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)

In this paper, I described clinical and basic problems on neurology of the aged patients. These studies have been done in various institutions with many co-workers. 1) A PET study revealed some age differences on CBF, CMRO2, or CMRgl. But these results are not so rigid in which much of individual variations should be considered in interpretation. Calendar age is not always compatible to biological age. 2) Saccular aneurysms in the brain artery were found in 7.3% of 1200 routine autopsy series of the aged subjects. Aneurysms with external diameter exceeding 6 mm had been fatally ruptured in 14 (78%) of 18 subjects. 3) Variations of the pyramidal crossing are found responsible for bizarre clinical manifestations. Non-crossing component was more prominent in the right pyramidal tract; consequently, right pyramidal tracts including ventral and lateral one seemed to have more extensive representation in the spinal cord level. 4) I123-IMP SPECT study showed a reduced uptake in the area 4 or area 4-6 of the ALS patients. 5) I introduced a new simplified Wartenberg's maneuver, which is useful for detection of subtle pyramidal dysfunctions. 6) Cases with central pontine myelinolysis and those of paraneoplastic syndrome were presented with an emphasis on their patho-chemical mechanisms. 7) Lewis-Sumner syndrome showing multifocal persistent conduction block is not rare in the aged, in which we have already had some useful therapeutic methods. 8) Dementia complicated with neurodegenerative disease was discussed on its clinical and chemical features of mental disturbances. In ALS-dementia, CSF-homovanilic acid reduced significantly than in the control and L-dopa was effective in some patients. 9) Vascular and Alzheimer-type dementias were presented and discussed on their pathogenetic mechanism according to our recent studies with review of literature.
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PMID:[Neurological diseases in the aged]. 209 73

We examined the 123I-iodoamphetamine SPECT for 3 patients with ALS, who were clinically diagnosed. Patient 1 was a 31-years-old man, who had bilateral muscle weakness of his upper extremities, and spasticity in lower extremities. Patient 2 was a 51-years-old woman, who had marked weakness of her upper extremities and bulbar sign. Patient 3 was a 68-years-old man, who had severe degree of marked weakness of his upper extremities and mild bulbar signs. Cerebral cognitive function were all normal in three patients. Computed tomographic and magnetic resonance imagings showed moderate degree of cortical atrophy in patient 1, but no abnormalities in patients 2 and 3. In 123I-IMP SPECT, however, hypoperfusion were recognized on the bilateral fronto-parietal border zone areas in these three patients with ALS. It was suggested that patients with ALS showed varying degrees of impaired perfusion in the fronto-parietal border zone areas in spite of normal cognitive functions.
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PMID:[123I-iodoamphetamine single photon emission computed tomography in three patients with amyotrophic lateral sclerosis]. 223 55

N-Isopropyl-p-123I-amphetamine (123I-IMP) single photon emission computer tomography studies were performed on 17 patients suffering from amyotrophic lateral sclerosis (ALS). All patients displayed varying degrees of impaired fixation of 123I-IMP in the whole brain. These findings were not related to the clinical variants of ALS and the age of the patients. A correlation between the severity of the disease and the degree of impaired fixation could be observed. In addition, patients suffering from ALS for longer periods showed more pronounced impairment of 123I-IMP fixation.
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PMID:N-isopropyl-p-123I-amphetamine single photon emission computer tomography in motor neuron disease. 279 43

A 57 year-old woman with amyotrophic lateral sclerosis (ALS) and an apraxia of eyelid closure was reported. Her first symptom was muscle weakness in the right arm. Since neurological examination showed mainly upper motor neuron sign with neurogenic pattern in the needle electromyograms of the tongue and limb muscles, she was diagnosed as ALS. Two years and 6 months later from onset, she showed an inability to close her eyelids voluntarily or on command with normal reflex closure. MRI of the brain showed atrophy in the frontal lobe and anterior of the temporal lobe, where 123 I-IMP SPECT revealed hypoperfusion. Considering previous reports, there might be an ALS subset who showed atrophy of frontal lobe together with apraxia of eyelid closure.
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PMID:[An apraxia of eyelid closure in association with frontal lobe atrophy in a patient with amyotrophic lateral sclerosis]. 1758 5

OBJECTIVES - We investigated the regional cerebral blood flow in amyotrophic lateral sclerosis with dementia (ALS-D) patients, using single photon emission computed tomography (SPECT). MATERIALS AND METHODS - The (123)I-IMP SPECT data for 5 ALS-D and 16 ALS patients were analyzed using three-dimensional stereotactic surface projection (3D-SSP). RESULTS - 3D-SSP demonstrated marked prefrontal hypoperfusion in all the five ALS-D cases and significant bilateral prefrontal hypoperfusion in group comparisons. CONCLUSIONS - This study revealed prefrontal hypoperfusion in ALS-D cases to be an obvious abnormality with scientific objectivity.
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PMID:Constant blood flow reduction in premotor frontal lobe regions in ALS with dementia - a SPECT study with 3D-SSP. 1792 28

To investigate the occurrence of a writing defect, omission of kana letters (OKL), in intellectually normal Japanese patients with amyotrophic lateral sclerosis (ALS), and define the neuroimaging profile of OKL. Sixteen Japanese adults (10 men and 6 women), similar in age (mean 62.9 +/- 9.9 years) and level of education (mean 12.6 +/- 2.13 years), with early-stage, classical ALS (mean duration 15.9 +/- 5.45 months) were investigated, including tests of motor function and ALS progression; intellectual function including writing ability; and neuroimaging, with follow-up of 1 year. Main outcome measures were as follows: Raven's Colored Progressive Matrices (RCPM: intellect and psychomotor speed); one-minute verbal fluency measurement; paired associate word-learning test (PAWLT); Western Aphasia Battery (WAB); moraic segmentation test; magnetic resonance imaging (MRI); and (123)I-isopropyl amphetamine ((123)I-IMP) or (99m)Tc-ethylcysteinate dimmer (ECD) single photon emission tomography (SPECT). Three patients (18.8%) showed OKL (WAB), with disturbance in moraic segmentation. One patient showed decreased blood flow to the bilateral frontal lobes by (123)I-IMP-SPECT. Patients with OKL did not differ significantly from those without in the RCPM (intellect), RCPM (time), verbal fluency, or PAWLT (p = 0.10, 0.84, 0.63, 0.55). Although motor dysfunction and weakness progressed during follow-up, none developed symptoms of dementia. The OKL may develop as a relatively early cognitive symptom in intellectually normal Japanese patients with classical ALS. The neuroimaging profile of OKL remains uncertain.
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PMID:Agraphia in intellectually normal Japanese patients with ALS: omission of kana letters. 1937 59