Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

MRI was performed in 21 patients and single photon emission computed tomography (SPECT) with N-isopropyl-p-123I iodoamphetamine in 16 patients, to visualize upper motor neurone lesions in amyotrophic lateral sclerosis. T2-weighted MRI revealed high signal along the course of the pyramidal tract in the internal capsule and cerebral peduncle in 4 of 21 patients. SPECT images were normal in 4 patients, but uptake was reduced in the cerebral cortex that includes the motor area in 11.
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PMID:MRI and SPECT findings in amyotrophic lateral sclerosis. Demonstration of upper motor neurone involvement by clinical neuroimaging. 140 16

The enzymatic activity of acetylcholinesterase (AchE) in the cerebrospinal fluid (CSF) is considered to be a marker of central cholinergic neuron integrity. Then, we evaluated CSF AchE activity in 90 cases of neurological diseases involving cholinergic system and their related disease, and 28 control cases without central organic lesions or abnormal findings in routine CSF study. AchE activity was evaluated according to Ellman's method using acetylthiocholine iodide as a substrate and tetraisopropyl-pyrophosphoramide, a specific inhibitor of butyrylocholinesterase. CSF AchE of Alzheimer type dementia (AD/SDAT, N = 12: 21.9 +/- 4.7 nmol/ml/min) showed no significant change from those of both control group (22.1 +/- 3.9) and vascular dementia (9: 21.7 +/- 6.7). In extrapyramidal diseases, reduction of the activity was observed in Huntington's chorea (HC, 4: 16.3 +/- 1.4) and progressive supranuclear palsy (PSP, 4: 17.6 +/- 1.7), whereas normal activity was shown in Parkinson's disease (PD, 19: 22.5 +/- 4.6), dentatorubropallidoluysian atrophy (DRPLA, 4: 22.6 +/- 4.2) and striatonigral degeneration (SND, 4: 20.4 +/- 4.3). In olivopontocerebellar atrophy (OPCA, N = 16), we disclosed reduced CSF AchE activity (15.8 +/- 2.4) which had significant correlations with the atrophy of the pontine base (r = 0.6017, p less than 0.02) and cerebellar vermis (r = 0.5450, p less than 0.05) in MRI. AchE activity in cerebellar cortical atrophy (CCA, 5: 20.6 +/- 2.2) remained within the control values. Normal activity was demonstrated in both amyotrophic lateral sclerosis (6: 24.3 +/- 7.3) and spinal muscular atrophy (4: 22.9 +/- 3.9).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[CSF acetylcholinesterase activity in central neurological diseases involving cholinergic systems]. 162 49

The authors report the case of a 32-year-old woman who developed symptoms and signs possible manifestations of a familial motor neuron disease with left sided pyramidal signs and marked wasting and weakness in the ipsilateral upper girdle, progressively extended to the contralateral upper limb. The CT-scan showed only frontal cortical atrophy. MRI disclosed a restricted area of increased signal intensity in the centrum semiovale of the subcortical white matter of the right prefrontal cortex. This young woman did not disclose any risk factors for cerebrovascular diseases nor other disorders of the CNS; therefore the authors are of the opinion that the white matter changes observed on MRI are not occasional findings, but are related to the pathologic process occurring in consequence of her neurodegenerative disorder (possible ALS).
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PMID:MRI findings in a patient with a familial form of motor neuron disease. 186 28

