Table 1.

Histologic 43 kDa transactive responsive sequence DNA binding protein pathology findings in patients with progressive muscular atrophy (Cases #1 and 2) and motor neuron disease isolated to the lower motor neuron (Cases #3–6)

(a) Spinal cord, brainstem, subcortex
Patient #	Spi				Med			Pon	Mid		Bas		Tha	Cer
	Cer	Tho	Lum	Sac	Dor	Inf	Oth		Sub	Oth	Str	Len		Gre	Whi	Den
1							*		*
2													*
3						*							*
4
5					*
6								*

(b) Cortex
Patient #	Mot		Sen		Vis		Fro		Ang		Sup		Orb
	Gre	Whi	Gre	Whi	Gre	Whi	Gre	Whi	Gre	Whi	Gre	Whi	Gre	Whi
1
2
3
4									*
5
6

Patient #	Cin		Tra		Hip		Amy	Per
	Gre	Whi	Gre	Whi	CA1	Fas		Gre	Whi
1
2
3									**
4
5
6

None	Rare	Mild	Severe	Severe

Not done

Spi spinal cord, Cer cervical, Tho thoracic, Lum lumbar, Sac sacral, Med medulla, Dor dorsal motor plate, Inf inferior olive, Oth other, Pon pons, Mid Midbrain, Sub subiculum, Bas basalganglia, Str striatum, Len lentiform, Tha thalamus, Cer cerebellum, Gre gray matter, Whi white matter, Den denate nucleus, Mo motor gyrus, Sen sensory cortex, Vis visual cortex, Fro frontal Gyrus, Ang angular gyrus, Sup superior-midtemporal gyurs, Orb orbitofrontal gyrus, Cin cingulate gyrus, Tra (trans-)entorhinal region, Hip hippocampus, CA1 CA1-subiculum, Fas Fascia dentata, Amy amygdala, Per Periamygdala

^*Focal,

^**perivascular