Clinical Evidence in Spastic Hypertonia Associated with Cerebral Palsy

Krach LE, Nettleton A, Klempka B. Satisfaction of individuals treated long-term with continuous infusion of intrathecal baclofen by implanted programmable pump. Pediatr Rehabil. 2006;9(3):210-218.

Methods

• N = 100 (88 had CP)
• Subject and/or caregiver survey
• Subjects had undergone ITB Therapy pump implantation 1 to 4 years prior to the study and were still receiving therapy at survey completion
• Patient age range: 5 to 42

Results

• Greater than half reported an improvement in the following motor skills: walking (74%), ability to position (70%), ability to transfer (59%) and use of arms (53%)
• More than half noted improvements in dressing (70%) and toileting (53%) and feeding improved in 48%
• 71% reported that their goals had been fully met; 14% reported that their goals had been partially met; 15% reported that they were unsure or that their goals were not met
• Nearly half (47%) reported an improvement in general outlook on life
• 82% indicated they would repeat their decision to implant the pump
• 29% experienced improved trunk control while 28% experienced decreased trunk control
• Drooling improved in 13% of patients, but worsened in 27%
• Increases in participation in a variety of activities was noted, with 57% reporting increased participation in recreational activities
• Several patients cited concerns with pump size and the frequency of follow-up needed for refills
• 21% experienced decreased bowel control and 12% reported improved bowel control
• Frustration improved in 21% of patients but worsened in 22%
• 22 subjects experienced 32 events related to system hardware or surgery, including catheter obstruction, migration or fracture (12%) and catheter-pump connector tear (7%)
• 6% of subjects developed infections and required explantation of the pump. All opted to have pumps re-implanted.

Krach LE, Kriel RL, Gilmartin RC, et al. GMFM 1 year after continuous intrathecal baclofen infusion. Ped Rehabil. 2005;8 (3):207-213.

Methods

• N = 31
• Prospective, open-label, non-controlled multicenter study
• All subjects had a diagnosis of spastic cerebral palsy with Ashworth scores > 3 in lower extremities
• Patient age range: 4 to 29

Results

• GMFM scores were improved in all patients at 12 months post-implant in all dimensions except running and walking
• There was a steady decrease in the mean improvement in GMFM as improvement in AS decreased
• Statistically significant decrease in Ashworth scores for CP classes II to V (p < 0.05)
• Subjects and caregivers most often reported improvement in motor control, positioning, and endurance; less than half observed improvement in speech, oral control, self-cares, transfers, or walking
• Drug-related adverse events were similar to those previously noted in an earlier report of this group of subjects related to hip location (Krach LE, et al. Hip status in cerebral palsy after one year of continuous intrathecal baclofen infusion. Pediatr Neurol. 2004;30(3):163-168)
• System complications were not reported

Gooch JL, Oberg WA, Grams B, Ward LA, Walker ML. Care provider assessment of intrathecal baclofen in children. Dev Med Child Neurol. 2004;46(8):548-552.

Methods

• N = 80 (62 had CP with severe spasticity of cerebral origin)
• Case series of consecutive patients
• Received ITB Therapy for a minimum of 1 year at time of evaluation
• Patient age range: about 4 to 21

Results

• Change in tone reduction of 0 to 0.9 in upper and of 1 to 1.9 in lower extremities as measured by Ashworth Scale
• 84% (43/51) experienced no loss of range of motion; 16% (8/51) experienced some loss of range of motion
• Patients who chose the following goals showed these improvements:
• 91% experienced decreased pain/improved comfort
• 91% prevented worsening of deformity
• 88% experienced improvement in ease of care
• 81% strongly agreed they would have the procedure performed again; 14% slightly agreed
• All care providers reported improvement scores in the Caregiver Questionnaire
• Non-system related complications included cognitive status change (2), severe spasms (1), and overdose (1)
• A total of 70 device-related complications requiring surgery occurred in 63 patients. The most common in this study were catheter occlusion/angulation (12), dislodgement (12), and disconnection at the pump (11), catheter tear/separation (9), and pump pocket infection with removal (8)
• The chance of requiring a repeat surgery due to complications was 19%
• The most common system related complications not requiring surgery included CSF leak, flipped pump, and suture site inflammation
• 4 patients died of causes unrelated to the pump

Krach LE, Kriel RL, Gilmartin RC, et al. Hip status in cerebral palsy after one year of continuous intrathecal baclofen infusion. Pediatr Neurol. 2004;30(3):163-168.

