Introduction: Essential thrombocytosis (ET) is a myeloproliferative disease ongoing with megakary... more Introduction: Essential thrombocytosis (ET) is a myeloproliferative disease ongoing with megakaryocytic hyperplasia in bone marrow and with an increased risk of both bleeding and thrombosis. Macroand microvascular events can be seen in ET. Macrovascular events are frequently arterial, and less commonly venous. Case: A 30-year-old woman presented with unhealed pain on the right side of the head for the preceding 2 months. No abnormal finding was determined at neurological examination apart from bilateral papillary edema. Biochemistry tests were normal except count of platelet. MR venography revealed thrombosis in the superior sagittal sinus and transverse sinus. Presence of JAK2 V617F mutation and presence of BCR/ABL were analysed owing to elevated platelet counts, and JAK2 V617F mutation resulted positive. She was started acetazolamide 250 mg for the headache and the patient was started hydroxyurea 1x500 mg. No decrease in platelet counts was observed with hydroxyurea, and the dose was raised to 1500 mg, at which platelet counts changed back into normal. Discussion and Conclusion: Essential thrombocytosis; can often be diagnosed before serious complications develop since it is easily detected at routine tests. Macrovascular events include coronary artery disease, cerebrovascular event, deep vein thrombosis. CVT is not commonly encountered among the complications seen in ET. The patient was started the cytoreductive agent hydroxyurea and in maintenance treatment. While CVT is not common in ET, JAK2 mutation must be investigated in the presence of CVT among young women patients and in the presence of thrombocytosis.
BackgroundWith the increasing use of biological treatment in inflammatory rheumatic diseases, som... more BackgroundWith the increasing use of biological treatment in inflammatory rheumatic diseases, some neurological side effects, especially central and peripheral nervous system demyelinating disorders, are seen.ObjectivesTo evaluate whether there is an increase in the frequency of neurological disorders with biological treatment compared to the normal population, and the relationship between discontinuation of biological agents and neurological symptoms.MethodsAdult patients using biological treatment followed in the Rheumatology outpatient clinic of Dokuz Eylul University between January 2011 and January 2020 were included. The relationship between biological agents and type, severity and duration of neurological symptoms, and the laboratory, imaging (cranial CT, MRI) and electrophysiological findings of the patients were retrospectively reviewed. Current treatments for rheumatologic disease and neurological findings, and disease activity were investigated.ResultsIn our study, neurol...
Complement is likely to have a role in refractory generalised myasthenia gravis, but no approved ... more Complement is likely to have a role in refractory generalised myasthenia gravis, but no approved therapies specifically target this system. Results from a phase 2 study suggested that eculizumab, a terminal complement inhibitor, produced clinically meaningful improvements in patients with anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis. We further assessed the efficacy and safety of eculizumab in this patient population in a phase 3 trial. We did a phase 3, randomised, double-blind, placebo-controlled, multicentre study (REGAIN) in 76 hospitals and specialised clinics in 17 countries across North America, Latin America, Europe, and Asia. Eligible patients were aged at least 18 years, with a Myasthenia Gravis-Activities of Daily Living (MG-ADL) score of 6 or more, Myasthenia Gravis Foundation of America (MGFA) class II-IV disease, vaccination against Neisseria meningitides, and previous treatment with at least two immunosuppressive therapies or ...
... Comparison of Turkish version of mini mental state examination and short orientation-memory-c... more ... Comparison of Turkish version of mini mental state examination and short orientation-memory-concentration test of cognitive impairment in Alzheimer Disease Meral Oğuz, Görsev Yener, Barış Baklan, Fatma Uzunel, Müslüm Yılmaz, İhsan Şengün J Neurol Sci [Turk] 2003; 20(1 ...
BackgroundThe genetic and epidemiological features of hereditary ataxias have been reported in se... more BackgroundThe genetic and epidemiological features of hereditary ataxias have been reported in several populations; however, Turkey is still unexplored. Due to high consanguinity, recessive ataxias are more common in Turkey than in Western European populations.ObjectiveTo identify the prevalence and genetic structure of hereditary ataxias in the Turkish population.MethodsOur cohort consisted of 1296 index cases and 324 affected family members. Polymerase chain reaction followed by Sanger sequencing or fragment analysis were performed to screen for the trinucleotide repeat expansions in families with a dominant inheritance pattern, as well as in sporadic cases. The expansion in the frataxin (FXN) gene was tested in all autosomal recessive cases and in sporadic cases with a compatible phenotype. Whole‐exome sequencing was applied to 251 probands, selected based on the family history, age of onset, and phenotype.ResultsMutations in known ataxia genes were identified in 30% of 1296 prob...
