0000000000541052
AUTHOR
Godbolt A. K
Sporadic and familial dementia with Ubiqitin Positive Tau negative inclusions: Clinical features of one histopathological abnormality underlying frontotemporal lobar degeneration
BACKGROUND: Frontotemporal lobar degeneration comprises a group of diseases with clinical presentations and underlying histopathologies that overlap. Familial disease occurs in up to 50% of frontotemporal lobar degeneration cases. One of several underlying histopathological abnormalities is of ubiquitin-positive tau-negative inclusions, similar to those in motor neuron disease. OBJECTIVE: To compare clinical features of familial and sporadic cases in this pathological subgroup. DESIGN AND PATIENTS: Case note review of dementia patients with ubiquitin-positive tau-negative inclusion pathological abnormalities proven by autopsy. SETTING: United Kingdom tertiary referral center. MAIN OUTCOME M…
Survival in Alzheimer disease
We thank Dr Lesser for his comments. We agree that deaths in older populations with AD are often from comorbid conditions, and we would not wish to overinterpret the limited survival data from this study. We also accept that determining symptom onset is an inexact science. However, we would suggest that in this small cohort the disease course of about a decade cannot be considered an aggressive one. Since publication of our study, 2 additional subjects have died 8.9 and 16.8 years after onset thereby increasing the mean time from onset to death to 9.4 years. We agree, however, that further study is required to resolve these issues and in particular to assess genetic factors that may influen…