Frontotemporal dementia
| Frontotemporal dementia | |
|---|---|
| Other names | FTD, Frontotemporal Degeneration, Frontal Lobe Dementia, Pick's disease |
| FTD primarily affects the Frontal and Temporal lobes of the brain. | |
| Specialty | Neurology, Psychiatry |
| Usual onset | Commonly 45–64 years, but can occur earlier |
| Causes | frontotemporal lobar degeneration |
| Risk factors | Family history of FTD or ALS; risk-associated mutations of GRN, MAPT, C9orf72 genes, and other less-common genes; ALS diagnosis; environmental risk factors currently unknown |
| Diagnostic method | Clinical diagnosis of exclusion based on progressive behavioral, cognitive, communication, or movement-based symptoms, with no other explanation. Supportive evidence from neuroimaging or genetic testing. Confirmed diagnosis via brain autopsy. |
| Differential diagnosis | Alzheimer's disease, Parkinsons' disease, Lewy body dementia, Vascular dementia, Mild cognitive impairment, Depression, Generalized anxiety disorder, other mental disorders |
| Treatment | As no cures or disease-modifying treatments have been approved, symptom management is the primary focus for FTD. Person-centered care is often used to address heterogeneous symptoms that vary between people. |
| Medication | No drugs have been approved for the condition, but medications may be prescribed to manage symptoms. |
| Prognosis | Life expectancy is highly variable but is typically 7–13 years. |
| Frequency | In 2011 in the United States, 50,000-60,000 |
Frontotemporal dementia (FTD), also known as frontotemporal degeneration, and historically as Pick’s disease, is a family of progressive neurodegenerative disorders that affect the frontal and temporal lobes. The FTD family includes behavioral variant FTD (bvFTD), primary progressive aphasia (PPA) and its semantic and nonfluent/agrammatic variants (svPPA and nfvPPA), primary progressive apraxia of speech (PPAOS), progressive supranuclear palsy (PSP), and corticobasal syndrome (CBS). Through a mutual risk gene, FTD and amyotrophic lateral sclerosis (ALS) share a clinical spectrum, with it possible for symptoms of both disorders to co-occur. Symptoms of FTD will typically match a specific disorder at first, though symptoms of other disorders will inevitably begin to show as the disease progresses to different areas of the brain. FTD disorders are a common young-onset dementia occurring under the age of 60, often developing when people are raising families and managing careers. As of 2017 there is no cure or approved symptomatic treatment for FTD, although some off-label drugs and behavioral methods are prescribed.
Features of FTD were first described by Arnold Pick between 1892 and 1906. The name Pick's disease was coined in 1922, but is reserved for the behavioral variant of FTD, in which characteristic Pick bodies and Pick cells are present. These were first described by Alois Alzheimer in 1911. Common signs and symptoms include significant changes in social and personal behavior, disinhibition, apathy, blunting and dysregulation of emotions, and deficits in both expressive and receptive language.
Each FTD subtype is relatively rare. FTDs are mostly early onset syndromes linked to frontotemporal lobar degeneration (FTLD), which is characterized by progressive neuronal loss predominantly involving the frontal or temporal lobes, and a typical loss of more than 70% of spindle neurons, while other neuron types remain intact.