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Images in Neurology |

Rapidly Cycling Auras and Episodic Focal Dystonia in Anti-LGI1 Autoimmune Encephalitis

Tadeu A. Fantaneanu, MD1; Shamik Bhattacharyya, MD1; Tracey A. Milligan, MD1; Page B. Pennell, MD1
[+] Author Affiliations
1Department of Neurology, Brigham and Women’s Hospital, Boston, Massachusetts
JAMA Neurol. 2016;73(9):1150. doi:10.1001/jamaneurol.2016.1085.
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This case report describes a 57-year-old man with hemochromatosis and panhypopituitarism who presented with a 6-month history of word-finding difficulty, short-term memory loss, and rapidly cycling symptoms.

A 57-year-old man with hemochromatosis and panhypopituitarism presented with a 6-month history of word-finding difficulty, short-term memory loss, and rapidly cycling symptoms consisting of a rising abdominal sensation into the chest and head associated with an electric-like “jolt” and speech difficulty lasting 4 to 8 seconds. Occasional, brief right-arm movements were observed by his family (Video). He reported 50 to 70 episodes daily. He was referred to the psychiatry and neurology departments at our hospital, and a video electroencephalogram (EEG) was ordered but did not reveal EEG changes with his events. He was referred for a second opinion to the neurology department, where, again, his episodes were captured without an EEG correlate. Magnetic resonance imaging of his brain, the results of which were initially interpreted as normal, revealed hyperintense mesial temporal lobes and basal ganglia (Figure, A and B), further confirmed by hypermetabolism on subsequent positron emission tomographic scans (Figure, C and D). Cerebrospinal fluid samples were confirmatory for anti–voltage-gated potassium channel (LGI1 subtype, 0.10 nmol/L) antibodies, and he underwent immunosuppressive therapy, which consisted of intravenous immunoglobulin (0.4 g/kg/d, 5-day course), high-dose intravenous methylprednisolone sodium succinate (1000 mg/d, 3-day course), and an infusion of rituximab (1000 mg, 1 injection); levetiracetam and gabapentin were also added to his regimen, with resolution of his rapid auras and focal dystonia. Whole-body positron emission tomography and ultrasonography of the testes did not reveal a neoplastic source.

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Neuroimaging Scans

Axial magnetic resonance imaging (MRI) fluid-attenuated inversion recovery scans reveal T2-weighted hyperintensities in the bilateral mesial temporal lobes (A) and the basal ganglia (B). Fluorodeoxyglucose F 18–positron emission tomographic (FDG-PET) scans reveal hypermetabolism in the left mesial temporal lobe (C) and the basal ganglia (D).

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Episodic Dystonia

Video EEG recording showing a typical event consisting of a rapid dystonia involving the right arm and associated with the patient’s described cycling auras.

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