Ase Mygland

To investigate multiple sclerosis (MS) patients’ satisfaction with out-patient follow-up in a general neurological hospital department. Patients with definite MS living in Vest-Agder county, Norway were invited to answer a questionnaire comprising one question regarding overall satisfaction, and 24 questions regarding demographics, disease characteristics, and experiences with different aspects of the health care services. Out of 330 invited patients, 159 responded (48%). Mean overall satisfaction with health care was 3.5 (SD = 1.03) on a 1–5 Likert scale (1 = not at all, 5 = to a very large extent). The best sub scores were given on confidence in the physician’s competence (mean = 4.01), the physician speaks in an understandable way (mean = 4.07), expectation of good treatment (mean = 3.72), and perception of being submitted to wrong treatment (mean = 1.5). The worst scores were given on satisfaction with frequency of outpatient appointments (mean = 2.89) and delay of outpatient appointments (mean = 3.07). Four factors were associated with high overall satisfaction; receiving the disease modifying drug natalizumab (B = 0.549, p = 0.004), satisfaction with frequency of outpatient appointments (B = 0.242, p < 0.001), experience that the physician facilitates talking about what the patient finds important (B = 0.218, p = 0.001), and confidence with the physician’s competence (B = 0.453, p < 0.001). The patients were rather satisfied with the content of follow-up, and less satisfied with the structure. Regular and predictable contact with a trustworthy physician that facilitates that the patient is able to talk about what is important was associated with higher overall satisfaction.

An 81-year-old woman treated with simvastatin for several years followed by atorvastatin for about 1 year presented with fatigue, weakness and unsteady gait. The finding of elevated creatine kinase (CK) and symmetric muscle weakness around shoulders and hips led to suspicion of a toxic statin-associated myopathy. Atorvastatin was withdrawn, but her weakness persisted. Owing to persisting weakness, an autoimmune myopathy (myositis) was suspected, but initially disregarded since a muscle biopsy showed necrotic muscle fibres without inflammatory cell infiltrates and myositis-specific autoantibodies were absent. After 18 months with slowly progressive weakness and increasing CK values, awareness of new knowledge about autoimmunity as a cause of necrotic myopathy, led to a successful treatment trial with intravenous immunoglobulines, followed by steroids and metothrexate. Antibodies to the target enzyme of statins (HMGCR (3-hydroksy-3-methylglutaryl-coenzyme A reductase)) were detected in her serum, and she was diagnosed with autoimmune necrotic myositis probably triggered by atorvastatin.

Background:  Lyme neuroborreliosis (LNB) is a nervous system infection caused by Borrelia burgdorferi sensu lato (Bb).Objectives:  To present evidence‐based recommendations for diagnosis and treatment.Methods:  Data were analysed according to levels of evidence as suggested by EFNS.Recommendations:  The following three criteria should be fulfilled for definite LNB, and two of them for possible LNB: (i) neurological symptoms; (ii) cerebrospinal fluid (CSF) pleocytosis; (iii) Bb‐specific antibodies produced intrathecally. PCR and CSF culture may be corroborative if symptom duration is Bb antibodies may be absent. PCR is otherwise not recommended. There is also not enough evidence to recommend the following tests for diagnostic purposes: microscope‐based assays, chemokine CXCL13, antigen detection, immune complexes, lymphocyte transformation test, cyst formation, lymphocyte markers. Adult patients with definite or possible acute LNB (symptom duration 6 months) for 3 weeks (good practice points). Children should be treated as adults, except that doxycycline is contraindicated under 8 years of age (nine in some countries). If symptoms persist for more than 6 months after standard treatment, the condition is often termed post‐Lyme disease syndrome (PLDS). Antibiotic therapy has no impact on PLDS (level A).

Background and purpose:  To chart remaining complaints 1 year after treatment for neuroborreliosis, and to identify risk factors for a non‐favorable outcome.Methods:  We followed patients treated for neuroborreliosis prospectively, and assessed outcome by a composite clinical score. The impact on outcome of clinical, demographic and laboratory factors were analyzed by univariate analyses and logistic regression.Results:  Out of 85 patients 41 (48%) had remaining complaints; 14 had objective findings and 27 subjective symptoms. Remaining complaints were associated with pre‐treatment symptom duration ≥6 weeks (OR = 4.062, P = 0.044), high pre‐treatment cerebrospinal fluid (CSF) cell count (OR = 1.005, P = 0.001), and female gender (OR = 3.218, P = 0.025). Presence of CSF oligoclonal bands (OCBs) was not analyzed in the logistic regression model due to many missing observations, but was found to be more frequent both pre‐treatment (P = 0.004) and after 12 months (P = 0.015) among patients with remaining complaints as compared to patients with complete recovery. Further evaluation showed that objective remaining findings, and not subjective symptoms, were associated with pre‐treatment symptom duration ≥6 weeks.No difference in outcome was observed between patients treated with IV ceftriaxone and patients treated with oral doxycycline.Conclusion:  Remaining complaints are common after neuroborreliosis. The majority of the complaints are subjective. Pre‐treatment symptom duration ≥6 weeks, high pre‐treatment CSF cell count, and female gender seem to be risk factors for remaining complaints. Presence of CSF OCBs may also predict a non‐favorable outcome, but this should be further studied. Whether subjective and objective complaints are associated with different risk factors is also an issue for future studies.

