IV immunoglobulin does not reverse established weakness in MS.

BACKGROUND: Immunoglobulin (Ig) administration induces remyelination in the Theiler's virus model of MS. METHODS: A randomized, double-blinded, placebo-controlled trial of IV immunoglobulin (IVIg) was performed in patients with MS who had persistent muscle weakness that had been stable for between 4 and 18 months to determine whether this would improve muscle strength (primary outcome: isometric muscle strength). Patients received either IVIg (0.4 g/kg) or placebo daily for 5 days, then single infusions every 2 weeks for 3 months (total, 11 infusions). Muscle groups identified by clinical measures to have unchanging significant weakness were the major targets for therapeutic response (targeted neurologic deficit [TND]). RESULTS: IVIg was well tolerated. An interim analysis after 67 patients were enrolled indicated no difference in the degree of change in strength between treatment groups in either the TND or non-TND muscle groups at 6 months, and the trial was terminated. There was no apparent benefit in relapse behavior or impairment measures during the 6-month observation period. Nor was there apparent benefit in either patients who remained clinically stable or in those with evidence of disease activity. Patients with active MS during the trial worsened in both TND and non-TND muscle groups. This worsening was seen regardless of whether the clinical manifestations of disease activity involved the TND muscle groups. CONCLUSIONS: IVIg does not reverse established weakness in MS. Measurements of isometric muscle strength were reliable (reproducible) indices of strength and may be sensitive, objective methods to document functional changes in impairment in future MS trials.

Inclusion body myositis masquerading as polymyositis: a case study.

A case of inclusion body myositis masquerading as unresponsive polymyositis is presented. A 56-year-old woman diagnosed with "biopsy-proven" polymyositis in 1991 was referred to our clinic in 1997 with progressive, painless weakness that was unresponsive to steroid therapy. Further evaluation, including electromyography and review of the original muscle biopsy specimen, found a diagnosis of inclusion body myositis, leading to a change in the patient's prognosis and management. Inclusion body myositis is frequently mistaken for polymyositis, despite the fact that it is now the most common inflammatory myopathy affecting people older than 50 years. The purpose of this report is to increase awareness of this disease, to enhance early diagnosis, and to ensure appropriate management. We discuss the clinical findings, pathogenesis, and physiatric management, as well as compare this disease with other idiopathic inflammatory myopathies.

Antidepressant exacerbation of spasticity.

Patients with spinal cord injury (SCI) may develop depression. This may be related to adjustment to living with an SCI in addition to dealing with complications of the injury, such as spasticity. Pharmacologic treatment of depression can be difficult because of neurochemical and receptor changes that are associated with SCI. Newer antidepressant agents are purported to have selective activity by alteration of serotonergic neurotransmission. A case report is presented that illustrates exacerbation of spasticity by this family of antidepressant medications. Mechanisms possibly explaining this exacerbation of spasticity are the effects of serotonin on motor neuron and reflex activity, denervation supersensitivity, and the serotonin syndrome. Understanding the relationship between serotonergic systems and spasticity can be important in treating depression in patients with spasticity.

Prevention of venous thromboembolism in patients with spinal cord injury: effects of sequential pneumatic compression and heparin.

