Frontotemporal dementia-a clinical overview

DR. MATCAU DIANA, DR. ARNAUTU SERGIU,PROF.DR.JIANU DRAGOS CATALIN

NEUROLOGY CLINIC TIMISOARA

DEFINITION

• Frontotemporal dementia (FTD) or frontotemporal degenerationsrefers to a group of disorders caused by progressive nerve cell loss in the brain's frontal lobes or temporal lobes.

Other names for frontotemporal dementia

• FTD • Pick's disease • frontal dementia • frontotemporal lobar degeneration • behavioural variant frontotemporal

dementia

• primary progressive aphasia • semantic dementia • progressive non-fluent aphasia

About frontotemporal dementia

The nerve cell damage caused by frontotemporal dementia leads to loss of function in these brain regions, which variably cause deterioration in behavior and personality, language disturbances, or alterations in muscle or motor functions.There are a number of different diseases that cause frontotemporal degenerations. The two most prominent are:

1) a group of brain disorders involving the protein tau;

2) a group of brain disorders involving the protein called TDP43.

For reasons that are not yet known, these two groups have a preference for the frontal and temporal lobes that cause dementia.

Types

I. FTD used to be called Pick's disease after Arnold Pick, M.D., a physician who in 1892 first described a patient with distinct symptoms affecting language. Some doctors still use the term "Pick's disease." Other terms you may see used to describe FTD include frontotemporal disorders, frontotemporal degenerations and frontal lobe disorders.

Behavior variant frontotemporal dementia (bvFTD) is characterized by prominent changes in personality, interpersonal relationships and conduct that often occur in people in their 50s and 60s, but can develop as early as their 20s or as late as their 80s. In bvFTD, the nerve cell loss is most prominent in areas that control conduct, judgment, empathy and foresight, among other abilities.

Types

II. Primary progressive aphasia (PPA) is the second major form of frontotemporal degeneration that affects language skills, speaking, writing and comprehension. PPA normally comes on in midlife, before age 65, but can occur in late life also. The two most distinctive forms of PPA have somewhat different symptoms:

• In semantic variant of PPA, individuals lose the ability to understand or formulate words in a spoken sentence.

• In nonfluent/agrammatic variant of PPA, a person’s speaking is very hesitant, labored or ungrammatical.

Types

III. Disturbances of motor (movement or muscle) function include three disorders that are a part of the frontotemporal degeneration spectrum that produce changes in muscle or motor functions with or without behavior (bvFTD) or language (PPA) problems:

• Amyotrophic lateral sclerosis (ALS), which causes muscle weakness or wasting.

• Corticobasal syndrome, which causes arms and legs to become uncoordinated or stiff.

• Progressive supranuclear palsy (PSP), which causes muscle stiffness, difficulty walking and changes in posture. It also affects eye movements.

Key differences between FTD and Alzheimer's• Age at diagnosis may be an important clue. Most people with FTD are diagnosed in their 40s and early 60s.

Alzheimer's, on the other hand, grows more common with increasing age.

• Memory loss tends to be a more prominent symptom in early Alzheimer's than in early FTD, although advanced FTD often causes memory loss in addition to its more characteristic effects on behavior and language.

• Behavior changes are often the first noticeable symptoms in bvFTD, the most common form of FTD. Behavior changes are also common as Alzheimer's progresses, but they tend to occur later in the disease.

• Problems with spatial orientation — for example, getting lost in familiar places ( more common in Alzheimer's than in FTD).

• Problems with speech: Although people with Alzheimer's may have trouble thinking of the right word or remembering names, they tend to have less difficulty making sense when they speak, understanding the speech of others, or reading than those with FTD.

• Hallucinations and delusions are relatively common as Alzheimer's progresses, but relatively uncommon in FTD.

Diagnosis• The diagnosis of bvFTD and PPA are based on expert evaluation by a doctor who is familiar with these disorders. The type of problems experienced by the patient and the results of neurological exams are the core of the diagnosis. Brain scans such as magnetic resonance imaging (MRI) and glucose positron emission scans are very helpful additional tests, but they must be interpreted in the context of the patient’s history and neurological exam.

• The following tests may be needed to make a diagnosis:

• an assessment of symptoms – it's normally helpful to have somebody who knows the person well to give an account of their symptoms, especially as someone with frontotemporal dementia may not be aware of changes in their behaviour

• an assessment of mental abilities – this will usually involve a number of tasks and questions

• blood tests – to rule out conditions with similar symptoms

• brain scans – such as an MRI scan, a CT scan or a PET scan; these can detect signs of dementia and help identify which parts of the brain are most affected, or help rule out other problems with the brain

• lumbar puncture – to test the spinal fluid; this may be useful to rule out Alzheimer's disease as the cause of symptoms

Causes and risks• Frontotemporal degenerations are inherited in about a third of all cases. Genetic counselingand testing are available now in individuals with family histories of frontotemporal degenerations. There are no known risk factors for any frontotemporal degenerations except for a family history or a similar disorder.

• Frontotemporal dementia is caused by clumps of abnormal protein forming inside brain cells. These are thought to damage the cells and stop them working properly.

