Infusion of intrathecal baclofen for generalized dystonia in cerebral palsy

1998 ◽  
Vol 88 (1) ◽  
pp. 73-76 ◽  
Author(s):  
A. Leland Albright ◽  
Margaret J. Barry ◽  
Michael J. Painter ◽  
Barbara Shultz

Generalized dystonia occurs in 15 to 25% of persons with cerebral palsy (CP) and responds poorly to medical and surgical treatments. Object. After the authors observed a woman whose dystonic CP was dramatically improved by continuous infusion of intrathecal baclofen, they designed this pilot study to evaluate the effect of this treatment on a group of patients with dystonic CP. Methods. The authors assessed the short-term response to intrathecal baclofen infusion in 12 patients with dystonic CP. An intrathecal catheter was inserted percutaneously and connected to an external microinfusion pump. The infusion began at a rate of 100 µg/day and was increased by 50 µg every 12 hours until the dystonia abated, adverse effects occurred, or the dose reached 900 mg/day with no improvement. Two observers, one blinded and one not blinded to the patient's treatment status, viewed videotapes made before and after the infusions and graded the dystonia in eight body regions, using a 5-point scale. Overall and regional scores were compared by using Wilcoxon signed-rank tests. Conclusions. Dystonia diminished in 10 of 12 patients whose average daily dose of intrathecal baclofen was 575 µg. Overall dystonia scores and scores for the extremities, trunk, and cervical regions were significantly better after infusion (p = 0.003). The two observers' scores were not significantly different. Programmable infusion pumps were subsequently implanted in eight patients for long-term therapy and improvement was sustained in six (p < 0.05). Intrathecal baclofen infusion is a promising treatment option for generalized dystonia associated with CP. The effects of intrathecal baclofen infusion on dystonia can be evaluated by using short-term continuous infusions.

1996 ◽  
Vol 85 (3) ◽  
pp. 452-457 ◽  
Author(s):  
Joe I. Ordia ◽  
Edward Fischer ◽  
Ellen Adamski ◽  
Edward L. Spatz

✓ The aim of this study was to determine the efficacy, safety, and cost-effectiveness of intrathecal baclofen delivered by a programmable pump for the chronic treatment of severe spasticity. Sixty-six patients with severe spasticity of spinal cord origin that was refractory to oral baclofen or who experienced intolerable side effects with this form of the drug were screened. The first nine participated in a double-blinded, randomized, placebo (normal saline)-controlled trial to determine response to a bolus dose of intrathecal baclofen. Subsequent patients were enrolled in an open-label treatment protocol without a placebo trial. All passed the screening, and the pump was implanted in 59 patients. Spasticity scores and medical costs before and after surgery were analyzed. In all patients, the mean Ashworth score for rigidity decreased from 4.3 preoperatively to 1.4 (p < 0.0005) with use of intrathecal baclofen. The spasm frequency score decreased from a mean of 3.6 to 0.5 (p < 0.0005). Activities of daily living, sleep, and skin integrity improved, and pain was eradicated in some. Constipation occurred in six patients. A reduction in dosage was necessitated by muscular hypotonia in three ambulatory patients, areflexic bladder and urinary retention in three others, and nausea, dizziness, and drowsiness in one. Catheter-related problems occurred 19 times in 15 patients. One pump was explanted because of infection in the pump pocket, and one was removed after it eroded through the skin. There were no pump failures. The use of intrathecal baclofen resulted in a decrease in the average length of subsequent hospitalizations. It is concluded that intrathecal baclofen delivered by an implanted programmable pump is a safe, effective, and cost-efficient method for treatment of severe intractable spinal spasticity.


