Pick’s Disease

Pick’s disease is the original term for frontotemporal dementia characterized by a slowly progressive deterioration of social skills and changes in personality, or with impairment of language. Currently the term Pick’s disease is reserved for a specific pathology with Pick bodies, which are abnormal collections of the protein tau in the brain. A diagnosis of Pick’s disease can only be confirmed through post-mortem examination of brain tissue.

Clinically it may be indistinguishable from the behavioral, aphasic or Parkinson-like presentations of FTD. Onset typically occurs sometime in the 50’s, though it can occur as early as age 20 or as late as age 80. As with all FTDs, the course of Pick’s disease will vary from one person to another. Not every symptom will be experienced by every person, nor will these symptoms develop in a pre-ordained sequence.

Key Clinical Features

Most Pick’s disease patients suffer from symptoms consistent with behavioral variant FTD (bvFTD); they may or may not also experience language and neurological difficulties. Other Pick’s disease patients may have predominant symptoms of Progressive Nonfluent Aphasia (PNFA). Very severe atrophy restricted to the frontal and temporal lobes can be seen on CT head or MRI brain and raises suspicion for this particular type of FTD.

Key Pathologic Features

The term Pick’s disease is reserved for a specific pathology with Pick bodies, which are abnormal collections of the protein tau in the brain.

Genetics

The majority of Pick’s disease cases are not hereditary.

Treatment

As with all forms of FTD, there is no cure for Pick’s disease, and in most cases its progression cannot be slowed. Although no medications have been proven effective specifically in FTD, many clinicians look to the medications and treatment approaches targeting behavioral disturbances as necessary.

For instance, some FTD patients benefit from selective serotonin reuptake inhibitors (SSRIs) used to treat obsessive-compulsive behaviors such as hoarding or craving sweets. Clinicians may also recommend antioxidants, such as coenzyme Q10, which are known to slow the progression of damage to brain cells in general, but there is little evidence to support this in Pick’s disease.

In the future, agents that address abnormal tau in the brain may prove useful in Pick’s disease.