de novo MAPT mutation G335A causes severe brain atrophy, 3R and 4R PHF-tau pathology and early onset frontotemporal dementia

Table 1 Semi-quantitative assessment and regional distribution of tau pathology on brain sections immunolabelled with AT8. -: absent; +: occasional; ++: moderate number; +++: frequent number. NFT, neurofibrillary tangles

	Tau pathology
Brain areas	NFT	Neuronal diffuse	Oligodendroglial	Astrocytic
Hippocampal pyramidal layer	+++	+++	++	+
Dentate gyrus	–	+	–	–
Subiculum	++	++	+	+
Transentorhinal cortex	+	+	+	+
Temporal cortex	++	++	++	+
Frontal cortex	++	+	++	+
Cingulate cortex	+	+	+	+
Visual cortex	–	–	–	–
Striatum	++	+	+++	+
Globus pallidus	++	+	+	+
Thalamus	++	+	+	+
Substantia nigra	+++	+	+	+
Pontine nuclei	++	+	+	+
Olivary Nuclei	–	+	–	–

ISSN: 2051-5960