TABLE 1.
CORRELATION BETWEEN THE RATE OF POLYMERIZATION OF MUTANTS OF NEUROSERPIN, THE NUMBER OF INCLUSIONS, AND THE SEVERITY OF THE ASSOCIATED DEMENTIA
| Mutation | Rate of Polymerization | Inclusions | Age of Onset (yr) | Clinical Features |
|---|---|---|---|---|
| Ser49Pro | + | + | 45–63 | Dementia, seizures |
| Ser52Arg | ++ | ++ | 20–40 | Dementia, myoclonus |
| His338Arg | +++ | +++ | 15 | Progressive myoclonus epilepsy |
| Gly392Glu | ++++ | ++++ | 13 | Progressive myoclonus epilepsy, chorea |
Reproduced by permission from Reference 16.