Frontotemporal lobar degeneration (FTLD) is a progressive neurodegenerative
disorder that involves the frontal and anterior temporal lobes. It is
characterized by prominent behavioral and language disorders. There are three
variants of FTLD: frontotemporal dementia (FTD), semantic dementia (SD) and
progressive non-fluent aphasia (PNFA). FTLD may be mistaken for AD (Alzheimer’s
disease) or psychiatric disorders especially in the early stages.ObjectivesTo describe the demographic characteristics of patients with FTLD, assessed
at the Behavioral and Cognitive Neurology Unit, and to show that FTLD is
commonly clinically under-diagnosed.MethodsAll patients diagnosed with FTLD (Consensus Criteria for FTLD), and who were
seen at the Behavioral and Cognitive Neurology Unit of Hospital das
Clínicas, in São Paulo, Brazil from January 2004 to August
2007 were included in the analyses.ResultsSixteen patients with FTLD (11 women) were included in this study. There were
12 patients with FTD, two with PNFA and two with SD. The mean duration of
the illness until diagnosis of FTLD was 4.1±2.3 years, ranging from
one to seven years. Nine patients had been initially seen by psychiatrists,
five by neurologists, and two by general physicians. The first diagnosis was
psychiatric disorder in six patients, AD in four, dementia in two, FTD in
two, and stroke and hydrocephalus for one patient each.ConclusionThe diagnosis of FTLD can be difficult and many patients may be misdiagnosed
in Brazil, especially in the initial stages. Educational programs on FTLD
for the medical community are warranted.