Frontotemporal Dementia - Signs and Symptoms

Signs and Symptoms

FTD is traditionally difficult to diagnose due to the heterogeneity of the condition. This heterogeneity means the signs and symptoms can vary dramatically between patients. Symptoms are classified into three groups which underlie the functions of the frontal and temporal lobes:

  • Behavioural variant FTD (bvFTD). Behavioural symptoms include lethargy and aspontaneity or oppositely disinhibition. Apathetic patients may become socially withdrawn and stay in bed all day or no longer take care of themselves. Disinhibited patients can make inappropriate (sometimes sexual) comments or perform inappropriate acts. Patients with FTD can sometimes get into trouble with the police because of inappropriate behaviour such as stealing. Recent findings indicate that psychotic symptoms are rare in FTD, possibly due to limited temporal-limbic involvement in this disorder. Among FTD patients, only approximately 2% have delusions, sometimes with paranoid ideation; hallucinations are rare. This is significantly less than AD patients, where approximately 20% have delusions and paranoia. See ref. "Psychotic symptoms."
  • Progressive nonfluent aphasia (PNFA). Patients present with a breakdown in speech fluency due to articulation difficulty, phonological and/or syntactic errors but preservation of word comprehension.
  • Semantic dementia (SD). Some patients remain fluent with normal phonology and syntax but increasing difficulty with naming and word comprehension.

Executive function is the cognitive skill of planning and organizing. Most FTD patients become unable to perform skills that require complex planning or sequencing. In addition to the characteristic cognitive dysfunction, a number of primitive reflexes known as frontal release signs are often able to be elicited. Usually the first of these frontal release signs to appear is the palmomental reflex which appears relatively early in the disease course whereas the palmar grasp reflex and rooting reflex appear late in the disease course.

FTD can occur in patients with motor neuron disease (typically amyotrophic lateral sclerosis) in a small number of cases. The prognosis for people with MND is worse when combined with FTD, shortening survival by about a year.

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