1996 ◽  
Vol 11 (1_suppl) ◽  
pp. S29-S35 ◽  
Author(s):  
A. Leland Albright

Intrathecal baclofen reduces spasticity in individuals with cerebral palsy. Intrathecal doses are far lower than oral doses and the effects are considerably greater, and the side effects are fewer. Response to intrathecal baclofen must be confirmed by a screening trial before implantation of a pump for chronic infusion. Intrathecal baclofen reduces spasticity in the upper and lower extremities and is often associated with improved gait and upper extremity function. Quality of life improves for patients and caregivers. The Medtronic pump has been exceedingly reliable and typically functions for 4 or 5 years. The currently available intrathecal catheter is associated with far fewer complications than the initial catheter. Baclofen overdoses are unusual and are usually caused by pump programming errors rather than pump malfunction. Preliminary studies suggest that continuous intrathecal baclofen infusion reduces generalized dystonia in cerebral palsy. Screening to determine response of dystonia to intrathecal baclofen is by continuous infusion. The doses required to reduce dystonia are higher than those for cerebral spasticity. Additional investigations are underway to quantify the effects of continuous intrathecal baclofen infusion on communication, disability, and dystonia. (J Child Neurol 1996;11(Suppl 1): S29-S35).


1998 ◽  
Vol 88 (6) ◽  
pp. 1009-1013 ◽  
Author(s):  
Peter C. Gerszten ◽  
A. Leland Albright ◽  
Graham F. Johnstone

Intrathecal baclofen infusion (IBI) is an effective treatment for spasticity secondary to cerebral palsy (CP). Object. To assess the need for orthopedic surgery of the lower extremities in such cases, the authors retrospectively reviewed the outcome in 48 patients with spastic CP who were treated with IBI. Methods. Pumps were placed in 40 patients (84%) suffering from spastic quadriplegia and eight patients (16%) with spastic diplegia. The patients' ages ranged from 5 to 43 years (mean 15 years). The mean follow-up period was 53 months (range 24–94 months). The mean baclofen dosage was 306 µg/day (range 25–1350 µg/day). At the time of pump placement, subsequent orthopedic surgery was planned in 28 patients (58%); however, only 10 (21%) underwent surgery after IBI therapy. In all 10 cases, the surgical procedure was planned at the time of initial evaluation for IBI therapy. In the remaining 18 patients, who did not subsequently undergo their planned orthopedic operation, it was believed that their lower-extremity spasticity had improved to the degree that intervention was no longer indicated. In addition, although six patients had undergone multiple orthopedic operations before their spasticity was treated, no patient required more than one operation after IBI treatment for spasticity. Conclusions. The authors conclude that IBI for treatment of spastic CP reduces the need for subsequent orthopedic surgery for the effects of lower-extremity spasticity. In patients with spastic CP and lower-extremity contractures, spasticity should be treated before orthopedic procedures are performed.


1999 ◽  
Vol 90 (5) ◽  
pp. 974-976 ◽  
Author(s):  
Juei-Jueng Lin ◽  
Ging-Yau Lin ◽  
Chunhsi Shih ◽  
Shinn-Zong Lin ◽  
Dar-Cheng Chang ◽  
...  

✓ This 29-year-old man with cerebral palsy complicated by generalized dystonia was treated by simultaneous bilateral posteroventral pallidotomy. Postoperatively, there was slow, but steady, improvement in the patient's dystonia and disability. However, the improvement in abnormal movements was only prominent for cervical dystonia and oromandibular dyskinesia. The patient's Burke-Fahn-Marsden dystonia scores were 51 preoperatively and 37, 33.5 and 33.5, at 3, 6, and 12 months postoperatively, respectively, demonstrating a maximum improvement of 34%. These results suggest that pallidotomy can be an alternative therapy for those patients suffering from intractable generalized dystonia.


