Cases reported "Quadriplegia"

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11/33. A novel mutation in KCNQ2 associated with BFNC, drug resistant epilepsy, and mental retardation.

    BACKGROUND: Benign familial neonatal convulsion (BFNC) is a rare autosomal dominant disorder caused by mutations in two genes, KCNQ2 and KCNQ3, encoding for potassium channel subunits underlying the M-current. This current limits neuronal hyperexcitability by causing spike-frequency adaptation. methods: The authors describe a BFNC family with four affected members: two of them exhibit BFNC only while the other two, in addition to BFNC, present either with a severe epileptic encephalopathy or with focal seizures and mental retardation. RESULTS: All affected members of this family carry a novel missense mutation in the KCNQ2 gene (K526N), disrupting the tri-dimensional conformation of a C-terminal region of the channel subunit involved in accessory protein binding. When heterologously expressed in cho cells, potassium channels containing mutant subunits in homomeric or heteromeric configuration with wild-type KCNQ2 and KCNQ3 subunits exhibit an altered voltage-dependence of activation, without changes in intracellular trafficking and plasma membrane expression. CONCLUSION: The KCNQ2 K526N mutation may affect M-channel function by disrupting the complex biochemical signaling involving KCNQ2 C-terminus. Genetic rather than acquired factors may be involved in the pathophysiology of the phenotypic variability of the neurologic symptoms associated with BFNC in the described family.
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12/33. carbamazepine toxicity following Oxybutynin and dantrolene administration: a case report.

    OBJECTIVE: To report a case of carbamazepine toxicity following the administration of Oxybutynin and dantrolene. STUDY DESIGN: A case report. SETTING: The Spinal rehabilitation Department, Loewenstein Hospital, Raanana, israel. methods: A patient with C6D tetraplegia who sustained intoxication because of drug interaction is presented. She had been treated by carbamazepine 1000 mg/day for neuropathic pain for 2 years without clinical or laboratory signs of toxicity. After administration of Oxybutynin concomitantly with an increase in the dose of dantrolene, she presented the clinical symptoms and laboratory finding of carbamazepine intoxication. Trying to adjust the treatment to the patient's requirements, carbamazepine together with Oxybutynin and dantrolene was readministrated in lower doses. RESULTS: The combination of these drugs, even small doses, caused toxicity. Adding dantrolene and Oxybutynin elevated the blood level of carbamazepine, possibly by inhibition of cytochrome P450. CONCLUSION: A possible pharmacokinetic interaction between dantrolene and Oxybutynin should be borne in mind when considering carbamazepine medication for a patient with a spinal cord lesion.
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13/33. Tuberculous radiculomyelitis (arachnoiditis) associated with tuberculous meningitis.

    Abstract. A 17-year-old man who presented with progressive quadriparesis is reported. About 8 months prior to admission, he had miliary tuberculosis, and that improved with anti-tuberculous therapy. He had also developed tuberculous meningitis and tuberculous myelitis, respectively. He regularly took anti-tuberculous drugs until this illness. Neurological findings were compatible with cervical cord lesion. CSF analysis indicated a predominate lymphocytic pleocytosis with a high protein level and low sugar profile. MRI findings revealed a multi-loculated arachnoid cyst at C1-C3 level with pressure affecting the adjacent spinal cord and evidence of myelitis at C3-T1 level. Hemi-larminectomy and removal of the arachnoid cyst were performed, but without improvement. A CSF culture yielded M. tuberculosis, that was susceptible to anti-tuberculous drugs.
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14/33. A Markov computer simulation model of the economics of neuromuscular blockade in patients with acute respiratory distress syndrome.

    BACKGROUND: Management of acute respiratory distress syndrome (ARDS) in the intensive care unit (ICU) is clinically challenging and costly. neuromuscular blocking agents may facilitate mechanical ventilation and improve oxygenation, but may result in prolonged recovery of neuromuscular function and acute quadriplegic myopathy syndrome (AQMS). The goal of this study was to address a hypothetical question via computer modeling: Would a reduction in intubation time of 6 hours and/or a reduction in the incidence of AQMS from 25% to 21%, provide enough benefit to justify a drug with an additional expenditure of $267 (the difference in acquisition cost between a generic and brand name neuromuscular blocker)? methods: The base case was a 55 year-old man in the ICU with ARDS who receives neuromuscular blockade for 3.5 days. A Markov model was designed with hypothetical patients in 1 of 6 mutually exclusive health states: ICU-intubated, ICU-extubated, hospital ward, long-term care, home, or death, over a period of 6 months. The net monetary benefit was computed. RESULTS: Our computer simulation modeling predicted the mean cost for ARDS patients receiving standard care for 6 months to be $62,238 (5%-95% percentiles $42,259-$83,766), with an overall 6-month mortality of 39%. Assuming a ceiling ratio of $35,000, even if a drug (that cost $267 more) hypothetically reduced AQMS from 25% to 21% and decreased intubation time by 6 hours, the net monetary benefit would only equal $137. CONCLUSION: ARDS patients receiving a neuromuscular blocker have a high mortality, and unpredictable outcome, which results in large variability in costs per case. If a patient dies, there is no benefit to any drug that reduces ventilation time or AQMS incidence. A prospective, randomized pharmacoeconomic study of neuromuscular blockers in the ICU to asses AQMS or intubation times is impractical because of the highly variable clinical course of patients with ARDS.
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15/33. Conversion locked-in syndrome after implantation of a spinal cord stimulator.

