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1/9. autonomic dysreflexia: one more way EMS can positively affect patient survival.

    autonomic dysreflexia is a life-threatening medical condition that affects people with spinal cord injuries above T6. Caused by the division of the autonomic nervous system, it can result in disastrous hypertension. Although complicated in nature, AD can be quickly treated and reversed by prehospital providers. The prompt emptying of a patient's bladder and/or bowels will resolve most occurrences. Other factors that can't be resolved in the prehospital setting may cause AD. In these situations, quickly transport the patient to a definitive care facility and consider the use of antihypertensive agents. Bladder catheterization and digital bowel emptying are not everyday EMS skills. They are, however, skills within the range of EMS abilities. Providers should contact their medical directors or training supervisors to obtain the training necessary to carry out both techniques. Having these skills will arm you with the necessary abilities to mitigate an episode of autonomic dysreflexia.
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2/9. Micturition disturbance in acute idiopathic autonomic neuropathy.

    OBJECTIVE: To define the nature of micturition disturbance in patients with acute idiopathic autonomic neuropathy (AIAN). methods: Micturitional symptoms were observed during hospital admissions and the in outpatient clinics in six patients with clinically definite AIAN (typical form in four, cholinergic variant in one, autonomic-sensory variant in one). Urodynamic studies included medium-fill water cystometry, external sphincter electromyography, and a bethanechol test. RESULTS: Four patients had urinary retention and two had voiding difficulty as the initial presentation. patients with retention became able to urinate within a week (two to seven days). The major symptoms at the time of urodynamic studies (three weeks to four months after disease onset in most cases) were voiding difficulty and nocturnal frequency. None had urinary incontinence. Complete recovery from the micturition disturbance took from three months to >18 years. The recovery period was shorter in a patient with cholinergic variant, and it was longer in two patients who had a longer duration of initial urinary retention. Micturition disturbance tended to improve earlier than orthostatic hypotension. The major urodynamic abnormalities were detrusor areflexia on voiding (5), denervation supersensitivity to bethanechol (3); low compliance detrusor (1); and impaired bladder sensation (2). None had neurogenic motor unit potentials of the external sphincter muscles. CONCLUSIONS: In patients with AIAN, urinary retention and voiding difficulty are common initial presentations. The underlying mechanisms seem to be pre- and postganglionic cholinergic dysfunction with preservation of somatic sphincter function. The bladder problems tend to improve earlier than orthostatic hypotension.
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3/9. Parkinsonism, dysautonomia, and intranuclear inclusions in a fragile X carrier: a clinical-pathological study.

    A new tremor-ataxia syndrome, fragile X-associated tremor/ataxia syndrome (FXTAS), has been described among carriers of premutation expansions (55-200 CGG repeats) of the fragile X mental retardation 1 (FMR1) gene. The prevalence of FMR1 premutation alleles has been reported to be 1 in 813 among men. patients with FXTAS may also have features of parkinsonism. Postmortem findings have been described in eight patients with FXTAS and detailed descriptions of the pathological features of this syndrome have been published in two of these. We present a detailed description of the postmortem findings in a third patient. The patient had parkinsonism and was a carrier of a premutation expansion in the FMR1 gene. As in previous reports, the most prominent finding was the presence of eosinophilic nuclear inclusions in neurons and astrocytes, loss of purkinje cells, and regional vacuolation of the cerebral white matter. As in one previous report, nuclear inclusions were also present in ependymal and choroid plexus cells. A new finding is that of nuclear inclusions in both the adeno- and neurohypophysis. These findings confirm the diffuse nature of this pathology. Further studies of clinical-pathological correlation in a larger sample of brains would provide additional insight into the mechanisms of the tremor, ataxia, and parkinsonism in these patients.
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4/9. Autonomic function and motility in intestinal pseudoobstruction caused by paraneoplastic syndrome.

