000331422_sm_Appendix.PDF (71.32 kB)
Supplementary Material for: Adult-Onset Leukoencephalopathy with Axonal Spheroids and Pigmented Glia Can Present as Frontotemporal Dementia Syndrome
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posted on 2011-10-05, 00:00 authored by Wong J.C., Chow T.W., Hazrati L.-N.Background/Aims: We review the characteristics of adult-onset leukoencephalopathy with axonal spheroids and pigmented glia(ALSP) and determine prevalence of behavioral variant of frontotemporal dementia (bvFTD) features in ALSP. Methods: Clinical and pathological information was abstracted from histopathologically confirmed ALSP cases identified by a systematic literature search. A new case of ALSP presenting as bvFTD was also described. Results: We retrieved 51 ALSP cases. Mean age of onset was 42.2 years. Mean disease duration was 6.2 years, with 24 cases lasting 4 years or fewer. Fourteen cases had 3 or more of the 6 key bvFTD features. White matter hyperintensities on T2-weighted MRI, motor symptoms, seizures and amnesia were common. Conclusion: ALSP can underlie FTD syndrome, as well as rapidly progressive dementia.