Long term effect (more than five years) of intrathecal baclofen on impairment, disability, and quality of life in patients with severe spasticity of spinal origin.

OBJECTIVES: To evaluate long term change in impairment, disability, and health related functional status in patients with severe spasticity who received intrathecal baclofen. METHODS: A long term (more than five years) observational longitudinal follow up study assessing 21 patients who received intrathecal baclofen given by programmable pump. Patients had chronic disabling spasticity which did not respond to oral antispasmolytic agents. Clinical efficacy was assessed by the Ashworth scale and spasm score; disability by the expanded disability status scale (EDSS), ambulation index (AI), and incapacity status scale (ISS); and health related quality of life by the sickness impact profile (SIP) and the Hopkins symptom checklist (HSCL). RESULTS: Compared with pretreatment values, there was a significant improvement in clinical efficacy (Ashworth scale and spasm score, p<0.05) but a small but significant worsening of disability (EDSS, AI, and ISS, p<0.05). Comparing pretreatment with 26 weeks after pump implantation, a worsening was observed in disability (EDSS and ISS, p<0.05) and perceived health status (SIP, psychosocial dimension, p<0.05). CONCLUSIONS: Long term administration of intrathecal baclofen delivered by an implanted programmable pump resulted in improved clinical efficacy but not in improvement in disability or perceived health status.

Cisatracurium pharmacodynamics in patients with oculopharyngeal muscular dystrophy.
Caron MJ, Girard F, Girard DC, Boudreault D, Brais B, Nassif E, Chouinard P, Ruel M, Duranceau A.
Department of Anesthesiology, Centre Hospitalier de l'Universite de Montreal, Hopital Notre-Dame, 1560 Sherbrooke E., Montreal, Quebec, Canada H2L 4M1.
The pharmacodynamics of muscle relaxants / relaxant in patients with oculopharyngeal muscular dystrophy (OPMD) have never been studied. We designed this study to compare the pharmacodynamics of cisatracurium in OPMD patients versus a control group. Forty patients were enrolled: 20 OPMD patients requiring general anesthesia for cricopharyngeal myotomy and 20 age-matched controls undergoing an operation of similar duration and expected blood loss. Anesthesia was standardized, and both groups received a bolus of cisatracurium 0.1 mg/kg. Onset time, time to 10% T1 recovery, and the intervals 10%-25% and 25%-75% were calculated for both groups. A subgroup analysis was performed in patients with a more severe form of OPMD. Demographic and intraoperative data were similar. Onset time was significantly longer in OPMD patients compared with the control group (4.6 +/- 1.5 min versus 3.4 +/- 1.0 min; P = 0.001). There was no difference in recovery times or indices between groups, regardless of the severity of the disease. In conclusion, there was no difference in the duration of a cisatracurium-induced neuromuscular block between OPMD patients and a control group. A delayed onset of action of the drug may occur.


Extended thymectomy in patients with myasthenia gravis with high thoracic epidural anesthesia alone.
Tsunezuka Y, Oda M, Matsumoto I, Tamura M, Watanabe G.
Department of General and Cardiothoracic Surgery, Kanazawa University School of Medicine, Takaramachi 13-1, Kanazawa 920-8641, Japan. tsuney@nifty.com
Successful extended thymectomy was performed in three patients with myasthenia gravis under only high thoracic epidural anesthesia with voluntary breathing. It was not necessary to intubate a tracheal tube during operation for any of the patients. Neither muscle relaxants / relaxant nor volatile anesthetic agents were required. The mean operating time was 2.0 +/- 0.5 hours. The drainage tubes were removed the day after operation in all patients. In two patients the arterial oxygen saturation (SaO2) and the arterial partial pressure of carbon dioxide (PaCO2) and oxygen (PaO2) were stable; in the third patient the SaO2 was temporarily decreased to 92 mmHg when bilateral mediastinal pleura were opened. The right pleural defect was then covered with a large wet towel, which was pressed on the defect, and thoracic drainage was performed. The left pleural defect was repaired with 3-0 Vicryl after suctioning the air in the pleural space, after which the SaO2 recovered. All patients were able to drink water and walk within 1 hour after the operation. This procedure is advantageous in that the use of muscle relaxants / relaxant and volatile anesthetic agents prevented the laryngeal injury that results from translaryngeal intubation; in turn we avoided causing postoperative respiratory insufficiency. This may be suitable for the operation of some patients with MG, but further studies are required to define the indication.

