Amyotrophic lateral sclerosis with dementia

Four patients with amyotrophic lateral sclerosis (ALS) and dementia are reported. Mental symptoms antedated motor signs and were investigated with a neuropsychology battery, which brought out different patterns of cognitive impairment. A case presented with frontal dementia, another with a predominant aphasic, apraxic, amnesic syndrome, while the remainders showed cognitive decline in association with blunt affect. Motor signs were characterized by a precocious involvement of the upper motor system.     

Amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is a progressive, degenerative disease of the motor system characterized by signs and symptoms of upper and lower motor dysfunction. This results in the presence of focal amyotrophies and pareses affecting voluntary muscles. Patients die after a few years, in most cases by respiratory failure. ALS is the most frequent motor neuron disease; however, its etiology and pathogenesis are only known in fragments. Its genetic basis is only partially understood and major gaps remain in the understanding of its pathogenesis with the basic principle of selective vulnerability and potentially resulting therapeutic consequences.     

Amyotrophic lateral sclerosis with antecedent poliomyelitis

Histopathological and virological studies were performed on autopsy tissue from a 47-year-old man who had a history of acute poliomyelitis at age 15 years and died after a three-year course of amyotrophic lateral sclerosis (ALS). The poliovirus serologic tests suggested prior infection with poliovirus type 3 but no ongoing poliovirus infection. The CNS showed typical features of ALS with no inclusion bodies or inflammatory cells. Attempts to isolate poliovirus in the CNS were unsuccessful and results of immunofluorescence studies for poliovirus antigen were negative. Molecular hybridization experiments using a DNA copy of the complete poliovirus genome failed to demonstrate poliovirus-related RNA or DNA sequences in the CNS. These studies, using sensitive techniques, indicate that there was no evidence of the continuing presence of poliovirus in this patient with ALS and antecedent poliomyelitis.     

Amyotrophic lateral sclerosis]

The literature on amyotrophic lateral sclerosis from the years 1995/96 is reviewed including the topics of the World Congress of Neurology in Buenos Aires in September 1997. The problems of a) changed criteria of diagnosis and course of SLA after the time of the first definitions by classical neurological authors, b) the concepts concerning the aetiology and mechanisms of the disease, c) the present studies on drugs and their tentative clinical applications are discussed.     

Amyotrophic lateral sclerosis associated with pregnancy.

Amyotrophic lateral sclerosis (ALS) is the most common, progressive motor neurone disease but is rare in the obstetric population. Only 4 cases have been described in the English literature since 1975. We describe a 29 year old woman who presented with ataxia, lower limb weakness and dysarthria 4 weeks after the birth of her first child. The symptoms had onset during the pregnancy but had not been considered remarkable. There were clinical features of upper and lower motor neurone involvement without any sensory loss. MRI of brain and spine was normal. CSF analysis was negative. EMG studies confirmed the presence of widespread anterior horn cell dysfunction compatible with ALS. The patient was commenced on Riluzole and has progressed clinically, at 12 months post diagnosis.     

Amyotrophic lateral sclerosis severity scale

The amyotrophic lateral sclerosis (ALS) severity scale has been developed to provide an ordinal staging system and a means of rapid functional assessment for patients with ALS. The scale allows an examiner to evaluate the symptoms of ALS numerically in four categories that describe speech, swallowing, lower extremity, and upper extremity abilities. These scores, combined with a vital capacity measured on a hand-held respirometer, provide a rapid, accurate assessment of a patient's disease status and can be used for treatment planning. The ALS severity scale has been shown to have an average estimated reliability coefficient of 0.95 between examiners. Speech ratings were correlated greater than 0.80 for objective speech measures. Rates of progression of the total score in a small group of patients ranged from -3.4 to -24.0 points/year with a mean of -11.3 points/year.     

Amyotrophic lateral sclerosis and prolactinoma

A 59-year-old male with amyotrophic lateral sclerosis is presented. Investigations revealed the co-existence of a pituitary adenoma of the prolactinoma type. This combination has not been reported before. The possible relation between endocrinological disturbances and this neurological disease is discussed.     

Amyotrophic lateral sclerosis and neuroregeneration

Totally three articles focusing on "defective deep somatosensory pathway in patients with amyotrophic lateral sclerosis,screening proteins that interact with mutant superoxide dismutase 1 from familial amyotrophic lateral sclerosis,and the mechanism by which     

Amyotrophic lateral sclerosis and glutamate

Amyotrophic lateral sclerosis is a progressive fatal disorder devastating the spinal cord and brain in humans. Excitotoxicity has been suggested to be involved in the pathogenesis of amyotrophic lateral sclerosis. This hypothesis has driven a wealth of basic research and stimulated development of neuroprotective therapies for chronic neurodegenerative disorders. As a result of these efforts, riluzole, an antiglutamatergic drug, has been established in the therapy of amyotrophic lateral sclerosis. A transgenic mouse showing features of amyotrophic lateral sclerosis has been subsequently engineered enabling studies of the disease in vivo. However, despite considerable progress, the etiology of amyotrophic lateral sclerosis remains obscure and the disturbances in excitatory neurotransmission should by no means be regarded as exclusive to the pathogenesis of the disease.