Neuropathologic Classification of Neurodegenerative Disorders Associated with Frontotemporal Dementia (FTD)

McKhann et al reported neuropathologic findings from the international Work Group on Frontotemporal Dementia and Pick's Disease.

Patient selection: clinical course compatible with FTD and frontotemporal neuronal loss with gliosis

Parameters:

(1) tau-positive inclusions

(2) number of microtubule binding repeats in the insoluble tau

(3) ubiquitin positive inclusions

(4) motor neuron disease (MND) and/or motor neuron disease type inclusions

Tau Positive Inclusions	Insoluble Tau	Ubiquitin Positive Inclusions	Motor Neuron Disease	Differential Diagnosis
present	predominant 3 repeats	NA	NA	1, 2, 3
present	predominant 4 repeats	NA	NA	2, 3, 4, 5,
present	3 and 4 repeats (mixed)	NA	NA	2, 3, 6
absent	absent	present	MND	3, 7
absent	absent	present	MND-type inclusions without MND	3, 8
absent	absent	absent	NA	3, 9

Type	Diagnosis
1	Pick disease
2	frontotemporal dementia with parkinsonism linked to chromosome 17
3	unidentified familial or sporadic frontotemporal disorder
4	corticobasal degeneration
5	progressive supranuclear palsy
6	neurofibrillary tangle dementia
7	frontotemporal dementia with MND
8	frontotemporal dementia with MND-type inclusions but without MND
9	frontotemporal lobar degeneration (dementia lacking distinct histopathological features)

Caveats from the Work Group:

(1) Neuropathologic features of FTD cannot establish the presence of FTD in the absence of documented clinical information.

(2) There is no definite cause-and-effect relationship with the neuropathologic changes and the manifestations of dementia.

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