Table 3

Cognitive evaluation of patients with NIID

TotalClinical phenotype
Dementia-dominant typeMovement disorder-dominant typeMuscle weakness-dominant typeParoxysmal symptom-dominant type
General cognition
 MMSE sore, median (IQR)24.00 (18.00–27.00)16.50 (13.75–22.00)25.00 (20.00–27.00)28.00 (26.00–29.00)27.00 (25.00–28.00)
 MMSE
(<education-matched average)
62/159 (42.9%)46/58 (79.3%)14/45 (31.1%)2/19 (10.5%)0/37 (0.0%)
 MoCA score, median (IQR)17.00 (12.00–22.00)11.00 (8.75–17.00)17.00 (12.75–20.00)24.00 (21.00–26.00)20.00 (15.50–24.50)
 MoCA
(<education-matched average)
91/106 (85.8%)35/37 (94.6%)29/32 (90.6%)7/13 (53.8%)20/24 (83.3%)
Detailed MoCA item/scores
Visuospatial/ executive/5, median (IQR)2.00 (1.00–3.00)1.00 (0.00–2.00)2.00 (1.00–3.00)3.00 (3.00–3.50)2.50 (1.00–3.00)
Naming/3, median (IQR)3.00 (2.00–3.00)2.00 (0.25–3.00)3.00 (2.00–3.00)3.00 (2.00–3.00)2.00 (2.00–3.00)
Attention/6, median (IQR)4.00 (3.00–6.00)3.00 (2.00–4.00)4.00 (2.00–6.00)6.00 (4.50–6.00)5.00 (3.75–5.25)
Language/3, median (IQR)1.00 (1.00–2.00)1.00 (0.00–2.00)2.00 (1.00–2.00)3.00 (2.00–3.00)1.00 (0.00–2.00)
Abstraction/2, median (IQR)1.00 (0.00–2.00)0.00 (0.00–1.75)1.00 (1.00–2.00)1.00 (1.00–2.00)1.00 (0.00–2.00)
Memory/5, median (IQR)0.00 (0.00–2.00)0.00 (0.00–1.00)1.00 (0.00–2.00)3.00 (1.50–4.00)0.50 (0.00–2.25)
Orientation/6, median (IQR)5.00 (4.00–6.00)4.00 (2.00–5.00)5.00 (4.00–6.00)6.00 (6.00–6.00)6.00 (4.75–6.00)
Frontal lobe function
 FAB score, median (IQR)12.25 (9.00–15.00)9.00 (7.00–12.00)12.00 (10.00–15.00)15.00 (12.25–16.00)12.50 (10.00–15.00)
 FAB (<age and education-matched average)45/101 (44.6%)23/35 (65.7%)11/32 (34.4%)3/12 (25.0%)8/22 (36.4%)
  • MMSE, Mini-Mental State Examination; MoCA, Montreal Cognitive Assessment; NIID, neuronal intranuclear inclusion disease.