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. Author manuscript; available in PMC: 2009 Oct 14.
Published in final edited form as: J Neuropathol Exp Neurol. 2008 Jul;67(7):635–648. doi: 10.1097/NEN.0b013e31817d751c

TABLE 2.

Clinicopathologic Correlations in 12 Patients

Group n Clinical Diagnoses Family History Sex Average Age at Onset, years Average Duration, years NIIs
1 4 2 FTDbv +/+ M/M 56.5 5.0 +/+
2 PPA +/- M/F +/+
2 5 3 FTDbv +/+/+ M/M/F 65.4 7.6 +/+/-
1 PPA - F +
1 “dementia” Unknown M +
3 3 All FTD with ALS +/-/+ M/M/F 62 3.3 +/-/-

Group 1, pathologic FTLD-U with PGRN mutations; Group 2, pathologic FTLD-U without PGRN mutations; Group 3, pathologic FTLD-MND without PGRN mutations.

ALS, amyotrophic lateral sclerosis; F, female; FTDbv, FTD behavioral variant; FTLD, frontotemporal lobar degeneration; FTLD-U, FTLD with ubiquitinated inclusions; M, male; MND, motor neuron disease; NIIs, neuronal intranuclear inclusions; PGRN, progranulin; PPA, primary progressive aphasia.