Table 2.

Imaging, clinical electrophysiology, treatment, and outcome

Patient	Brain MRI	EEG	EMG/NCS	Treatments	Outcome (duration of follow-up)
1	Bilateral increased T2 signal involving mesial temporal lobes, and mild cortical atrophy	Left frontal polar and bitemporal epileptiform discharges but no electrographic seizures	Normal motor and sensory responses (aside from median neuropathy at the wrist). No evidence of peripheral nerve hyperexcitability	Corticosteroids, rituximab	Improving cognition; mild residual memory deficits; seizures controlled with antiepileptics (6 months)
2	Normal	-	Normal motor and sensory nerve conduction studies. Needle EMG showed spontaneous motor unit discharges and fibrillation potentials, but no neuromyotonia. Repetitive stimulation was normal	Prednisone, cyclosporine, thymectomy, rituximab	All symptoms resolved. Mental status normal (5 years)
3	-	-	Decreased sensory and motor amplitudes, normal conduction velocities, fasciculations, 5–150 Hz spontaneous motor unit discharges, increased motor unit duration, amplitude, and polyphasic motor units.	carbamazepine, amitriptyline, pregabalin (all for peripheral nerve hyperexcitability).	Mild disability from continuing sensory- motor polyneuropathy and cramps (4 years).
4	Normal	Mild diffuse background slowing		Methylprednisolone , plasma exchange, IVIG, low-dose cyclophosphamide, rituximab	Markedly improved cognition, seizures controlled, bulbar weakness improved (6 months)
5	Age-appropriate atrophy	Normal	At presentation: myokymia, fasciculations, chronic and acute denervation. After one month of treatment: marked improvement of myokymia, no signs of denervation	Corticosteroids, plasma exchange	Cognitive symptoms persist (not oriented to time), improving muscle movements (12 months)
6	T2 increased intensities affecting left medial temporal lobe	Focal slowing and sharp waves over left temporal region		methylprednisolone	Recovered from seizures and memory deficits; mild mood instability (6 months)
7	At presentation: normal. At 2 years: diffuse cortical and subcortical atrophy	Right temporal sharp waves	At presentation: normal. At 5 years: normal NCS, diffuse fasciculations, normal motor unit morphology. At 7 years: normal sensory responses, decreased motor amplitudes, normal conduction velocities, wide-spread fasciculations, fibrillation potentials, and 5–150 Hz motor unit discharges	None	Lost to follow-up after 7 years of worsening symptoms.
8	Normal	-	-	IVIG (6 monthly cycles)	Normal (6 months)