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Abstracts / Journal of the Neurological Sciences 333 (2013) e422–e480
(e.g. Diabetes mellitus, Parkinsonism). Abnormalities and final diagnosis were noted, along with medication and patient cooperation. The relevance of AST to the clinical problem was ranked on a 3-point scale. Results: 162 patients underwent AST. Orthostatic dizziness was the most common referring diagnosis (53.1%) followed by known/ suspected disease assessment (33.3%), thermoregulatory (8.6%) and neurological (4.3%). 47.5% of ASTs were abnormal, 40.7% were normal and 11.7% inconclusive. The most frequent abnormal final diagnosis was isolated orthostatic hypotension (OH) (27.3%). 25.5% of diabetics were eventually diagnosed with diabetic autonomic neuropathy. AST was very helpful in 56.2%, somewhat helpful in 32.7% and inconclusive in 11.1%. Among inconclusive ASTs, 61.1% were attributed to poor performance of test maneuvers, 16.7% to medications and 16.7% to both. All patients with irregular heart rhythms had inconclusive results. Conclusion: AST was abnormal in more than half. The most common abnormality was isolated OH. Patients referred for known/suspected disease assessment were most likely to have abnormal AST. Conversely, patients with thermoregulatory problems were most likely to have normal AST. Poor performance of test maneuvers and irregular heart rhythm were confounders in the general population, while medication interfered with AST of Parkinsonism.
and 12 years old and its characteristics are defined by small patients series. CLCN1 genetic alterations that occur are multiple and usually heterozygous. We present a juvenile-onset Becker myotonia series. Material and methods: Observational study of Becker type myotonia genetically diagnosed patients series. We analyzed the age of onset and clinical features. Results: 3 patients (2 males and 1 female) with a mean age of onset of 1, 4 and 3 years old. Only one of them had family disease history. All had weakness at the beginning of the activity but without paralysis and with subsequent warm-up phenomenon. All of them had clinical and electrical myotonia. There was no clinical or EMG signs of muscular distrophy. CPK levels were normal. Genetic studies identified a homozygous mutation in the male patient with family history, a heterozygous mutation for the female patient and 2 different heterozygous mutations for the gene CLCN1 in the other male. Conclusions: Becker type myotonia congenita may have its onset in adolescence with similar symptoms than those described in earlier ages of onset. Genetic alterations are very variable.
doi:10.1016/j.jns.2013.07.1549
Abstract - WCN 2013 No: 744 Topic: 7 - Neuromuscular disorders Could acetate Eslicarbacepina be effective in neuropathic pain?
Abstract - WCN 2013 No: 742 Topic: 7 - Neuromuscular disorders Late onset post-radiotherapy brachial plexopathy F.J. Rodriguez de Rivera, F. Palomo, F. Vivancos, J. Arpa, E. Díez Tejedor. Department of Neurology, Hospital Universitario La Paz, Madrid, Spain Introduction: Risk of developing post-radiotherapy brachial plexopathy from breast cancer is less than 2%. It usually appears around 10.5 months (range 1.5–77) after the start of treatment and only in 25% of cases alterations in EMG are identified. A case of brachial plexopathy developed 13 years after radiotherapy treatment. Material and methods: Female patient, 66 years old with pain and muscle weakness in the distal portion of left arm and hand for 7 months. She had been treated 13 years ago for an infiltrating ductal carcinoma in the left breast with reconstruction and chemotherapy and subsequent radiotherapy. Results: Electromyography: Left brachial plexopathy C7–D1. MRI Cervical: C5–C6 degenerative disc disease without signs of CNS involvement. MRI brachial plexus: Fibrosis post-radiotherapy encompassing left brachial plexus fascicles. Laboratory study: Normal. Conclusions: Post-radiotherapy plexopathy may develop years after completion of treatment probably due to the evolution of tissue fibrosis. doi:10.1016/j.jns.2013.07.1550
Abstract - WCN 2013 No: 743 Topic: 7 - Neuromuscular disorders Juvenile-onset Becker type myotonia congenita F.J. Rodriguez de Rivera, I. Sanz, J. Arpa, E. Diez Tejedor. Department of Neurology, Hospital Universitario La Paz, Madrid, Spain Introduction: Becker type myotonia congenita is a very rare (1:100,000) autosomal recessive disease which usually begins between the ages of 4
doi:10.1016/j.jns.2013.07.1551
F.J. Rodriguez de Rivera, E. Díez Tejedor. Department of Neurology, Hospital Universitario La Paz, Madrid, Spain Introduction: Eslicarbacepina Acetate metabolite is a prodrug of an active Oxcarbazepine metabolite and with Carbamazepine, which share their sodium channels blockers mechanism. Theoretically it could be effective in patients with neuropathic pain. Material and methods: An observational study in patients with neuropathic pain ressistant to other drugs who are treated with Eslicarbacepina for 12 weeks. Pathology causing the pain, the effectiveness and side effects were analyzed. Results: 15 patients (4 female and 11 male) with a mean age of 64.3 years. 3 patients with Trigeminal Neuralgia, 12 patients with nondiabetic PNP. Previous treatments: Pregabalin 80%, 33.3% Gabapentin, Carbamazepine 20%. Visual Analog Pain Scale Pre Eslicarbacepina treatment 6.6/10, post-treatment 3.3/10. Withdrawal of medication in 3 patients (20%) with PNP: 2 due to ineffectiveness and 1due to diarrhea. Conclusions: Acetate Eslicarbacepina could be effective in neuropathic pain. Clinical trials are needed to determine its effectiveness level and its potential use in monotherapy. doi:10.1016/j.jns.2013.07.1552
Abstract - WCN 2013 No: 735 Topic: 7 - Neuromuscular disorders Clinical and immunological predictors of prognosis for Japanese patients with thymoma-associated myasthenia gravis S. Suzukia, K. Utsugisawab, Y. Naganeb, N. Suzukia. aDepartment of Neurology, Keio University School of Medicine, Tokyo, Japan; bDepartment of Neurology, Hanamaki General Hospital, Iwate, Japan Background: About 30%–50% of patients with thymomas also have autoimmune myasthenia gravis (MG). There are no immunological markers to predict the prognosis of patients with thymomaassociated MG (T-MG). Anti-Kv1.4 antibody, one of the striatal