Methods

• N = 33
• Prospective, non-controlled, open-label multicenter study
• All subjects had a diagnosis of spastic cerebral palsy and had Ashworth scores > 3 in their lower extremities
• Patient age range: 4 to 31

Results

• 90.9% of hips showed no deterioration or had improvement of their migration percentage class
• Average change in absolute migration percentage for all patients was 1.3%, which is statistically less than the expected 5% reported in literature (p = 0.006)
• At least 2/3 of hips in all CP classifications of acetabular index remained unchanged
• Mean lower extremity Ashworth score decreased from 3.56 at baseline to 2.17 at 12 months post-implant (p = 0.0001)
• 12% of hips in all CP classifications improved
• 1/3 of the hips manifested progression in hip subluxation
• In nonambulatory subjects with CP grades III to V, 62.5% were unchanged or better with regard to change of absolute migration percentage
• 1/3 of hips with initial migration percentage > 30 improved after ITB Therapy infusion
• 12% of hips with initial migration percentage < 30 got worse
• 29 of 33 subjects experienced at least 1 adverse event within the first year post-implant, some of which may have been drug-related. 5 subjects had serious drug-related adverse events
• 19 subjects had 29 system or operative-related complications within the year after implantation. 5 subjects experienced 2 or more such complications during the year

Albright AL, Gilmartin R, Swift D, Krach LE, Ivanhoe CB, McLaughlin JF. Long-term intrathecal baclofen therapy for severe spasticity of cerebral origin. J Neurosurg. 2003;98(2):291-295.

Methods

• N = 68 (54 had CP)
• Prospective, open-label, multicenter, follow-up study
• Patients were examined at least every 3 months and observed for an average of 70 months

Results

• Spasticity in both upper and lower extremities decreased significantly (p < 0.005)
• Upper extremity assessed at 6, 12, and 24 months (p < 0.001)
• Lower extremity assessed at 6, 12, 18, 24, 30, and 36 months (p < 0.0001)
• Number of patients available for analysis at 7 to 10 years was too small for statistically significant analysis, but the average Ashworth scores in that timeframe were similar to scores in the first 6 years of follow-up
• 2/3 of the 68 patients experienced at least 1 adverse event. The most commonly reported drug-related adverse events were hypotonia, somnolence, nausea and vomiting, headache, and seizures
• 8 of the 9 patients experiencing seizures did so before receiving ITB Therapy; the seizure frequency did not appear to change
• The most commonly reported adverse events in the titration phase were hypotonia (17.6%), somnolence (16.2%), nausea and vomiting (11.8%), and headache (10.3%)
• The most commonly reported adverse events in the maintenance phase were hypotonia (25%), somnolence (19.1%), and seizures (13.2%)
• The most common procedural complications (occurred within 60 days after operation) were seromas (16.2%) and CSF leaks (14.7%) 
• The most common system-related complications were associated with the catheters: 16.2% fractured, 8.8% kinked or occluded, and 5.9% dislodged
• 2 deaths occurred; both were unrelated to the device. 3 pumps were explanted due to infection. 3 patients withdrew from the study due to complications.

Awaad Y, Tayem H, Munoz S, Ham S, Michon AM, Awaad R. Functional assessment following intrathecal baclofen therapy in children with spastic cerebral palsy. J Child Neurol. 2003;18(1):26- 34.

