[Immunopathology and treatments of Guillain-Barré syndrome and of chronic inflammatory demyelinating polyneuropathy]. / Mécanismes immunitaires et traitements du syndrome de Guillain-Barré et des polyradiculonévrites inflammatoires chroniques.

The concepts of Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) have changed over the last decade. The spectrum of GBS ranges from acute inflammatory demyelinating polyneuropathy to pure motor, sensory-motor or bulbar variants and the Miller Fisher syndrome. Also CIDP includes different variants in addition to the typical clinical picture with symmetrical proximal and distal weakness, such as a form with predominant distal weakness, a pure sensory form, an asymmetric form and a form with predominant cranial nerve involvement. Detailed immunopathologic features have been described in GBS and CIDP: most current investigations are centered on the hypothesis of molecular mimicry in GBS and together with the pathogenic role of cell-mediated immunity different antibodies have been discovered in GBS which interfere with nerve impulse conduction on neuromuscular transmission. The immunopathogenesis of CIDP remains fragmentary and insufficient for a unified hypothesis. Activated macrophages and T-cells with the participation of T-1 helper cell related cytokines seem to play a fundamental role in demyelination. The nature of antigen presenting cells, T-cell receptors, adhesion molecules and the proinflammatory cytokines need to be explored to design more specific immunotherapies. Established treatments in GBS include intravenous immunoglobulin and plasma exchange. Randomized trials have shown the efficacy of prednisone, intravenous immunoglobulin and plasma exchange in CIDP. New insight in the pathogenetic role of the cytokine-network in CIDP opens new therapeutical possibilities with the modification of the T-1 helper cell reaction with interferon.

[Neurological complications of HIV infection. Review: new diagnostic, therapeutic and prognostic aspects]. / Neurologische Komplikationen der HIV-Infektion. Aktuelle Ubersicht: neue diagnostische, therapeutische und prognostische Aspekte.

About one third of patients with HIV infection show neurological complications with considerable morbidity and high mortality. This is an actualized review of the most important neurological manifestations resulting from primary HIV infection, from secondary opportunistic infections, or as complications of antiretroviral therapy. The primary neurological manifestations, including HIV-associated dementia complex, myelopathies, peripheral neuropathies and myopathies, the more common opportunistic infections, primary central nervous system lymphoma and cerebrovascular diseases, are discussed in the light of new evidence in diagnosis, therapy and prognosis. Cognitive and psychiatric symptoms, visual changes, headache, seizures, dizziness, involuntary movements, gait disturbances, cranial neuropathies and focal deficits are the common neurological symptoms in HIV infection which are described under the aspect of differential diagnosis. It is important to bear in mind that nearly all information available to date on this subject concerns HIV patients in the period before combination therapies (including protease inhibitors). The introduction of highly active antiretroviral therapy (HAART) with protease inhibitors in 1995, and non-nucleoside reverse transcriptase inhibitors, have opened up new therapeutic modalities with a new emphasis on earlier detection and treatment of neurological complications. The prognosis of different HIV-associated neurological diseases has considerably improved, as recently shown in the case, for example, of progressive multifocal leucoencephalopathy.

[Intermittent paraparesis as manifestation of a bilateral chronic subdural hematoma]. / Fluktuierende Paraparese als Manifestation eines bilateralen chronischen Subduralhämatoms.

Chronic subdural haematomas are mainly related to slight or moderate head trauma with consecutive lesion of bridge or cortical veins and bleeding in the subdural space. Further predisposing factors are known impairment of coagulation (coagulopathies, treatment with anticoagulants, alcohol abuse), risk factors for degenerative disease of the arteries (diabetes mellitus, arterial hypertension), and development of pressure gradients (hydrocephalus, epileptic seizures, lumbar puncture, CSF drainage and cerebral atrophy). Chronic subdural haematomas appear bilaterally in 20 to 25% of cases. We report on a 69-year-old male with a 4-day history of intermittent, proximal, painless paraparesis (BMA grade M2-5) without a trigger event. Sensibility was normal in all qualities and vigilance was not disturbed. Computed tomography of the neurocranium revealed a bitemporally located chronic subdural haematoma with extension to parietal on both sides. Trepanation was performed over the tuber parietale and temporoparietally on both sides, with release of 150 ml fluid. The neurologic deficits regressed totally within 12 hours postoperatively. To the best of our knowledge, we are the first to describe the clinical paradox of intermittent, painless paraparesis with preserved sensibility and without disturbances of vigilance, as manifestation of a chronic subdural haematoma possibly leading to impairment of cerebral blood flow in the area of the middle cerebral artery. Small changes in systemic blood pressure lead to changes in cerebral perfusion pressure due to vessel compression by the haematoma, thus explaining the intermittent character of the clinical presentation.

