Until recently, the damaging processes of ALS were considered specific to the motor system, sparing higher cortical functions associated with thinking, memory, and personality. Frontotemporal dementia is a change in personality and in mental processes. It appears in several degenerative diseases of the nervous system, such as Parkinson's disease. Researchers are finding that this dementia appears to accompany ALS and may even precede it in some cases.
Frontotemporal refers to the forward part of the brain that sits above the eyes and behind the temples. It is the last region of the cerebral cortex to mature, which explains why teens often cannot make good "adult" decisions. Lowering of the function of this region can lead to impulsive, compulsive, and emotional behavior. Dementia from damage to this area differs from the dementia in Alzheimer's disease, in that it is more subtle.
Many investigators are showing that this type of dementia can occur together with motor neuron disease, and in some instances, the personality change precedes the motor symptoms. The changes can hamper a person's ability to cope with the demanding nature of an illness such as ALS. More knowledge of the incidence and nature of this dementia will aid practitioners in caring and planning for patients with ALS.
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