What is frontotemporal dementia, the condition Bruce Willis suffers from

Frontotemporal Dementia is a neurological disorder that primarily hits the nerves of the frontal and temporal lobes of the brain. All you want to know about the disorder.

Frontotemporal Dementia is a gradual loss of brain tissue in the frontal and temporal lobes of the brain and develops over a period for many years before the symptoms start to show. The disease has brought in focus after Hollywood star Bruce Willis, 67, was diagnosed with it recently as announced by his family. Last year, Willis was diagnosed with aphasia which causes difficulty with language and speech. This was perhaps the early sign of his dementia. Symptoms of frontotemporal dementia may get progressively worse if not addressed on time. Behavioural changes, motor disorder, Speech and language problems can be seen in the disorder. (Also read: What is Aphasia? Here's what you need to know)

Frontotemporal Dementia is a neurological disorder that primarily hits the nerves of the frontal and temporal lobes of the brain

What is frontotemporal dementia

"Frontotemporal Dementia is a neurological disorder that primarily hits the nerves of the frontal and temporal lobes of the brain. These areas of the brain are usually linked to personality, behaviour and language. Symptoms of frontotemporal dementia may vary from person to person. If left unnoticed, Symptoms may get progressively worse over time. People suffering from this disorder may show symptoms such as behavioural changes, Motor disorder, Speech and language problems," says Dr Najeeb Ur Rehman, Senior Consultant Neurology, Marengo QRG Hospital, Faridabad.

"Frontotemporal Dementia is an umbrella term for a group of brain disorders that primarily affects the frontal and temporal lobes of the brain Damage to neurons leads to early atrophy and shrinkage of these lobes of the brain," says Dr. Vishal Bhasme- Consultant Neurosurgeon at Ruby Hall Clinic.

Symptoms of frontotemporal dementia

"Many symptoms, such as strange behaviours, emotional issues, communication difficulties, difficulties at work, or difficulties walking, are possible effects. FTD is uncommon and tends to occur early in life, unlike other types of dementia. About 60% of FTD patients are between the ages of 45 and 64," says Dr Bhasme.

Behavioural changes

The most commonly reported signs of frontotemporal dementia as per Dr Rehman are extreme changes in behaviour and personality.

"These are increasingly inappropriate social behaviour, loss of empathy and other interpersonal skills like having sensitivity to another's feelings, lack of judgment, loss of inhibition, lack of interest (apathy) which can be mistaken for depression, repetitive compulsive behaviour like tapping, clapping or smacking lips, a reduction in personal hygiene, changes in eating habits, generally overeating or developing a preference for sweets and carbohydrates, eating inedible objects and compulsively wanting to put things in the mouth," says Dr Rehman.

Speech and language problems

Some subtypes of frontotemporal dementia may lead to language problems or impairment or loss of speech. Primary progressive aphasia, semantic dementia and progressive agrammatic (non-fluent) aphasia are all known as frontotemporal dementia.

"These conditions may cause problems such as developing difficulty in using and understanding written and spoken language like having trouble finding the right word to use in speech or naming objects, trouble naming things, no longer knowing word meanings, developing hesitant speech that may sound telegraphic and making mistakes in sentence construction," says Dr Rehman.

Motor disorders

Motor-related problems are tremor, rigidity, muscle spasms or twitches, poor coordination, difficulty swallowing, muscle weakness, inappropriate laughing or crying and falls or walking problems.

Causes of frontotemporal dementia

In frontotemporal dementia, the frontal and temporal lobes of the brain get constricted. Moreover, certain substances may accumulate in the brain. The exact cause of these changes is not clear. There are genetic mutations that may be associated with frontotemporal dementia. You may be at higher risk of developing frontotemporal dementia if you have a family history of dementia.

"Frontotemporal dementia (FTD) is currently a largely unknown disease, as its underlying causes remain unresolved. Patients with FTD typically have high levels of proteins called tau and TDP-43 in their neurons - the cells that control nerve function. However, there are still many unanswered questions about this condition. The neurons die because the build-up of these proteins causes damage. However, in some cases, mutations or changes in genes can be identified as the cause of FTD," says Dr Bhasme.

Diagnosis of frontotemporal dementia

"No single test may identify frontotemporal dementia therefore signs and symptoms of the disease are pondered," says Dr Rehman.

- To identify other causes of impairment, lab tests, neurological testing and brain imaging are conducted.

- Blood tests may help rule out other conditions like liver or kidney disease. If the patient has symptoms of sleep apnoea (loud snoring and pauses in breathing while sleeping), he/she may be recommended to undergo a sleep study to rule out obstructive sleep apnoea as a cause of your symptoms.

- Neuropsychological testing is also done to test your reasoning and memory skills as it is especially helpful in determining the type of dementia at an early stage. This test may also help differentiate frontotemporal dementia from other causes of dementia.

- Brain scans (Magnetic resonance imaging-MRI, Fluorodeoxyglucose positron emission tracer -FDG-PET scan) may help identify visible conditions like clots, bleeding or tumours that may be causing signs and symptoms.

Treatment

"At present, there is no cure or specific treatment available for frontotemporal dementia. Certain medications and speech therapy may help control symptoms of frontotemporal dementia. Some types of antidepressants, such as trazodone may be prescribed to lessen the behavioural problems linked to frontotemporal dementia. Antipsychotic medications like olanzapine (Zyprexa) or quetiapine (Seroquel) may also be advised to treat the behavioural problems of frontotemporal dementia. Patients experiencing language difficulties may be recommended to undergo speech therapy to learn alternate strategies for communication," says Dr Rehman.

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