OBJECTIVE: To estimate the incidence of and risk factors for venous thromboembolism in patients with acute traumatic spinal cord injury (SCI) and evaluate the effectiveness of sequential pneumatic compression devices (SCD), gradient elastic stockings (GES), and heparin in preventing thromboembolism. DESIGN: Prentice's case-cohort design. SETTING: All patients admitted to our hospital between 1976 and 1995 with acute traumatic SCI. MAIN OUTCOME MEASURES: Demographic characteristics, venous thromboembolism risk factors, methods of surveillance and prophylaxis, and thromboembolic events during the first 6 weeks following injury. RESULTS: Venous thromboembolism occurred in 84 of 428 patients (19.6%). Venous thromboembolism increased from 21% between 1976 and 1979 to 31% between 1980 and 1984, then decreased to 16% between 1985 and 1989 and to 8% between 1990 and 1995. Routine surveillance for venous thromboembolism increased through 1983, and SCD/GES use increased after 1983, with a concurrent decline in incidence of thromboembolism. Multivariate analysis showed that SCD/GES reduced the risk of deep venous thrombosis (DVT) or pulmonary embolism (relative risk, 0.5; 95% CI, 0.28 to 0.90). Multivariate analysis suggested a reduced risk of DVT in patients receiving heparin therapy within the first 14 to 42 days after injury, but estimates of reduced risk were not statistically significant (p = .064 for first 14 days, p = .13 for heparin anytime). CONCLUSION: The SCD/GES combination and heparin are each effective in preventing venous thromboembolism in individuals' acute traumatic SCI. Effectiveness of heparin prophylaxis may be greatest during the first 14 days after injury, whereas benefit from SCD continues to 6 weeks after injury.

Carpal tunnel syndrome in pregnancy: frequency, severity, and prognosis.

OBJECTIVE: To determine the frequency, severity, prognosis, and patterns of carpal tunnel syndrome (CTS) in pregnancy. DESIGN: Descriptive retrospective chart review using the Rochester Epidemiology Project medical record diagnostic indexing system to identify patients with new CTS occurring during pregnancy from 1987 to 1992 at our institution. SETTING: Obstetrical practice, where two thirds of pregnant women in the county receive primary obstetrical care. PATIENTS: Women pregnant during 1987 to 1992 who had a new diagnosis of CTS. Women with pregnancies at other dates or women who had CTS with onset before or after pregnancy were excluded. OUTCOME MEASURES: Age, underlying medical problems, gestation interval, weight gain, number of pregnancies, presenting symptoms, onset and duration of symptoms before diagnosis, trimester of CTS diagnosis, treatment and response, and results of electrophysiologic studies are described. RESULTS: Of 10,873 pregnant patients receiving antenatal care for 14,579 pregnancies, 50 (.34%) fulfilled the inclusion criteria. Their mean age was 30.5 +/- 4.0 yrs. Twelve patients (24%) were primigravid. Mean weight gain was 12.1 +/- 5.7 kg. CTS was diagnosed most frequently during the third trimester (n = 25, 50%). Symptom onset, when recorded, occurred with even distribution during each trimester: first, n = 11 (32%); second, n = 11 (32%); third, n = 12 (35%). For 37 patients in whom symptom duration was recorded, duration before diagnosis was 9.3 +/- 9.0 weeks. Paresthesia (88%) was most often bilateral (68%), and 67% of patients had pain. The Tinel sign was present over the median nerve at the wrist in 95%. Only nine patients had nerve conduction studies performed. During pregnancy, 37 women were treated nonsurgically with wrist orthoses, steroid injections, or both. Of treated patients for whom follow-up data were available, 25 of 26 improved, and 4 of 26 required surgery. Thirteen women had no treatment during pregnancy; three underwent surgery in the postpartum period. All 7 women in whom conservative treatment failed who underwent surgery had resolution of symptoms. CONCLUSION: These results represent the frequency and patterns of clinically significant CTS in a large population of pregnant women. CTS severe enough to warrant treatment occurs infrequently in pregnancy and generally resolves spontaneously postpartum or responds to conservative treatment.

A new dynamic triceps-driven orthosis (DTDO): achieving elbow flexion in patients with C5 deficits.

This report describes a unique orthosis designed to assist activities of daily living for a patient with severe proximal upper limb weakness caused by traumatic central cord syndrome. The orthosis-the dynamic triceps-driven orthosis (DTDO)-provides elbow flexion using contralateral elbow extension to move a cable threaded across the shoulders and wrist cuffs bilaterally. The device is a simple, inexpensive design that can be reproduced by any orthotist. The DTDO has been used successfully for other patients with severe weakness in C5- and C6-innervated muscles.

