Table 1. Clinical Characteristics of 13 SQSTM1 Mutation Carriers.

		Age at Onset, y
Family/Patient	First Symptoms	First Symptoms	bvFTD	Clinical Symptoms of PDB	Clinical Symptoms of ALS	Disease Duration, y	Age at Last Evaluation, y	Additional Clinical Symptoms	Results of ENMG
F297/003	Behavioral disorders	63	63	Absent	Absent	7	70a	Oculomotor limitation, buccofacial apraxia, parkinsonism	Not performed
F297/005	Behavioral disorders	73	73	Absent	Absent	11	84a	Absent	Normal (at 79 y)
F297/016	Behavioral disorders	60	60	Absent	Absent	8	68a	Absent	Not performed
F297/018	Behavioral disorders	71	71	Absent	Absent	2	73	Absent	Not performed
F523/005	Behavioral disorders	48	48	Absent	Absent	10	58	Absent	Not performed
F523/003	Behavioral disorders	55	49	Absent	Absent	8	63	Absent	Not performed
F523/007	Behavioral disorders	49	49	Absent	Absent	4	53	Absent	Not performed
F480/010	Behavioral disorders, dysarthria	71	71	Absent	73	4	75a	Absent	Motor neuron disease (at 74 y)
F480/011	Behavioral disorders	67	67	Absent	Absent	3	70	Absent	Not performed
FR1324/010	PDB	51	59	51	Absent	14	65	Absent	Normal (at 63 y)
FR1324/005	PDB	55	79	58	Absent	29	84	Absent	Not performed
FR1324/006	PDB	<60	63	<60	Absent	NA	65a	Absent	Not performed
FR1324/003	Dementia	65	65	69	Absent	23	89	Absent	Not performed

Abbreviations: ALS, amyotrophic lateral sclerosis; bvFTD, behavioral variant of frontotemporal dementia; ENMG, electroneuromyography; PDB, Paget disease of bone.

^aAge at death (in years).