• Pick’s Disease / Frontotemporal Dementia

    by  • 12/12/2013 • Psychiatry

    Definition:  is a rare neurodegenerative disease that is just one of the causes of the clinical syndrome known as frontotemporal lobar degeneration.

    Classification / type of disease:

    Epidemiology:  common cause of dementia in 55-65yo

    Presenting symptoms / signs:  associated more with the frontotemporal dementia and progressive nonfluent aphasia subtypes than the semantic dementia subtype.

    Additional signs/symptoms:

    • Uncharacteristic impulsive behaviour
    • Easily distractible
    • Lack of personal hygiene (stops bathing, brushing teeth, combing hair, etc without prompting from wife)
    • Incontinence
    • Depression
    • Parkinsonism
    • Appears unkempt/dishevelled
    • Uncharacteristically rude to friends and family
    • Eats with fingers occasionally
    • No focal neurological signs/symptoms.
    • Prominent grasp and snout reflex.

    Diagnostic investigations:

    • To differentiate between Alzheimer’s disease and Pick’s disease =
    • All six isoforms of tau proteins are expressed in alzheimers.
    • The presence of neurofibrillary tangles that are a hallmark of Alzheimer’s can be stained with antibodies to basic fibroblast growth factoramyloid P, and heparan sulfate glycosaminoglycan.
    • In Pick’s disease, a personality change occurs prior to any form of memory loss, unlike Alzheimer’s, where memory loss typically presents first.
      • This is used clinically to determine whether patient is suffering from Alzheimer’s or Pick’s.
    • Blood and urine tests:
      • Dementia screen which should include B12, TFTs, ANF and TPHA (if appropriate).
    • Genetic tests for Huntington’s disease may be indicated.
    • Further tests may include cerebrospinal fluid (CSF) examination via LP (for chronic meningitis and HIV-related disease) and, if inattention is prominent, exclusion of Lyme disease (Lyme serology) and metastatic carcinoma may be necessary.
    • Brain biopsy in exceptional circumstances

    Imaging may be necessary:

    • Structural scans = CT or more preferred - MRI 
    • Functional scans = PET

     

    Aetiology: Pick’s disease causes progressive destruction of nerve cells in the brain and causes tau proteins in neurons to accumulate into silver-staining, spherical aggregations known as “Pick bodies” (aka balloon cells) that are a defining characteristic of the disease

    Differentials:

     

    Transmission:

    Treatment:

    • Stopping drugs which may be exacerbating memory problems or confusion (anticholinergics, CNS drugs).
    • Treatment of symptoms of depression.
    • Considering thiamine/vitamins.
    • Help with social planning / support

     

    Prognosis: 6 year median survival after onset

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