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Clinical Neuroscience

MARCH 30, 2020

CANOMAD syndrome with respiratory failure

SALAMON András, DÉZSI Lívia, RADICS Bence, VARGA Tímea Edina, HORTOBÁGYI Tibor, TÖMÖSVÁRI Adrienn, VÉCSEI László, KLIVÉNYI Péter, RAJDA Cecília

CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, disialosyl antibodies) syndrome is a rare polyneuropathy. IgM paraproteins react with ganglioside-containing disialylated epitopes resulting in dorsal root ganglionopathy and B-lymphocyte infiltration of cranial and peripheral nerves. Clinical features include ataxia, slight muscle weakness, areflexia, sensory- and cranial nerve symptoms. Case studies have reported the efficacy of rituximab and intravenous immunoglobulin (IVIg) treatments. We present the case of a 57-year-old man, who had difficulty walking, with numbness and clumsiness in all limbs. He had areflexia, vibratory sensation loss and ataxia. Laboratory tests showed IgM monoclonal components and disialosyl antibodies in the serum. Nerve conduction studies indicated severe sensorimotor demyelinating polyneuroradiculopathy. Despite IVIg and rituximab treatments, the patient’s disease course gradually worsened and he died of respiratory failure. Neuropathological examination revealed dorsal column- and dorsal root atrophy with mixed mononuclear cell infiltration. This article aims to draw attention to this syndrome, and the use of early potent immunosuppressive treatment to improve patients’ quality of life.

Lege Artis Medicinae

DECEMBER 18, 2016

[Intravenous immunoglobulin immunotherapy in immune mediated habitual abortions]

FÜLÖP Vilmos, PETRÁNYI Győző

[INTRODUCTION - Based upon international and domestic research data authors summerize the humoral and cellular im­munregulatory disorders which can be found in the background of “immune me­diated abortions” (IMA). PATIENTS AND METHODS - Within the frame of a home research program a special examination protocol was elaborated in order to sepatare alloimmune habitual abortions from autoimmune and non immune backgrounds. After all other causes were excluded erythrocyte antibody inhibition assay (EAI) was used for measuring the serum level of FcgRII receptor blocking IgG antibody, because its lack an important diagnostic parameter. Among the cell mediated immunofuntional tests the mixed lymphocyte culture reaction (MLC) was the most useful. During the roughly last 16 years 67 out of 76 selected alloimmun IMA patients were administered Intratect IVIG treatment without any particular selection among them. IVIG treatment was first applied on the completed 5-6th week of pregnancy and doses of 0,3-0,4g/kg bodyweight per oc­casion were given 3 times with 3-week intervals. RESULTS - Altogether a significant rise in the serum level of blocking antibodies was shown after each IVIG treatment although a slight decrease was seen after every given dose. Of the 67 IMA patients 54 carried infants to term during the study period. In 4 cases of abortion no cause was identified with post hoc diagnosis. Thus the success rate of this type of IVIG therapy was 93.1% (58/54). Conclusion - In approprietly selected alloimmun IMA cases the IVIG generated immunoregulatory and antiimflammatory pathways may contribute to its net positive reproductive effect.]

Clinical Neuroscience

NOVEMBER 30, 2016

Patient characteristics and the effects of intravenous immunoglobulin in patients with Guillain-Barre syndrome

GUZEY ARAS Yesim, TANIK Osman, DOGAN GÜNGEN Belma, KOTAN Dilcan

Purpose - The purpose of this study is to determine the diagnosis- and treatment-related characteristics in Guillain-Barré syndrome (GBS) and to evaluate the effects of early intravenous immunoglobulin (IVIg) treatment on disability, mortality and prognosis. Materials and methods - Adult patients who were diagnosed with GBS in our clinic between January 2000 and January 2014 were retrospectively scanned. While the patients undergoing IVIg treatment were included in the study, the other neuropathic diseases were excluded. Patients were divided into two groups based on the administration time of the IVIg treatment; Group 1 (<7 days) and Group 2 (≥ 7 days) Group 1 consisted of patients undergoing IVIg treatment within 7 days after presentation of symptoms and Group 2 consisted of patients undergoing IVIg treatment on and after 7th day following presentation of symptoms. The scores from Hughes Functional Grading Scale (HFGS) on admission and one month laterwere recorded in all patients in order to evaluate the disability and prognosis in terms of demographic and clinical laboratory characteristics. Results - In this study, 49 GBS patients were included (31 patients in Group 1 and 18 patients in Group 2). Demyelinating form of GBS was determined in 22 (44.8%) patients.). While there was no difference between both groups (p: 0.288, p: 0.762, p: 0.693 respectively) in terms of intensive care and rehabilitation requirement and progression, only 2 patients in Group 1 died. While HFGS mean score on admission in all the patient groups was 3.27±0.974, their HFGS mean score at month 1 was 2.53±1.226. There was no difference between the groups in terms of HFGS mean scores on admission and at month 1. Within each groups, there was a significant improvement between initial (on admission) HFGS scores and HFGS scores acquired at month 1. Conclusion - In this study, demyelinating form was more frequent than axonal form. A total of 2 g/kg dose of IVIg treatment administered for 5 days as a standard in GBS patients ensured a significant improvement on both disability and early and late administration and early administration of the treatment does not lead to any difference in intensive care unit and rehabilitation requirements.

