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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 76-year-old right-handed woman complained of speech disturbance and difficulity of singing was admitted to our hospital. Examination showed motor aphasia and mild cognitive impairment. After she was discharged, dementia and weakness of the extremities had rapidly progressed. She was readmitted eight month after the first visit, when she was almost abulic, her skeletal and bulbar muscles were remarkably atrophic, and hyperreflexia of the extremities were seen. Electromyographcal study showed neurogenic pattern. These findings suggest amyotrophic lateral sclerosis (ALS) with dementia. Pathological findings were atrophy at the anterior horn of the spinal cord. The brain was diffusely atrophic. The extent of degenerative change was not lateralized. This case is a discriminative type of ALS with dementia, that its first symptom is motor aphasia.
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PMID:[A case of amyotrophic lateral sclerosis presenting as motor aphasia in its early stage]. 1698 78

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease classically defined by the impairment of the voluntary motor system and ubiquitin-positive intraneuronal aggregates in anterior horn cells. Frontotemporal dementia (FTD) is a common form of neurodegenerative dementia and presents with personality change associated in a significant subgroup of patients with cortical ubiquitin-only neuropathology (FTD-U). Careful study of ALS as well as FTD patient cohorts suggests clinical as well as pathological overlap of ALS with FTD. The idea that this reflects a shared pathogenesis has received strong support from the identification of new genetic loci on chromosome 9p and of mutations in specific genes (CHMP2B and DCN1) in families with co-segregation of ALS and FTD. The identification of two further genetic causes of FTD-U with (rare) ALS (PGRN) or without ALS (VCP) also provides a starting point for exploring the pathways associated with ubiquitin-mediated protein mishandling in FTD-U and ALS. Pure ALS, through ALS with cognitive impairment and ALS-FTD to pure FTD-U, may represent a continuous spectrum of ubiquitin-associated neurodegenerative disease.
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PMID:Recent advances in the genetics of amyotrophic lateral sclerosis and frontotemporal dementia: common pathways in neurodegenerative disease. 1698 82

Cognitive impairment in nondemented ALS patients has been demonstrated, although its incidence remains to be determined. FTD is the most frequently form of dementia in ALS. The clinical profile of patients with dementia or mild cognitive deficit evokes neuropsychological deficits and behavioural changes resulting from executive dysfunction. The psychometric evaluation, centred on executive disturbances, goes with behavioural scales in order to accurately appreciate the repercussion of cognitive and behavioural changes on daily life.
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PMID:[Amyotrophic lateral sclerosis: cognitive and behavioral evaluation]. 1712 3

Neuropsychological assessment in the ALS population is relatively recent probably because this disease was traditionally considered to spare cognitive abilities. However, evidence is emerging from neuropsychological testing that some patients with classical ALS, who are thought not to be demented, develop cognitive deficits and particularly frontal executive disturbances. Neuroimaging gives support to frontal lobe involvement in ALS. Nevertheless, cognitive impairment does not concern all ALS patients and appears to be mild when it is described compared with rather severe frontotemporal dementia observed in association with ALS. Most of the references in this work question the cognitive perspective in the ALS population but few of them can provide a real useful tool for cognitive investigation in this disease.
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PMID:[Neuropsychological assessment in amyotrophic lateral sclerosis]. 1712 4

Psychostimulants have been used to treat many symptoms associated with advanced cancer. The primary role of psychostimulants in such cases is the treatment of symptoms such as cancer-related fatigue, opioid-induced sedation, depression, and cognitive dysfunction associated with malignancies. These uses for psychostimulants came after approval for treatment of disorders such as attention deficit disorder. Modafinil, a new psychostimulant, is following a similar path after its approval for use in attention deficit disorder in 1998. Modafinil has been used to treat fatigue associated with neurodegenerative disorders such as multiple sclerosis and amyotrophic lateral sclerosis. It is now being increasingly used for cancer-related symptoms targeted by psychostimulants. Preliminary evidence from literature review suggests that modafinil is efficacious in improving opioid-induced sedation, cancer-related fatigue, and depression. There is no evidence to support its use in the treatment of cognitive dysfunction related to cancer or to support its having analgesic properties. Well-designed, randomized, controlled clinical trials are still needed to further elucidate the precise role of this drug in the care of patients with cancer. Specifically, large placebo-controlled trials with modafinil must be conducted in patients with cancer, with specific attention paid to pain control, depression, cognitive function, and adverse effects.
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PMID:Modafinil: is it ready for prime time? 1731 46

