Jay M. Meythaler

Practice parameter: immunotherapy for Guillain-Barré syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology.

Objective: To provide an evidence-based statement to guide physicians in the management of Guillain–Barré syndrome (GBS). Methods: Literature search and derivation of evidence-based statements concerning the use of immunotherapy were performed. Results: Treatment with plasma exchange (PE) or IV immunoglobulin (IVIg) hastens recovery from GBS. Combining the two treatments is not beneficial. Steroid treatment given alone is not beneficial. Recommendations: 1) PE is recommended for nonambulant adult patients with GBS who seek treatment within 4 weeks of the onset of neuropathic symptoms. PE should also be considered for ambulant patients examined within 2 weeks of the onset of neuropathic symptoms; 2) IVIg is recommended for nonambulant adult patients with GBS within 2 or possibly 4 weeks of the onset of neuropathic symptoms. The effects of PE and IVIg are equivalent; 3) Corticosteroids are not recommended for the management of GBS; 4) Sequential treatment with PE followed by IVIg, or immunoabsorption followed by IVIg is not recommended for patients with GBS; and 5) PE and IVIg are treatment options for children with severe GBS.

Long-term continuously infused intrathecal baclofen for spastic-dystonic hypertonia in traumatic brain injury: 1-year experience☆☆☆

OBJECTIVEnTo determine if the long-term use of continuously infused intrathecal baclofen (ITB) over a 1-year period will control spastic-dystonic hypertonia in patients with traumatic brain injury (TBI).nnnSETTINGnTertiary care outpatient and inpatient rehabilitation center directly attached to a university hospital.nnnSUBJECTSnPersons with TBI and intractable spasticity and dystonia for more than 6 months duration recruited in a consecutive manner.nnnDESIGNnTBI patients were admitted to the study after screening via a bolus injection of either intrathecal normal saline or 50 microg of baclofen. Data for Ashworth rigidity scores, spasm scores, and deep tendon reflex scores were collected for both the upper extremities (UE) and lower extremities (LE). Patients whose LE Ashworth scores decreased an average of 2 points were then offered implantation of a computer-controlled pump for continuous ITB. Changes over time were assessed statistically via Friedmans analysis for ordinal data and ANOVA for linear data. Differences between set points in time were also assessed via Wilcoxon signed rank.nnnDATA SETnSeventeen patients (average age 29+/-11 yrs) with spasticity and/or dystonia treated over 1 year via a computer-controllable intrathecal delivery system for the delivery of ITB.nnnRESULTSnAfter 1 year of continuous ITB treatment the average LE Ashworth score decreased from 3.5+/-1.3 (SD) to 1.7+/-0.9 (p < .0001), spasm score from 1.8+/-1.3 to 0.2+/-0.5 (p< .0001), and reflex score from 2.5+/-1.1 to 0.1+/-0.3 (p < .0001). The average UE Ashworth score decreased from 2.9+/-1.5 to 1.6+/-1.0 (p < .0001), spasm score from 1.2+/-1.5 to 0.2+/-0.6 (p < 0.0001), and reflex score from 2.2+/-0.5 to 1.0+/-0.8 (p < .0001). The average ITB dose required to attain these effects at 1 year was 302 microg continuously infused per day.nnnCONCLUSIONnContinuous intrathecal infusion of baclofen is capable of maintaining a reduction in spasticity and dystonia in both the upper and lower extremities of TBI patients.

Rehabilitation of Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) is the most common cause of acute neuromuscular paralysis in developed countries. GBS is a significant cause of new long-term disability for at least 1,000 persons per year in the United States, and more elsewhere. Given the young age at which GBS sometimes occurs and the relatively long life expectancies following GBS, it is likely that at least 25,000 and perhaps 50,000 persons in the US are experiencing some residual effects of GBS. Approximately 40% of patients who are hospitalized with GBS will require admission to inpatient rehabilitation. For GBS persons necessitating admission to inpatient rehabilitation, the requirement of prior ventilator support most strongly predicts an extended length of stay on inpatient rehabilitation. Other issues that affect rehabilitation are dysautonomia, cranial nerve involvement, and various medical complications associated with GBS. Deafferent pain syndrome is common in the early stages of recovery. Multiple medical complications, including deep venous thrombosis, joint contractures, hypercalcemia of immobilization, and decubitii, may develop in the early stages of recovery and interfere with the rehabilitation program. Anemia is a frequent finding in the first few months of illness but does not appear to interfere with functional recovery. Therapy should not overfatigue the motor unit, which has been associated with paradoxical weakening. Little is known of the long-term implications of the disability caused by GBS. Work similar to that performed for postpolio syndrome and spinal cord injury should be started in the rehabilitation setting.

