Case report: Orthostatic leg tremor as the initial manifestation in a patient with metabotropic glutamate receptor-5 encephalitis without cortical dysfunction: complexities in identification and treatment.

Objective: Metabotropic glutamate receptor 5 (mGluR5) encephalitis is such a rare type of autoimmune encephalitis that its diagnosis remains a challenge. Case report: A 19-year-old female patient initially presented with anxiety and orthostatic leg tremors without cortical dysfunction. We found that this patient was positive for mGluR5 antibodies in both serum (1:1,000) and cerebrospinal fluid (1:32). After comprehensive intervention, the patient showed complete recovery at the 18-month follow-up. Discussion: This report expands our knowledge of the possible presentations of mGluR5 encephalitis for early diagnosis, which makes it possible to prevent serious consequences and improve the prognosis.

Efficacy of low to moderate doses of oxcarbazepine in adult patients with newly diagnosed partial epilepsy.

PURPOSE: The objective of this study was to explore the efficacy of low dose of oxcarbazepine (OXC) in adult patients with newly diagnosed partial epilepsy in an actual clinical setting. The associated factors influencing the poor control of seizures were also evaluated. METHODS: The epilepsy database (2010-2014) from the Epilepsy Clinic of West China Hospital was retrospectively reviewed. RESULTS: A total of 102 adult patients with newly diagnosed, previously untreated partial epilepsy initially treated with OXC were included, and divided into good response group (64) and poor response group (38) according to whether they were seizure-free for at least 12 months. There were 27 (26.5%) patients becoming seizure-free with OXC 600 mg/day monotherapy. The remaining 75 patients had doses of either increasing OXC to 900 mg/day (n = 59) or the addition of another antiepileptic drug (AED) (n = 16), with another 20 (19.6%) and six (5.9%) patients becoming seizure-free, respectively (P = 0.788). In addition, two (2.0%) and nine (8.8%) patients became seizure-free with OXC > 900 mg/day monotherapy and OXC ≥ 900 mg/day combination therapy, respectively. Multivariate binary logistic regression analysis revealed that the time from onset of epilepsy to treatment initiation is significantly associated with seizure control (P = 0.02). CONCLUSION: Our results indicated that OXC at low to moderate doses is effective for the treatment of Chinese adult patients with newly diagnosed, previously untreated partial epilepsy, and a longer time interval from the onset of epilepsy to the start of treatment significantly predicts poor seizure control.

Intractable hiccup caused by spinal cord lesions in demyelination disease.

OBJECTIVE: This study aimed to summarize the clinical features of patients who presented intractable hiccup (IH) without brain and medulla oblongata (MO) lesions. METHOD: This study included six patients who were diagnosed with inflammatory demyelinating myelitis, categorized as neuromyelitis optica (NMO), multiple sclerosis (MS), and myelitis. Patients who presented IH with cervical lesions but without MO lesions were also included. Clinical profiles, laboratory data, and magnetic resonance imaging findings were analyzed. RESULTS: Three out of six patients were diagnosed with NMO, whereas the remaining three were diagnosed with acute myelitis, recurrent myelities, and MS, respectively. The duration of hiccup was from 2 to 23 days (average = 9.33 ± 8.64 days). Five patients (83.33%, patients 1-5) had long segmental lesions and one had a patchy lesion. None of these patients had any MO lesions. Half of them were successfully treated with high-dose methylprednisolone combined with gamma-aminobutyric acid (GABA) inhibitor. CONCLUSION: IH occurred in patients without MO lesion. However, the mechanism remained unclear. Immune factors of demyelinating neuropathy stimulated the hiccup reflex arch. Cervical cord lesions may activate the hiccup center. In general, IH can be controlled by IVMP combined with GABA inhibitor. Unilateral phrenic nerve block may elicit no effect.

Interrater reliability of the international consensus definition of drug-resistant epilepsy: a pilot study.

We evaluated the interrater reliability of the consensus definition of drug-resistant epilepsy proposed by the International League Against Epilepsy. According to the definition framework, outcome of each antiepileptic drug (AED) trial was categorized as "seizure freedom" or "treatment failure." This level 1 assessment was used to determine the level 2 classification, which defined drug-resistant epilepsy as the failure of adequate trials of two or more AED schedules to achieve sustained seizure freedom. Two raters classified treatment outcomes of 150 patients independently. The patients had received a total of 428 trials of AEDs. Categorization of level 1 outcome to individual AED trials by the raters was consistent in 413 (96.5%). For the level 2 classification of drug-resistant or drug-responsive epilepsy, there was absolute agreement between the raters in 141 patients (94%), with a κ index of 0.91 (P<0.001). The definition appeared to have a high degree of interrater reliability in this setting.