Health care utilization in multiple sclerosis: a population-based study in Olmsted County, MN.

OBJECTIVE: The authors sought to determine acute ambulatory- and hospital-billed charges for the Olmsted County, Minnesota Multiple Sclerosis (MS) Disability Prevalence Cohort and compare them to those incurred by the general population. METHODS: Billed charges for 155 people with clinically definite or laboratory-supported MS were compared with those of age- and gender-matched non-MS controls. Billing data, including all inpatient and outpatient acute and rehabilitative medical care charges over a 5-year period surrounding a December 1, 1991 prevalence date, were analyzed. Data were correlated with level of disability using the Minimal Record of Disability for MS. RESULTS: Median total annual billed charges for most individuals with MS, including those with less severe ($1,277) and relapsing-remitting illness ($1,348), did not differ from those for controls ($1,327, p=0.075). Only those with severe MS (22.6%) had median annual medical charges higher than controls ($5,440, p < 0.001). Male patients with MS had higher median annual total charges ($2,353) than male controls ($762, p=0.003). Total charges for female patients with MS ($1,440) were not different from those for female controls ($1469). Median annual outpatient charges were 15% more for the MS group ($1,418) than for controls ($1,231). Patients with MS had a mean of 0.2 hospital admissions annually compared with 0.1 annual admissions per control patient. Among variables collected on persons with MS, the Expanded Disability Status Scale was the strongest predictor of level of charges (p < 0.001). CONCLUSION: Acute ambulatory- and hospital-billed charges for most patients with MS do not differ from those of the general population.

Hyperthermia, rhabdomyolysis, and disseminated intravascular coagulation associated with baclofen pump catheter failure.

A 29-year-old man with C6 tetraplegia (ASIA A) using an implanted baclofen pump and intrathecal catheter infusion system for spasticity control developed severe spasticity, hyperthermia, hypotension, rhabdomyolysis, and disseminated intravascular coagulation after catheter disconnection. Tracheal intubation and mechanical ventilation were necessary. Extensive workup for a concurrent infection was negative except for urine cultures. The patient remained febrile for 10 days despite empirical antibiotic trials. Administration of high-dose benzodiazepines was inadequate for spasticity control. Spasticity control and his clinical condition, including body temperature, did not improve until his catheter was surgically replaced and intrathecal baclofen administration was resumed. The pharmacopathology of abrupt baclofen withdrawal and the similarities between this presentation, sepsis, neuroleptic malignant syndrome, and malignant hyperthermia are discussed. High-dose dantrolene was not used; however, based on similarities between this patient's presentation and neuroleptic malignant syndrome, it may have been the drug of choice.

Management of impairment, disability, and handicap due to multiple sclerosis.

In this article, we update management measures for patients with multiple sclerosis (MS) that can improve or prevent impairment, disability, and handicap and include those factors that a primary-care physician can implement or facilitate. The medical literature since 1989 was reviewed. Although new drug trials hold promise to decrease impairment from MS, well-coordinated interdisciplinary care to minimize disability and handicap most profoundly affect the quality of life for patients with MS. MS is usually not severely disabling, and appropriately timed intervention can prevent secondary impairment and reduce disability and handicap. Pharmacologic, physical, and psychosocial issues--ranging from spasticity, pain, weakness, and tremor to neurogenic bowel management and sexuality--are addressed. General wellness measures remain important. The influence of the Americans With Disabilities Act is discussed, and specific adaptive equipment and social resources are outlined. The ultimate goals of management of patients with MS are functional independence and efficient use of medical and community resources: a focus on "ability" rather than "disability." Although impairment can limit function, wellness and adjustment have no boundaries.

Transient traumatic quadriplegia in a 29-year-old man without predisposing anatomic lesion.