    BACKGROUND: The locked-in syndrome is defined as quadriplegia and anarthria (loss of articulate speech) with the preservation of consciousness. It is typically caused by a lesion to the ventral pons. conversion disorder is the deficit of voluntary motor or sensory function requiring an extensive work-up to exclude any organic cause. methods AND RESULTS: After surgery for an implantation of a spinal cord stimulator, a 42-year-old woman presented with quadriplegia and lower facial diplegia, but was able to open and blink her eyes. We found no organic causes to explain her condition after appropriate radiological studies looking for intracranial or intraspinal causes, and reversal drugs were administered with no immediate effect. Over the course of several hours, the patient gradually recovered and was discharged the following day. A psychology consultation was obtained during her stay and she was found to meet the criteria for a conversion disorder to explain her condition. CONCLUSIONS: Before considering a psychological cause, all organic factors should be excluded with proper tests and consultations, as conversion disorder is a diagnosis of exclusion. We report a patient who, after implantation of a spinal cord stimulator, manifested locked-in syndrome resulting from a conversion disorder.
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16/33. Toxicological analysis of a fatal baclofen (Lioresal) ingestion.

    A fatality following ingestion of the drug baclofen (Lioresal) is described. baclofen was identified in urine by gas chromatography/mass spectrometry. After derivatization with trinitrobenzene sulfonic acid, baclofen was quantitated in serum and urine by high-performance liquid chromatography. The concentration of baclofen was 17 mg/L in serum and 760 mg/L in urine collected approximately 12 h after the overdose. To our knowledge, this is only the second reported fatality involving a baclofen overdose. The previous case did not include quantitation of baclofen in any biological fluid.
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keywords = overdose, drug
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17/33. 'Duplicate limbs' sensation in acute traumatic quadriplegia.

    A 64-year-old man sustained acute quadriplegia due to a traffic accident, while in the midst of a petit mal seizure. After recovery from the initial medical complications he developed a duplicate limb phenomenon. The patient felt that another pair of upper and lower limbs had grown from his body, parallel to the paralysed limbs. To the best of our knowledge this duplicate phenomenon in all limbs has not been described before in a traumatic quadriplegic patient. It is our impression that this phenomenon is a rare example of preoccupation with the paralytic limbs, of sensory deprivation with a (possible) unusual drug reaction.
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18/33. Successful treatment of hyperkalemic quadriplegia associated with spironolactone.

    Hyperkalemic flaccid quadriplegia and cardiotoxic disturbances developed during antihypertensive therapy with spironolactone in a 76-year-old woman with chronic renal insufficiency. hyperkalemia was successfully overcome and followed by the disappearance of all cardiac and muscular disorders. Progression of the renal disease leading to a decrease of creatinine clearance from 85 ml/min to 4.5 ml/min, during a three-year observation period per se had apparently no influence on the serum level of potassium. Our studies suggested that no primary insufficiency of the renin-aldosterone and glucocorticoid systems had been responsible for the hyperkalemia, therefore, it could be contributed entirely to the effect of spironolactone abolishing the K secreting capacity of the already decreased renal mass. Further studies revealed that blocking action of the drug on H secretion ("renal tubular acidosis") may also have had a role-in addition to the K retention-in the development of the spironolactone-induced hyperkalemia.
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19/33. Urological problems in the management of quadriplegic women.

    During the past 25 years enormous progress has been made in the management of the neuropathic bladder, largely as a result of the adoption of intermittent catheterisation, together with improvements in catheter-related techniques, and the judicious use of antibacterial drugs and sphincter surgery. A few quadriplegic women can be trained to do self-intermittent catheterisation, using a special technique. For the majority of these women, however, there is no practical alternative at present to indwelling catheters. Bladder spasms resulting from the mechanical stimulation of the catheter and/or repeated infections may be difficult to control, and illustrative cases demonstrating some problems encountered are presented. There is an obvious need for an external collecting device for these women. The development in the field of such devices are presented and evaluated.
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20/33. Reversible electroencephalogram changes associated with administration of baclofen in a quadriplegic patient: case report.

    We present the case of a spastic quadriplegic who developed mental symptoms which resolved when his baclofen was discontinued. Of interest was the presence of EEG abnormalities similar to those described in cats receiving this drug. These abnormalities, previously unreported in humans, resolved upon discontinuing baclofen therapy.
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