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CARDINAL MANIFESTATIONS OF NEUROLOGIC DISEASE
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centers, but Sakakibara and colleagues have reported that half of their patients with brainstem strokes had urinary symptoms, for which lesions in the pons were most often responsible; the precision of anatomic localization of the strokes was limited and the symptoms varied Therapy of Disordered Micturition Several drugs are useful in the management of accid and spastic disturbances of bladder function In the case of a accid paralysis, bethanechol (Urecholine) produces contraction of the detrusor by direct stimulation of its muscarinic cholinergic receptors In spastic paralysis, the detrusor can be relaxed by propantheline (Pro-Banthine, 15 to 30 mg three times daily), which acts as a muscarinic antagonist, and by oxybutynin (Ditropan, 5 mg two to three times daily), which acts directly on the smooth muscle and also has a muscarinic antagonist action Atropine, which is mainly a muscarinic antagonist, only partially inhibits detrusor contraction More recently, alpha1 sympathomimetic blocking drugs such as terazosin, doxazosin, and tamsulosin have been used to relax the urinary sphincter and facilitate voiding Their widest use has been in men with prostatic hypertrophy, but they may be bene cial in patients with dyssynergia of the sphincter from neurologic disease Several other drugs may be useful in the treatment of neurogenic bladder but can be utilized rationally only on the basis of sophisticated urodynamic investigations (Krane and Siroky) Often the patient must resort to intermittent self-catheterization, which can be safely carried out with scrupulous attention to sterile technique (washing hands, disposable catheter, etc) Some forms of chronic antibiotic therapy and acidi cation of urine with vitamin C (1000 g/day) are practical aids, but their use has gone through cycles of popularity based on various studies with differing results In selected paraplegic patients, the implantation of a sacral anterior root stimulator may prove to be helpful in emptying the bladder and achieving continence (Brindley et al)
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internal sphincter, and polymyositis and myasthenia gravis, which may impair the function of the external sphincter (Schuster) The inability to control atulence may be an early sign of sphincteric weakness in myasthenia Also, sphincteric damage may complicate hemorrhoidectomy The serotonin receptor agonist cisapride had been used in partially restoring gastrointestinal motility in some cases of neurogenic ileus as, for example, the early stages of the Guillain-Barre syndrome and in pediatric bowel diseases Because of arrhythmias and a few cases of sudden cardiac death, its administration is currently allowed only when given by experienced pediatric gastroenterologists Many cases of ileus, even to the extent of megacolon, have a pharmacologic basis, being due to the use of drugs that paralyze the parasympathetic system or narcotics that have a direct effect on the motility of gastrointestinal smooth muscle Congenital Megacolon (Hirschsprung Disease) This is a rare disease affecting mainly male infants and children It is due to a congenital absence of ganglion cells in the myenteric plexus The internal anal sphincter and rectosigmoid are involved most often and are the parts affected in restricted forms of Hirschsprung disease (75 percent of cases), but the aganglionosis is sometimes more extensive The aganglionic segment of the bowel is constricted and cannot relax, thus preventing propagation of peristaltic waves, which, in turn, produces retention of feces and massive distention of the colon above the aganglionic segment Enterocolitis is the most serious complication and is associated with a high mortality Some cases of megaloureter are attributed to a similar defect Hirschsprung disease in most cases has been traced to a mutation of the RET oncogene; the variability in clinical severity corresponds to polymorphisms in the responsible gene Other genes, such as the one that codes for the endothelin receptor, are implicated in a small group with the disease
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