Eyelid Tremor in a Patient with Anti-Caspr2 Antibody-Related Encephalitis

Abstract

We describe the first case of a patient with eyelid tremor probably associated with anti-contactin-associated protein-like 2 (Caspr2) antibody. Encephalitis associated with anti-voltage-gated potassium channel antibody is now attributed to autoantibodies against leucine-rich glioma inactivated 1 (Lgi1) and less frequently against Caspr2. Eyelid tremor or blepharoclonus is a rare or underdiagnosed involuntary movement that has been found in patients with infarction in the thalamus or drug-induced or idiopathic parkinsonism. Since patients with anti-Caspr2 antibody-related encephalitis occasionally have extrapyramidal signs, we speculate that the eyelid tremor was also caused by anti-Caspr2 antibody in our patient. Partial resolution of his symptoms by plasmapheresis also supported the involvement in immunological processes.

Case Report: Prominent Brainstem Involvement in Two Patients With Anti-CASPR2 Antibody-Associated Autoimmune Encephalitis

“…Besides, peripheral nerve hyperexcitability and Morvan’s syndrome are also reported with clinical manifestations of neuropathic pain, neuromyotonia, or autonomic dysfunction ( 3 , 4 ). In recent years, the clinical spectrum of anti-CASPR2 antibody-associated disorders has been extensively expanded with the discovery of novel manifestations including parkinsonism ( 5 ), non-paraneoplastic cerebellar ataxia ( 4 , 6 , 7 ), Guillain–Barré syndrome ( 8 ), chorea ( 9 ), neuropathic pain ( 10 ), Creutzfeldt–Jakob disease ( 11 ), amyotrophic lateral sclerosis with frontotemporal dementia syndrome ( 12 ), orthostatic myoclonus ( 13 ), and eyelid tremor ( 14 ). In this study, we report for the first time two cases of anti-CASPR2 antibody-associated autoimmune encephalitis with prominent brainstem involvement, which may further expand the clinical profile of this rare autoimmune disease.…”

Eyelid Nystagmus and Other Involuntary Movements of the Upper Lids; What's in a Name?

“…Eyelid tremor has been infrequently reported. 8 , 9 Jungehülsing and Ploner 8 described a patient with a right paramedian thalamic infarct and rhythmic jerks of the lids with a frequency of 7 Hz on voluntary eye closure and abolished with forced eye closure. This was termed “tremor” by the authors, although no video was provided with the publication.…”

“…This was termed “tremor” by the authors, although no video was provided with the publication. Ueno and colleagues 9 reported a patient with anti‐Caspr2 antibody‐related encephalitis presenting with parkinsonism and “eyelid tremor” at a frequency of 1–2 Hz. However, the accompanying video appears to show repetitive semi‐rhythmic blinking rather than typical tremor, perhaps better classified as a type of blepharoclonus.…”