Table 1

Randomised controlled trials of pharmacological interventions in FTD spectrum disorders

Study	Condition studied	Cohort size	Trial design	Intervention	Control	Length	Outcome of interest
Leclair-Visonneau et al 38	PSP syndrome	28	DB, PG, MC	Sodium valproate 1500 mg/day	Placebo	24 months	Symptom progression
Nuebling et al 39	PSP syndrome	44	DB, PG, SC	Rasagiline 1 mg/day	Placebo	12 months	Symptom progression
Apetauerova et al 40	PSP syndrome	61	DB, PG, MC	Coenzyme Q10 2400 mg/day	Placebo	12 months	Safety and efficacy on disease progression
Pardini et al 41	BvFTD	26	SB, CX, SC	Souvenaid 125 mL/day	Placebo	12 weeks	Frontal lobe function
Hughes et al 42	BvFTD	12	DB, CX, SC	Citalopram Single-dose 30 mg	Placebo	2 sessions*	Frontal lobe function
Finger et al 43	BvFTD and SD	23	DB, PG, SC	Oxytocin 24, 48, 72 IU Twice daily	Placebo	1 week	Safety and tolerability, symptom progression
Tolosa et al 44	PSP syndrome	146	DB, PG, MC	Tideglusib 600 or 800 mg/day	Placebo	52 weeks	Safety and disease progression
Höglinger et al 45†	PSP syndrome	37	DB, PG, MC	Tideglusib 600 or 800 mg/day	Placebo	52 weeks	Disease progression on brain imaging
Boxer et al 46	PSP syndrome	313	DB, PG, MC	Davunetide 30 mg twice daily	Placebo	52 weeks	Safety and efficacy on disease progression
Boxer et al 47	BvFTD and SD	81	DB, PG, MC	Memantine 10 mg twice daily	Placebo	26 weeks	Symptom progression
Jesso et al 48	BvFTD	20	DB, CX, SC	Oxytocin Single-dose 24 IU	Placebo	1 week	Emotion recognition
Vercelletto et al 49	BvFTD	52	DB, PG, MC	Memantine 10 mg twice daily	Placebo	52 weeks	Symptom progression
Bensimon et al 50	PSP syndrome and MSA	767 (363 with PSP)	DB, PG, MC	Riluzole 50–200 mg/day	Placebo	35 months	Survival and disease progression
Stamelou et al 51	PSP syndrome	21	DB, PG, SC	Coenzyme Q10 5 mg/kg/day	Placebo	6 weeks	Symptom progression and energy metabolite on MRS
Kertesz et al 52	BvFTD and PPA	36	OL and DB, PG, SC	Galantamine 16–24 mg/day	Placebo	OL: 18 weeks DB: 8 weeks	Symptom progression
Rahman et al 53	BvFTD	8	DB, CX, SC	Methylphenidate Single-dose 40 mg	Placebo	2 sessions*	Frontal lobe function
Deakin et al 54	BvFTD	10	DB, CX, SC	Paroxetine 40 mg/day	Placebo	7 weeks	Frontal lobe function
Moretti et al 55	FTD	40	OL, PG, SC	Rivastigmine 3–9 mg/day	Standard treatment	12 months	Symptom progression
Lebert et al 56	BvFTD	31	DB, CX, MC	Trazodone 300 mg/day	Placebo	6 weeks	Symptom progression
Moretti et al 57	FTD	16	OL, PG, SC	Paroxetine 20 mg/day	Piracetam 1200 mg/day	14 months	Symptom progression
Litvan et al 58	PSP syndrome	21	DB, CX, SC	Donepezil 10 mg/day	Placebo	6 weeks	Symptom progression
Frattali et al 59	PSP syndrome	6	DB, CX, SC	Physostigmine 0.5–2 mg Every 2 hours	Placebo	3–4 days	Oral motor functions
Rascol et al 60	PSP syndrome	14	DB, CX, MC	Efaroxan 2 mg Three times a day	Placebo	6 weeks	Motor symptom progression

*Studies involved administration of a single dose of the investigational drug or placebo followed by same-day cognitive assessment.
†This was a substudy of the above study.
BvFTD, behavioural variant frontotemporal dementia;CX, crossover;DB, double-blind;FTD, frontotemporal dementia;MC, multicentre;MRS, magnetic resonance spectroscopy;MSA, multisystem atrophy;OL, open-label;PG, parallel-group;PPA, primary progressive aphasia;PSP, progressive supranuclear palsy;SB, single-blind;SC, single-centre; SD, semantic dementia.