Table 1.

Evaluation of DM1 neuropathology based on density and regional distribution of tau‐immunopositive neurons. Neuron density was scored as: none (−), low degree (+), moderate degree (++) and high degree (+++) (see Figure 2).

	Three‐repeat tau		Four‐repeat tau
	Case 1	Case 2	Case 1	Case 2
CA1	(+)	(+)	(+)	(++)
Entorhinal cortex	(+++)	(+)	(++)	(+++)
Amygdala	(+++)	(+)	(+)	(++)
Frontal lobe cortex	(+)	(−)	(−)	(+)
Temporal lobe cortex	(+)	(−)	(−)	(+)
Parietal lobe cortex	(−)	(−)	(−)	(+)
Basal ganglia	(+)	(−)	(−)	(+)
Subthalamic nucleus	(++)	(−)	(−)	(+)
Oculomotor nucleus	(+++)	(−)	(−)	(++)
Substantia nigra	(++)	(−)	(−)	(+)
Dorsal vagal nucleus	(+)	(−)	(−)	(+)