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)

Pick syndrome, non-Alzheimer type dementia with primary degeneration in the anterior portion of the brain, has been shown to include several disease-entities besides classic Pick's disease. Fronto-temporal dementia (FTD) proposed by Lund & Manchester group is a concept that includes Pick's disease, and its "clinical diagnostic features" are useful because they distinguish dementia with frontal and anterior temporal involvement from Alzheimer type dementia. FTD is classified into three types, the frontal lobe degeneration (FLD) type, Pick type and motor neuron disease (MND) type, and neuropathological diagnostic features of each type are presented. In Japan, however, cases neuropathologically corresponding to the FLD type, which has mild frontal dominant degeneration with heavy heredity, have not been previously reported. Classic Japanese Pick's disease is consistent with Pick type, however, cases of Pick's disease with and without Pick body exist in nearly the same number in Japan. The latter may include heterogeneous types, which should be elucidated. MND type corresponds to Japanese cases, called Yuasa-Mitsuyama disease or dementia with motor neuron disease, and could be actually diagnosed because such patients do not exhibit severe personality change and accompany symptoms of amyotrophic lateral sclerosis or spinal progressive muscular atrophy in their clinical course. In addition, Pick syndrome includes progressive subcortical gliosis, corticobasal degeneration, basophilic inclusion body disease and fronto-temporal dementia with parkinsonism linked chromosome 17. Progressive aphasia and semantic dementia are neuropsychologically important concepts, however, they are symptom-complex and are dominated only by where the degeneration begins and how it progresses. They should not be confused with pathologically confirmed disease-entities.
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PMID:[Where fronto-temporal dementia should be placed in the history of Pick's disease and related disorders]. 1097 14

Frontotemporal dementia (clinical Pick's disease) is a relatively common, but underdiagnosed degenerative disease in the presenium. Estimated prevalence ranges from 6-12% of dementias. The behavioural, aphasic and extrapyramidal presentations are labeled FTD-behavioural variant, Primary Progressive Aphasia (PPA) and Corticobasal Degeneration/Progressive Supranuclear Palsy (CBD/PSP). The diagnostic features and course of each are described and their overlap in the evolution of the illness is emphasized. The neuropathology ranges from the most common tau negative ubiquitin positive amyotrophic lateral sclerosis (ALS) type inclusions to the tau positive classical Pick bodies and more or less distinct changes of PSP and CBD. The genetics of the relatively frequent tau mutations and the yet unsolved problem of tau negative families are discussed. The tau negative cases tend to be associated with the behavioural presentation and semantic dementia and the tau positive ones with PPA and the CBD/PSP syndrome. However the overlap is too great to split the disease. A glossary to navigate the proliferating terminology is included.
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PMID:Progress in clinical neurosciences: Frontotemporal dementia-pick's disease. 1673 22

Neurologic disorders can produce "secondary" mania, and clinicians must distinguish secondary mania from bipolar disorders (BD). Patients with new and late onset mania require an evaluation that includes a thorough history, a neurologic examination, neuroimaging, and other selected tests. Neurologic causes of mania include strokes in the right basotemporal or inferofrontal region, strokes or tumors in the perihypothalamic region, Huntington's disease and other movement disorders, multiple sclerosis and other white matter diseases, head trauma, infections such as neurosyphilis and Creutzfeldt-Jakob disease, and frontotemporal lobar degeneration. The term Frontotemporal Lobar Degeneration (FTLD) is suggested for neurodegenerative diseases characterized by focal degeneration such as Primer Progressive Aphasia (PPA), Frontal Lobe Dementia, PPA- Amyotrophic Lateral Sclerosis (ALS), and Corticobasal Degeneration. In this article, we report a frontotemporal dementia (FTD) case that referred with manic symptoms. The female patient was 46 years old, married, graduated from primary school, and had been admitted with complaints of hyperactivity, excessive talking, and decreased sleep for one week. She presented first with complaints that began three years ago that included the inability to remember names, recognize faces, use household appliances, and follow rules. She had also been repeating the same words and behaviors. Prosopagnosia, aphasia, and a positive family history of ALS were discussed with related index in our case.
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PMID:[Aphasia, prosopagnosia and mania: a case diagnosed with right temporal variant semantic dementia]. 2344 43

Amyotrophic lateral sclerosis (ALS) is the most common neurodegenerative disease that belongs to the group of motor neuron diseases. Motor deficits like reduce in tongue strength, may coexist with cognitive deficits compatible with frontotemporal dementia (FTD), also known as frontotemporal lobar degeneration (FTLD). FTD is a neurodegenerative syndrome with two main clinical variants: behavioral (bvFTD) and language or Primary Progressive Aphasia (PPA). ALS and FTD have significant clinical and neuropathological overlapping so that for some researchers they are "the ends of the same disease spectrum". A key intervention in this patient population is the speech language therapy (SLT), a specific form of cognitive intervention, which evaluates communication skills and designs a personalized intervention plan to improve communication abilities. It has been used in patients with aphasia of different etiologies and has been shown to be effective. There is limited research in SLT interventions in patients in FTD-ALS spectrum, and the initial findings indicate success to some extent. Due to progressive neurodegeneration in FTD-ALS spectrum, the main goal of the intervention is not the complete rehabilitation of linguistic deficits but the reduction and, if possible, the delay of language decline in order to improve patient's communication and the quality of his/her life. In this paper, we critically review the reported approaches of speech language therapy (SLT) for monitoring language impairments and the impact of interventions in patients with FTD-ALS spectrum. Initial findings are supporting more systematic treatment of speech and language impairment in patients in the FTD-ALS spectrum.
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PMID:Speech and language intervention for language impairment in patients in the FTD-ALS spectrum. 3087 31