Fronto-temporal dementia and motor neuron disease: a neuropsychological study.
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.
Gentileschi
V
V
Third Neurological Department of the University of Milan, Italy.
Muggia
S
S
Poloni
M
M
Spinnler
H
H
D003704:Dementia / Q000503:physiopathology / Q000523:psychology*; D005500:Follow-Up Studies; D005625:Frontal Lobe / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000503:physiopathology / Q000523:psychology*; D009483:Neuropsychological Tests; D013702:Temporal Lobe / Q000473:pathology*