frontotemporal dementia with motor neuron disease
Frontotemporal dementia with motor neuron disease (FTD-MND) is a type of frontotemporal lobar degeneration characterized by the insidious onset (between the ages of 38-78 years) of dementia-associated psychiatric symptoms (e.g. personality changes, uninhibited behavior, irritability, aggressiveness), memory difficulties, global intellectual impairment, emotional disorders and transcortical motor aphasia that eventually leads to mutism, in addition to the manifestations of motor neuron disease such as neurogenic muscular wasting (similar to what is seen in amyotrophic lateral sclerosis). The disease is progressive, with death occurring 2-5 years after onset.
MONDO:0005395
movement disorder
is_a
MONDO:0015547
hereditary dementia
is_a
MONDO:0024237
inherited neurodegenerative disorder
is_a
FTD-ALS
exact
FTD-MND
exact
FTDALS
exact
frontotemporal dementia with ALS
exact
frontotemporal dementia with amyotrophic lateral sclerosis
exact
SPAC6C3.02c SPAC1565.08