Frontotemporal dementia with motor neuron disease

Orpha code: 275872OMIM code: 616439

Definition

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; see this term). The disease is progressive, with death occurring 2-5 years after onset.

Disease data
Classification

Disease

Synonyms
FTD-ALS
FTD-ALS
FTD-MND
Otępienie czołowo-skroniowe ze stwardnieniem zanikowym bocznym
FTD-MND
Frontotemporal dementia with amyotrophic lateral sclerosis
ORPHA code
275872
OMIM code
616439
ICD10 code
G31.0
ICD11 code
6D83

No additional description.

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