1981 ◽  
Vol 55 (6) ◽  
pp. 935-937 ◽  
Author(s):  
Giuseppe Salar ◽  
Salvatore Mingrino ◽  
Marco Trabucchi ◽  
Angelo Bosio ◽  
Carlo Semenza

✓ The β-endorphin content in cerebrospinal fluid (CSF) was evaluated in 10 patients with idiopathic trigeminal neuralgia during medical treatment (with or without carbamazepine) and after selective thermocoagulation of the Gasserian ganglion. These values were compared with those obtained in a control group of seven patients without pain problems. No statistically significant difference was found between patients suffering from trigeminal neuralgia and those without pain. Furthermore, neither pharmacological treatment nor surgery changed CSF endorphin values. It is concluded that there is no pathogenetic relationship between trigeminal neuralgia and endorphins.


2003 ◽  
Vol 98 (1) ◽  
pp. 21-31 ◽  
Author(s):  
Oliver Tucha ◽  
Christian Smely ◽  
Michael Preier ◽  
Georg Becker ◽  
Geraldine M. Paul ◽  
...  

Object. There is presently no specific information available concerning the nature and course of cognitive deficits caused by intracranial meningiomas. In this prospective study the authors examined the cognitive functioning of patients with frontal meningiomas. Methods. Fifty-four patients with frontal meningiomas were examined neuropsychologically before and after neurosurgery. The test battery consisted of standardized instruments including those assessing memory, attention, visuoconstructive abilities, and executive functions. The time period between pre-and postoperative assessment ranged from 4 to 9 months. The patients' performance was compared with the results in 54 healthy adults who were also assessed twice by using the same test battery in a period ranging from 4 to 9 months. In addition, the effect on cognition of meningioma lateralization, localization, lesion size, edema, brain compression, time course, and the occurrence of preoperative seizures was analyzed. Conclusions. Except in the case of working memory, comparisons of pre- and postoperative assessments of cognition revealed no differences in memory, visuoconstructive abilities, or executive functions, although a postoperative improvement in attentional functions was observed. The results of this study indicate that the surgical removal of frontal meningiomas does not impair patients' cognitive functioning. Furthermore, improvements in attentional functions may occur in these patients.


1977 ◽  
Vol 46 (4) ◽  
pp. 506-511 ◽  
Author(s):  
Richard D. Penn ◽  
Mary Liz Etzel

✓ The changes in motor function in cerebral palsy patients produced by chronic anterior lobe cerebellar stimulation were documented with the Milani-Comparetti developmental scale, which allows comparison between functional gains and reflex patterns. Two patients with marked motor retardation and primitive reflexes were followed serially over several months of stimulation. Using these measures, the efficiency of chronic stimulation can be assessed, although many years will be needed to evaluate its role in treating motor disabilities.


1997 ◽  
Vol 86 (1) ◽  
pp. 139-142 ◽  
Author(s):  
Martin M. Bednar ◽  
Cordell E. Gross ◽  
Sheila R. Russell ◽  
David Short ◽  
Patricia C. Giclas

✓ Although complement activation is associated with tissue injury during inflammatory and ischemic states, complement activation in states of acute cerebral ischemia before and after administration of tissue plasminogen activator (TPA) has not yet been examined and is the focus of this investigation. Twenty-four New Zealand White rabbits weighing 3 to 3.5 kg were used for this study. Of these, 20 were subjected to intracranial autologous clot embolization via the internal carotid artery. Three hours postembolization, rabbits received an intravenous infusion of TPA (6.3 mg/kg, 20% bolus with the remainder infused over a 2-hour interval; 12 animals) or vehicle (eight animals). All animals were observed for a total of 7 or 8 hours postembolization. These two groups were compared to a cohort undergoing sham operation with subsequent TPA infusion (four animals). Plasma samples to quantify complement component C5 hemolytic activity (C5H5O) were obtained at the following time points: 30 minutes before and after clot embolization; 1 hour before and 1 hour after the initiation of therapy with TPA or vehicle and at the completion of the protocol; 7 to 8 hours after clot embolization. The C5 activation was not detected as the result of acute cerebral ischemia. However, animals receiving TPA with or without concomitant clot embolization exhibited C5 activation as assessed by a reduction in C5 hemolytic function, both 1 hour after initiation of TPA infusion (78.7 ± 10.3% and 77.5 ± 9.9% of baseline value, respectively; mean ± standard error of the mean [SEM]) and at the end of the protocol, 2 hours after the completion of the TPA infusion (72.5 ± 8.8% and 53.3 ± 8.1%, respectively; mean ± SEM, p < 0.05, each group). This study supports the conclusion that TPA, but not acute cerebral ischemia, may activate the complement cascade in this rabbit model of thromboembolic stroke.


