Pick’s Disease

Pick’s disease is the original term for frontotemporal degeneration characterized by a slowly progressive deterioration of behavior, personality, or language. Currently the term Pick’s disease is used for a specific pathology with Pick bodies, which are abnormal collections of the protein tau in the brain.

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 nonfluent/agrammatic primary progressive aphasia. Piguet et al (2011) counted 62% of 21 cases with autopsy-confirmed Pick’s disease had bvFTD and the rest had progressive aphasia (4 PNFA, 3 semantic dementia, and 1 with a mixture of the two). Of note, 6 of the bvFTD cases had memory loss early in the course of illness. The 8 aphasic patients lived approximately five years longer than the bvFTD group.

Onset typically occurs sometime in the 50s, 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. There is some evidence that patients with Pick’s disease can survive longer than patients with FTD caused by another, more common type of tau abnormality. Not every symptom will be experienced by every person, nor will these symptoms develop in a pre-ordained sequence. 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. In the future, agents that address abnormal tau in the brain may prove useful in Pick’s disease.