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Diagnostic criteria for corticobasal degeneration*

Diagnostic criteria for corticobasal degeneration*
  Clinical research criteria for probable sporadic CBD Clinical criteria for possible CBD
Presentation Insidious onset and gradual progression Insidious onset and gradual progression
Minimum duration of symptoms, years 1 1
Age at onset, years ≥50 No minimum
Family history (two or more relatives) Exclusion Permitted
Permitted phenotypes
  1. Probable CBS
    or
  2. FBS or NAV plus at least one CBS feature:
    • Limb rigidity or akinesia
    • Limb dystonia
    • Limb myoclonus
    • Orobuccal or limb apraxia
    • Cortical sensory deficit
    • Alien limb phenomena
  1. Possible CBS
    or
  2. FBS or NAV
    or
  3. PSPS plus at least one CBS feature:
    • Limb dystonia
    • Limb myoclonus
    • Orobuccal or limb apraxia
    • Cortical sensory deficit
    • Alien limb phenomena
Genetic mutation affecting tau (eg, MAPT) Exclusion Permitted
CBD: corticobasal degeneration; CBS: corticobasal syndrome; FBS: frontal behavioral-spatial syndrome; MAPT: microtubule-associated protein tau; NAV: nonfluent/agrammatic variant of primary progressive aphasia; PSPS: progressive supranuclear palsy syndrome.
* Exclusion criteria for both clinical research criteria for probable sporadic CBD and possible CBD:
  1. Evidence of Lewy body disease: classic 4 Hz Parkinson disease resting tremor, excellent and sustained levodopa response, or hallucinations.
  2. Evidence of multiple system atrophy: dysautonomia or prominent cerebellar signs.
  3. Evidence of amyotrophic lateral sclerosis: presence of both upper and lower motor neuron signs.
  4. Semantic- or logopenic-variant primary progressive aphasia.
  5. Structural lesion suggestive of focal cause.
  6. Granulin mutation or reduced plasma progranulin levels; TDP-43 mutations; FUS mutations.
  7. Evidence of Alzheimer disease (this will exclude some cases of CBD with coexisting amyloid; data from one brain bank suggest that excluding cases with evidence of amyloid may result in missing approximately 14 percent of CBD cases [Dickson D, 2012, personal communication]): laboratory findings strongly suggestive of Alzheimer disease such as low cerebrospinal fluid beta-amyloid 42-to-tau ratio or positive 11C-Pittsburgh compound B PET; or genetic mutation suggesting Alzheimer disease (eg, presenilin, amyloid precursor protein).
¶ Possible CBD emphasizes clinical presentations consistent with CBD but ones that may also overlap with other tau-based pathologies.
From: Armstrong MJ, Litvan I, Lang AE, et al. Criteria for the diagnosis of corticobasal degeneration. Neurology 2013; 80:496. DOI: 10.1212/WNL.0b013e31827f0fd1. Copyright © 2013 American Academy of Neurology. Reproduced with permission from Wolters Kluwer Health. Unauthorized reproduction of this material is prohibited.
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