Table 1.

Summary of the demographic, clinical and neuroimaging information of the study subjects

Patient ref no.	Family 66			Family 97
	66-E	66-K	66-L	97-E
Gender/AAE (y)	Female/35	Male/28	Male/31	Female/30
AAO (y)	26	22	23	25
Initial symptoms	Depression/bradykinesia	Depression/tremor	Bradykinesia	Neuropsychiatric symptoms
Bradykinesia	+++	++	+++	++
Tremors	Yes	Yes	No	No
Postural instability	+++	++	+++	+++
Dystonia	No	No	No	No
Loss of ambulation	3 y after onset	ambulatory	3 y after onset	2 y after onset
Pyramidal signs	++	+	++	+
Cerebellar signs	No	No	No	No
Levodopa response	Yes -minimal	Yes -minimal	Yes -minimal	Yes -moderate
Levodopa-induced dyskinesia	++	–	++	–
Frontotemporal atrophy on MRI	++	+	++	++
Iron accumulation	No	No	No	No
PET scan	Frontotemporal lobe	Frontotemporal lobe	Not done	Frontotemporal lobe
Hoehn & Yahr stage	4.5	4	5	4
Neuropsychiatric symptoms	++	++	+	+++
Emotional Liability	+++	+	+	+++
Cognitive decline	+	++	+	+
Semantic disorder	+	+	+	–
Autonomic symptoms	Yes	Yes	Yes	No
Bladder disturbances	Urgency	Urge incontinence	Urgency	–
Sweating	Yes	Yes	No	–
Flushing	No	Yes	No	–
Sleep disorders	Yes	Yes	Yes	Yes
REM-sleep behavior disorder	Yes	No	No	–
Vivid dreaming	No	Yes	No	Yes
Sleep fragmentation	Yes	Yes	Yes	–
Death	Alive	Alive	Died after 8 y	Alive

AAE age at examination; y years; AAO age at onset; REM rapid eye movement; + mild; ++ moderate; +++ severe