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Frontotemporal Lobar degeneration including Frontotemporal Dementia
What are the symptoms?
FTLD consists of three syndromes: Frontotemporal Dementia (FTD) or the behavioural variant of FTLD; and two syndromes which present with language problems, Progressive Non-Fluent Aphasia (PNFA) and Semantic Dementia (SD).
Frontotemporal Dementia (FTD) or behavioural variant FTLD
FTD varies in the way it affects individuals. There are however a common core of symptoms. Some or all of these may be present at different stages of the disease. The more common symptoms include:
Progressive Non-Fluent Aphasia (PNFA)
In this condition there are progressive difficulties with speech production. This may be the only problem between the ages of two and ten. Reading and writing can become affected as the disease progresses. Patients are usually aware of their speech/language deficit. It is caused by loss of cells in the speech areas of the brain (in most people this is on the left side of the brain). Comprehension of speech is normally unaffected early on but can become a feature later. Similarly, behavioural symptoms can occur as the disease progresses although these are rarely a major problem. This condition is sometimes called Primary Progressive Aphasia (PPA) although this term is also used to mean both PNFA and SD by some doctors.
Semantic Dementia (SD)
SD also presents as a problem with language although it differs from PNFA in being a difficulty with understanding the meaning of words. The first symptom is therefore often difficulty finding the correct words. Speech is still fluent but the person may talk about things in a vague or circumlocutory manner. They often use the wrong word or say 'thing' instead. As the disease progresses there is increased difficulty comprehending what is being said to them. As with PNFA, behavioural symptoms can become a feature later on.
In a small number of patients FTLD overlaps with either Motor Neurone disease (MND) or a disease called Corticobasal Ddegeneration (CBD). Symptoms of MND can include weakness of the limbs or problems with swallowing. Patients with CBD can get stiffness and/or jerking of one or more of their limbs. Furthermore, some patients with FTLD will later develop symptoms similar to Parkinson's disease such as slowing of movements, tremor and stiffness of the limbs.
Background
| What are the causes? 
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