Elisabeth Farbu

EFNS guideline on diagnosis and management of post-polio syndrome. Report of an EFNS task force.

Post‐polio syndrome (PPS) is characterized by new or increased muscular weakness, atrophy, muscle pain and fatigue several years after acute polio. The aim of the article is to prepare diagnostic criteria for PPS, and to evaluate the existing evidence for therapeutic interventions. The Medline, EMBASE and ISI databases were searched. Consensus in the group was reached after discussion by e‐mail. We recommend Halsteads definition of PPS from 1991 as diagnostic criteria. Supervised, aerobic muscular training, both isokinetic and isometric, is a safe and effective way to prevent further decline for patients with moderate weakness (Level B). Muscular training can also improve muscular fatigue, muscle weakness and pain. Training in a warm climate and non‐swimming water exercises are particularly useful (Level B). Respiratory muscle training can improve pulmonary function. Recognition of respiratory impairment and early introduction of non‐invasive ventilatory aids prevent or delay further respiratory decline and the need for invasive respiratory aid (Level C). Group training, regular follow‐up and patient education are useful for the patients’ mental status and well‐being. Weight loss, adjustment and introduction of properly fitted assistive devices should be considered (good practice points). A small number of controlled studies of potential‐specific treatments for PPS have been completed, but no definitive therapeutic effect has been reported for the agents evaluated (pyridostigmine, corticosteroids, amantadine). Future randomized trials should particularly address the treatment of pain, which is commonly reported by PPS patients. There is also a need for studies evaluating the long‐term effects of muscular training.

Brain atrophy and disability progression in multiple sclerosis patients: a 10-year follow-up study.

Objectives To identify MRI biomarkers associated with long-term disability progression in patients with multiple sclerosis (MS), and to define the rate of evolution of global, tissue-specific and regional atrophy in patients with MS over long-term. Methods MRI of the brain and clinical neurological assessment was performed in 81 patients at time of first visit and after 5 and 10 years of follow-up. MRI was acquired on 1.5 T scanners. T1-lesion and T2-lesion volumes (LVs) were calculated. Global and tissue-specific atrophy changes were longitudinally assessed, using a direct measurement approach, by calculating percentage volume changes between different time points. Regional tissue volumes for the subcortical deep grey matter (SDGM) structures were also obtained. Disability progression was defined as an increase in Expanded Disability Status Scale of ≥1.0 compared to baseline at 5-year and 10-year follow-up. Results Over 5 years, patients with disability progression showed significantly increased loss of whole brain (−3.8% vs −2.0%, p<0.001), cortical (−3.4% vs −1.8%, p=0.009) and putamen volume changes (−10.6% vs −3.8%, p=0.003) compared to patients with no disability progression. No significant change in white matter (WM) volume was observed when comparing progressing and non-progressing patients. Over 10 years, there was a trend for greater decrease in whole brain volume (−5.5% vs −3.7%, p=0.015) in the progressing patients. No significant changes in LV measures were detected between the patients with and without disability progression. Conclusion This long-term study shows that whole brain, cortical and putamen atrophy occurs throughout the 10-year follow-up of this MS cohort and is more pronounced in the group that showed disability progression at 5, but not at 10 years of follow-up. Overall, GM atrophy showed better association with disease progression than WM atrophy over 5-year and 10-year follow-up.

Post‐polio syndrome patients treated with intravenous immunoglobulin: a double‐blinded randomized controlled pilot study

Post‐polio syndrome (PPS) is characterized by new muscle weakness, atrophy, fatigue and pain developing several years after the acute polio. Some studies suggest an ongoing inflammation in the spinal cord in these patients. From this perspective, intravenous immunoglobulin (IvIg) could be a therapeutic option. We performed a double‐blinded randomized controlled pilot study with 20 patients to investigate the possible clinical effects of IvIg in PPS. Twenty patients were randomized to either IvIg 2 g/kg body weight or placebo. Primary endpoints were changes in pain, fatigue and muscle strength 3 months after treatment. Surrogate endpoints were changes in cerebrospinal fluid (CSF) cytokine levels. Secondary endpoints were pain, fatigue and isometric muscle strength after 6 months. Patients receiving IvIg reported a significant improvement in pain during the first 3 months, but no change was noted for subjective fatigue and muscle strength. CSF levels of tumour necrosis factor‐α (TNF‐α) were increased compared with patients with non‐inflammatory neurological disorders. In conclusion, in this small pilot study no effect was seen with IvIg treatment on muscle strength and fatigue, however IvIg treated PPS patients reported significantly less pain 3 months after treatment. TNF‐α was increased in the CSF from PPS patients. The results are promising, but not conclusive because of the low number of patients studied.

Post-polio syndrome and total health status in a prospective hospital study.

