A 45-year-old lady presented with 3 weeks history of very brief episodic involuntary posturing on initiating movements. The episodes consisted of mild neck flexion with outstretching of both upper limbs lasting 1–2 s. She was fully conscious and there were no facial movements. These occurred exclusively on movement and had several episodes per day. They were absent in sleep. She also had short-lasting episodes of goosebumps all over body. She was fully conscious during these episodes of goosebumps which use to last 10-20 seconds. Clinical examination revealed episodic piloerection and mild episodic memory decline. A video EEG showed paroxysmal Kinesigenic myoclonus [Video 1]. Interictal EEG was normal and ictal EEG showed EMG artifacts only. MRI brain showed limbic encephalitis [Figure 1]. Serum and CSF anti LGI1 antibody was positive. She was treated with pulse methylprednisolone followed by oral prednisolone, azathioprine and oxcarbazepine. She responded in 3 weeks, both piloerection and involuntary movements disappeared. Medications were tapered off after 1 year and she is in remission at 2-year follow-up.
Figure 1.
MRI Brain FLAIR axial image showing limbic encephalitis
Prototypical paroxysmal dyskinesia in LGI1 encephalitis is facio brachial dystonic seizure (FBDS).[1] Paroxysmal kinesogenic dyskinesia has been rarely described in anti-LGI1 encephalitis and other autoimmune encephalitis.[1,2] Pilomotor seizures are unusual autonomic seizures usually associated with temporal lobe epilepsy (TLE). Rarely frontal and hypothalamic origin also have been reported. Unilateral piloerection may indicate an ipsilateral focus, whereas bilateral piloerection does not have any clear lateralizing value.[3] Association of Piloerection to autoimmune encephalitis is recently recognized, anti-LGI1 encephalitis being the commonest.[1,4]
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