Table 1.

Demographic features, non-speech bulbar and non-bulbar neurologic findings

Patient	Sex	Age at onset	Duration of symptoms at final follow-up (years)	Non-speech bulbar neurological findings	Non-speech non- bulbar neurological findings	EMG findings
1	F	87	2	lip and tongue weakness, pseudobulbar affect, mild periorbital weakness, occasional dysphagia		normal
2	M	69	5	reduced range, strength, and speed of lip and tongue movements, mild right facial droop, minor dysphagia	right UMN pattern weakness, Hoffman sign on right	normal
3	F	62	4	mild tongue weakness and slowness, subtle nonverbal oral apraxia, pseudobulbar affect	bilateral (right > left) upper extremity spasticity with slow AMRs	normal
4	M	57	1	pseudobulbar affect, drooling, dysphagia		normal
5	F	79	2	right lower face and tongue weakness, dysphagia	UMN limb weakness	normal
6	F	46	3.5	tongue weakness, brisk jaw jerk		normal
7	F	79	3	drooling, dysphagia, mild lip weakness	mild left upper extremity spasticity with slow AMRs	normal
8	F	63	1	mild lower facial weakness		normal
9	F	74	3	dysphagia, drooling, facial and tongue weakness, neck flexor weakness	spasticity left>right leg	normal
10	M	58	7	equivocal nonverbal oral apraxia	spastic paraparesis and left upper extremity spasticity, left Babinski; mild postural/kinetic tremor	evidence for longstanding motor neuronopathy or neuropathy without fasciculation or fibrillation potential
11	F	60	3	dysphagia, pseudobulbar affect, nonverbal oral apraxia	mildly brisk, symmetric reflexes and decreased limb AMRs	normal
12	M	46	1	dysphagia, tongue weakness and slowness, bifacial weakness	left side UMN type weakness, left Babinski	normal
13	F	72	1	dysphagia, pseudobulbar affect, mild tongue and lower facial weakness	brisk (non-pathologic) reflexes, mild slow limb AMRs	normal
14	F	66	5	dysphagia, drooling, minimal jaw opening, mild left facial weakness	left upper extremity spasticity with slow AMRs; 2–3 beat left ankle clonus;	normal
15	M	63	1	dysphagia, pseudobulbar affect, tongue and facial weakness	mild left upper extremity UMN weakness	normal
16	M	48	2	minor dysphagia, drooling, brisk jaw, jerk, jaw clonus, pseudobulbar affect, tongue weakness		normal
17	F	56	1	minor dysphagia, tongue and lip weakness, positive suck and snout reflexes		normal
18	M	71	2	pseudobulbar affect, nonverbal oral apraxia		normal
19	F	50	2	dysphagia, pseudobulbar affect, tongue weakness, brisk jaw jerk reflex	mild hyperreflexia in upper and lower extremities (not pathologic)	normal
20	F	63	2	dysphagia, pseudobulbar affect, jaw clonus, reduced tongue strength and speed	left upper and lower extremity spasticity with slow AMRs; spastic/apraxic gait	normal
21	F	66	1	dysphagia, subtle jaw clonus, mild tongue weakness, brisk jaw jerk reflex	mild hyperreflexia in upper and lower extremities (not pathologic)	normal
22	M	56	6	drooling, minor dysphagia, pseudobulbar affect, jaw clonus	left UMN pattern weakness, bilateral limb spasticity (left > right), spastic gait circumducting left leg; subtle left Babinski	n/a
23	M	62	1.5	dysphagia, pseudobulbar affect, facial, tongue, and velar weakness	frontal release signs, hyper-reflexia, reduced right arm swing, unsustained clonus at both ankles	n/a
24	F	55	1	minor dysphagia, jaw and tongue weakness		normal
25	F	70	1	dysphagia, pseudobulbar affect, tongue and palatal weakness		normal

n/a: not administered