    This report documents the occurrence of chronic intestinal pseudoobstruction in association with a small cell carcinoma of the lung with evidence of pre- and postganglionic sympathetic dysfunction in one patient with brain metastases, and with sympathetic and parasympathetic postganglionic dysfunction in a second patient. A strategy is outlined for the identification and characterization of disordered neural control of gut motility. This strategy utilizes gastrointestinal motility studies to confirm gut neuropathy, autonomic function tests, and plasma norepinephrine responses to intravenous edrophonium to identify the level of dysfunction. These cases are compared with others in the literature, and the occult nature of these cancers, the spectrum of symptoms suggesting autonomic dysfunction on presentation, and the occasional response of the neurologic deficit to treatment of the malignancy are highlighted.
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5/9. Transient regional and migratory osteoporosis. A possible neural mechanism.

    Transient osteoporosis of the hip is a rapidly developing painful osteoporosis of benign nature and as yet unexplained pathophysiology. We add 5 new cases of transient osteoporosis, 4 of which are regional and 1 migratory, to 70 cases abstracted from the literature. Our review of the literature confirmed that there are two major subgroups of transient osteoporosis: regional and migratory. Two of our cases exhibited abnormal neurological findings. A possible neural mechanism related to regional osteoporosis should be further explored by a meticulous neurological and electrophysiological workup of such patients.
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6/9. Hypothalamic astrocytoma. syndrome of hyperphagia, obesity, and disturbances of behavior and endocrine and autonomic function.

    A 26-year-old woman had hyperphagia, obesity, aggressive behavior, visual hallucinations, reversal of wake-sleep patterns, hypothermia, hypothyroidism, and amenorrhea. She died of pancreatitis, probably secondary to hypothermia. autopsy revealed a low-grade astrocytoma in the third ventricle and medial anterior and mid hypothalamus, primarily on the right. Although she exhibited thyroid and ovarian hypofunction, the patient had intact median eminence and pituitary function, suggesting end-organ failure, possibly of an autoimmune nature.
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7/9. Severe tetanus--in spite of tetanus toxoid.

    A 66-year-old man sustained an injury to his right foot while gardening. Despite receiving tetanus toxoid one hour later and adequate wound toilet, he developed severe tetanus complicated with autonomic dysfunction six days later. He died 20 days after admission. This case shows that tetanus toxoid alone may not be sufficient to prevent tetanus in wounded patients. Careful consideration must be given to the immune status of the patient and to the nature of the wound sustained. Incompletely immunised patients or patients with unknown immune status who sustain a tetanus prone wound should be protected with both tetanus toxoid and tetanus immunoglobulin.
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8/9. skin sympathetic nerve activity in acquired idiopathic generalized anhidrosis.

    We recorded skin sympathetic nerve activity (SSNA) microneurographically from the right tibial nerve of a patient with acquired idiopathic generalized anhidrosis (AIGA). The patient did not show any spontaneous sweating or pilocarpine- and nicotine-induced sweat response. Histopathologic examination showed degenerated eccrine glands associated with surrounding inflammatory cellular infiltration. Electrical nerve stimulation produced a two-peak pattern of SSNA reflex discharge representing sudomotor and vasoconstrictor components. The frequency of spontaneous SSNA bursts (burst rate), presumably of a sudomotor nature, at the ambient temperature of 25 degrees C was significantly higher than in a healthy control subject and was further increased at a temperature of over 38 degrees C. Thus, sudomotor sympathetic nerve activity is well preserved or even increased in AIGA. We conclude that anhidrosis of AIGA results from the generalized sweat gland dysfunction rather than decreased sympathetic outflow to the skin.
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9/9. Autonomic hyperreflexia associated with exacerbation of reflex sympathetic dystrophy.

    Autonomic hyperreflexia is a condition of massive paroxysmal reflex sympathetic outflow in response to noxious stimuli occurring below the major splanchnic sympathetic outflow in patients with spinal cord injuries. This heightened sympathetic outflow can result in dramatic sudomotor and vasomotor manifestations. The unique nature of this sympathetic-mediated response following traumatic spinal cord injury provides a model for the study of the autonomic nervous system in normal and pathological conditions. A case of autonomic hyperreflexia exacerbating the pain of reflex sympathetic dystrophy in a patient with tetraplegia illustrates the role of the sympathetic nervous system in the pathophysiology of sympathetic-mediated pain and supports current treatment options for these conditions.
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