Spasticity in adults living in a developmental center.

OBJECTIVES: To ascertain the prevalence of spasticity among adults living in a developmental center and to document the development of spasticity treatment plans for this population. DESIGN: Descriptions of the clinical features of medical disorders and a prevalence survey. SETTING: Residential developmental center. PARTICIPANTS: One hundred three adults. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Characteristics described included the prevalence of spasticity in this population, the specific spasticity diagnosis, functional goals for spasticity treatment identified by the participants' multidisciplinary teams, and the specific treatment indicated by the neurologist. RESULTS: Of the 103 people diagnosed by the neurologist, 24 had diplegic spasticity, 4 had hemiplegic spasticity, 44 had quadriplegic spasticity, and 31 had no spasticity. Functional goals identified by multidisciplinary teams were undergarment change (46.3% of the persons for whom goals were identified), splinting hands (11%), dressing (57.4%), hygiene (20.4%), wheelchair positioning (25.9%), ambulation improvement (14.8%), and transfers (9.3%). After physical and occupational therapy, the first invasive treatments indicated for people with spasticity included botulinum toxin injections (60%), intrathecal baclofen (26.4%), orthopedic surgery (5.6%), and medication (1.4%). No treatment was recommended for 25% of the spasticity patients. CONCLUSIONS: The prevalence of spasticity was 35% in this developmental center population of 205 individuals. A multidisciplinary team approach to the evaluation of adults with spasticity who live in a developmental center makes it possible to identify functional goals that are amenable to spasticity treatment and minimizes treatment that does not target specific functional goals.

Ryanodine receptors in peritoneal mast cells: possible role in the modulation of exocytotic activity.

Previous studies have shown that ryanodine in low concentrations and caffeine increase intracellular [Ca(2+)] in the absence of external Ca(2+), suggesting Ca(2+) release from intracellular stores through ryanodine receptors (RyR). In the present study we employed amperometry to examine the effect of RyR agonists and antagonists on serotonin release elicited with compound 48/80 (10 micro g/ml). Ryanodine (1 micro M) or, similarly, 20 mM caffeine, in the absence of external Ca(2+), enhanced the amperometric response to compound 48/80 and all the individual amperometric spike parameters. Ryanodine (50 micro M), dantrolene (20 micro M) and tetracaine (50 micro M), putative antagonists of the RyR, attenuated the amperometric response significantly, decreasing the number and frequency of events as well as their amplitude. This is the first demonstration that Ca(2+) availability from RyR-operated Ca(2+) sources may contribute to the modulation of secretory activity in mast cells, affecting not only the cellular exocytotic response, but also the characteristics of single amperometric events. Immunocytochemical labelling, using a monoclonal RyR antibody, confirmed the presence of RyR in this preparation.

Subdural catheter migration may lead to baclofen pump dysfunction.

OBJECTIVES: To report an unusual cause of intrathecal drug delivery failure in baclofen pump device. STUDY DESIGN: A case report of an SCI patient treated with intrathecal baclofen, presenting a drug withdrawal. SETTING: Regional spinal cord injuries centre in Geneva (Switzerland). METHODS: We present a case of a 38-year-old male with complete T9 spastic paraplegia for 15 years, treated with intrathecal baclofen for 11 years. He recently presented to our centre with a spastic hypertonic episode, associated with rhabdomyolysis. RESULTS: Standard investigations were unrevealing. However, a CT scan performed after injecting a radio-opaque solution by the side port of the pump, showed an unexpected catheter migration into the subdural space. Surgical revision reversed withdrawal symptoms. CONCLUSIONS: Subdural catheter migration must be considered in the differential diagnosis of intrathecal drug delivery system failures. We recommend the use of the CT scan after contrast injection, to detect the localization of the distal catheter tip and confirm the normal diffusion into the subarachnoid space.