Visual disturbances and transurethral resection of the prostate: the TURP syndrome.

A 73-year-old man with a history of a cerebral and a cardiac vascular disease and atrial flutter developed visual disturbances characterized by vision being dark in both eyes and by seeing as through a color photographic negative immediately after an uncomplicated transurethral resection of the prostate (TURP) for prostatic hyperplasia under spinal anesthesia. There was complete remission of the symptomatology after 2.5 h. A cerebrovascular workup was negative. Considering postoperative hyponatremia and hypoosmality, we discuss the possible role of glycine-induced visual disturbances as described in the TURP reaction syndrome, to our knowledge an entity almost unknown in the neurologic literature. Glycine-induced visual disturbances should therefore be considered in the differential diagnosis of bilateral transient visual loss.

[Neurosarcoidosis of the central nervous system. Analysis of the long-term follow-up of 8 cases]. / Neurosarkoidose des zentralen Nervensystems. Analyse des Langzeitverlaufes in 8 Fällen.

We present retrospective and follow-up data of eight patients with sarcoidosis involving the central nervous system (CNS). The diagnosis was established definitely by histological evidence in seven cases. Biopsies were taken directly from the CNS or the covering meninges in two of them. Additionally, we included a further case with the characteristic triad of arthralgia, erythema nodosum and pulmonary hilar adenopathy on X-ray, representing Löfgren's syndrome. Systemic manifestation of sarcoidosis was hardly recognized in some cases and sometimes became evident only in the long term after recurring manifestations. The symptomatology in our cases can be summarized as follows: Mostly, meningopolyneuritic and meningoencephalopathic syndromes occurred, with, in addition, one case of chronic meningitis and two cases of spinal involvement. In two cases, CNS symptoms represented the initial manifestation of the disease. Furthermore, the stringent interdependency of non-neurologic and neurologic episodes of the disease was not recognized easily. Under these circumstances, the histological confirmation was often difficult. Thus, the latency between the first neurological signs of the disease and the diagnosis of neurosarcoidosis may be long, as in our cases (mean 27 months). The course of the disease was followed for a mean 8.1 (range 1-15) years. Seven patients showed at least partial recovery from progressive disease after corticosteroid therapy. One patient developed, subacutely, a fatal pontine-medullary syndrome. The potentially poor prognosis emphasizes the importance of diagnostic efforts.

[Neurology at the threshold of the 21st century]. / Die Neurologie an der Schwelle zum 21. Jahrhundert.

In the controversial field of a continuously changing neurology with the problem of rising medical costs, the perspectives of modern neurology at the beginning of a new century are described by four aspects: 1. Relatively new are the studies of neuropsychiatric symptoms in neurologic diseases. An immediate diagnosis and treatment of these symptoms can improve the outcome of the neurologic disease. 2. An example for the progress in investigative methods by medical technology is magnetic resonance imaging which allows a more refined diagnosis and which improves and reevaluates the importance of the clinical examination. There is a warning on the other hand about unnecessary and badly planned examinations. 3. Advances in clinical neurology are mentioned by describing neurogenetic diseases, infectious neurologic diseases and new forms of dementia. 4. Some therapeutical aspects concerning neuroprotection and neurologic intensive care are discussed. Finally, the aims for the training of future neurologists are briefly outlined.

Pamidronate for pain control in patients with malignant osteolytic bone disease: a prospective dose-effect study.

