Neurology: Case of the Month

Test Yourself — Patient 35

Progressive Non-fluent Aphasia (formerly Primary Progressive Aphasia)

1. Typical frontotemporal lobe degeneration syndromes include all of the following, EXCEPT:
   • [ A ] Frontotemporal dementia
   • [ B ] Lewy body dementia
   • [ C ] Semantic dementia
   • [ D ] Progressive nonfluent aphasia
2. One of the familial forms of FTDP (frontotemporal degeneration with parkinsonism) has been mapped to which chromosomal locus?
   • [ A ] 5q11-13
   • [ B ] 17q21-22
   • [ C ] 17p11.2
   • [ D ] 19q13.2
3. Which candidate gene for FTDP lies within the region specified in question (2)?
   • [ A ] Beta-amyloid precursor protein (beta-APP)
   • [ B ] Apolipoprotein E
   • [ C ] Microtubule-associated protein (tau)
   • [ D ] Alpha-synuclein
   • [ E ] Presenilin-1
4. Which clinical scenario is most consistent with a diagnosis of primary progressive aphasia?
   • [ A ] Early mutism, early amnesia, and late behavioral changes
   • [ B ] Early loss of social skills followed by anomia and apraxia
   • [ C ] Memory loss followed by rapid onset of aphasia
   • [ D ] Progressive impairment of expressive language, with mutism and behavioral changes as late features
5. Supportive features for a diagnosis of progressive aphasia include all, EXCEPT:
   • [ A ] Primitive reflexes, bradykinesia, rigidity, and tremor
   • [ B ] Spatial disorientation, logoclonic speech, myoclonus, and cerebellar ataxia
   • [ C ] Mildly asymmetric EEG and functional imaging
   • [ D ] Onset before age 65, with a positive family history
   • [ E ] Co-occurrence of motor neuron disease

 

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