Background – Chronic polyneuropathy is a common disorder with heterogenic clinical presentation and many different etiologies. Diagnostic investigation is challenging. Materials and methods – An algorithm for diagnostic investigation of chronic polyneuropathy is presented. It was designed to secure practical usefulness for general neurologists, identification of the most common causes with an adequate number of laboratory tests, and more focused investigation when necessary. The proposal is based on review of articles found by PubMed search using the terms ‘‘peripheral neuropathy, cause, and investigation’’, relevant book chapters, and own clinical experience. Results – All patients should undergo a routine investigation for the most common causes of polyneuropathy by asking for diabetes, heredity, alcohol abuse, toxic medications and agents, symptoms of Sjögren's syndrome, renal failure, and the following laboratory tests; glucose, haemoglobin, leucocytes, thrombocytes, ESR, creatinin, ALAT, GT, vitamin B12, serum electrophoresis, TSH and thyroxin. If routine investigation is negative, a targeted approach based on clinical type and electrophysiological findings is recommended. The most common type with slowly progressive, symmetric sensory symptoms beginning in the feet can often be classified as cryptogenic without further investigation. Polyneuropathies with subacute onset, progressive weakness, asymmetric symptoms, proximal weakness, selective involvement of sensory fibers or demyelinating pathology, or other organ manifestations, have various etiologies that necessitate individual focused investigation. Interpretation – Diagnostic investigation of polyneuropathy can be simplified and systematized.

Chronic polyneuropathy is a common disorder with heterogenic clinical presentation and many possible etiologies. This review presents diagnostic guidelines for physicians without specialist competence in neuromuscular disorders. The article is based on a review of articles identified in PubMed using the search terms "peripheral neuropathy", "cause" and "investigation", relevant book chapters and own clinical experience. All patients should undergo a routine investigation for the most common causes of polyneuropathy, such as diabetes, heredity, alcohol abuse, toxic medications and agents, symptoms of Sjögren's syndrome and renal failure. The following laboratory assessments should be done if the medical history proves negative: glucose, haemoglobin, leucocytes, thrombocytes, ESR, creatinin, ALAT, GT, vitamin B12, serum electrophoresis, TSH and thyroxin. If the routine investigation proves negative, a targeted approach based on clinical type and electrophysiological findings is recommended. The most common sensory type with slowly progressing, symmetric sensory symptoms beginning in the feet can often be classified as cryptogenic without further investigation. Polyneuropathies with subacute onset, progressive weakness, asymmetric symptoms, proximal weakness, selective involvement of sensory fibres or demyelinating pathology, or other organ manifestations, have different etiologies that necessitate individually focused investigations.

A sensitive method to detect intrathecal IgG production is important in diagnosing inflammatory central nervous system (CNS) diseases, including multiple sclerosis (MS). To compare cerebrospinal fluid (CSF) electrophoresis with isoelectric focusing (IEF), immunofixation-peroxidase electrophoresis (IFPE) and high-resolution agarose electrophoresis with protein-staining (HRAGE). Paired serum and CSF samples from 307 consecutive patients attending a general neurology clinic were examined with IEF, IFPE and HRAGE. Clinical diagnosis was based on review of the patients' medical records after an average of 4 years. The sensitivity for detecting any inflammatory (autoimmune or infectious) CNS disease (52 patients) was 67% for IEF, 50% for IFPE and 29% for HRAGE. The sensitivity for detecting MS (14 patients) was 93%, 86% and 29% respectively. The sensitivity for detecting clinically isolated syndrome (eight patients) was 75%, 25% and 13% respectively. The number of oligoclonal bands in IEF was higher in inflammatory than in non-inflammatory neurological diseases or symptoms, but similar in MS and other inflammatory diseases. IEF is the method of choice in diagnosing intrathecal IgG synthesis.