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1/113. Primary sjogren's syndrome with severe central nervous system disease.

    OBJECTIVE: central nervous system (CNS) involvement in primary sjogren's syndrome (pSS) is controversial with regard to frequency, significance, and etiology. methods: We describe a young woman with pSS and severe CNS disease and review the literature on the pathophysiology, clinical significance, symptoms, diagnostic examinations, and treatment of CNS disease with concomitant pSS (CNS-SS). RESULTS: Our patient with pSS had a 5-month history of benign lymphadenopathy and myositis, after which she developed severe CNS disease, vasculitic lesions on her hands, and a neurogenic bladder attributable to spinal cord involvement. The diagnosis was based on the clinical picture and the results of a brain magnetic resonance imaging (MRI) scan, electroencephalography (EEG), and cerebrospinal fluid (CSF) analysis. The disease did not respond to corticosteroids, but the administration of cyclophosphamide resulted in recovery. In the literature, the incidence of CNS-SS varies widely, from rare to incidence rates of 20% to 25%. The clinical picture is diverse, ranging from mild cognitive symptoms to fatal cerebrovascular accidents. The pathophysiology of CNS-SS is unclear, specific diagnostic methods are not available, and diagnosis is based on the clinical picture and a combination of examinations. MRI is the most sensitive test and cerebral angiography the most specific. CSF reflects involvement of the leptomeninges, and EEG is nonspecific. There are no controlled studies of the treatment of CNS-SS. Regimens for vasculitis are commonly used. CONCLUSIONS: CNS-SS is uncommonly recognized and difficult to diagnose. Increasingly accurate and available diagnostic examinations will yield more information about the association of CNS disease with pSS.
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ranking = 1
keywords = spina, spinal
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2/113. cauda equina syndrome due to lumbosacral arachnoid cysts in children.

    We describe the clinical, neuroradiological and surgical aspects of two children in whom symptoms attributable to cauda equina compression were caused by spinal arachnoid cysts. The first patient presented with recurrent urinary tract infections due to neurogenic bladder dysfunction, absent deep tendon reflexes and sensory deficit in the lower limbs. The second child presented with unstable gait as a result of weakness and diminished sensation in the lower extremities. Spinal magnetic resonance imaging revealed a lumbosacral arachnoid cyst in both patients. During surgery the cysts were identified and excised. Two years after surgery, the sensory deficits of the first patient have disappeared and patellar and ankle reflexes can be elicited, but there is no improvement in bladder function. Neurological examination of the second patient was normal. We conclude that the diagnosis of cauda equina syndrome should prompt a vigorous search for its aetiology. Lumbosacral arachnoid cysts are a rare cause of cauda equina syndrome in children.
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keywords = spina, spinal
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3/113. Interfacing the body's own sensing receptors into neural prosthesis devices.

    Functional electric stimulation (FES) is today available as a tool in muscle activation used in picking up objects, in standing and walking, in controlling bladder emptying, and for breathing. Despite substantial progress over nearly three decades of development, many challenges remain to provide a more efficient functionality of FES systems. The most important of these is an improved control of the activated muscles. Instead of artificial sensors for feedback, new developments in electrodes to do long-term and reliable recordings from peripheral nerves emphasize the use of the body's own sensors. These are already installed and optimised through millions of years of natural evolution. This paper presents recent results on a system using electrical stimulation of motor nerves to produce movement and using the natural sensors as feedback signals to control the stimulation that can replicate some of the functions of the spinal cord and its communication with the brain. We have used the nerve signal recorded from cutaneous nerves in two different human applications: (1) to replace the external heel switch of a system for correction of spastic drop foot by peroneal stimulation, and (2) to provide an FES system for restoration of hand grasp with sensory feedback from the fingertip. For the bladder function, the sacral root stimulator is a useful control tool in emptying the bladder. To decide when to stimulate, we are at present carrying out experiments on pigs and cats using cuff electrodes on the pelvic nerve and sacral roots to record the neural information from bladder afferents. This information can potentially be used to inhibit unwanted bladder contractions and to trigger the FES system and thereby bladder emptying. Future research will show whether cuffs and other types of electrodes can be used to reliably extract signals from the large number of other receptors in the body to improve and expand on the use of natural sensors in clinical FES systems.
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keywords = spina, spinal
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4/113. Single kidney outcome and management in persons with spinal cord injury.