Cognitive impairment in patients with amyotrophic lateral sclerosis (ALS) is well documented, but behavioral abnormalities are not well defined. The Frontal Systems Behavior Scale (FrSBe) was used to assess changes in apathy, disinhibition, and executive dysfunction in 45 patients with ALS. Results suggest a high incidence of behavioral change, most notably in apathy associated with the onset of the disease and independent of mood. Apathy and verbal fluency were correlated, lending further support to the hypothesis that an underlying continuum exists between ALS and frontotemporal dementia (FTD), which is characterized predominantly by behavioral disturbance. The FrsBe is useful for detecting behavioral change and abnormalities in patients with ALS.
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PMID:Detecting neurobehavioral changes in amyotrophic lateral sclerosis. 1736 37

Amyotrophic lateral sclerosis (ALS) is a multifactorial disease, with many genetic and environmental factors contributing to its outcome. The population of Israel is comprised of immigrants from all over the world as well as by Arabs. People with different ethnic backgrounds who live in the same environment provide a unique opportunity to analyze genetic and environmental influences on ALS. We performed a retrospective analysis of 374 sporadic ALS patients whose origin was European in 211, North African in 53, Oriental in 43, Balkan in 19, Arab in 9, and Yemenite in 7, comparing their age at disease onset, gender, disease form at onset, survival, smoking habits, cognitive dysfunction and apolipoprotein E genotype. Patients of North African origin were significantly younger and had a shorter duration of disease relative to their age compared to other ethnic groups, adjusted for age. The difference between the patient groups might be related to a genetic burden in North African patients and warrants further investigation.
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PMID:Earlier onset and shorter survival of amyotrophic lateral sclerosis in Jewish patients of North African origin. A clue to modifying genetic factors? 1740 94

Amyotrophic lateral sclerosis (ALS) may be accompanied by cognitive impairment; when present, it is mainly in the form of frontotemporal impairment. We report on two cases with clinically defined ALS that subsequently developed dementia. Neuropathological examination showed not only the typical neuropathological hallmarks characteristic of ALS but, surprisingly, also showed neurofibrillary tangles and neuritic plaques in sufficient numbers to fulfill the diagnostic criteria of definite Alzheimer's disease.
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PMID:Amyotrophic lateral sclerosis and Alzheimer's disease--clinical and neuropathological considerations in two cases. 1759 41

Amyotrophic lateral sclerosis (ALS) is classically described as a pure motor disease; however, there is growing evidence of a range of cognitive impairment. Cognitive abnormalities include deficiencies in frontal executive skills, varying from mild deficits to meeting criteria for diagnosis of frontotemporal dementia (FTD). Cognitive impairment occurs in sporadic and familial forms of ALS. Patients may present with cognitive deficits before, after, or at the onset of motor neuron disease. Structural and functional imaging studies have shown extramotor cortical degeneration corresponding to levels of frontal executive impairment on neuropsychologic testing. In addition, ALS and a subset of FTD patients display common pathological findings on immunohistochemistry staining. It is believed that these disorders represent a continuum between motor and nonmotor cortical degeneration. The purpose of this article is to review the literature on cognitive deficits in ALS. Identifying changes in cognition is critical for physicians and caregivers of ALS patients, as cognitive decline may interfere with patient compliance. Diagnosis and treatment of cognitive symptoms in ALS patients may improve quality of life.
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PMID:Cognition and amyotrophic lateral sclerosis (ALS). 1771 61

The clinical disorders associated with frontotemporal lobar degeneration (FTLD) are increasingly recognized as an important cause of early-onset dementia. Patients usually present with progressive changes in personality, behavior, or language, progressing to general cognitive impairment and ultimately death. In the past decade, improved clinical and histopathologic characterization uncovered extensive heterogeneity, and multiple clinical and pathologic FTLD subtypes were defined. Simultaneously, the discovery of four causal FTLD genes emphasized the genetic complexity associated with FTLD. More recently, the field of FTLD has gained increased attention as a result of two major findings. First, mutations in the progranulin gene (PGRN) were recognized as a major cause of FTLD with ubiquitin-positive and tau-negative inclusions (FTLD-U), and subsequently the TAR DNA-binding protein-43 (TDP-43) was identified as a key protein within the ubiquitinated inclusions in FTLD-U and amyotrophic lateral sclerosis (ALS). In this report, we outline the progress made in the study of the genetic etiologies and neuropathologic substrates in FTLD.
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PMID:The genetics of frontotemporal lobar degeneration. 1776 35


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