Sudden unexpected death in epilepsy (SUDEP) is a major clinical problem in epilepsy patients in the United States, especially those with chronic, uncontrolled epilepsy. Several pathophysiological events contributing to SUDEP include cardiac arrhythmias, respiratory dysfunction, and dysregulation of systemic or cerebral circulation. There is a significant body of literature suggesting the prominent role of cardiac arrhythmias in the pathogenesis of SUDEP. There is evidence to say that long-standing epilepsy can cause physiological and anatomical autonomic instability resulting in life-threatening arrhythmias. Tachyarrhythmias, bradyarrhythmias, and asystole are commonly seen during ictal, interictal, and postictal phase in epilepsy patients. It is unclear if these rhythm disturbances need attention as some of them may be just benign findings. Evidence regarding prolonged cardiovascular monitoring or the benefit of pacemaker/defibrillator implantation for primary or secondary prevention in epilepsy patients is limited. Awareness regarding pathophysiology, cardiac effects, and management options of SUDEP will become useful in guiding more individualized treatment in the near future. (PACE 2011; 1-8).
Abstract. Ewing's sarcoma is the second most common primary sacral tumor. Ewing's sarcomas are rare, aggressive tumors with a tendency towards recurrence following resection and early metastasis. Although peak incidences are between the ages of 10 and 20 years, patients of younger or older age account for almost 30% of the cases. We report the case of a 52-year-old healthy female who presented with a 2-week history of pain in her right posterior thigh that was unable to be relieved by non-steroidal anti-inflammatory medicine and physical therapy. Magnetic resonance imaging demonstrated an irregular right presacral mass and core needle biopsy revealed a small, round blue cell neoplasm. Staging workup was normal and an open biopsy was positive for the ES translocation (22q12). The patient was treated with 17 cycles of vincristine, adriamycin and cytoxan with mesna rescue, alternating with ifosfamide and etoposide in addition to external beam radiation. Post-treatment imaging demonstrated complete resolution of the tumor. Six weeks post-treatment the patient presented with a recurrent tumor. This case emphasizes the importance of timely establishment of initial diagnosis, early metastasis in treatment responsive patients and under-utilization of positron emission tomography-computed tomography (PET-CT) during the treatment to detect sub-clinical metastasis. IntroductionEwing's sarcoma (ES) is the second most common bone tumor after osteosarcoma in children and adolescents. ES are aggressive tumors with a tendency towards recurrence following resection and pronounced proclivity toward early hematogenous metastases to lungs and bone. No hereditary or congenital syndromes, environmental or known risk factors have been associated with the occurrence of ES. In 90% of cases, Ewing's sarcoma family tumor (ESFT) cells harbor the translocation t(11;22)(q24;q12), and in the remaining 10% the variant translocation is t(21;12)(22;12) (1,2). Although peak incidence occurs between the ages of 10 and 20 years, patients of younger or older ages account for almost 30% of the cases (3). Poor prognostic factors include tumor ≥8 cm, pelvic primary, presence of metastases and age >15 at the time of diagnosis (4). Older patients with sarcoma also have a higher risk of thromboembolism (5). Case reportA 52-year-old healthy female presented with a 2-week history of pain in her right posterior thigh. The pain originated in the patient's right buttock and radiated to the back of the knee without a radicular component. Motor strength, sensory function and reflexes were normal. Musculoskeletal examination was within normal limits without tenderness of the hips or back.Despite the use of non-steroidal anti-inflammatory medications and physical therapy the pain persisted. On subsequent reassessment, a radicular component was present. Magnetic resonance imaging (MRI) demonstrated an irregular-shaped right presacral mass with heterogeneous short T1 inversion recovery (STIR) hyperintensity (Fig. 1A) and T1 hypointensity (Fig. 1B). Additional evalua...
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