Table 1.
Patient | Sex | CAG | AO Motoric | AO Psychic | Duration | TFC | IS % | Rigidity | Swallowing problems |
Case 1 | M | 22/46 | 28 | 22 | 11 | 2 | 20 | ++ | +++ |
Case 2 | F | 20/54 | 37 | ND | 6 | 5 | 70 | + | - |
Case 3* | M | 17/48 | 23 | 23 | 8 | 3 | 40 | ++ | ++ |
Case 3* | M | 17/48 | 23 | 23 | 12 | 2 | 30 | ++ | +++ |
Case 4 | M | 19/48 | 33 | ND | 11 | 4 | 40 | +++ | ++ |
Case 5 | M | 17/43 | 50 | 45 | 6 | 3 | 40 | ++ | ++ |
Case 6 | F | 25/52 | 31 | ND | 10 | 3 | 40 | ++ | ++ |
Case 7 | F | 17/50 | 28 | 26 | 7 | 6 | 70 | + | - |
Case 8 | M | 17/50 | 30 | 30 | 11 | 3 | 30 | +++ | +++ |
Clinical characteristics of the patients. AO = age of onset, TFC = Total functional capacity (UHDRS), IS = Independence score (UHDRS), CAG = CAG-ranges (low/high); Intensity: - = none, + = mild, ++ = moderate, +++ = severe. ND = no data. One patient (*) was treated twice with an increased dose of valproic acid within a 4 year period, see text.