in glial cells. High levels of methallothionein, a sign of exposure to heavy metals, have been found in brain tissue of deceased ALS patients. The effects of Cd on enzyme systems that mediate neurotoxicity and motor neuron disease suggest a cause effect relationship between Cd and ALS in this worker.- - - - - - - - - - ranking = 6.4124001133256keywords = brain (Clic here for more details about this article) |
10/84. Sporadic amyotrophic lateral sclerosis showing abnormal somatosensory evoked potentials: a report of three cases.OBJECTIVE: Although dysfunction of the sensory systems in sporadic amyotrophic lateral sclerosis (ALS) has been reported, the clinical characteristics of such cases still remain unknown. We therefore performed a clinico-electrophysiological analysis of sporadic ALS patients. MATERIAL AND methods: Twelve ALS patients (aged 36-66 years), who had their somatosensory evoked potentials (SEPs) evaluated, were reviewed and their clinical characteristics were delineated. In addition, needle EMG, sensory nerve conduction velocities, motor evoked potentials (MEPs) and cervical MRI or plain X-ray of the neck were also recorded. RESULTS: Three cases were segregated from the other 9 patients because of predominant upper motor neuron signs with pseudobulbar palsy and abnormal posterior tibial nerve and/or median nerve SEPs. The MEPs were also abnormal in these 3 patients and the brainstem auditory evoked potentials were abnormal in one patient. EMG revealed less involvement in the lower motor neurons. CONCLUSION: Sporadic ALS with a predominant upper motor neuron sign and also demonstrating pseudobulbar palsy with abnormal SEPs, may therefore form a clinical subtype of ALS.- - - - - - - - - - ranking = 2.1374667044419keywords = brain (Clic here for more details about this article) |
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