2005 ◽  
Vol 102 ◽  
pp. 234-240 ◽  
Author(s):  
Chihiro Ohye ◽  
Tohru Shibazaki ◽  
Sumito Sato

Object.The authors studied the effects of gamma knife thalamotomy (GKT) on Parkinson disease-related tremor and essential tremor before and after reloading of radioactive cobalt.Methods.Based on experience in stereotactic thalamotomy aided by depth microrecording, the target was located at the lateral border of the thalamic ventralis intermedius nucleus (VIM). For more precise targeting, the percentage representation of the thalamic VIM in relation to the entire thalamic length is useful. The location of the target was determined on magnetic resonance (MR) imaging and computerized tomography scanning. A maximum dose of 130 Gy was delivered to the target by using a single isocenter with the 4-mm collimator. In more recent cases, a systematic follow-up examination was performed at 3, 6, 12, 18, and 24 months after GKT.Since 1993, the authors have treated 70 patients with PD. Throughout the series the same dosimetric technique has been used. The course after GKT was compared between the 25 cases with PD treated before reloading and the 35 cases treated after reloading. In the majority (80–85%) treated after reloading, tremor and rigidity were reduced around 6 months after GKT. In the cases treated before reloading this effect took approximately 1 year. The thalamic reaction on MR imaging showed the same two lesion types in both series: a restricted and a diffuse. After reloading the restricted lesion was more frequent and the lesion volume was smaller.Conclusions.The shorter delay in clinical improvement and smaller lesion size may be related to an increased radiation dose.


2004 ◽  
Vol 100 (1) ◽  
pp. 2-6 ◽  
Author(s):  
Vaijayantee Kulkarni ◽  
Vedantam Rajshekhar ◽  
Lakshminarayan Raghuram

Object. The authors studied whether cervical spine motion segments adjacent to a fused segment exhibit accelerated degenerative changes on short-term follow-up magnetic resonance (MR) imaging. Methods. Preoperative and short-term follow-up (mean duration 17.5 months, range 10–48 months) cervical MR images obtained in 44 patients who had undergone one- or two-level corpectomy for cervical spondylotic myelopathy were evaluated qualitatively and quantitatively. The motion segment adjacent to the fused segment and a segment remote from the fused segment were evaluated for indentation of the thecal sac, disc height, and sagittal functional diameter of the spinal canal on midsagittal T2-weighted MR images. Thecal sac indentations were classifed as mild, moderate, and severe. New indentations of the thecal sac of varying severity (mild in 17 patients [38.6%], moderate in 10 [22.7%], and severe in six [13.6%]) had developed at the adjacent segments in 33 (75%) of 44 patients. The degenerative changes were seen at the superior level in 11 patients, inferior level in 10 patients, and at both levels in 12 patients and resulted from both anterior and posterior element degeneration in the majority (23 [69.6%]) of patients. The remote segments showed mild thecal sac indentations in seven patients and moderate indentations in two patients (nine [20.5%] of 44). Compared with the changes at the remote segment, the canal size was significantly decreased at the superior adjacent segment by 0.9 mm (p = 0.007). No patient sustained a new neurological deficit due to adjacent-segment changes. Conclusions. On short-term follow-up MR imaging, levels adjacent to the fused segment exhibited more pronounced degenerative changes (compared with remote levels) in 75% of patients who had undergone one- or two-level central corpectomy.


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