In cases of Parkinson's disease, a high incidence of dementia and simultaneous pathologic changes of Alzheimer's type have been reported. X-ray CT and MRI have such good spatial resolution that they can be expected to be useful for evaluation of brain atrophy. Positron emission tomography (PET) used with 18F-2-deoxy-2-fluoro-D-glucose is considered to reflect regional function. By these techniques, brain atrophy and local cerebral metabolic rate of glucose (LCMR-glc) in patients with Parkinsonism with dementia was studied, and also compared with age-matched normal controls and senile dementia of Alzheimer type. In seven cases of Parkinson's disease with dementia, LCMRs-glc were statistically decreased in all regions in comparison with ten normal controls. LCMRs-glc in six Parkinson's disease without dementia were higher than those of demented Parkinson's disease, but significantly lower than normal controls in all regions except basal ganglia. Some aged normal controls presented cortical atrophy and a significant difference could not be seen in evaluation by MRI among these three groups. There was also no correlation between LCMR-glc and cortical atrophy. There was no significant difference of LCMR-glc between six Guamnian cases of Parkinsonism-Dementia complex (PD complex) without ALS and four cases of PD complex with ALS, and these values were significantly lower than five Guamanian and ten Caucasian normal controls. In PD complex with and without ALS, remarkable cortical atrophy and ventricular dilatation were recorded in comparison with normal controls, and correlation between decrement of LCMR-glc and cortical atrophy was indicated in frontal, parietal and temporal lobe. In Parkinson's disease with dementia and PD complex in Guam, LCMRs-glc in all regions of brain were generally lower than normal controls. These findings were different from Alzheimer's disease in which LCMR-glc have been reported to be low especially in cerebral cortex. On the other hand, cortical atrophy and ventricular dilatation evaluated by MRI and CT was apparent in PD complex, but these changes were not remarkable in Parkinson's disease. Cortical atrophy did not always correlate with the decrease of LCMR-glc and changes of LCMR-glc could reflect clinical signs such as Parkinsonism and dementia. Both PET as a functional imaging method and MRI, CT as an anatomical imaging method are useful to access the study of these diseases.
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PMID:[Comparison study of positron emission tomography, X-ray CT and MRI in parkinsonism with dementia]. 279 56

The aberrant pyramidal tracts in the pontine medial lemnisci were studied, using standard Sudan III stain, in six cases of chronic pyramidal tract degeneration. Three of the six cases had bilateral or unilateral cerebral destructive lesions, one cervical hematomyelia with rare retrograde pyramidal tract degeneration, one classical amyotrophic lateral sclerosis, and one atypical motor neuron disease with striatonigral degeneration. Except for the latter two cases the aberrant pyramidal tract degeneration was confirmed bilaterally or on the side ipsilateral to the pyramidal tract degeneration in the pontine base. This degeneration could also be found, on careful examination, with other stains, i.e., H & E, Luxol fast blue-periodic acid Schiff and modified Bielschowsky. Significant change was not observed in the medullary medial lemniscus in any case. The different results observed in the aberrant pyramidal tract between the destructive and degenerative disorders might be pathogenetically important. Reservation, however, may be required since the number of the cases of degenerative disorders in this study was limited. A possible factor for this difference is the survival length which might have erased degradation products altogether. Another factor is the sensitivity of Sudan III in comparison with the Marchi's method which might demonstrate more subtle evidence of degeneration but with its intrinsic capricious staining characteristics. The physiological role of the aberrant pyramidal tract, which has been neglected in the recent textbooks of neuroanatomy, may become of clinical interest with high-quality MRI in cases such as isolated cranial motor nerve palsy without concomitant paralysis of the extremities.
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PMID:[Aberrant pyramidal tract: a study with Sudan III stain]. 280 32

Sternocleidomastoid muscle EMG (SCM-EMG) and dermatomal somatosensory evoked potential (DSSEP) were evaluated as diagnostic aids in differentiation between amyotrophic lateral sclerosis (ALS) and cervical spondylotic myelopathy (CSM) in this study. 104 inpatients were divided into three groups: those diagnosed clinically as either (A) ALS (36 cases), or (B) CSM (32 cases) and (C) those needed further investigation (36 cases). EMG of limb muscles and sternocleidomastoid of both sides in all, and of tongue muscles in 58/104 of three groups; DSSEP in 56/104 of all three groups; MRI/CTM in 12/36 of group A and all patients of group B and C. SCM-EMG abnormal in 34/36 of group A but normal in all 32 of group B. SCM-EMG also abnormal in 32/36 of group C and 8 of them showed postoperative aggravation. DSSEP normal in 12/12 of group A while abnormal in 19/20 of group B. DSSEP abnormal only in 6/24 of group C. MRI/CTM showed spinal compression in all cases presenting abnormal DSSEP and close correspondence between upper margins of pathological involvement as revealed using these two methods. Both SCM-EMG and DSSEP have proved to be valuable differential aids between ALS and CSM.
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PMID:The electrophysiological study of differential diagnosis between amyotrophic lateral sclerosis and cervical spondylotic myelopathy. 755 28