Introduction: Essential thrombocytosis (ET) is a myeloproliferative disease ongoing with megakary... more Introduction: Essential thrombocytosis (ET) is a myeloproliferative disease ongoing with megakaryocytic hyperplasia in bone marrow and with an increased risk of both bleeding and thrombosis. Macroand microvascular events can be seen in ET. Macrovascular events are frequently arterial, and less commonly venous. Case: A 30-year-old woman presented with unhealed pain on the right side of the head for the preceding 2 months. No abnormal finding was determined at neurological examination apart from bilateral papillary edema. Biochemistry tests were normal except count of platelet. MR venography revealed thrombosis in the superior sagittal sinus and transverse sinus. Presence of JAK2 V617F mutation and presence of BCR/ABL were analysed owing to elevated platelet counts, and JAK2 V617F mutation resulted positive. She was started acetazolamide 250 mg for the headache and the patient was started hydroxyurea 1x500 mg. No decrease in platelet counts was observed with hydroxyurea, and the dose was raised to 1500 mg, at which platelet counts changed back into normal. Discussion and Conclusion: Essential thrombocytosis; can often be diagnosed before serious complications develop since it is easily detected at routine tests. Macrovascular events include coronary artery disease, cerebrovascular event, deep vein thrombosis. CVT is not commonly encountered among the complications seen in ET. The patient was started the cytoreductive agent hydroxyurea and in maintenance treatment. While CVT is not common in ET, JAK2 mutation must be investigated in the presence of CVT among young women patients and in the presence of thrombocytosis.
BackgroundWith the increasing use of biological treatment in inflammatory rheumatic diseases, som... more BackgroundWith the increasing use of biological treatment in inflammatory rheumatic diseases, some neurological side effects, especially central and peripheral nervous system demyelinating disorders, are seen.ObjectivesTo evaluate whether there is an increase in the frequency of neurological disorders with biological treatment compared to the normal population, and the relationship between discontinuation of biological agents and neurological symptoms.MethodsAdult patients using biological treatment followed in the Rheumatology outpatient clinic of Dokuz Eylul University between January 2011 and January 2020 were included. The relationship between biological agents and type, severity and duration of neurological symptoms, and the laboratory, imaging (cranial CT, MRI) and electrophysiological findings of the patients were retrospectively reviewed. Current treatments for rheumatologic disease and neurological findings, and disease activity were investigated.ResultsIn our study, neurol...
Complement is likely to have a role in refractory generalised myasthenia gravis, but no approved ... more Complement is likely to have a role in refractory generalised myasthenia gravis, but no approved therapies specifically target this system. Results from a phase 2 study suggested that eculizumab, a terminal complement inhibitor, produced clinically meaningful improvements in patients with anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis. We further assessed the efficacy and safety of eculizumab in this patient population in a phase 3 trial. We did a phase 3, randomised, double-blind, placebo-controlled, multicentre study (REGAIN) in 76 hospitals and specialised clinics in 17 countries across North America, Latin America, Europe, and Asia. Eligible patients were aged at least 18 years, with a Myasthenia Gravis-Activities of Daily Living (MG-ADL) score of 6 or more, Myasthenia Gravis Foundation of America (MGFA) class II-IV disease, vaccination against Neisseria meningitides, and previous treatment with at least two immunosuppressive therapies or ...
... Comparison of Turkish version of mini mental state examination and short orientation-memory-c... more ... Comparison of Turkish version of mini mental state examination and short orientation-memory-concentration test of cognitive impairment in Alzheimer Disease Meral Oğuz, Görsev Yener, Barış Baklan, Fatma Uzunel, Müslüm Yılmaz, İhsan Şengün J Neurol Sci [Turk] 2003; 20(1 ...
BackgroundThe genetic and epidemiological features of hereditary ataxias have been reported in se... more BackgroundThe genetic and epidemiological features of hereditary ataxias have been reported in several populations; however, Turkey is still unexplored. Due to high consanguinity, recessive ataxias are more common in Turkey than in Western European populations.ObjectiveTo identify the prevalence and genetic structure of hereditary ataxias in the Turkish population.MethodsOur cohort consisted of 1296 index cases and 324 affected family members. Polymerase chain reaction followed by Sanger sequencing or fragment analysis were performed to screen for the trinucleotide repeat expansions in families with a dominant inheritance pattern, as well as in sporadic cases. The expansion in the frataxin (FXN) gene was tested in all autosomal recessive cases and in sporadic cases with a compatible phenotype. Whole‐exome sequencing was applied to 251 probands, selected based on the family history, age of onset, and phenotype.ResultsMutations in known ataxia genes were identified in 30% of 1296 prob...
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