Protective effects of dantrolene against myocardial injury induced by isoproterenol in rats: biochemical and histological findings.
Acikel M, Buyukokuroglu ME, Erdogan F, Aksoy H, Bozkurt E, Senocak H.
Department of Cardiology, Faculty of Medicine, Ataturk University, 25240 Erzurum, Turkey. macikel@atauni.edu.tr
PURPOSE: We investigated whether dantrolene might protect the heart against myocardial injury (MI) induced by isoproterenol (ISO), using an experimental model in rats. METHODS: Twenty-eight rats were randomized to treatment with saline only (control group, n=8), ISO only (ISO group, n=8), low-dose dantrolene (LDD)+ISO (LDD group, n=6) and high-dose dantrolene (HDD)+ISO (HDD group, n=6). ISO (150 mg/kg/day, s.c.), LDD (5 mg/kg/day, i.p.) and HDD (10 mg/kg/day, i.p.) were given once a day for two consecutive days. At the end of the second day, blood samples were taken from abdominal aorta shortly after the rats were anesthetised for cardiac troponins T (cTnT) and I (cTnI) assay, and the hearts were removed and observed microscopically. RESULTS: cTnT and cTnI levels were increased in the ISO group when compared with the control group (p<0.001). LDD and HDD significantly reduced cTnT and cTnI levels when compared with the ISO group. Elevations of cTnT and cTnI appeared to relate to the severity of histological changes. The rate of animals that exhibited marked MI was higher in the ISO group than in the control group (p<0.001). The rats in both LDD and HDD groups showed less histological changes when compared to the ISO group (p<0.01). There was no significant difference between the control group and both LDD and HDD groups. CONCLUSIONS: This study shows that dantrolene has a significant effect in the protection of the heart against MI induced by ISO. We believe that pretreatment with dantrolene may contribute to developing novel strategies in the cardiotoxicity animal models and in the prevention of the cardiotoxic effects of elevated levels of catecholamines.

Can first responders achieve and maintain normocapnia when sequentially ventilating with a bag-valve device and two oxygen-driven resuscitators? A controlled clinical trial in 104 patients.

BACKGROUND AND OBJECTIVE: To evaluate the capability of first responders to achieve and maintain normal ventilation of the lungs of victims employing a bag-valve device and two oxygen-driven resuscitators. METHODS: Prospective, controlled, blinded, single-centre clinical trial using a bag-valve device and one of two FR-300 devices, with 20 cmH2O working pressure, and flows of either 24 or 30 L min(-1). One hundred and four patients were analysed. Induction of anaesthesia followed by ventilation of the lungs with a bag-valve device and an Oxylator in manual and automatic modes performed by a fireman first responder. Each series was repeated for three conditions (anaesthesia; anaesthesia plus muscle relaxation, both with facemask; anaesthesia plus relaxation using an endotracheal tube). RESULTS: Patients age 49 +/- 17 yr; 47% males, 48-132 kg. Normocapnia was achieved and maintained in 66% (bag-valve device), 82% (Oxylator). CONCLUSIONS: The use of an oxygen-driven device improves the ability of first responders to achieve and maintain normocapnia even when distracted. Use of the Oxylators improves performance (P < 0.001) vs. the bag-valve device significantly.

Anesthesia for cesarean section in a patient with spinal muscular atrophy.

We describe the anesthetic management for cesarean section and tubal ligation of a 23-year-old primipara with type II spinal muscular atrophy (benign Werdnig Hoffmann). She was wheelchair-bound, had severe restrictive lung disease, and severe kyphoscoliosis, with Harrington rods extending from the thoracic to the sacral spines. A general anesthetic was given. We used propofol and alfentanil for rapid-sequence induction of anesthesia. We did not use any muscle relaxants / relaxant intraoperatively. Postoperative care was provided in the intensive care unit. The patient made a good recovery.

Quinine associated blindness.

BACKGROUND: Quinine is commonly prescribed to the elderly for the treatment of benign nocturnal cramps, yet its use is not without complications. OBJECTIVE: This article presents a case of quinine toxicity producing bilateral blindness, followed by a review of the adverse reactions associated with quinine use and its efficacy in treating benign nocturnal muscular cramps. DISCUSSION: Visual loss has been associated with quinine serum concentrations above 10 microg/mL (therapeutic range 2-5 microg/mL). Other adverse reactions include neurological symptoms, haemolysis, acute renal failure and arrhythmia. There is conflicting evidence for the efficacy of quinine for leg cramps in randomised controlled studies, however, meta-analysis of these studies suggests some benefit. Although severe side effects are rare at therapeutic doses, the possibility of overdose needs to be considered when prescribing and an individual risk benefit analysis needs to be made. Benefits and adverse reactions should be closely monitored and medication ceased if appropriate.