Table 4

Prevalence of neuropsychiatric symptoms in patients with NIID

NPI symptomsTotalClinical phenotype
Dementia-dominant typeMovement disorder-dominant typeMuscle weakness-dominant typeParoxysmal symptom-dominant type
NPI score, median (IQR)3.00 (0.00–11.00)4.00 (1.00–20.00)5.00 (1.00–13.00)0.00 (0.00–2.00)4.00 (0.00–7.00)
NPI (>=1)86/121 (71.1%)34/45 (75.6%)29/37 (78.4%)5/13 (38.5%)18/26 (69.2%)
Delusion19/121 (15.7%)9/45 (20.0%)4/37 (10.8%)1/13 (7.7%)5/26 (19.2%)
Hallucinations20/121 (16.5%)11/45 (24.4%)3/37 (8.1%)1/13 (7.7%)5/26 (19.2%)
Agitation/aggression21/121 (17.4%)10/45 (22.2%)6/37 (16.2%)1/13 (7.7%)4/26 (15.4%)
Depression/dysphoria36/121 (29.8%)18/45 (40.0%)12/37 (32.4%)0/13 (0.0%)6/26 (23.1%)
Anxiety45/121 (37.2%)15/45 (33.3%)18/37 (48.6%)2/13 (15.4%)10/26 (38.5%)
Euphoria16/121 (13.2%)7/45 (15.6%)5/37 (13.5%)0/13 (0.0%)4/26 (15.4%)
Apathy33/121 (27.3%)18/45 (40.0%)10/37 (27.0%)1/13 (7.7%)4/26 (15.4%)
Disinhibition22/121 (18.2%)11/45 (24.4%)5/37 (13.5%)0/13 (0.0%)6/26 (23.1%)
Irritability/liability47/121 (38.8%)18/45 (40.0%)15/37 (40.5%)3/13 (23.1%)11/26 (42.3%)
Aberrant motor behaviour18/121 (14.9%)10/45 (22.2%)7/37 (18.9%)0/13 (0.0%)1/26 (3.8%)
Nighttime disturbance24/121 (19.8%)12.45 (26.7%)10/37 (27.0%)0/13 (0.0%)2/26 (7.7%)
Eating disturbance24/121 (19.8%)11/45 (24.4%)7/37 (18.9%)3/13 (23.1%)3/26 (11.5%)
  • NIID, neuronal intranuclear inclusion disease; NPI, Neuropsychiatric Inventory.