Table 1.

Demographic data

Female gender	116 (49.4)
Caucasian race	212 (90.2), unknown n = 12
Education, years	13.9±2.4, unknown n = 4
Family history of neurological illness	60 (26), adopted n = 5
Age at onset, years	34.7±7.9
Age at presentation, years	36.7±7.8
Years from onset to presentation	2.0±2.2
Short test of mental status score at presentation (maximum 38)	26.4±8.1 (n = 155, range 3–38), unable n = 48, not recorded n = 32
Onset of cognitive decline
Gradual	181 (77.0)
Subacute	47 (20.0)
Acute	7 (3.0)
Course of cognitive decline
Progressive	217 (92.3), rapid n = 22 (9.4)
Relapsing-progressive	14 (6.0)
Relapsing-remitting	4 (1.7)
Etiologies
Neurodegenerative	73 (31)
Autoimmune/inflammatory	50 (21)
Vascular	14 (6)
Metabolic	25 (11)
Infectious	11 (5)
Other	18 (8)
Unknown	44 (19)

Figures in parentheses are percentages.