Behavioral variant of frontotemporal dementia

Alternate names

Pick disease of the brain; Lobar atrophy of the brain; Dementia with lobar atrophy and neuronal cytoplasmic inclusions; bvFTD; Pick's disease

Definition

Pick’s disease is a neurological condition characterized by a slowly progressive deterioration of behavior, personality, or language.

Summary

• People with Pick's disease have abnormal substances (called Pick bodies) inside nerve cells in the damaged areas of the brain.
• Pick bodies contain an abnormal form of a protein called tau.
• This protein is found in all nerve cells, but people with Pick's disease have an abnormal amount or type of this protein.

Cause

The underlying cause of Pick's disease is unknown.

Inheritance

In some cases, the disease runs in families.

Signs and symptoms

• Behavioral variant frontotemporal dementia (bvFTD), involves changes in personality, behavior, and judgment.
• People with this dementia may have problems with cognition, but their memory may stay relatively intact.
• They can act strangely around other people, resulting in embarrassing social situations.
• Often, they don’t know or care that their behavior is unusual and don’t show any consideration for the feelings of others.
• They may also do impulsive things that are out of character or engage in repetitive behaviors.
• Over time, language and/or movement problems may occur, and the person needs more care and supervision.

Diagnosis

FTD is diagnosed based on symptoms and results of tests, including:

• Assessment of the mind and behavior (neuropsychological assessment)
• Brain MRI
• Electroencephalogram (EEG)
• Examination of the brain and nervous system (neurological exam)
• Examination of the fluid around the central nervous system (cerebrospinal fluid) after a lumbar puncture
• Head CT scan
• Tests of sensation, thinking and reasoning (cognitive function), and motor function
• Newer methods that test brain metabolism or protein deposits may better allow for more accurate diagnosis in the future
• Positron emission tomography (PET) scan of brain
• A brain biopsy is the only test that can confirm the diagnosis.

Treatment

• There is no specific treatment for FTD.
• Medicines may help manage mood swings.
• Sometimes, people with FTD take the same medicines used to treat other types of dementia.
• In some cases, stopping or changing medicines that worsen confusion or that are not needed can improve thinking and other mental functions.

Medicines include:

• Analgesics
• Anticholinergics
• Central nervous system depressants
• Cimetidine
• Lidocaine
• Medicines may be needed to control aggressive, dangerous, or agitated behaviors.
• Behavior modification can help some people control unacceptable or dangerous behaviors. This consists of rewarding appropriate or positive behaviors and ignoring inappropriate behaviors (when it is safe to do so).
• Talk therapy (psychotherapy) does not always work. This is because it can cause further confusion or disorientation.
• Reality orientation, which reinforces environmental and other cues, may help reduce disorientation.
• Depending on the symptoms and severity of the disease, monitoring and help with personal hygiene and self-care may be needed. Eventually, there may be a need for 24-hour care and monitoring at home or in a special facility. Family counseling can help the person cope with the changes needed for home care.

NIH genetic and rare disease info

• NIH info on Behavioral variant of frontotemporal dementia

Behavioral variant of frontotemporal dementia is a rare disease.

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