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1/9. Sudden death due to a paraganglioma of the organs of Zuckerkandl.

    A 20-year-old woman died suddenly in a hospital emergency room after presenting with nausea, vomiting, back pain, and hypertension. At autopsy, an extra-adrenal pheochromocytoma (paraganglioma) of the organs of Zuckerkandl was found, with microscopic focal myocardial necrosis similar to that described in death from adrenal pheochromocytomas. Tumors of the organs of Zuckerkandl are extremely rare; less than 100 such cases have been reported in the world's literature, and only six, including the present case, have presented as a sudden, unexpected death. The symptoms of catecholamine storm may mimic those of acute drug intoxications, leading to misdiagnosis by both clinical physicians and pathologists.
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2/9. seizures secondary to autonomic dysreflexia.

    seizures are a little-recognized component of the syndrome of autonomic dysreflexia. Three patients who exhibited seizures during episodes of dysreflexia independent of intracerebral pathology are described. The presence of seizures in patients with traumatic myelopathy T-6 should alert the physician to a possible dysreflexic episode, and measures should be taken to avert such an episode.
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3/9. Preterm labor in the quadriplegic parturient.

    The occurrence of preterm labor is not uncommon in the pregnant quadriplegic. early diagnosis is hampered by the inability of most quadriplegics to sense uterine contractions in the usual way. A patient we recently treated for preterm labor learned to recognize contractions by the associated symptoms of autonomic hyper-reflexia: flushing, headache, and piloerection. Tocolytic therapy was successful and a favorable neonatal outcome occurred. Increased awareness by the physician and the pregnant quadriplegic patient is encouraged so that symptoms of autonomic hyper-reflexia may be recognized as potentially indicative of uterine contractions.
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4/9. Ewings sarcoma as an etiology for persistent back pain in a 17-year-old girl after trauma to the back.

    back pain--a common complaint familiar to all physicians--can be either a manifestation of musculoskeletal dysfunction or a symptom of a more serious underlying disease. Unfortunately, the diagnosis can be difficult, and back pain that is not resolved by conservative treatment requires aggressive investigation. The author presents the case history of a 17-year-old patient whose back pain had been extensively evaluated by a series of specialists to no avail until further workup revealed the problem to be Ewing's sarcoma. The steps required to diagnose elusive back pain are presented, together with a brief discussion of cauda equina syndrome and conus medullaris syndrome, components of both of which were found in this patient.
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5/9. serotonin syndrome complicating migraine pharmacotherapy.

    serotonin syndrome, a condition with numerous clinical neurological manifestations, is the result of central serotonergic hyperstimulation. Features of the syndrome include mental status and behavioral changes (agitation, excitement, hypomania, obtundation), motor system involvement (myoclonus, hemiballismus, tremor, hyperreflexia, motor weakness, dysarthria, ataxia) and autonomic symptoms (fever, chills, diarrhea). serotonin syndrome has been reported exclusively in patients on medications for psychiatric illness and Parkinsonism, despite the fact that the putative action of many antimigraine agents also involves the serotonin system. We herein report six patients with migraine who developed symptoms suggestive of the serotonin syndrome. Five were taking one or more serotomimetic agents for migraine prophylaxis (sertraline, paroxetine, lithium, imipramine, amitriptyline). In each case the symptoms and signs developed in close temporal proximity with use of a migraine abortive agent known to interact with serotonin receptors. In three instances the agent was subcutaneous sumatriptan and, in three, intravenous dihydroergotamine. In each instance the symptoms were transient and there was full recovery. With the ever increasing use of migraine medications active at serotonin receptor sites, cases of serotonin syndrome will likely occur more frequently. It is important that physicians treating migraine are aware of the serotonin syndrome and are able to recognize its varying presentations.
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6/9. Pourfour du Petit syndrome: a case following a traffic accident with severe cranioencephalic trauma.

    Poufour du Petit syndrome is an extraordinarily unusual clinical condition produced by hyperactivity of the sympathetic cervical chain as a consequence of irritation of these nerves. It causes an ipsilateral mydriasis, which, in patients suffering a head injury as in the case reported here, can confuse the diagnosis and disconcert physicians.
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7/9. Autonomic dysfunction as the presenting feature of guillain-barre syndrome.

    Autonomic dysfunction has been demonstrated in various conditions associated with peripheral neuropathy such as acute intermittent porphyria, amyloidosis, and guillain-barre syndrome (GBS). In the latter, hypertension is an associated complication that typically occurs after neurological signs are already present. We report a case of a patient with autonomic dysfunction as the presenting feature who was admitted to the coronary unit with chest pain and hypertension. Subsequently, he developed progressive symmetric muscle, weakness, sensory changes, and areflexia. GBS was then diagnosed based on the clinical picture, albuminocytologic dissociation in the cerebrospinal fluid, and electrodiagnostic abnormalities suggestive of demyelinative polyneuropathy with conduction block. Few cases in the literature have reported autonomic dysfunction as the presenting feature of GBS, such as in this case. In a previously asymptomatic patient, acute onset of autonomic dysfunction should alert the physician to the possibility of an acute polyneuropathy, such as GBS.
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8/9. pregnancy and delivery in tetraplegic women.

    Increase in survival of spinal cord injured (SCI) women, society's acceptance that their lives should be similar to those of non-disabled women and their better general health are increasing the number of SCI women who become pregnant and will be delivered of a child. Vaginal delivery is preferred. Any SCI woman whose level is at T6 or higher is at risk for acute autonomic hyperreflexia as a result of uterine contractions. If induction is with Pitocin/oxytocin, the risk is even greater. communication with the woman's obstetrician is essential. The patient should be provided with a packet of information to share with the obstetrician. This should be followed with a phone call from the SCI physician to the obstetrician. Effective management includes epidural anesthesia; vacuum extraction is helpful in the expulsion stage. episiotomy is usually not needed since the pelvic floor is relaxed. In addition, there is an increased incidence of dehiscence since SCI women should be mobilized early and need to transfer in and out of a wheelchair. blood pressure needs to be taken during the peak of contraction. This needs to be compared to prenatal blood pressures. If prenatal blood pressure is 80/60 or 90/60 but during contraction is 130/80 with a pounding headache, that indicates autonomic hyperreflexia which is an indication for epidural anesthesia. With improvement of acute care and more effective rehabilitation, pregnancy and delivery in spinal cord injured (SCI) women will occur more frequently. No one has any great experience with this situation and most articles report only a few cases. Even the report by Goller and Paeslack4 dealt with 175 cases from 42 centers in 24 countries. Most women were paraplegic and several who were injured early in their pregnancy had abnormal babies (possibly due to x-rays taken for spinal injury). Our spinal cord injury staff were pleased when we had two tetraplegic patients who were pregnant. It helped confirm our belief that life and its functions continue after paralysis. Staff members were involved in prenatal care, were present during delivery and were involved with postnatal care. Even more important is the fact that rehabilitation from the start was oriented with child care in mind. Occupational therapists used their skills and imagination to develop a program for newborn baby care by the tetraplegic mother.
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9/9. Isolated venlafaxine-induced serotonin syndrome.

    serotonin syndrome is a potentially fatal complication of serotonergic drug therapy. Usually, serotonin syndrome occurs with the concomitant use of two serotonergic drugs; this case report describes a patient with a classic presentation of serotonin syndrome induced solely by a venlafaxine overdose. Emergency physicians need to be aware that the serotonin syndrome may occur not only with serotonergic drug combinations but also with overdoses of a single potent serotonergic agent such as venlafaxine.
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