Not all types of dementia cause memory loss: what happens when it strikes early

What happens when a person's personality, behavior, and language suddenly start changing for the worse? At first glance, these shifts might be chalked up to stress, depression, or a midlife crisis. However, if these changes persist and progressively worsen, they may be indicative of frontotemporal dementia (FTD), a group of disorders that specifically target the frontal and temporal lobes of the brain.

Dementia encompasses a range of disorders affecting cognitive functions such as memory, language, attention, planning, and judgment. While Alzheimer's disease is the most prevalent form of dementia, FTD is another significant type that affects different lobes of the brain, leading to distinct clinical presentations.

In FTD, the frontal and temporal lobes are primarily affected. These areas are crucial for personality, behavior, decision-making, and language. K. Subramaniyan, a clinical lead and senior consultant neurologist, explains that "these areas are important for personality, behavior, decision-making, and language."

FTD presents in several clinical forms. The behavioral variant is characterized by changes in personality, social conduct, empathy, judgment, and impulse control. On the other hand, language variants primarily involve difficulty finding or understanding words, or producing speech.

In contrast to Alzheimer's, where memory problems are usually the first noticeable symptom, in FTD, early signs are predominantly related to behavior, personality, judgment, and language. Memory and routine activities may remain relatively intact, even as behavior increasingly impacts family and workplace relationships.

Recognizing FTD can be challenging for families, as early symptoms may resemble deliberate behavior or a psychiatric issue. A person might become socially inappropriate, impulsive, or apathetic, losing interest in relationships and previously valued activities. Loss of empathy can be particularly distressing, as a previously caring individual may become indifferent to the feelings of family members.

Other alterations can include poor judgment, excessive spending, risky investments, compulsive or repetitive behavior, altered eating habits, an affinity for sweets, neglect of personal hygiene, irritability, and aggression.

Language problems may also arise, including difficulty finding words, reduced speech, or difficulty understanding them. Shrivarthan R., a consultant neurologist, highlights that "if a previously normal person gradually develops unexplained changes in behavior, personality, empathy, judgment, or language, families should consider seeking a medical evaluation rather than assuming it is simply a personality or psychiatric problem."

Diagnosing FTD is primarily based on the pattern of symptoms, a detailed medical history, neurological examination, cognitive and neuropsychological assessments, and brain imaging. Family members' information is particularly crucial as individuals with FTD might not be aware of their own behavioral changes.

The diagnosis of FTD is clinical, relying on detailed history-taking and formal neuropsychological testing. These tests evaluate executive functions, language, judgment, and social cognition, in addition to memory. In some cases, MRI may reveal shrinkage, or atrophy, in the frontal and temporal regions, presenting as a characteristic 'knife-blade atrophy.' However, early scans might sometimes appear normal.

Imaging can also help rule out other conditions causing similar symptoms, such as tumors, hydrocephalus, chronic subdural haemorrhage, and encephalitis. If the diagnosis remains uncertain, functional imaging like an FDG-PET scan may provide additional insights.

In some cases, genetic testing may be considered, particularly when there's a strong family history or an unusually early onset of symptoms. However, identifying a mutation does not currently translate to a disease-modifying treatment.

Treatment for FTD focuses on managing symptoms, maintaining independence, and supporting caregivers. Medications may be prescribed to address symptoms such as irritability, compulsive behavior, aggression, or psychosis. SSRIs, for instance, can help some patients with irritability, compulsive behaviors, or disinhibition, though they primarily treat symptoms rather than the underlying disease.

The primary treatment, as noted by Dr. Shrivarthan, is supportive care. This includes managing apathy, aggression, psychosis, compulsion, and behavior changes. Behavioral and environmental strategies are also vital, such as implementing a predictable routine to reduce difficulties, simplifying communication when language is affected, and utilizing speech and language therapy to retain communication skills.

Occupational therapy can help adapt daily activities and the home environment, especially when judgment is impaired, necessitating monitoring of access to money and reconsideration of driving privileges.

Family members play a crucial role in the management of FTD. They need to understand that these behavioral changes stem from brain-related changes and are not intentional. Caregiver support is equally important, as addressing caregiver burnout is crucial in managing patients with FTD.