TABLE 1.
Reference | Patient ID/gender | Family DNAJC5 mutation* | Age of disease onset (years), first manifestation | Age of death |
---|---|---|---|---|
Boehme et al8 | IV-2/M | c.346_348 del CTC | 39, seizures | 47 |
Boehme et al8 | IV-3/M | c.346_348 del CTC | 32, seizures | 43 |
Boehme et al8 | IV-5/F | c.346_348 del CTC | 30, seizures | NR |
Boehme et al8 | IV-7/F | c.346_348 del CTC | 32, hand weakness | 39 |
Boehme et al8 | IV-13/M | c.346_348 del CTC | 31, seizures | NR |
Boehme et al8 | IV-15/F | c.346_348 del CTC | 33, difficulty speaking | NR |
Nijssen et al10 | 1/F | c.344T>G | 44, dementia and epilepsy | NR |
Nijssen et al10 | 2/F | c.344T>G | 46,myoclonus, epilepsy | 59 |
Nijssen et al10 | 3/F | c.344T>G | 42, seizures | 56 |
Nijssen et al10 | 4/M | c.344T>G | 36, myoclonus | 56 |
Nijssen et al10 | 5/F | c.344T>G | 32, myoclonus | NR |
Nijssen et al10 | 6/M | c.344T>G | 25, myoclonus | NR |
Josephson et al11 | II-1/M | c.344T>G | NR, seizures | 43 |
Josephson et al11 | III-2/M | c.344T>G | NR, seizures | 58 |
Josephson et al11 | III-4/M | c.344T>G | NR, seizures | 57 |
Josephson et al11 | IV-1/M | c.344T>G | 37, myoclonus | 46 |
Josephson et al11 | IV-2/F | c.344T>G | 35, seizures | 45 |
Josephson et al11 | V-3/F | c.344T>G | 32, abnormal EEG | NR |
Josephson et al11 | V-4/F | c.344T>G | 40, seizures | 53 |
Josephson et al11 | V-5/F | c.344T>G | 36, seizures | 41 |
Josephson et al11 | VI-6/F | c.344T>G | 35, seizures | NR |
Josephson et al11 | VI-7/M | c.344T>G | 37, seizures | NR |
Burneo et al12 | B1/F | c.346_348 del CTC | NR, seizures | 81 |
Burneo et al12 | C1/F | c.346_348 del CTC | NR, seizures | 48 |
Burneo et al12 | C2/M | c.346_348 del CTC | NR, seizures | 81 |
Burneo et al12 | D1/F | c.346_348 del CTC | NR, seizures | 39 |
Burneo et al12 | E1/F | c.346_348 del CTC | 28, seizures | 47 |
Burneo et al12 | E3/M | c.346_348 del CTC | NR, seizures | 49 |
Burneo et al12 | E4/M | c.346_348 del CTC | 27, seizures | 46 |
Burneo et al12 | E5/F | c.346_348 del CTC | 29, seizures | 47 |
Burneo et al12 | E6/F | c.346_348 del CTC | 28, seizures | NR |
Burneo et al12 | F1/M | c.346_348 del CTC | 22, seizures | NR |
Burneo et al12 | F4/M | c.346_348 del CTC | 30, myoclonus | NR |
Velinov et al15 | VI-2 | c.346_348 del CTC | 26, OCD, irritability | 54 |
Velinov et al15 | BD319 | c.344T>G | 30s, seizures | 54 |
Jedličková et al17 | Mother | c.370_399 dup | 42, NR | 56 |
Jedličková et al17 | Proband 1 | c.370_399 dup | 31, seizures, memory loss | NR |
Jedličková et al17 | Proband 2 | c.370_399 dup | 34, seizures, memory loss | NR |
Not reported | IBR1 | c.346_348 del CTC | 22, seizures | NR |
Not reported | IBR2 | c.346_348 del CTC | 25, myoclonus | NR |
Not reported | IBR3 | c.346_348 del CTC | 21, seizures | NR |
Jarrett et al18 | III-2/F | c.346_348 del CTC | 36, seizures | 55 |
Jarrett et al18 | IV-2/F | c.346_348 del CTC | 31, memory loss and clumsiness | NR |
Jarrett et al18 | IV-3/M | c.346_348 del CTC | 30, seizures | NR |
Jarrett et al18 | IV-1/F | c.346_348 del CTC | 33, myoclonus | NR |
Note: Mutations identified in the patient or in a family relative.
Abbreviation: NR, not reported; OCD, Obsessive compulsive disorder.