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diagnostic criteria for frontotemporal dementia

Essential features: 1) insidious onset & gradual progression 2) early decline in social interpersonal conduct 3) early impairment in regulation of personal conduct 4) early emotional blunting 5) early loss of insight Supportive features: 1) behavior a) decline in personal hygiene & grooming b) mental rigidity & inflexibility c) distractability & impersistence d) hyperoral behavior & dietary changes e) perseveration & stereotypy 2) speech & language a) lack of spontaniety & economy of speech b) echolalia c) mutism 3) signs a) primitive reflexes (frontal release signs) b) incontinence c) akinesia, rigidity & tremor d) low & labile blood pressure 4) neuropsychiatric testing a) impairment on frontal lobe tests b) absence of: 1] severe amnesia 2] aphasia 3] perceptuospatial disorder 5) normal conventional EEG despite dementia 6) MRI or PET show predominant frontal lobe &/or anterior temporal lobe abnormality 7) onset before age 65 8) positive family history in a 1st degree relative 9) associated motor neuron disease (minority of patients) a) bulbar palsy b) muscle atrophy c) fasciculations Notes: - performance of MMSE may be near normal [3]

Related

frontotemporal dementia; frontotemporal lobar degeneration; frontotemporal neurocognitive disorder (FTD, FTLD)

General

criteria

References

  1. Cummings JL, The Neuropsychiatry of Alzheimer's Disease and Related Dementias, Martin Dunitz, 2003
  2. Piguet O et al. Sensitivity of current criteria for the diagnosis of behavioral variant frontotemporal dementia. Neurology 2009 Feb 24; 72:732. PMID: 19237702
  3. Medical Knowledge Self Assessment Program (MKSAP) 17, American College of Physicians, Philadelphia 2015