    This case study examined the outcomes of persons with spinal cord injury (SCI) who had a single kidney. A Urologic database, including 1655 persons with SCI between 1969 and 1997, was examined and 22 persons were identified with single kidneys. Twenty persons had adequate follow-up. Renal function was measured by total and individual kidney effective renal plasma flow (ERPF). Of 11 persons who had a single kidney prior to injury or as a result of an associated injury, all maintained a normal ERPF for an average of 8.6 years. Of 9 persons who had removal of a kidney following their injury for other diseases or urinary complications, 3 were deceased, but 2 had a normal ERPF in the remaining kidney prior to death. One with vesicoureteral reflux had decreased renal function in the remaining kidney. Recurrent renal calculi in a single kidney carries risks for decreasing renal function, urosepsis, and death.
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ranking = 2.5
keywords = spina, spinal
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5/113. Bladder cancer arising in a spina bifida patient.

    We report the case of a 52-year-old patient with spina bifida, neurologic bladder, and a history of recurrent urinary tract infections (UTIs) in whom a bladder cancer was incidentally discovered. Cytology, cystoscopy, and cystography showed nonspecific, extensive inflammatory lesions. Cystography demonstrated a complex of diverticulae and cellules. Pathologic examination of a diverticulectomy specimen revealed a grade III pT3b transitional and squamous cell carcinoma. Because of the similar disease causation (recurrent UTIs, stones, and indwelling catheterization), we suggest extension of the guidelines proposed for patients with spinal cord injuries (ie, annual serial bladder biopsies) to patients with nontraumatic neurogenic bladder.
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ranking = 26.57495424353
keywords = spina bifida, bifida, spina, spinal
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6/113. Laparoscopic bladder auto-augmentation in an incomplete traumatic spinal cord injury.

    OBJECTIVES: To assess the urodynamic and clinical outcome of a laparoscopic auto-augmentated bladder. methods: Laparoscopic bladder autoaugmentation in a 27-year-old woman with an incomplete spinal cord injury at T12 level with urge incontinence caused by a hyperreflexic bladder. RESULTS: Six months later the patient voids by Valsalva's manoeuvre every 3 h and remains dry day and night. The radio-urodynamic study, performed 2 months later, revealed an intact bladder with a diverticulum of anterior wall and a capacity of 510 ml with filling rate of 100 ml/min without evidence of leakage of infusion water. CONCLUSION: Laparoscopic retropubic auto-augmentation allows a brief hospital stay and minor postoperative discomfort. Moreover the laparoscopic approach should not complicate or preclude subsequent enterocystoplasty if necessary.
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ranking = 2.5
keywords = spina, spinal
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7/113. Early autonomic dysreflexia.

    INTRODUCTION: During the stage of spinal shock the conventional view is that autonomic activity is abolished. Here, evidence is presented that autonomic activity is still present. patients: Four patients with acute cord transactions are presented: one new case and three from the literature. DEFINITIONS: The definitions of spinal shock and autonomic dysreflexia are given. methods: All four cases showed acute autonomic dysreflexia between 7 and 31 days after acute cord transection at a stage when the tendon reflexes were abolished. RESULTS: Two cases showed a severe rise in blood pressure; the two earlier cases, before blood pressure was routinely recorded, profuse sweating. In two cases autonomic dysreflexia was obtained when the bladder was overdistended with 1000 ml and 1600 ml. In the other two cases it occurred in response to traumatic catheterisation. This was found when supramaximal stimuli were applied. It has not been recorded routinely as, with modern management, the bladder does not get overdistended or traumatised. DISCUSSION: Other evidence, the blood pressure, and urethral tone is presented to show that sympathetic reflex activity of the cord is not abolished during spinal shock. CLINICAL SIGNIFICANCE: The clinical importance of this is that autonomic dysreflexia can be seen at an early stage and it should be considered in the differential diagnosis of a sick patient immediately after spinal injury.
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ranking = 2
keywords = spina, spinal
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8/113. Sigmoid colon rupture secondary to Crede's method in a patient with spinal cord injury.

