What happens when a previously stable person begins making reckless financial decisions, becomes indifferent to family members or uses socially inappropriate language? Such changes may be mistaken for stress, depression or a midlife crisis. When progressive, however, they can signal frontotemporal dementia (FTD), a group of disorders that predominantly affect the frontal and temporal lobes of the brain.How is FTD different from Alzheimer’s?Dementia is an umbrella term for disorders affecting cognitive functions such as memory, language, attention, planning and judgment. Alzheimer’s disease is the most common form, but there are several types.In FTD, the frontal and temporal lobes are predominantly affected. “These areas are important for personality, behaviour, decision-making and language,” says K. Subramaniyan, clinical lead and senior consultant, neurology, SRM Prime Hospital, Chennai.FTD has different clinical presentations. In the behavioural variant, changes in personality, social conduct, empathy, judgment and impulse control predominate. In language variants, difficulty finding or understanding words, or producing speech, may be the main problem.“In Alzheimer’s disease, one of the early signs is usually memory impairment, particularly impairment of recent memory. In frontotemporal dementia, however, the early problems are predominantly related to behaviour, personality, judgment and language,” says S. Balasubramaniam, senior consultant neurologist and stroke specialist, Prashanth Hospitals, Chennai.Memory and routine activities may remain relatively intact even as behaviour increasingly affects family and workplace relationships.What should raise concerns?FTD can be difficult for families to recognise because early symptoms may resemble deliberate behaviour or a psychiatric problem. A person may become socially inappropriate, impulsive or apathetic, losing interest in relationships and activities they once valued. Loss of empathy can be particularly distressing, with a previously caring person becoming indifferent to the feelings of family members. Other changes can include poor judgment, excessive spending, risky investments, compulsive or repetitive behaviour, altered eating habits, overeating, a preference for sweets, neglect of personal hygiene, irritability and aggression.“Language problems may also emerge, including difficulty finding words, reduced speech or difficulty understanding words,” says Shrivarthan R., consultant, neurology, MGM Healthcare.“If a previously normal person gradually develops unexplained changes in behaviour, personality, empathy, judgment or language, families should consider seeking a medical evaluation 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 based on the pattern of symptoms, detailed history, neurological examination, cognitive and neuropsychological assessments and brain imaging. Information from family members is particularly important because people with FTD may not recognise their own behavioural changes.“The diagnosis of FTD is clinical with detailed history taking and formal neuropsychological testing,” says Sreenivas U..M, consultant neurologist, Apollo Speciality Hospitals, Chennai. “A lot of stress needs to be emphasised to differentiate it from primary psychiatric illness.”Neuropsychological testing assesses executive functions, language, judgment and social cognition, in addition to memory. This can help identify FTD when conventional memory-based screening appears relatively normal.MRI may show shrinkage, or atrophy, in the frontal and temporal regions. In some patients, this produces a characteristic ‘knife-blade atrophy’. Early scans, however, can sometimes be normal.Imaging can also help rule out other conditions that cause similar symptoms, including tumours, hydrocephalus, chronic subdural haemorrhage and encephalitis. When the diagnosis remains uncertain, functional imaging such as an FDG-PET scan may provide additional information.Genetic testing may be considered in selected patients, particularly those with a strong family history or unusually early onset. However, identifying a mutation does not currently mean that a disease-modifying treatment is available.What treatment helps?There is currently no treatment that can reverse FTD or stop its progression. Management focuses on controlling symptoms, maintaining independence and supporting caregivers.Medications may be used for symptoms such as irritability, compulsive behaviour, aggression or psychosis. SSRIs, for instance, may help some patients with irritability, compulsive behaviours or disinhibition, but they treat symptoms rather than the underlying disease.“The primary treatment is supportive,” says Dr. Shrivarthan. “Giving agents to address apathy, aggression, psychosis, compulsion and behaviour changes is the only established treatment at present.”Behavioural and environmental strategies are also important. A predictable routine may help reduce difficulties. Communication can be simplified when language is affected, while speech and language therapy can help retain communication skills.Occupational therapy can help adapt daily activities and the home environment. When judgment is impaired, families may also need to monitor access to money and reconsider driving. “Family members need to understand that these behavioural changes are a result of changes in the brain and are not simply intentional behaviour,” says Dr. Balasubramaniam.Caregiver support is equally important. “Counselling the caregivers and addressing caregiver burnout is crucial in the management of any patient with FTD,” says Dr. Shrivarthan.Who should be tested?FTD can occur without a family history, but an estimated 10–40% of cases may have one, depending on the population and definition used.Several relatives developing similar symptoms at a young age should prompt a discussion about genetic counselling. “If FTD develops early, genetic analysis to identify any target mutations can help contribute to identification of at-risk individuals,” says Dr. Shrivarthan. The implications vary according to the specific gene and mutation, making genetic counselling important before predictive testing.There is currently no established treatment to prevent FTD in an asymptomatic person who carries a disease-causing mutation. Families may need help with financial and legal planning, future care, workplace concerns and safety.Early recognition may not change the course of FTD, but it can change how the condition is understood.