Twice repeated ischemic brain infarct in patient with primary antiphospholipid syndrome and S-protein deficiency

Nie ma jednolitej koncepcji dotyczącej roli dziedzicznego niedoboru naturalnych inhibitorów koagulacji (do których należy białko S) w występowaniu udarów niedokrwiennych u młodych osób. W pracy przedstawiono przypadek obecnie 50-letniego mężczyzny z obciążającym wywiadem rodzinnym (matka i ojciec) w kierunku choroby zakrzepowej, u którego 3-krotnie wystąpiło zapalenie żył kończyn dolnych, a w 46 i 47 roku życia kolejne udary niedokrwienne lewego, a następnie prawego płata skroniowego. Ponadto, w wieku 24 lat przebył kilka krótkotrwałych epizodów zaburzeń widzenia. Wykonane badania (przeciwciała antykardiolipinowe klasy IgG i IgM) pozwoliły na rozpoznanie pierwotnego zespołu antyfosfolipidowego. Analiza układu krzepnięcia ujawniła niedobór białka S (50- i 45-procentowy) najprawdopodobniej o charakterze dziedzicznym, gdyż wykluczono możliwe nabyte przyczyny obniżonego stężenia tego białka. Wydaje się, że, obok zespołu antyfosfolipidowego, niedobór białka S u wymienionego wyżej pacjenta był dodatkowym czynnikiem sprzyjającym zakrzepom.There is no uniform conception dealing with the role that hereditary deficit of natural coagulation inhibitors (to which belong S-protein) plays in cases of brain ischemic infarcts in young persons. We present the case of man (at present 50-years-old) with history of thrombotic disease in family (mother and father), who experienced triple inflammation of pelvic limbs veins and in his 46th and 47th years of life experienced in succeeding ischemic strokes of the left and next of the right temporal lobe. Moreover since his 24th year he had transitory episodes of sight disturbances. Examinations carried out (IgG and IgM class anticardiolipin antibodies) permitted to diagnose the primary antiphospholipid syndrome. Analysis of coagulation showed decreased serum concentration (50% and 45%) likely of hereditary nature because acquired causes of diminished serum S concentration of this protein were excluded. It seems, that S-protein deficiency in our patient was next to antiphospholipid syndrome and additional factor favouring thrombosis

Efficacy and Safety of Intravenous Immunoglobulin Treatment in Selected Neurological Diseases—One Centre’s Experience Based on the Therapy of 141 Patients

Background: Intravenous immunoglobulins (IVIg) are the first-choice drugs for the treatment of certain neuroimmune diseases. The aim of this study was to evaluate the efficacy and safety of IVIg in patients with selected nervous system diseases. Methods: The study enrolled patients who received IVIg in programmes financed by the National Health Fund in Poland. The status of patients upon inclusion and during treatment was assessed using scales dedicated to specific neurological diseases. Results: The study enrolled 141 patients aged 56.28 ± 14.72 (51.77% female): 21 patients with myasthenia gravis (MG), 65 with chronic inflammatory demyelinating polyneuropathy (CIDP), 30 with Guillain–Barré syndrome (GBS), 12 with neuromyelitis optica spectrum disorder (NMOSD) and 13 patients with autoimmune encephalitis (AE). Neurological improvement was found in 14 (66.66%) MG patients (with a reduction of at least three points on the Quantitative Myasthenia Gravis Score (QMGS) within 14 days from the completion of the cycle), and in 34 (52.3%) GBS patients (with a reduction of at least one point on the Medical Research Council Scale within 14 days from the completion of the cycle). The parameters with the strongest effect on clinical improvement in MG patients were age [OR 1.033, CI 95% [0.09–1.09], p = 0.049] and baseline QMGS [OR 0.505; CI 95% [0.24–0.87], p = 0.038]. In the majority of CIDP patients (27, 97%) and NMOSD patients (6, 50%), neurological stabilisation was observed (without clinical improvement, defined for CIDP patients as an increase of at least two points on the Lovett Scale after three courses of IVIg were administered, and for NMOSD patients as an increase of at least one point on the Medical Research Council Scale and/or a shift of at least 0.3 logMAR after three courses of treatment). Deep-vein thrombosis was only one serious adverse event in the total group of patients treated with IVIg. Conclusions: The use of IVIg in patients with MG and GBS mostly results in neurological improvement, while in patients with NMOSD and CIDP, it mostly results in disease stabilisation. This could indicate the predominant anti-idiotypic antibody activity of IVIg in acute neuroimmune diseases or during exacerbations in chronic autoimmune diseases. The therapy of AE in comorbid neoplastic disease is burdened with an elevated risk of failure for IVIg. The results of our study confirm the improved safety of IVIg for selected neurological diseases