[Neuromyelitis optica spectrum disorder and pregnancy]. / Neuromyelitis-optica-Spektrum-Erkrankung und Schwangerschaft.

BACKGROUND: Neuromyelitis optica spectrum disorders (NMOSD) are autoimmune inflammatory diseases of the central nervous system that mainly affect women. In some of these patients NMOSD occurs during fertile age. For this reason, treating physicians may be confronted with questions concerning family planning, pregnancy and birth. OBJECTIVE: This study provides an overview on the influence of NMOSD on fertility, pregnancy complications and pregnancy outcome. The effect of pregnancy on NMOSD course and therapy options during pregnancy are discussed. MATERIAL AND METHODS: A search of the current literature was carried out using the PubMed database. RESULTS AND CONCLUSION: Animal studies have shown lower fertility rates in NMOSD; however, studies investigating fertility in NMOSD patients are lacking. Pregnancy in NMOSD patients are associated with an increase in postpartum disease activity and a higher grade of disability after pregnancy. Some studies showed higher risks of pregnancy complications e. g. spontaneous abortions and preeclampsia. With a few limitations, acute relapses during pregnancy can be treated with methylprednisolone and/or plasma exchange/immunoadsorption. Stopping or continuing immunosuppressive therapy with azathioprine or rituximab during pregnancy should be critically weighed considering previous and current disease activity. Therefore, a joint supervision by a specialized center is recommended, particularly in specific situations such as pregnancy.

[Therapeutic options for autoimmune encephalomyelitis]. / Therapieoptionen bei immunvermittelten Enzephalomyelitiden.

Autoantibodies to neuronal tissue are becoming increasingly more important in the evaluation and classification of several neurological diseases, e.g. neuromyelitis optica, paraneoplastic syndromes of the central nervous system (CNS), stiff person syndrome or autoimmune epilepsy. As these disorders are rare, no evidence-based recommendations for therapy are available. Currently, immunomodulating or immunosuppressive drugs are administered in most cases. In paraneoplastic syndromes treatment of the underlying cancer is of considerable importance. This overview summarizes current experiences and recommendations in the treatment of autoimmune neurological disorders.

[Elective termination of respiratory therapy in amyotrophic lateral sclerosis]. / Elektive Termination der Beatmungstherapie bei der amyotrophen Lateralsklerose.

BACKGROUND: Due to the growing use of artificial respiration in amyotrophic lateral sclerosis (ALS), physicians are increasingly confronted with patients seeking discontinuation of therapy. Yet there are few systematic investigations of the withdrawal of ventilation therapy. PATIENTS AND METHODS: In a retrospective investigation of nine German ALS patients, clinical data were recorded from the discontinuation of noninvasive ventilation (n=4) and mechanical ventilation (n=5). RESULTS: In cases of residual spontaneous breathing, intensified symptom control of dyspnea and anxiety was possible with intravenous morphine sulfate at a low dose rate (10 mg/h) but high cumulative dose (185-380 mg). The terminal phase after removing the mask was protracted (22:10 h to 28:00 h). In cases of minimal or absent spontaneous breathing the disconnection was realized in deep sedation, which required a moderate total dose of morphine sulfate (120 mg) but a high dosage rate (up to 300 mg/h). The terminal phase in deep sedation was short (15-80 min). CONCLUSION: The elective termination of ventilation requires differentiated pharmacologic palliative care. More controlled studies are required in order to establish evidence-based guidelines for the termination of ventilation.

[Progressive muscle atrophy. A rarely diagnosed variant of amyotrophic lateral sclerosis]. / Progressive Muskelatrophie. Eine unterdiagnostizierte Variante der amyotrophen Lateralsklerose.

Progressive muscle atrophy (PMA) is a degenerative disease of the lower motor neuron. The course of the illness and the fatal prognosis correspond to those of amyotrophic lateral sclerosis (ALS). Neuropathologic and genetic findings support categorizing PMA within the spectrum of ALS, even though no clinical sign of a disorder of the upper motor neuron is demonstrable. The diagnosis of PMA is based on advanced extremity pareses and atrophies with a high progression rate. Respiratory insufficiency is determinative of the prognosis. Absent or late affection of bulbar functions is characteristic of the disease. Intraneuronal bunina bodies and ubiquitine-positive inclusions, which are established morphologic characteristics of ALS, are found post mortem. The treatment options of riluzol medication, respiratory therapy, and nutrition are analogous to those for typical ALS.