Table 6.
Subtypes of Sporadic Creutzfeldt–Jakob Disease
| Subtype* (frequency) | Age of onset Years (range) | Duration Months (range) | Presentation | Movement disorders | Regions predominantly affected |
|---|---|---|---|---|---|
| MM1/MV1(65%) | 68 (31–86) | 5 (1–24) | Cognitive/behavioral, visual changes | Ataxia Myoclonus | Neocortex (particularly occipital lobe), subcortical nuclei and cerebellum |
| VV2(20%) | 64 (40–83) | 6.5 (3–18) | Cerebellar dysfunction. Later, cognitive/behavioral changes | Gait and limb ataxia Oculomotor abnormalities | Cerebellum and subcortical nuclei |
| MV2(10%) | 65 (36–83) | 17 (4–48) | Cognitive/behavioral or motor | Ataxia Parkinsonism Myoclonus | Cerebellum and subcortical nuclei. Less cortical involvement |
| MM2 (Thalamic)(<5%) | 52 (26–71) | 16 (8–36) | Insomnia, cognitive | Ataxia Myoclonus | Thalamus and inferior olive |
| MM2 (Cortical)(<5%) | 64 (49–77) | 16 (9–36) | Cognitive, apraxia, aphasia | Myoclonus | Neocortex |
| VV1(1%) | 44 (19–55) | 21 (17–42) | Cognitive/behavioral | Ataxia Parkinsonism | Cortex and striatum |
*
Subtypes are based on the combination of methionine/valine polymorphism at codon 129 in the PRNP (MV, MM, VV) and the electrophoresis pattern of PrP after the exposure of prion protein to proteinase K digestion (classified as 1 or 2).
Frequency, age of onset, and duration values for this table were obtained from literature review.