Abnormal high signal in the corticospinal tracts on MRI has been described in amyotrophic lateral sclerosis. We report a case with further high signal in fibres of the corpus callosum on proton density and T2-weighted spin-echo images, closely matching findings of earlier pathological reports.
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PMID:Involvement of corpus callosum in amyotrophic lateral sclerosis shown by MRI. 766 61

We investigated the mechanisms of muscle fatigue in ALS. In the muscles of ALS patients and healthy control subjects, we examined (1) fatigue using measurements of muscle force, (2) energy metabolism using phosphorus-31 magnetic resonance spectroscopy, and (3) activation using neurophysiologic measures and MRI. During 25 minutes of intermittent isometric exercise of the tibialis anterior muscle, both maximum voluntary and tetanic force declined more in patients than in controls, indicating greater fatigability in ALS. There was a similar decline of voluntary and tetanic force, suggesting that much of the fatigue was not central. Evoked compound muscle action potential amplitudes were preserved during exercise in both groups, indicating no failure of neuromuscular transmission; this result suggests that the source of fatigue was not at the neuromuscular junction or within the muscle membrane. In spite of greater fatigability, changes during exercise in energy metabolites and proton signal intensity tended to be less in ALS patients compared with controls, suggesting impaired muscular activation. We conclude that the greater muscle fatigue in ALS patients results from activation impairment, due in part to alterations distal to the muscle membrane.
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PMID:Physiology of fatigue in amyotrophic lateral sclerosis. 772 63

Magnetic resonance imaging by gradient echo method demonstrated lesions of the lateral corticospinal tract at cervical cord levels in three ALS patients. Patient 1 was a 43-year-old woman with common form of ALS. She developed right-side predominant pyramidal signs, and right-side predominant prolongation of central motor conduction time. MRI showed hypersignal intensity areas in the dorsal region of the lateral column at the 4th and 5th cervical segments with right-side predominancy. Patient 2 was a 65-year-old man with pseudopolyneurtic form of ALS, who showed lower motor neuron signs without a pyramidal sign. MRI of the 3rd and 4th cervical cord segments demonstrated bilateral hypersignal intensity areas in the dorsal part of the lateral column. Patient 3 was a 62-year-old man with common form of ALS, who showed marked bilateral pyramidal signs with Babinski's sign. MRI of the 5th cervical spinal cord segment demonstrated bilateral hypersignal intensity areas in the dorsolateral column. Thus obtained MR images of the spinal cord corresponded well to the postmortem-confirmed degeneration of the spinal corticospinal tract. MRI of the spinal cord performed by gradient echo method would provide additional information on the upper motor neuron involvement in ALS.
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PMID:[The corticospinal tract lesion of amyotrophic lateral sclerosis--magnetic resonance imaging of the spinal cord]. 782 Sep 58

To evaluate dysarthria in patients with ALS, we used MRI (gradient rephasing echo method) and compared it with the computed acoustic analysis. Five ALS male patients of progressive bulbar palsy type and five normal male were asked to phonate the five Japanese vowels, /a/./i/./u/./e/./o/. MRI of the sagittal tongue and vocal tract was obtained by the gradient rephasing echo method (0.2 Tesla, TR:30 ms, TE:10 ms, FA 25 degrees C, Hitachi). We could clearly visualize the change of tongue shape and the narrow site of the vocal tract for each vowel phonation. In normal subjects, the tongue shape and the narrow site of the vocal tract were distinguishable between each vowel, but unclear in ALS. Acoustic analysis showed that the first formant frequency of /i/./u/ in ALS was higher than normal and the second formant frequency of /i/./e/ in ALS was significantly lower than normal. The discrepancy from the normal first, second and third formant frequency for each vowel of ALS was most seen in /i/./e/. It was speculated that /i/ and /e/ were the most disturbed vowels in ALS. The first and second formant frequency of vowel depends on the tongue shape and the width of the oral cavity. Therefore the results of the acoustic analysis in ALS indicated poor movement of tongue in /i/./u/./e/ and were compatible with the findings of the sagittal tongue MRI. The sagittal view of the tongue in the gradient rephasing echo MRI and the acoustic analysis are useful in evaluation dysarthria in ALS.
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PMID:[Analysis of dysarthria in amyotrophic lateral sclerosis--MRI of the tongue and formant analysis of vowels]. 820 Jan 37


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