Table 1.

Summary of TDP-43 and Tau Pathologic Findings

	Case 1	Case 2
pTDP-43 distribution	MND: Primary motor cortex is most involved; sparse pathology in spinal cord; no pathology in brainstem FTLD: Temporal >> frontal cortex; layer 2 predominance; minimal white matter pathology; minimal neostriatum pathology	MND: Primary motor cortex, brainstem, and spinal are all moderately involved FTLD: Temporal > frontal cortex; all cortical layers; prominent white matter pathology; moderate brainstem pathology; variable neostriatum pathology (globus pallidus > caudate > putamen)
pTDP-43 morphology	Dense perinuclear donut-like morphology encompassing the majority of the cytoplasm with centrally placed nuclei Scattered short neurites No intranuclear inclusions Skein-like inclusions in motor neurons Rare glial inclusions	Granular cytoplasmic neuronal staining Granular parenchymal staining but minimal neurites Dense Lewy-body like inclusions in pigmented nigral neurons No intranuclear inclusions Numerous glial inclusions
FTLD-type	FTLD-TDP-43 Type A/B	FTLD-TDP-43 Type B or Type E
ADNC	Low (Thal 2, Braak precluded, CERAD absent)	Low (Thal 2, Braak IV, CERAD absent)
Lewy body pathology	Not identified	Not identified
Tau pathology	Features of argyrophilic grain disease - Oligodendroglial coiled bodies - Grains - Perinuclear ptau accentuation in CA2 hippocampal neurons

Abbreviations: ADNC = Alzheimer disease neuropathologic change; FTLD = frontotemporal lobar degeneration; MND = motor neuron disease.