We report a case of a 31-year-old woman with an intramedullary thoracic dermoid, who had progressive spastic paraparesis in her lower extremities over a period of three years, as well as loss of bowel and bladder control. She had been operated for "spina bifida" at three days of age but had no neurologic deficit. MRI of the thoracic spine showed an intramedullary hypointense lesion at T7-T8, associated with an intramedullary lipoma at a higher level. Intracapsular resection of an intramedullary dermoid cyst was performed. We review the literature on intramedullary dermoids differentiating them from the more common, but still rare, lumbosacral subdural juxtamedullary dermoids. We discuss the associated malformations with these lesions, as well as their pathophysiology and treatment. Although uncommon, dorsal intramedullary dermoids should be suspected and treated in a timely manner, especially in presence of other congenital anomalies.
Aim. Assess quality-of-life after vagal nerve stimulation and determine patient characteristics associated with improvement in quality-of-life. Methods. Sixteen patients (11 children, 5 adults) who had vagal nerve stimulation at our center were studied. Quality-of-life was assessed pre-and post-vagal nerve stimulation using the Quality-of-Life in Childhood Epilepsy questionnaire for children and the Epilepsy Surgery Inventory-55 for adults. Results. Sixteen patients who did not qualify for resective surgery were included; seven (43.75%) were males and 9 (56.25%) were females. Mean age at onset of seizures was 3.96 ± 4.00 years and at surgery was 15.78 ± 10.78. Follow-up time was 1.26 ± 0.92 years. Fourteen patients (87.5%) were mentally retarded. Ten (62.5%) had cryptogenic etiology and 6 patients (37.5%) symptomatic etiology. Fifty percent had localization-related epilepsy. Six of 7 patients with generalized cryptogenic etiology (85.71%) had Lennox-Gastaut syndrome. Seizures dropped from 122.31 ± 159.49 to 67.84 ± 88.22 seizures/month. Seizure reduction (> 50%) correlated with improvement in total quality-of-life (p = 0.034). Post-vagal nerve stimulation, the total group scored significantly higher in the social domain (p = 0.039). In patients with localization-related epilepsy, significant improvements were detected in the social domain (p = 0.049) and in total quality-of-life (p = 0.042). Conclusion. Despite a diverse and small population size, we observed significant improvements in the social domain 1.26 years post-vagal nerve stimulation. In addition, there was an improvement in total quality-of-life amongst patients with partial seizures. Finally, seizure reduction was associated with quality-of-life improvement. Our results support previous studies from the West reporting improvement in quality-of-life following vagal nerve stimulation, contradict those studies that did not show such differences, and are the first coming from a developing country.
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