Cyproheptadine for intrathecal baclofen withdrawal

OBJECTIVEnTo evaluate the efficacy of cyproheptadine in the management of acute intrathecal baclofen (ITB) withdrawal.nnnDESIGNnDescriptive case series.nnnSETTINGnUniversity hospital with a comprehensive in- and outpatient rehabilitation center.nnnPARTICIPANTSnFour patients (3 with spinal cord injury, 1 with cerebral palsy) with implanted ITB infusion pumps for treatment of severe spasticity, who had ITB withdrawal syndrome because of interruption of ITB infusion.nnnINTERVENTIONSnPatients were treated with 4 to 8mg of cyproheptadine by mouth every 6 to 8 hours, 5 to 10mg of diazepam by mouth every 6 to 12 hours, 10 to 20mg of baclofen by mouth every 6 hours, and ITB boluses in some cases.nnnMAIN OUTCOME MEASURESnClinical signs and symptoms of ITB withdrawal of varying severity were assessed by vital signs (temperature, heart rate), physical examination (reflexes, tone, clonus), and patient report of symptoms (itching, nausea, headache, malaise).nnnRESULTSnThe patients in our series improved significantly when the serotonin antagonist cyproheptadine was added to their regimens. Fever dropped at least 1.5 degrees C, and heart rate dropped from rates of 120 to 140 to less than 100bpm. Reflexes, tone, and myoclonus also decreased. Patients reported dramatic reduction in itching after cyproheptadine. These changes were associated temporally with cyproheptadine dosing.nnnDISCUSSIONnAcute ITB withdrawal syndrome occurs frequently in cases of malfunctioning intrathecal infusion pumps or catheters. The syndrome commonly presents with pruritus and increased muscle tone. It can progress rapidly to high fever, altered mental status, seizures, profound muscle rigidity, rhabdomyolysis, brain injury, and death. Current therapy with oral baclofen and benzodiazepines is useful but has variable success, particularly in severe cases. We note that ITB withdrawal is similar to serotonergic syndromes, such as in overdoses of selective serotonin reuptake inhibitors or the popular drug of abuse 3,4-methylenedioxymethamphetamine (Ecstasy). We postulate that ITB withdrawal may be a form of serotonergic syndrome that occurs from loss of gamma-aminobutyric acid B receptor-mediated presynaptic inhibition of serotonin.nnnCONCLUSIONnCyproheptadine may be a useful adjunct to baclofen and benzodiazepines in the management of acute ITB withdrawal syndrome.

Cost-effectiveness of routine screening for proximal deep venous thrombosis in acquired brain injury patients admitted to rehabilitation.

OBJECTIVEnDetermine the prevalence of proximal deep venous thrombosis (DVT) among acquired brain injury patients on admission to rehabilitation and assess cost-effectiveness of routine ultrasound screening for proximal DVT in those patients.nnnDESIGNnProspective study, sequential case series.nnnSETTINGnUniversity tertiary care rehabilitation center.nnnDATA SETnOne hundred sixteen traumatic brain injury patients (TBI) and 48 nontraumatic brain injury patients were admitted to our brain injury (BI) unit over a 21-month period and screened for a DVT on admission to rehabilitation utilizing real time, Doppler, and color Doppler ultrasound. Patients with a previous clinically recognized and treated DVT were excluded. No patients had been treated with prophylactic anticoagulation or intermittent anticoagulation since their BI and all patients were within 4 months of the original injury.nnnRESULTSnFourteen patients in total (8.5%) were found to have a DVT in the thigh or popliteal area, 9 of the 116 patients in the TBI group (7.8%), and 5 of the 48 patients in the nontraumatic BI group (10.4%). Statistically, there was no significant difference in the total number of detected proximal lower extremity DVTs between the TBI and the nontraumatic BI groups (Fishers exact test). In the TBI group 22 patients had associated lower extremity or pelvic fractures, but this factor appeared not to be significant, because only 1 of the 22 patients was discovered to have a DVT. The average admission Glascow Coma Score (GCS) of the TBI group was 8.6 but there was no correlation with the GCS and prevalence of DVT. The additional cost of screening for and treating the additional DVTs utilizing color Doppler ultrasound is conservatively estimated to be

Abrupt withdrawal from intrathecal baclofen: Recognition and management of a potentially life-threatening syndrome ☆ ☆☆ ★

674.84 per patient admitted to the BI rehabilitation unit. It can be estimated the cost of saving one life is

Supportive care for patients with Guillain-Barré syndrome

129,527.83.nnnCONCLUSIONnDVT screening in this patient population appears to be more cost-effective than mass screening programs for either breast cancer or colorectal cancer. The cost per year of life saved, estimated at only

Prospective assessment of tizanidine for spasticity due to acquired brain injury

2,977.65 (

Concept of spastic hypertonia.

129,527.83/43.5 years) for DVT screening for brain injury patients on admission to rehabilitation, is lower than those costs proposed for comparable programs with significant social support.