• The proteins mainly build up in the frontal and temporal lobes of the brain at the front and sides. These are important for controlling language, behaviour, and the ability to plan and organise.

• It's not fully understood why this happens, but there's often a genetic link. About one in every three people with frontotemporal dementia has a family history of dementia.

Symptoms of frontotemporal dementiaFrontotemporal dementia usually causes changes in behaviour or language problems at first.

These come on gradually and get worse slowly over time.

Signs of frontotemporal dementia can include:

•Personality and behavior changes

•Language problems

•Problems with mental abilities

•Memory problems

•Physical problems

I. Personality and behaviour changes Most patients with frontotemporal dementia develop a number of unusual behaviours they're not aware of, these can include:

• being insensitive or rude

• acting impulsively or rashly

• loss of inhibitions

• seeming subdued

• losing interest in people and things

• losing drive and motivation

• inability to empathise with others, seeming cold and selfish

• repetitive behaviours, such as

humming, hand-rubbing and foot-tapping, or routines such as walking exactly the same route repetitively

• overeating, a change in food preferences, such as suddenly liking sweet foods, and poor table manners

• neglecting personal hygiene

• As the condition progresses, people with frontotemporal dementia may become socially isolated and withdrawn.

II. Language problems – Some people experience problems with speech and language, including:

• using words incorrectly – for example, calling a sheep a dog

• loss of vocabulary

• repeating a limited number of phrases

• forgetting the meaning of common words

• slow, hesitant speech

• difficulty making the right sounds to say words

• getting words in the wrong order

• automatically repeating things other people have said

Some people gradually lose the ability to speak, and can eventually become completely mute.

III. Problems with mental abilities – Problems with thinking don't tend to occur in the early stages of frontotemporal dementia, but these often develop as the condition progresses. These can include:

• difficulty working things out and needing to be told what to do

• poor planning, judgement and organisation

• becoming easily distracted

• thinking in a rigid and inflexible way

• losing the ability to understand abstract ideas

• difficulty recognising familiar people or objects

• memory difficulties, although this isn't common early on

IV. Physical problems - These problems can make daily activities increasingly difficult, and the person may eventually be unable to look after themselves.

In the later stages, some people with frontotemporal dementia develop physical problems and difficulties with movement.

These can include:

• slow, stiff movements, similar to Parkinson's disease• difficulty swallowing• loss of bladder control• loss of bowel control

Some people have frontotemporal dementia overlapping with other neurological (nerve and brain) problems, including:• motor neurone disease – causes increasing weakness, usually with muscle wasting • corticobasal degeneration – causes problems controlling limbs, loss of balance and co-ordination, slowness and

reduced mobility • progressive supranuclear palsy – causes problems with balance, movement, eye movements and swallowing.

V. Memory problems – these only tend to occur later on, unlike more common forms of dementia, such as Alzheimer's disease

Treatment

There are no specific treatments for any of the frontotemporal subtypes. There are medications that can reduce agitation, irritability and/or depression. These treatments should be used to help improve quality of life.

FTD inevitably gets worse over time and the speed of decline differs from person to person. For many years, individuals with FTD show muscle weakness and coordination problems, leaving them needing a wheelchair — or bedbound. These muscle issues can cause problems swallowing, chewing, moving and controlling bladder and/or bowels. Eventually people with frontotemporal degenerations die because of the physical changes that can cause skin, urinary tract and/or lung infections.

Treatments include:

• medicines – to control some of the behavioural problems

• therapies – such as physiotherapy, occupational therapy, and speech and language therapy for problems with movement, everyday tasks and communication

• dementia activities – such as memory cafés, which are drop-in sessions for people with memory problems and their carers to get support and advice

• support groups – who can offer tips on managing symptoms from dementia experts and people living with frontotemporal dementia, and their families

The following medicines may help:

• antidepressants – antidepressants called selective serotonin reuptake inhibitors (SSRIs) may help control the loss of inhibitions, overeating and compulsive behaviours seen in some people

• antipsychotics – these are rarely used, but if needed they can help control severely challenging behaviour that's putting the person with dementia or others around them at risk of harm

The medications for Alzheimer's disease aren't effective for frontotemporal dementia.

Support and other therapies

In addition to medication, there are a number of therapies and practical measures that can help make everyday living easier for someone with dementia.

These include:

• occupational therapy – to identify problem areas in everyday life, such as getting dressed, and help work out practical solutions

• speech and language therapy – to help improve any communication or swallowing problems

Support and other therapies

• physiotherapy – to help with movement difficulties

• relaxation techniques – such as massage, and music or dance therapy

• social interaction, leisure activities and other dementia activities –such as memory cafés, which are drop-in sessions for people with memory problems and their carers to get support and advice

• strategies for challenging behaviour – such as distraction techniques, a structured daily routine, and activities like doing puzzles or listening to music

• incontinence products if needed

Outlook for frontotemporal dementia

How quickly frontotemporal dementia gets worse varies from person to person and is very difficult to predict.

People with the condition can become socially isolated as the illness progresses. They may not want to spend time in the company of others, or may behave in rude or insulting ways.

Home-based help will usually be needed at some stage, and some people will eventually need care in a nursing home.

The average survival time after symptoms start is around eight years. But this is highly variable and some people live much longer than this.

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