Frontotemporal dementia (FTD) is a disease process that results in progressive damage to the frontal and/or temporal lobes of the brain which are generally associated with personality, behavior and language. There is a gradual, progressive decline in behavior, language and/or movement with memory usually relatively preserved. Some people with frontotemporal dementia experience dramatic changes in their personality and become socially inappropriate, impulsive or emotionally indifferent. Others lose the ability to use and understand language.
Signs and Symptoms
The signs and symptoms of frontotemporal dementia (FTD) can vary greatly between individuals. General symptoms can include:
- Extreme changes in behavior and personality
- Speech and language difficulties
- Problems with movement
Subtypes of FTD
There are many subtypes of FTD and are identified clinically according to the symptoms that appear first and most prominently. Primary subtypes include:
Behavioral variant (bvFTD)
- Increasing socially inappropriate actions
- Loss of empathy and other interpersonal skills
- Lack of judgement and inhibition
- Repetitive compulsive behavior
- Decline in personal hygiene
- Changes in eating habits, predominately overeating, dietary compulsions.
- Lack of awareness of thinking or behavioral changes.
The majority of bvFTD cases have not been linked to genetic mutations.
Primary Progressive Aphasia (PPA)
- Nonfluent/Agrammatic variant - characteristics include slowing of speech production, grammar difficulties, halting speech and speech sound errors, eventual decline of reading and writing abilities and muteness.
- Semantic variant - characteristics include difficulty naming and understanding objects but fluent, spontaneous speech remains intact.
- Logopenic variant - characterized by impaired single word retrieval and impaired repetition of phrases and sentences.
Progressive Supranuclear Palsy (PSP)
- Loss of balance and gait instability resulting in falls
- Slowness and stiffness of movements
- Difficulty swallowing and speech difficulties
- Immobile, “masked face”
- Forced laughing or crying is prolonged
- Absence or slowness of movements; lack of spontaneous movements
- Rigidity—resistance to imposed movement
- Tremor, often complex
- Abnormal posture of the extremities
- Some patients will exhibit cognitive and language symptoms as well.
FTD with Motor Neuron Disease (FTD/MND)
- Muscle weakness, twitching and cramps
- Tripping or falling
- Shortness of breath
- Muscle atrophy
- Difficulty swallowing
- Slurred speech, nasal or breathy speech
- Tight and stiff muscles
- Exaggerated reflexes
- Outbursts of laughing or crying
FTD & ALS
Researchers recognize an important connection between FTD and Amyotrophic Lateral Sclerosis (ALS/Lou Gehrig’s Disease). Usually, FTD has little effect on movement, however, in about 10-15% of patients with FTD, the disease also involves the nerve cells controlling voluntary movement called motor neurons.
Early and Accurate Diagnosis is Important
People with FTD often don’t recognize that they have a problem. It is often a family member who initiates an appointment with a family doctor. They may be referred to a neurologist or phycologist. FTD is often misdiagnosed as a psychiatric disorder, a movement disorder such as Parkinson’s or Alzheimer’s. Diagnosis accuracy is important as some medications used to treat other disorders may be harmful in a person with FTD.
There is no known treatment to slow or stop the progression of FTD. Supportive care is essential. Certain medications may be used to treat symptoms and speech therapy may be beneficial for those with Primary Progressive Aphasia.
Sources: www.theaftd.org www.mayoclinic.org
Frontotemporal Dementia Facts
- FTD affects an estimated 50,000—60,000 Americans.
- FTD represents an estimated 10% - 20% of all dementia cases. It is recognized as one of the most common dementias occuring in a younger population.
- Onset often occurs in a person’s 50’s or 60’s, but has been seen as early as 21 and as late as 80 years. Roughly 60% of cases occur in people 45-64 years old.
- The course of the disease ranges from 2 to over 20 years, with a mean course of 8 years from the onset of symptoms.
- The prevalence worldwide is uncertain with estimates of FTD amongst people ages 45—64 between 15—22 per 100,000.