Frontotemporal Lobar Degeneration (FTLD) encompasses several overlapping disorders.
Categories:
(1) frontotemporal dementia and Parkinsonism linked to chromosome 17 (FTDP-17)
(2) Pick's disease
(3) frontotemporal lobar degeneration with ubiquitin-positive inclusions and without motor neuron disease (FTLD-U)
(4) frontotemporal lobar degeneration with ubiquitin-positive inclusions and with motor neuron disease (FTLD-MND)
(5) frontotemporal lobar degeneration without ubiquitin-positive deposits and without distinctive histology (DLDH = dementia lacking distinctive histology)
|
Category |
Immunostaining |
Key Feature |
|
FTDP-17 |
tau positive |
parkinsonism, mutation chromosome 17, may have Pick-like bodies |
|
Pick's disease |
tau positive, phosphorylated neurofilament positive |
Pick bodies |
|
FTLD-U |
tau negative, ubiquitin positive inclusions |
absence of motor neuron disease |
|
FTLD-MND |
tau negative, ubiquitin positive inclusions |
presence of motor neuron disease |
|
DLDH |
tau negative, ubiquitin negative |
absence of distinctive histologic features |
where:
• The inclusions in FTLD-U and FTLD-MND are located in the nucleus and/or cytoplasm of neurons in the superficial cortical and dentate granular cell layers.
Specialty: Neurology
ICD-10: ,
