From: Cognitive impairment profile in adult patients with Niemann pick type C disease
Functions | Tests | n | Mean | SD | Median | IQR | Normsa |
---|---|---|---|---|---|---|---|
Global cognitive efficiency | Impaired (Y/N) | 17/4 | |||||
MMSE (/30) | 20 | 20.6 | 6.8 | 22 | 17.2–25 | ≥ 27 | |
Mattis (/140) | 12 | 103.6 | 28.6 | 117 | 110–125 | ≥ 123 | |
Executive functions | Impaired (Y/N) | 17/3 | |||||
FAB (/18) | 19 | 11.6 | 4.1 | 12 | 11–14 | ≥ 16 | |
Wisc. (criteria) (/6) | 9 | 3.6 | 1.9 | 3 | 2–6 | ≥ 5.6 | |
Lexical fluency | 12 | 6.5 | 4.4 | 5.5 | 3–9.7 | ≥ 17–19 | |
Semantic fluency | 11 | 13.3 | 5.8 | 11 | 10–15 | ≥ 26–29 | |
Attention | Impaired (Y/N) | 11/1 | |||||
Direct verbal spans | 11 | 4.7 | 1.3 | 5 | 4–6 | 7 ± 2 | |
Indirect verbal spans | 11 | 2.6 | 1.7 | 3 | 2–3 | 5 ± 2 | |
Direct visual spans | 9 | 4.1 | 1.5 | 4 | 3–5 | 7 ± 2 | |
Indirect visual spans | 9 | 3.2 | 1.8 | 3 | 2–4.5 | 5 ± 2 | |
Memory (FCSRT) | Impaired (Y/N) | 6/7 | |||||
Immediate Recall (/16) | 8 | 13.6 | 1.5 | 14 | 12.5–14 | ≥ 14 | |
Free recall (/48) | 8 | 22.5 | 5.7 | 22.5 | 17.5–28.2 | ≥ 17 | |
Total recall (/48) | 9 | 43.4 | 5.6 | 45 | 40.5–47 | ≥ 40 | |
Cued free recall (/16) | 8 | 8 | 3.6 | 8.5 | 6.2–10.7 | ≥ 10 | |
Cued total recall (/16) | 8 | 14.5 | 2.5 | 15.5 | 13.5–16 | ≥ 15 | |
Language | Impaired (Y/N) | 8/3 | |||||
BNT 34 (/34) | 4 | 21.3 | 8.7 | 19.5 | 14–30.2 | ≥ 27 | |
Praxis | Impaired (Y/N) | 10/3 | |||||
Visuo-contructive functions | Impaired (Y/N) | 5/5 | |||||
Rey figure (score) | 6 | 28.3 | 8.1 | 31.5 | 25.5–32.2 | ≥ 29 | |
Rey figure (second) | 6 | 319.2 | 149.9 | 341 | 179.5–459.7 | ≤ 360 |