Frontotemporal Dementia

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Synonyms: Pick's Complex, Pick's Dementia, Pick's Disease

See separate related article Dementia.

This is a progressive dementia, first described by Arnold Pick in 1892, which typically affects the frontal and/or temporal lobes (unlike Alzheimer's disease and other dementias localised to posterior parietal lobes). This is now considered part of an overlapping collection of syndromes which are more common than has been thought in the past.[1] The term Pick Complex now incorporates other frontotemporal dementias. It is a common cause of dementia before the age of 65.[2]

Pick's disease is defined by severe atrophy, neuronal loss and gliosis with Pick cells (ballooned neurons) and Pick bodies (argentophilic neuronal inclusions) found disproportionately in the frontal and temporal cortical regions.[3][4]

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  • Pick's disease is, after Alzheimer's disease and diffuse Lewy body disease, one of the most common cortical neurodegenerative dementias. It is the fourth most common cause of dementia if vascular or non-neurodegenerative dementia is included.
  • Clinically, 10-15% of patients with dementia may have features suggesting Pick's disease, but only about a third of these meet neuropathological criteria specific for Pick's disease.
  • In some series, most patients with autopsy-confirmed Pick's disease have been diagnosed in life with another neurodegenerative disease (usually Alzheimer's disease).[5]
  • It may occur more frequently in Scandinavian countries. Familial forms linked to chromosome 17q are more common in people of Scandinavian origin where it makes up as much as 17% of dementias.
  • More men than women may be affected.
  • It affects a younger age group than Alzheimer's with peak incidence at age 55-65.[6]

Onset of behavioural and cognitive effects is insidious. In the first 2 years there are:

  • Psychiatric problems following a frontal lobe pattern. This may produce aggression, socially inappropriate behaviour, and stereotypical behaviour (orbitofrontal area). Lack of concern and apathy can occur (dorsomedial area).
  • Depression may occur early on and often starts before amnesia.
  • Speech and language problems develop early and deterioration is rapid. Memory problems are less severe than the behavioural and language difficulties early in the disease.
  • Incontinence tends to develop early on unlike in Alzheimer's disease
  • Parkinsonism may develop but not as prominently as in Lewy body disease.[6]

On examination:

  • Patients are often very unkempt and may exhibit inappropriate jocularity ('Witzelsucht') or disinhibition.
  • Patients may exhibit echolalia (repeating the examiner's words) and echopraxia (copying the examiner's gestures).
  • Neurological examination may show primitive reflexes (grasp, sucking, etc.), and akinesia with plastic rigidity. Resting tremor suggests Parkinsonism.

Mental state examination may reveal:

  • Nonfluency of verbal output and difficulty with naming objects (anomia).
  • Sparse, spontaneous and grammatically correct speech (logopenia).

Visuospatial and visual orientation skills are relatively well preserved.

Clinical diagnostic features of frontotemporal dementia are listed in annex 6 of the Scottish Intercollegiate Guidelines Network (SIGN) guidance.[7]

Includes:

  • Blood and urine tests:
    • Dementia screen which should include B12, TFTs, ANF and TPHA (if appropriate).
    • If encephalopathy is suspected note particularly FBC, LFTS, biochemistry, ammonia level, erythrocyte sedimentation rate (ESR) and urine toxicology.
    • If Parkinsonism present add ceruloplasmin and serum copper (to exclude Wilson's disease) with a peripheral blood screen for acanthocytes.
  • Genetic tests for Huntington's disease may be indicated.
  • Further tests may include cerebrospinal fluid (CSF) examination (for chronic meningitis and HIV-related disease) and, if inattention is prominent, exclusion of Lyme disease (Lyme serology) and metastatic carcinoma may be necessary.
  • Measurement of biomarkers in the CSF has been useful in Alzheimer's disease (raised levels of phosphorylated tau protein and low levels of beta-amyloid are found). However, levels of tau and beta-amyloid in the CSF of patients with frontotemporal dementias have been less useful in diagnosis and assessing prognosis.[6][8]
  • Brain biopsy in exceptional circumstances may be required (diagnosis in doubt, substantial benefit to patient or family from tissue diagnosis, treatment with adverse effects being considered (eg autoimmune therapy for neurosarcoidosis).

Imaging may be necessary:

  • CT scan of the brain if MRI is contra-indicated.
  • MRI is preferred, as CT scans can miss relevant pathology - for example, metastases and subcortical infarcts.
  • Functional scans such as positron emission tomography (PET) scans may be used to demonstrate acquired disease (for example, for employment-related concerns).

Lumbar puncture with extensive testing of CSF is done routinely by some specialists in the field.

This should incorporate:

  • Stopping drugs which may be exacerbating memory problems or confusion (anticholinergics, CNS drugs).
  • Treatment of symptoms of depression.
  • Considering thiamine/vitamins.
  • Proceeding with first- and second-line investigations as appropriate.
  • Helping with organisation of social and family care.
  • Provision of information to the family, with full discussion of investigations, results, diagnosis and management.

Referrals to other agencies will be required, including geriatric medicine, psychiatry, psychology, social work and community nursing.[9]

Subdural haematoma is a greater risk after lumbar puncture in Pick's disease.[6]

Slow progression of symptoms, with increased disability both at work and home, is usual. Some patients develop creative and artistic interests. It runs a shorter course than Alzheimer's disease with a 6-year median survival after onset. Some forms where speech is predominantly affected may progress more slowly.[10]

Where there is a strong family history of frontotemporal dementia, genetic testing should be discussed. Genetic counselling should be undertaken before testing is undertaken.

Further reading & references

  1. Kertesz A; Pick Complex: an integrative approach to frontotemporal dementia: primary progressive aphasia, corticobasal degeneration, and progressive supranuclear palsy. Neurologist. 2003 Nov;9(6):311-7.
  2. Galariotis V, Bodi N, Janka Z, et al; Frontotemporal dementia--Part I. History, prevalence, clinical forms. Ideggyogy Sz. 2005 May 20;58(5-6):164-71.
  3. The Internet Pathology Laboratory; Web Path; Views of brain specimens
  4. Frontotemporal Dementias - Pick's Disease, in Neuropathology - Chapter 9 Degenerative Diseases
  5. Litvan I, Agid Y, Sastry N, et al; What are the obstacles for an accurate clinical diagnosis of Pick's disease? A clinicopathologic study. Neurology. 1997 Jul;49(1):62-9.
  6. Barrett AM; Pick Disease, eMedicine, Jun 2008
  7. Management of patients with dementia, Scottish Intercollegiate Guidelines Network - SIGN (Feb 2006)
  8. Schipper HM; Biological markers and Alzheimer disease: a canadian perspective. Int J Alzheimers Dis. 2010 Aug 8;2010. pii: 978182.
  9. Dementia: Supporting people with dementia and their carers in health and social care; NICE Clinical Guideline (2006)
  10. Hodges JR, Davies R, Xuereb J, et al; Survival in frontotemporal dementia. Neurology. 2003 Aug 12;61(3):349-54.

Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions. EMIS has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our conditions.

Original Author:
Dr Richard Draper
Current Version:
Last Checked:
23/11/2010
Document ID:
695 (v24)
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