    Crede's method is a manual suprapubic pressure exerted with a clenched fist or fingers, used to initiate micturition, in patients with spinal cord injury (SCI) who have neurovesical dysfunction. It is usually a benign maneuver unassociated with any major complications. This paper will illustrate a case report involving a sigmoid colon rupture secondary to Crede's method in a patient with SCI. Various techniques of Crede's method are briefly described. It is recommended that patients with quadriplegia avoid forceful use of Crede's method, as it may cause contusion of the abdominal wall and injuries to internal viscera, possibly leading to colonic rupture. It is believed that this is the first reported case of such an unusual complication of Crede's method in patients with SCI.
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ranking = 2.5
keywords = spina, spinal
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9/113. Neurological worsening due to infection from renal stones in a multiple sclerosis patient.

    Symptomatic bladder dysfunction occurs in the majority of patients with multiple sclerosis (MS). Although guidelines have been established for diagnosis and management of bladder dysfunction in these patients, they are sometimes overlooked in the primary care setting, leading to severe, life threatening complications. A 64-year-old male with a 31-year history of spastic quadriparetic MS and neurogenic bladder dysfunction managed with an indwelling catheter, presented to the hospital with worsening neurological function. He had developed increased weakness and cognitive impairment several weeks after being treated for a urinary tract infection (UTI). He had become unable to perform any activities of daily living or drive his power wheelchair. After an extensive work-up, he was found to have a large (14 x 18 x 30 cm) retroperitoneal abscess and multiple renal stones, including a large obstructing calculus in the collecting system near the ureteropelvic junction, and he underwent nephrectomy and abscess drainage. Of note, he had been found to have multiple renal stones and hydronephrosis on renal ultrasound 3 years earlier, but he had received no treatment. Following drainage of the abscess, his upper extremity neurological function returned to baseline, his cognitive status improved, and he regained the ability to perform activities of daily living. patients with paralysis from MS, much like those with traumatic spinal cord injuries, are at grave risk of mortality and morbidity from undiagnosed and under-treated urinary complications. This case demonstrates that evaluation and appropriate treatment for complications of neurogenic bladder should be part of routine care for patients with MS. Current recommendations for evaluation and management of bladder dysfunction in patients with MS will be reviewed.
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ranking = 0.5
keywords = spina, spinal
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10/113. Compression of femoral vein by the strap of a urine-collecting device in a spinal cord injury patient.

    OBJECTIVE: To report an unusual cause of femoral vein compression in a spinal cord injury (SCI) patient. DESIGN: A case report of a SCI patient in whom the strap of a urinal produced compression of femoral vein. Setting Regional spinal injuries Centre, Southport, england. SUBJECT: A 65-year old male, who had sustained paraplegia at T-10 level 33 years ago, attended the spinal unit for a routine follow-up intravenous urography (IVU). He was wearing a urinal, which was held tightly over the penis by means of two straps coursing over the inguinal regions. MAIN OUTCOME MEASURES: IVU was performed by injecting 50 ml of Ultravist-300 via a 23-gauge butterfly needle inserted in a vein over the dorsum of the left foot. After completion of the injection, an X-ray of the pelvis was taken to evaluate the right hip. This showed contrast in the vena profunda femoris, circumflex femoral veins and inter-muscular veins with evidence of compression of proximal femoral vein. RESULTS: It was suspected that the strap holding the urinal was causing compression of the femoral vein. Therefore, a venogram was performed 5 days later, when the patient had discarded the urinal and the straps. This showed free flow of contrast through the left femoral and iliac veins. CONCLUSION: Compression of femoral vein by a strap holding the urinal was discovered serendipitously in this patient during a routine follow-up. physicians and health professionals should bear in mind this rare complication when examining spinal cord injury patients who use this type of urine collecting device, and discuss with them alternative methods for urinary drainage.
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ranking = 3.5
keywords = spina, spinal
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