Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
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Drug
Enzyme
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Query: UMLS:C0002736 (
amyotrophic lateral sclerosis
)
19,048
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
The association between
Amyotrophic Lateral Sclerosis
(
ASL
) and FrontoTemporal Dementia (FTD) is well known. Most of reports describing
ASL
-FTD cases show a strong association between
ALS
and the behavioural form of FTD. Conversely, the association between
ALS
and pure
Semantic Dementia
or Progressive Non-Fluent Aphasia (PNFA) is extremely rare, ranging from 1 to 3%. A clinical phenotype characterized by a rapidly progressive aphasic dementia and motoneuron disease (MND) has been described in few case reports; since the updating of PNFA diagnostic criteria in 2011, no clinical report has been related. We want to describe a case of patient presented, at the onset, as PNFA who developed, one year later,
ALS
with bulbar onset. The patient was screened for the main genes causing or associated with MND and/or dementia but no variants with a pathogenetic effect were observed.
...
PMID:A case of progressive non-fluent aphasia as onset of amyotrophic lateral sclerosis with frontotemporal dementia. 3014 30