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Fronto‐Temporal Dementia and Motor Neuron Disease: a neuropsychological study
Author(s) -
Gentileschi V.,
Muggia S.,
Poloni M.,
Spinnler H.
Publication year - 1999
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.1999.tb00408.x
Subject(s) - neuropsychology , dementia , frontotemporal dementia , context (archaeology) , disease , psychology , neuropsychological assessment , amyotrophic lateral sclerosis , medicine , pediatrics , psychiatry , physical medicine and rehabilitation , cognition , pathology , paleontology , biology
The neuropsychological follow‐up study of a 58‐year‐old man suffering from Motor Neuron Disease (ALS/MND) and Fronto‐Temporal Dementia (FTD) is reported. Neuromuscular signs first appeared at the age of 51 and slowly progressed to late bulbar involvement; behavioural symptoms of the frontal type first appeared around age 53; lastly, several neuropsychological symptoms suggestive of worsening temporal involvement supervened at age 57. Our patient died at 59 of respiratory failure with the classic clinical and neuroradiological picture of FTD. A short discussion addresses the controversial issue of the coupling of ALS/MND with Dementia and its possible interpretation as the expression of a chance association of relatively common diseases, versus that of a single multifaceted disease. The role of a detailed neuropsychological assessment is highlighted, within the context of increasingly specific diagnostic criteria for FTD.