Right temporal variant frontotemporal dementia is pathologically heterogeneous: a case-series and a systematic review

Table 3 Pathological features of rtvFTD cases

	Case 1	Case 2	Case 3	Case 4	Case 5
Macroscopic analysis
Brain weight	1117 gr	1410 gr	1260 gr	1010 gr	975 gr
Atrophy	FT-Right	FT	FT-Right	FT	T
Substantia nigra	Normally pigmented	Normally pigmented	Slightly pale	Pale	Slightly pale
Locus coeruleus	Visible	Visible	Right < Left	Not visible	Visible
Atherosclerosis	No	Moderate	Severe	Mild	No
Microscopic analysis
Plaque and tangles	Negative	Negative	Thal 3	Negative	Negative
Congo red	Negative	Negative	Negative	Negative	Negative
Alpha synuclein	Negative	Negative	Negative	Braak 3	Negative
Tau	Negative	Negative	Positive	Negative	Negative
Pick Bodies	No	No	No	No	No
TDP-43	Positive	Positive	Negative	Negative	Positive
FUS	Negative	Negative	Negative	Positive	Negative
Accumulation	All layers	Predominantly layer 2	3R + 4R	FUS	Several long threads
Frontal	+ +	+ +	+ + +	+ + +	+
Temporal	+ + +	+ + +	+ + + *	+ + +	+ + +
Motor cortex	+ + +	–	–	n/a	–
Corticospinal tract	+ + +	–	–	–	–
Parietal	–	–	+ +	n/a	+
Occipital	–	–	+	–	–
Hippocampus	+ + +	+ + +	+ + +	+ + +	+ + +
Amygdala	+ + +	+ + +	+ + +	+ + +	+ + +
Caudate, putamen	–	+ +	+ + +	+ + +	+ +
Thalamus	–	+ +	+ +	+ + +	+ +
Brain stem	–	–	+ + +	+ + +	–
Cerebellum	–	–	–	–	–
Cervical cord	+ + +	–	–	–	–
Diagnosis	FTLD-TDP type B + MND	FTLD-TDP type E	FTLD-MAPT	FTLD-FUS	FTLD-TDP type C

rtvFTD: Right temporal variant frontotemporal dementia, FTLD: Frontotemporal lobar degeneration, MAPT: Microtubule associated protein tau, TDP-43: TAR DNA-binding protein 43, n/a: not available, F: Frontal, T: Temporal, R: Repeat
*Extensive tau positivity indicates a primary tauopathy. Pathological results are suggestive for tau mutation
+++: Severe, ++: Moderate, +: Mild, –: Normal

ISSN: 2051-5960