A 29-year-old man presented 45 minutes after sustaining a blow to the right side of his head when his motorcycle slid on gravel and flipped on the side of the road. After walking away from the accident, the patient noted burning pain in his neck. Shortly thereafter, complete quadriplegia with a sensory level at C4 developed. The patient was given a high dose of methylprednisolone and transferred to a tertiary care center. En route, neurological testing demonstrated rapid improvement, and on arrival, all abnormal motor and sensory findings had completely resolved. Radiographic examination and magnetic resonance imaging of the entire spine showed no abnormality. Torg's ratio was > 1 at vertebral level C3 through C6. There was no evidence of developmental stenosis, congenital fusion, cervical instability, or intervertebral disk protrusion.

Intrathecal baclofen therapy in stiff-man syndrome: a double-blind, placebo-controlled trial.

We performed a double-blind, placebo-controlled trial of intrathecal baclofen (ITB) in stiff-man syndrome. Three patients, unresponsive to current therapy, received 50 micrograms of ITB or placebo on sequential days. Following ITB, all patients demonstrated improvement in reflex EMG activity. The mean reduction in total EMG activity (from all muscles) following stimulation of the medial plantar nerve (cutaneous flexor reflex) was 72% following 50 micrograms of ITB compared with 18% following placebo (ANOVA: significance of F, p < 0.0001). The mean latency to onset of the response was also significantly prolonged for all muscles following ITB (ANOVA: significance of F, p < 0.05). Although reflex EMG activity was reduced in all patients, clinical improvement was evident in only one patient, who differed from the others studied by a longer duration of disease, greater severity of stiffness, less fear of falling, and greater electrophysiologic improvement.

Neurological abnormalities associated with remote occupational elemental mercury exposure.

We examined 502 subjects, 247 of whom had occupational elemental mercury exposures 20 to 35 years previously, to identify potential exposure-related neurological abnormalities. Few significant (p less than 0.05) differences existed between exposed and unexposed subjects. However, multiple linear regression analysis demonstrated several significant correlations between declining neurological function and increasing exposure as determined by urine mercury measurements from the exposure interval. Subjects with urine mercury peak levels above 0.6 mg/L demonstrated significantly decreased strength, decreased coordination, increased tremor, decreased sensation, and increased prevalence of Babinski and snout reflexes when compared with the remaining subjects. Furthermore, subjects with clinical polyneuropathy had significantly higher peak levels than normal subjects (0.85 vs 0.61 mg/L; p = 0.04), but not increased exposure duration (20.1 vs 20.8 quarters; p = 0.34), and 28% of subjects with peak levels above 0.85 mg/L had clinical evidence of polyneuropathy, compared with 10% of remaining subjects (p = 0.005). Although exposure was not age dependent, several neurological measures showed significant age-mercury interaction, suggesting that natural neuronal attrition may unmask prior exposure-related subclinical abnormalities.

Patterns of referral to a university hospital consultation service: failure to accurately predict need for physiatric services.

The purpose of this study was to determine the predictive value of screening the narrative sections of the consultation request form to determine the need for physiatric intervention rather than PT intervention alone. We conducted a review of 107 requests for consultation from various acute care services at our hospital. After reviewing the referring physician's narrative summaries, we determined that 36% of 107 patients would require physiatric evaluation, and that the remaining patients could be "passed through" to PT for treatment. After evaluation of the medical records and examination of the patients, 54% of patients required physiatric assessment and only 45% could be "passed through" to PT. The hypothesis that this narrative information would be an adequate predictor of need for physiatric consultation was rejected at the p less than 0.005 level (chi 2 = 18.63; df = 1). In addition, the referring service indicated whether it wished PT or physiatry to evaluate the patient on the consultation form. The hypothesis that screening for the need for physiatric intervention by the indicated preference of the referral was rejected at the p less than 0.0005 level (chi 2 = 20.45; df = 1). We concluded that we could not reliably predict when patients required physiatric or only PT intervention based on the consultation request narrative. Ongoing physiatric involvement on a consultative basis, educational conferences, and other forms of education for house staff and attending physicians may serve to improve understanding of physiatric services and physiatric utilization.