Table 1.

Clinical Features, CSF, EEG, and MRI Findings

Case*	Sex/age	Symptom presentation	CSF	Initial EEG	Initial Brain MRI (FLAIR)	GluR1/2 antibodies (main antigen)
14	F/65	Initial episode and relapses: short-term memory loss, confusion, agitation, aggressive behavior, confabulation. Transient downbeat nystagmus.	30 WBC, protein 97, positive OB and ISAb	Normal, repeated twice	Mild increased signal in medial temporal lobes.	GluR1/2 positive (GluR1)
24	F/44	Initial episode and relapses: short-term memory loss; confusion, combativeness. Focal motor seizures. Right beating nystagmus.	44 WBC, protein 91, glucose 70, no OB, positive ISAb	Diffuse theta activity; episodes of epileptic activity in left temporal lobe.	Initial MRI normal. Followup 5 days later: mild temporal lobe increased signal (right> left).	GluR1/2 positive (GluR2)
34	M/38	Initial episode and relapse: short-term memory loss, confusion, agitation, perseveration generalized tonic-clonic seizures	7 WBC, protein 50, positive OB and ISAb,	NA	Increased signal in the right medial and lateral temporal lobe, right frontal, left insular and left occipital regions.	GluR1/2 positive (GluR2)
4	F/64	Increased seizures, confusion, disorientation, lethargy, short-term memory loss.	75 WBC, protein 79	Slow activity in the right temporal region. No epileptic activity.	Post-operative changes in the right temporal lobe. New increased signal in the left medial temporal lobe.	GluR1/2 positive (GluR2)
55	F/44	Confusion, behavioral change, hypersomnia, progressive unresponsiveness. Mild gait unsteadiness, low grade fever.	15 WBC, normal protein	NA	NA** CT normal	GluR1/2 positive (GluR2>R1)
6	F/38	Short-term memory loss, confabulation	6 WBC, normal protein	Normal	Increased signal in medial temporal lobes, left septal nucleus, left cerebellum.	GluR1/2 positive (GluR2)
7	F/87	Initial episode: short-term memory loss, disorientation. At relapse: memory loss, generalized tonic-clonic seizures	Initially normal; protein 50 at relapse	Diffuse slow activity (7-8 c/sec), delta activity in anterior frontotemporal areas	Increased signal in medial temporal lobes; mild transient contrast enhancement in the left hippocampus.	GluR1/2 positive (GluR1)
8	F/61	Initial episode and relapse: Short-term memory loss, decreased level of consciousness.	24 WBC, protein 420	Theta activity in posterior temporal regions	Normal MRI	GluR1/2 positive (GluR2)
9	F/59	Progressive memory loss, behavioral change, agitation, confabulation (4 months). Mild dysdiadochokinesia	17 WBC, protein 51, positive OB	Bilateral sharp waves in temporal lobes; no seizures	Increased signal in medial temporal lobes, and medial orbitofrontal region.	GluR1/2 positive (GluR1)
10	F/67	Confusion, combativeness, insomnia, hallucinations, short term memory loss	32 WBC, normal protein, no OCB	Sharp waves in temporal lobes	Mild temporal lobe increased signal.	GluR1/2 positive (GluR2)

OB: oligoclonal bands; ISAb: Intrathecal synthesis of antibodies; CSF normal values: WBC <4/μl; Protein 16-46 mg/dL. All patients had normal glucose levels in the CSF; ND: not done. NA: not available.

^*The initial clinical features of patients #1, 2 and 3, were described in detail in⁴ (cases 2,5 and 6); the long term follow-up is provided here. Patient #5 corresponds to case 4 of Tables 1 and 2 in⁵

^**Limbic encephalitis confirmed at autopsy.