Methods

• N = 55 (39 received ITB Therapy)
• Prospective, open-label assessment up to 18 months
• All patients had a diagnosis of cerebral palsy with severe spasticity in the lower extremities
• Patients had to undergo a trial of oral anti-spasmodics for at least 6 months
• Patient age range: 4 to 32

Results

• Ashworth scores decreased from 3.26 ± 0.61 to 1.46 ± 0.59 after the bolus injection of ITB (p < 0.0005). Ashworth scores at 18 months were 1.60 ± 0.49 (p < 0.0005)
• PEDI scores: self-care domain increased from 34.18 ± 22.44 at baseline to 37.91 ± 29.00 at 18 months (p = 0.020)
• PEDI scores: mobility domain increased from 25.44 ± 20.41 at baseline to 31.02 ± 24.63 at 18 months (p = 0.023)
• 14 families reported better sleeping at night, better speech and communication were described for 3, better hand and head control for 1 patient, better use of walker in another, and increased attention span in 1; 3 families stated that weight gain occurred after pump implant. 1 family found seizure intensity decreased
• The most common drug-related adverse events reported were nausea (4) and constipation (6)
• Seizure frequency increased in 2; new onset seizures reported in 2 (all had underlying brain pathology)
• System and surgical procedure complications included meningitis (2), seroma (7), flipped pump (3), infections (3), and catheter dislodgement (3 events in 2 patients)
• The pump was removed in 4 patients due to adverse effects and/or lack of effect

Meythaler JM, Guin-Renfroe S, Law C, Grabb P, Hadley MN. Continuously infused intrathecal baclofen over 12 months for spastic hypertonia in adolescents and adults with cerebral palsy. Arch Phys Med Rehabil. 2001;82(2):155-161.

Methods

• N = 13
• Case series
• CP patients with intractable spastic hypertonia
• All patients either failed or experienced side effects from oral anti-spasticity medications
• Patient age range: 13 to 43

Results

• Average Ashworth scores (for upper and lower extremity) decreased (p < 0.0001)
• Lower extremity scores decreased from 3.4 ± 1.2 to 1.5 ± 0.7 at 12 months (p < 0.001)
• Upper extremity scores decreased from 3.0 ± 1.2 to 1.7 ± 1.0 (p < 0.0001)
• Lower extremity spasms decreased from 1.4 ± 1.6 to 0.6 ± 1.2 (p = 0.1024) and upper extremity spasms decreased from 1.2 ± 1.6 to 0.2 ± 0.6 (p = 0.0135) at 12 months
• Reflex scores decreased from 2.5 ± 1.2 to 0.7 ± 1.1 (p < 0.001) in the lower extremities and from 2.3 ± 0.7 to 0.5 ± 0.9 (p < 0.0001) in the biceps at 12 months
• All dependent patients were more comfortable, and easier to manage at home with regard to hygiene, ADLs, and assisted transfers
• 3 patients had improvement in their dysarthria
• Adverse events include: Postoperative headaches with nausea (2) and constipation (2)
• 2 patients developed seizures approximately 1 year after pump placement; there was no prior documented history of seizures in either of these patients

Gerszten PC, Albright AL, Johnstone GF. Intrathecal baclofen infusion and subsequent orthopedic surgery in patients with spastic cerebral palsy. J Neurosurg. 1998;88(6):1009-1013.

Methods

• N = 48 (40 received ITB Therapy)
• Retrospective analysis
• Spastic cerebral palsy patients
• Patient age range: 5 to 43

Results

• Orthopedic surgery was planned in 28 patients; 18 patients did not undergo their planned orthopedic operation after receiving ITB Therapy
• 6 patients had undergone multiple orthopedic procedures before their spasticity was treated; no patient required more than 1 operation following ITB Therapy
• 11 patients (23%) required a revision of their pump for malfunction secondary to catheter-related problems, including catheter fracture and catheter dislocation
• Drug-related adverse events were not reported in this article

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