TABLE 1

Patient demographic data and clinical features

Patient
no.
Age of
onset (yr)
Disease
duration (mo)
GenderPRNP genotype
at codon 129
sCJD
subtype
Clinical signs
at onset
Visual
symptomsa
Visuospatial
dysfunctionb
1601.5MMM1Cognitive/visualYesNo
26020MMV1-2VisuospatialYesYes
36915MMV2Behavior/memoryNoNo
47927MMV2CognitiveNoNo
5556FVV2ApraxiaYesNo
65610FMM1-2LanguageNoNo
7574FMM1MotorNoNo
85524FMV1-2BehaviorNoNo
9632FMM1LanguageNoYes
10696FMV1Behavior/memoryNoNo
116910FMM1-2Cognitive/apraxiaNoYes
  • a Visual symptoms noted at first through last UCSF visit.

  • b Visuospatial dysfunction based on neuropsychological testing or neurological examination.