In a prospective dose-escalation study tolerability and effectiveness of repeated infusions with intravenous pamidronate were investigated. A total of 80 patients with proven malignancy and pain due to osteolytic bone disease were enrolled. Doses of 30 mg, 45 mg, 60 mg and 90 mg pamidronate, given every 4 weeks, 3 weeks or 2 weeks were tested. Thus dose intensity was increased by giving higher doses and/or by shortening the intervals. A combined palliation score on the bases of pain score (WHO), analgesic score (WHO) and improvement of performance status (SAKK/ECOG) was rated by the physician on a six-point scale. Regression analysis showed a close correlation between dose intensity and effect (Pearson's R = 0.7: P < 0.0001). A statistically significant different palliative score for patients treated with low (below 15 mg/week), medium (16-30 mg/week) and high doses (above 31 mg/week) of pamidronate was found (P = < 0.01). A dose intensity below 10 mg pamidronate/week and single doses of 30 mg had no clinically relevant benefit, whereas dose intensities of 25-45 mg/week showed a significant palliative effect. We conclude that pamidronate should be given in a close intensity of 20 mg per week or more in patients with far advanced osteolytic bone disease. Best results are obtained with high doses of 60 mg or 90 mg pamidronate. Further investigations by prospective randomized trials are needed to determine the optimal dose and schedule of pamidronate infusions.

Attitudes of Swiss physicians in prescribing opiates for cancer pain.

Following clinical observations showing that opiates are sometimes not consistently administered for chronic cancer pain, a survey was conducted among 1200 physicians in the German-speaking part of Switzerland. Their opium-prescribing habits were assessed by means of a postal questionnaire. The results indicate that, among the majority of physicians completing the questionnaire, established guidelines and basic principles of pain control with opiates in cancer patients are largely understood. Oral morphine is chosen by 89% to initiate treatment of chronic cancer pain, and the correct use of slow-release morphine is known to 87% of the responding physicians. Unfortunately, an important minority of physicians does not follow established guidelines in the treatment of cancer pain, and up to 20% still feel that the danger of addiction, respiratory depression and other side-effects are important reasons for withholding opiates in this patient population. The results and their implications are discussed and compared with the current literature on cancer pain management.

Preliminary results of a study assessing asthenia and related psychological and biological phenomena in patients with advanced cancer.

Asthenia is a very common symptom of patients with advanced cancer, but its investigation is hindered by a lack of suitable validated measuring instruments. The goal of the present study was to construct and validate a questionnaire for the study of asthenia in cancer patients, as well as to establish correlations with other symptoms and physiological and biochemical parameters. A group of 31 patients with advanced cancer and a control group of 30 healthy volunteers were examined. The proposed questionnaire, based on visual analogue scales, questions with categorical answers and on the hospital anxiety and depression scale was validated by comparing results of the patient and control groups, by the test/retest method and by comparison with the evaluation of an observer. Correlation with various physiological and biochemical parameters was performed. The questionnaire distinguished well among the patients and control groups. VAS of asthenia proved quite stable over a period of 5 days. Correlations of asthenia with lack of appetite, the hospital anxiety and depression scale, weight, heart rate and serum cortisol levels could be established. No significant correlation between asthenia and various serum markers of inflammation and cytokines, including C-reactive protein, tumour necrosis factor, interleukin-1, and interleukin-2 receptors, could be found. The proposed questionnaire for evaluation of asthenia could be validated in a patient sample of limited size and a simplified questionnaire based on visual analogue scales is being developed for further investigations.

[Chronic tumor pain and opiates: a survey of prescription practice among physicians of German-speaking Switzerland]. / Chronische Tumorschmerzen und Opiate: Eine Umfrage zur Verordnungspraxis bei Arzten in der deutschsprachigen Schweiz.

Motivated by clinical experience that opioids are still not administered adequately in cancer pain, a survey about their use involving 1200 physicians of the German-speaking part of Switzerland was conducted. The results confirm that a part of the treating physicians uses opioids not rationally, but that the majority follows established guidelines in the treatment of cancer pain. The results are compared and discussed with the current literature on the management of cancer pain.