Frontotemporal Dementia

What it is

Frontotemporal dementia is a group of conditions caused by damage to the frontal and temporal lobes, the parts of the brain at the front and sides that govern personality, behaviour, judgement, emotion and language. Because these areas are affected first, frontotemporal dementia looks quite different from Alzheimer's disease in its early stages: rather than memory loss, the earliest signs are usually striking changes in personality and behaviour, or a progressive loss of language, while day-to-day memory may remain relatively intact for some time.

It is less common than Alzheimer's disease overall but is one of the more common causes of dementia in younger people, often beginning in the fifties and sometimes the forties. This younger onset, and the unusual early symptoms, make it one of the most misunderstood and most frequently misdiagnosed dementias.

What it can look like

There are two main pictures. In the behavioural form, personality and conduct change: a once-considerate person may become tactless, impulsive, apathetic or socially inappropriate, lose empathy, develop new compulsive habits or unusual eating patterns, and seem to lose insight into these changes. In the language forms, the person progressively loses the ability to find words, to speak fluently, or to understand the meaning of words, while other abilities are initially preserved.

Because the early changes are about behaviour or language rather than memory, and because the person is often relatively young, these symptoms are frequently mistaken for a midlife crisis, an affair, depression, stress, or a primary psychiatric illness, sometimes for years before the true cause is found. This delay is hard on families, who may blame the person for changes that are in fact driven by brain disease.

How common is it

Frontotemporal dementia is much less common than Alzheimer's disease in the population as a whole, but because it strikes younger, it accounts for a significant share of dementia in people under sixty-five. There is a stronger genetic component than in many other dementias, and a notable minority of people have a family history.

What causes it

It is caused by the build-up of abnormal proteins in the frontal and temporal lobes, leading to the death of brain cells there; the specific proteins differ between subtypes. Genetics play a larger role than in most dementias: a meaningful proportion of cases run in families, and specific gene changes are known, which is why family history is important and why genetic counselling is sometimes offered. For most people, though, the cause is not a clearly inherited one.

How it is diagnosed

Diagnosis can be challenging and is best done by a specialist familiar with the condition. It rests on the pattern of early behavioural or language change with relatively preserved memory, supported by an account from family, tests of thinking, and brain scans that can show shrinkage in the frontal and temporal regions. A key task is distinguishing it from primary psychiatric conditions and from other dementias, since the treatment and what families need to understand differ. Where there is a strong family history, genetic assessment may be considered.

How it is treated and supported

There is no cure and no treatment that slows the disease, and importantly, the medicines used for memory in Alzheimer's disease are generally not helpful here and can sometimes worsen symptoms. Care therefore centres on understanding, safety and support. A specialist may use medication to manage specific distressing symptoms. The most valuable interventions are non-medical: helping the family understand that the behaviour changes are caused by brain disease and not by choice or malice, which transforms how the changes are met; managing safety given the impulsivity and loss of judgement; speech and language therapy in the language forms; and substantial, sustained support for carers, who face a particularly difficult journey given the personality changes and younger age. The caregiver guidance in our Alzheimer's and dementia entry applies here too.

Frontotemporal dementia in the African context

Frontotemporal dementia is especially easy to misunderstand here, because it often strikes in midlife and changes a person's personality, behaviour, or language rather than their memory, so the early signs, uncharacteristic rudeness, loss of judgement, apathy, or odd conduct, are commonly read as a moral failing, a midlife crisis, marital trouble, or a spiritual or bewitchment problem rather than a brain disease. This can cost a person their job, marriage, and standing before anyone realises they are ill. Awareness is very low and services scarce. Recognising that a marked change in personality or language in a younger or middle-aged adult can be a neurological condition is what spares families years of blame and conflict.

Support for families and carers

This is one of the hardest dementias for families, and support matters greatly.

• Understand that hurtful or out-of-character behaviour is the illness, not a choice, which eases blame and grief.
• Keep routines, reduce triggers for difficult behaviour, and keep the environment calm and safe.
• Protect the family financially and legally early, since judgement and decision-making are often affected.
• Share the load and seek support, since the personality changes are emotionally exhausting. Our Get Support page can help.
• Look after your own wellbeing through a long and demanding course.