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Frontotemporal Lobar Degeneration

Overview

Frontotemporal lobar degeneration (FTLD), sometimes referred to as Pick’s disease, is a progressive disorder that causes atrophy (shrinkage) of the frontal and temporal lobes of the brain due to an abnormal build-up of tau protein or TDP-43 protein. Onset of this disease typically occurs at around 45 to 64 years of age. FTLD can cause changes in behavior, language difficulties, movement disorder or problems with walking and balancing.

  • Types

    • Behavior variant frontotemporal degeneration (bvFTD) causes changes in personality, behavior and emotion.
    • Primary progressive aphasia (PPA) affects language skills and the ability to speak, write, read and/or understand words.
    • Progressive motor function decline includes three disorders:
      • Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, which includes weakness and muscle loss
      • Corticobasal syndrome, characterized by involuntary movements, stiffness or changes in behavior, personality, speech or language
      • Progressive supranuclear palsy (PSP) causes problems with walking, imbalance and stiffness, or impaired eye movements
  • Symptoms

    • Behavioral- and mood-related symptoms, which include lack of interest or motivation, apathy, inappropriate emotional response (e.g., laughing at something sad), compulsive eating, uninhibited behavior, impulsivity or difficulty with planning or multi-tasking
    • Language symptoms, such as difficulty understanding words, word-finding or speech changes (e.g., slurring words or speaking softer)
    • Movement symptoms, including stiffness, tremor (shakiness), difficulty performing common actions (e.g., using eating utensils), difficulty walking, imbalance, and muscle weakness or cramps
  • Prevention

    There are no known preventive measures. Genetic counseling may be considered for those with inherited FTLD.

  • Risk Factors

    • Risk factors are not well understood, but obesity was identified as a potential risk factor in a recent study.
    • The condition is inherited in about one third of patients. Gene mutations linked to FTLD affect the MAPT, PGRN, VCP, CHMP2B, TARDBP, FUS or C9ORF72 genes.
  • Diagnosis

    • Medical history and symptom review. The provider will review your personal and family history of medical conditions, review your symptoms and inquire about your ability to perform daily tasks.
    • Physical examination. The doctor will perform a neurologic exam, cognitive testing and/or neuropsychological evaluation.
    • Diagnostic tests. MRI or CT scans of the brain may show brain atrophy at the frontal or temporal lobes. PET or SPECT scans measure areas of brain activity. EEG may show slowing of certain areas of the brain. Genetic testing may be performed for suspected familial forms of FTLD.
  • Treatment

    Unfortunately, there are currently no known treatments to stop or slow the progression of FTLD, but symptoms may be treated using an individualized approach depending on each patient’s needs. Recommendations may include:

    • Keeping a routine and staying mentally, socially and physically active
    • Medications to treat symptoms
    • Physical or occupational therapy for those with difficulties walking or performing activities of daily living
    • Resources for caregiver support
    • Speech therapy for language or swallowing difficulties
  • Follow-up Care

    • Periodic office visits can assess your need for medication changes or referrals.
    • Follow-up visits for abrupt changes in symptoms allow the doctor to evaluate for other causes of this change in symptoms.