New loss of function among patients with previous polio is frequently reported and has several causes. All patients referred to the Department of Neurology, Haukeland University Hospital, Bergen, for 13 months during 2000–2001 with diagnosis late effects of polio were examined prospectively to identify their symptoms and loss of function. Eighty‐five patients aged 47–91 years with mean of 61 years were included. The most common complaints were pain (44%), muscular weakness (27%), and fatigue (16%). Muscular weakness occurred in lower limbs in 75%, in respiratory muscles in only 5%. Walking in stairs was impaired in 72% and outdoor walking in 65%. Seventeen patients (19%) reported no loss of function.

Lifestyle and late effects after poliomyelitis. A risk factor study of two populations

Background – Patients with polio often experience new symptoms (muscle weakness, pain, fatigue and respiratory problems) many years after the acute disease. This study examined possible interactions between lifestyle factors (overweight, physical inactivity, smoking) and late polio with new symptoms.

Health related quality of life in patients recently diagnosed with multiple sclerosis

To describe a representative population of patients recently diagnosed with MS in terms of both motor and non‐motor disability. In particular we wanted to examine the HRQoL in this population to get a better understanding of what impact various clinical features have on the patients’ experience of distress in the early phase of the disease.

Risk of MS is not associated with exposure to crude oil, but increases with low level of education

Background: Offshore workers in the Norwegian upstream petroleum industry are exposed to a number of chemicals such as organic solvents, mineral oils and other hydrocarbons, possibly contributing to an increased risk of multiple sclerosis (MS). Objective: To estimate the risk of MS in this population compared with the general working population in Norway, adjusting for education. Methods: Using the Norwegian Registry of Employers and Employees we included all 27,900 offshore workers registered from 1981 to 2003 and 366,805 referents from the general working population matched by gender, age and community of residence. The cohort was linked to the Norwegian MS Registry and the Norwegian Education Registry. Results: There was no increased risk of MS among the offshore workers. We found a marked and linear inverse relationship between level of education and the risk of MS in the total study population, with a rate ratio of 0.48 (95% CI, 0.53 to 0.88) for workers with a graduate degree compared to workers with elementary school only. Conclusions: These findings do not support a major aetiological role of petroleum-based products, but rather point to smoking and other lifestyle factors related to the level of education as being important for the risk of MS.

Acute ischemic stroke--from symptom recognition to thrombolysis.

The understanding of stroke has changed in the recent years from rehabilitation to an emergency approach. We review existing data from symptom recognition to thrombolysis and identify challenges in the different phases of patient treatment.

Poliomyelitis: long‐time consequences for social life

In a study of 102 consecutive patients hospitalized for previous poliomyelitis, we found that 70 patients had continued education after elementary school and 18 were academics. This is a higher proportion than in the general Norwegian population. All 14 patients with paraparesis had continued education after elementary school, while as many as 12 of 18 patients with a university degree had widespread pareses in the acute phase. Of the patients 46 worked or had worked full‐time up to 60 years of age. Only 29 patients were receiving a disabled pension. Another 9 patients had neither been employed nor received any pension, all housewives. Nine of 14 patients with paraparesis were working full‐time, only 2 received disabled pension. Among the 35 patients with persisting widespread pareses, 11 were still in full‐time work and 7 were working part‐time. The employment rate among the patients in this study was nearly identical to the age‐correlated general employment rate in Norway. Our conclusion is that polio patients are doing well in society; they have taken education, are working, and are generally self‐supported. The degree of pareses does not seem to have been the most determining factor for their educational and professional activity.

Polio survivors--well educated and hard working.

Abstract 243 patients were diagnosed with acute poliomyelitis (polio) in Western Norway between 1950 and 1954; 186 were paralytic and 57 non-paralytic. This study examines how polio influenced their education, employment, profession, annual income, marital status and energy for leisure activities. 149 of the patients identified were alive and 98 of the matched controls responded to a questionnaire. Education length did not differ between acute paralytic polio patients, acute non-paralytic polio patients and controls. Fifty percent of the patients with residual weakness and 77 % of the patients with normal muscle power were employed, against 73 % of the controls (P=0.014). A higher proportion of patients without motor deficits had manual work than those with weakness or controls (P=0.002). There was no significant association between severity of weakness and education, employment and profession. Physical ability had been an important factor for the choice of education and profession for all the polio patients, but not for controls (P < 0.001). Annual income did not differ significantly between patients and controls. Residual weakness increased the chance of being single (P=0.023), although as many as 79 % had married. 53 % of the patients with weakness claimed that fatigue prevented hobbies, compared wich 31 % of the other patients and only 16 % of the controls (P < 0.001). There was no significant association between severity of weakness and fatigue. In conclusion, the polio patients are generally well educated, provide their own income and marry. However, their polio has influenced choice of education and profession, and polio patients with persisting weakness differ from controls and polio patients without motor deficits regarding employment and marital status.