Utility of an immunotherapy trial in evaluating patients with presumed autoimmune epilepsy.

OBJECTIVE: To evaluate a trial of immunotherapy as an aid to diagnosis in suspected autoimmune epilepsy. METHOD: We reviewed the charts of 110 patients seen at our autoimmune neurology clinic with seizures as a chief complaint. Twenty-nine patients met the following inclusion criteria: (1) autoimmune epilepsy suspected based on the presence of ≥ 1 neural autoantibody (n = 23), personal or family history or physical stigmata of autoimmunity, and frequent or medically intractable seizures; and (2) initiated a 6- to 12-week trial of IV methylprednisolone (IVMP), IV immune globulin (IVIg), or both. Patients were defined as responders if there was a 50% or greater reduction in seizure frequency. RESULTS: Eighteen patients (62%) responded, of whom 10 (34%) became seizure-free; 52% improved with the first agent. Of those receiving a second agent after not responding to the first, 43% improved. A favorable response correlated with shorter interval between symptom onset and treatment initiation (median 9.5 vs 22 months; p = 0.048). Responders included 14/16 (87.5%) patients with antibodies to plasma membrane antigens, 2/6 (33%) patients seropositive for glutamic acid decarboxylase 65 antibodies, and 2/6 (33%) patients without detectable antibodies. Of 13 responders followed for more than 6 months after initiating long-term oral immunosuppression, response was sustained in 11 (85%). CONCLUSIONS: These retrospective findings justify consideration of a trial of immunotherapy in patients with suspected autoimmune epilepsy. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that in patients with suspected autoimmune epilepsy, IVMP, IVIg, or both improve seizure control.

MRI findings in autoimmune voltage-gated potassium channel complex encephalitis with seizures: one potential etiology for mesial temporal sclerosis.

BACKGROUND AND PURPOSE: Autoimmune voltage-gated potassium channel complex encephalitis is a common form of autoimmune encephalitis. Patients with seizures due to this form of encephalitis commonly have medically intractable epilepsy and may require immunotherapy to control seizures. It is important that radiologists recognize imaging characteristics of this type of autoimmune encephalitis and suggest it in the differential diagnosis because this seizure etiology is likely under-recognized. Our purpose was to characterize MR imaging findings in this patient population. MATERIALS AND METHODS: MR imaging in 42 retrospectively identified patients (22 males; median age, 56 years; age range, 8-79 years) with seizures and voltage-gated potassium channel complex autoantibody seropositivity was evaluated for mesial and extratemporal swelling and/or atrophy, T2 hyperintensity, restricted diffusion, and enhancement. Statistical analysis was performed. RESULTS: Thirty-three of 42 patients (78.6%) demonstrated enlargement and T2 hyperintensity of mesial temporal lobe structures at some time point. Mesial temporal sclerosis was commonly identified (16/33, 48.5%) at follow-up imaging. Six of 9 patients (66.7%, P = .11) initially demonstrating hippocampal enhancement and 8/13 (61.5%, P = .013) showing hippocampal restricted diffusion progressed to mesial temporal sclerosis. Conversely, in 6 of 33 patients, abnormal imaging findings resolved. CONCLUSIONS: Autoimmune voltage-gated potassium channel complex encephalitis is frequently manifested as enlargement, T2 hyperintensity, enhancement, and restricted diffusion of the mesial temporal lobe structures in the acute phase. Recognition of these typical imaging findings may help prompt serologic diagnosis, preventing unnecessary invasive procedures and facilitating early institution of immunotherapy. Serial MR imaging may demonstrate resolution or progression of radiologic changes, including development of changes involving the contralateral side and frequent development of mesial temporal sclerosis.

Botulinum toxin-A injections via electrical motor point stimulation to treat writer's cramp: pilot study.

PURPOSE: Writer's cramp describes a task-specific dystonia, in which the act of writing initiates dystonic posturing of the hands. Previous studies have described the efficacy of injections of botulinum toxin type-A (BTX-A) under electromyographic guidance, in which the injected muscle is either voluntarily, or less often, electrically (electrical motor point stimulation, EMPS) activated to ensure that the needle is in the target muscle. We performed an open label, prospective study to assess the efficacy of BTX-A injections, performed with EMPS under electromyographic guidance. METHODS: Eight patients (seven male and one female) of mean age 44 (range 25-66) were recruited. All had idiopathic writer's cramp. Outcome measures, which included timed writing, objective assessment of dystonia (modified Ashworth scale and a visual analog scale rating) and patient assessment of functional disability, were assessed before injections and at six weeks follow-up. RESULTS: The total dose of BTX-A injected for writer's cramp ranged from 50 to 130 units, which was less than that reported in previous studies using muscle activation techniques (up to 300 units). Improvements were observed in all outcome measures. Patients reported mild (non-disabling) weakness of injected, but not of uninjected muscles. CONCLUSION: Lower dosages of BTX-A, administered using EMPS, offers the advantages of decreased cost and increased accuracy of targeting, while achieving good outcomes.