What happens when a previously stable person begins making reckless financial decisions, becomes indifferent to family members, or uses socially unacceptable language? Such changes may be mistaken for stress, depression or a midlife crisis. However, if they progress, they can signal frontotemporal dementia (FTD), a group of disorders that primarily affect the frontal and temporal lobes of the brain.
How is FTD different from Alzheimer’s disease?
Dementia is an umbrella term for disorders that affect cognitive functions such as memory, language, attention, planning and judgment. Alzheimer’s disease is the most common form, but there are several types.
FTD primarily affects the frontal and temporal lobes. “These areas are important for personality, behavior, decision-making and language,” says K. Subramanian, clinical director and senior consultant, department of neurology, SRM Prime Hospital, Chennai.
FTD has various clinical manifestations. The behavioral variant is dominated by changes in personality, social behavior, empathy, judgment, and impulse control. In language variants, the main problem may be difficulty finding or understanding words or speaking.
“In Alzheimer’s disease, one of the early signs is usually memory decline, especially decline in recent memory. However, in frontotemporal dementia, early problems are predominantly related to behavior, personality, judgment and speech,” says S. Balasubramaniam, senior consultant neurologist and stroke specialist at Prashanth Hospital, Chennai.
Memory and routine activities may remain relatively intact even as behavior increasingly impacts family and work relationships.
What should you be concerned about?
FTD can be difficult for families to recognize because early symptoms may resemble intentional behavior or a psychiatric problem.
The person may become socially inept, impulsive, or apathetic, losing interest in the relationships and activities that he once valued. Loss of empathy can be especially distressing as a previously caring person becomes indifferent to the feelings of family members. Other changes may include poor judgment, excessive spending, risky investments, compulsive or repetitive behavior, changes in eating habits, overeating, preference for sweets, neglect of personal hygiene, irritability and aggression.
“Language problems may also occur, including difficulty finding words, decreased speech, or difficulty understanding words,” says Srivartan R, consultant, neurology, MGM Healthcare.
“If a previously normal person gradually develops unexplained changes in behavior, personality, empathy, judgment or speech, families should consider seeking medical help rather than assuming it is simply a personality or psychiatric problem,” says Dr. Balasubramaniam.
How is FTD diagnosed?
There is no single test for FTD. Diagnosis is made based on the nature of symptoms, a detailed history, neurological examination, cognitive and neuropsychological assessments, and brain imaging. Information from family members is especially important because people with FTD may not be aware of changes in their behavior.
“The diagnosis of FTD is made on the basis of a clinical examination with a detailed history and formal neuropsychological testing,” says U M Srinivas, consultant neurologist, Apollo Super Specialty Hospitals, Chennai. “The importance of stress must be emphasized to distinguish it from primary psychiatric illness.”
Neuropsychological testing evaluates executive function, language, judgment, and social cognition in addition to memory. This may help identify FTD when conventional memory-based screening appears relatively normal.
MRI may show wrinkling or atrophy in the frontal and temporal regions. In some patients, this causes characteristic “knife blade atrophy.” However, early scans can sometimes be normal.
Imaging can also help rule out other conditions that cause similar symptoms, including tumors, hydrocephalus, chronic subdural hemorrhage, and encephalitis. If the diagnosis remains uncertain, functional imaging such as FDG-PET may provide additional information.
Genetic testing may be considered in selected patients, especially those with a strong family history or unusually early onset of the disease. However, identification of a mutation at this time does not mean that a disease-modifying treatment is available.
What treatment helps?
There is currently no treatment that can reverse FTD or stop its progression. Treatment focuses on controlling symptoms, maintaining independence, and supporting caregivers.
Medications may be used for symptoms such as irritability, compulsive behavior, aggression, or psychosis. SSRIs, for example, may help some patients with irritability, compulsive behavior, or disinhibition, but they treat symptoms, not the underlying condition.
“The main treatment is supportive,” says Dr. Srivartan. “The administration of agents to treat apathy, aggression, psychosis, compulsion, and behavior modification is the only recognized treatment at present.”
Behavioral and environmental strategies are also important. Having a predictable daily routine can help reduce difficulties. Communication can be made easier if speech is affected, and speech and language therapy can help maintain communication skills.
Occupational therapy can help adapt daily activities and home environment. When judgment is impaired, families may also have to control access to money and reconsider their views on driving. “Family members need to understand that these behavioral changes are the result of changes in the brain and not just intentional behavior,” says Dr. Balasubramaniam.
Carer support is equally important. “Counseling caregivers and addressing caregiver burnout is critical in the management of any patient with FTD,” says Dr. Srivartan.
Who should get tested?
FTD can occur without a family history, but it is estimated that it may occur in 10–40% of cases, depending on the population and definition used.
For several relatives who develop similar symptoms at a young age, genetic counseling should be discussed. “If FTD develops early, genetic testing to identify any target mutations can help identify people at risk,” says Dr. Srivartan. The consequences vary depending on the specific gene and mutation, so genetic counseling is important before predictive testing.
There is currently no established treatment to prevent FTD in an asymptomatic person with the disease-causing mutation. Families may need help with financial and legal planning, future care, workplace issues, and safety.
Early recognition cannot change the course of FTD, but it can change the understanding of the disease.