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Table e-1: Atypical clinical history or syndromes in patients with IgG NMDAR antibodies
No Age, sex Clinical features Ancillary tests NMDAR-ab,
serum/CSF 1 35, F Opsoclonus-myoclonus and ataxia associated with
ovarian teratoma
CSF 9 WBCs. Brain MRI with increased T2 signal in the corpus callosum
- / +
2 49, F New onset depression, followed by opsoclonus and myoclonus predominantly involving trunk; truncal and limb ataxia, slurred speech. Recovered with steroids
CSF 82 WBCs. EEG and brain MRI normal - / +
3 70, M Apathy, depression, memory loss, confusion that transiently improved with steroids. Relapsed 1 month later: confusion, bradykinesia, hypersomnia. Partial improvement when retreated with steroids.
CSF 13 WBCs. EEG slow activity
Brain MRI with increased FLAIR signal in bilateral basal ganglia, insula and hippocampi with focal enhancement
- / +
4 33, F Headache, nausea, vomiting, hyperthermia followed by ataxia, opsoclonus, myoclonus. Suspected seizures that led to intubation and admission to ICU.
CSF 360 WBCs, brain MRI and EEG normal - / +, additional AMPAR-ab in CSF
5 53, M For 6 months progressive memory impairment with multiple episodes of confusion.
CSF 5 WBCs. EEG normal. Brain MRI showed increased FLAIR signal in medial temporal lobes
- / +
6 39, F Headache for 1 month, weight loss, episodes of cramps in right extremities, predominantly in the hand, visual and auditory hallucinations
CSF 8 WBCs. EEG normal. Brain CT normal. - / +
7 17, F Subacute gait instability, dysmetria, nystagmus, diplopia
CSF 564 WBCs. Brain MRI with mild increased signal in cerebellum without enhancement
- / +
10 8 17, M* Paroxysmal exercise-induced right foot weakness:
several weeks later, admitted for possible viral encephalitis (viral studies negative) with convulsive status epilepticus.
CSF 2 WBC, mildly elevated protein concentration + / +
9 35, F** During pregnancy, acute development of dysphagia, dysarthria, dyspnea, numbness in face, tongue, and right hand. Subsequently transient delirium, gaze evoked nystagmus, mild oral dyskinesias, sinus bradycardia. Underwent C-section followed by immunotherapy. Full clinical recovery.
CSF 13 WBCs, brain MRI normal, EEG without epileptic activity.
+ / +