Clinical Neuroscience

MARCH 30, 2013

[Inclusion body myositis - a rarely recognized disorder]

DÉZSI Lívia, DANIELSSON Olof, GÁTI István, VARGA Edina, VÉCSEI László

[Inclusion body myositis is the most common disabling inflammatory myopathy in the elderly. It is more frequent in men and after the age of 50 years. Inflammatory and degenerative features coexist. There is a T-cell mediated autoimmunity driven by in situ clonally expanded cytotoxic CD8-positive T-cells invading non-necrotic muscle fibres expressing MHC-I antigen. The hallmarks of degeneration are the deposition of protein aggregates and the formation of vesicles. The course of the disease is slow and the diagnosis is usually set after several years. The muscle weakness and wasting is assymetric, affecting predominantly distal muscles of the upper extremity and proximal muscles of the legs. The signs and clinical course can be characteristic, but the diagnosis is established by muscle biopsy. There is currently no evidence based effective treatment for sIBM. Prednisone, azathioprine, methotrexate, cyclosporine and IFN-β failed. Oxandrolon did not improve symptoms. Treatment with intravenous immunglobuline (IVIG) induced in some patients a transient improvement of swallowing and of muscle strenght, but the overall study results were negative. A T-cell depleting monoclonal antibody (alemtuzumab), in a small uncontrolled study slowed down disease progression for a six-month period. Repeated muscle biopsies showed the reduction of T-cells in the muscle and the suppression of some degeneration associated molecules. An effective therapeutic mean should act on both aspects of the pathomechanism, on the inflammatory and the degenerative processes as well.]

Lege Artis Medicinae

JULY 14, 2008

[IMMUNE-MEDIATED NEUROLOGICAL DISORDERS]

CSÉPÁNY Tünde

[Multiple sclerosis, myasthenia gravis and chronic inflammatory neuropathies share the common feature of chronic course with potential development of disability due to the damage caused by immunological processes. Early detection and precise diagnosis is very important, because most patients respond well to proper immunomodulatory treatment. The diagnosis requires extensive knowledge of the disease and is based on the clinical symptoms recognised by the GP, as well as on complex assessment of the results of special neurophysiological, radiological and laboratory examinations. The present paper reviews the major immune-mediated neurological disorders and discusses their targeted immunological treatment.]

Lege Artis Medicinae

DECEMBER 19, 2008

[IMMUNOLOGICAL ASPECTS OF PREGNANCY FAILURES - NOVEL THERAPEUTIC APPROACHES]

FÜLÖP Vilmos

[Recurrent miscarriage and failure of in vitro fertilization and embryo transfer affect millions of women annually. It is one of the great problems of the medical community that in as many as 75% of these failures their cause is never established. Considering that recurrent abortions may have several causes including anatomical, morphological, genetic, medical, bacteriological as well as immunological abnormalities it is inevitable to pursue these kinds of examinations and tests. One part of the immunological background can be explained by autoimmune diseases while the other part of it is associated with immunopathological factors. Currently available practical testing batteries that can directly assess immunological background, as well as their importance are covered. Recently developed and mainly immunology based treatments are also described. The results generally show that immunoglobulin products can be used safely in pregnancy. In the author's institute the efficacy of this therapy has been 93.5% in cases of immune-mediated abortion. As an alternative to intravenous immunoglobulin (IVIG) therapies, partner specific platelet suspension can be used only in selected cases with proven immunological background. Therapy monitoring, that is, assessment of the functional efficacy of IVIG is possible by determining FcR blocking antibodies in the maternal serum. Based upon the functional percentage of serum blocking activity the dosage and number of treatments can be changed.]

Clinical Neuroscience

NOVEMBER 30, 2009

[Guillain–Barré syndrome in childhood]

KOLLÁR Katalin, LIPTAI Zoltán, ROSDY Beáta, MÓSER Judit

[Background - Guillain-Barré syndrome (GBS) is clinically well known since 1916. It can occur at any age. Its main characteristic is acute rapidly ascending flaccid paresis. It is a neuro-immunologic disorder with heterogeneous background. In Hungary we could not find reports about big paediatric population with GBS. Patient and method - We analysed retrospectively the data of 38 children diagnosed and treated with GBS at the Neurological Department of Paul Heim Children’s Hospital or at the Paediatric Department of St. László Hospital from January 2000 till April 2008. We analysed the clinical characteristics, seriousness of clinical signs, laboratory results, and electrophysiological features of them as well documented the preceding illness. We observed the effectiveness of our treatment; we measured the speed and time of the healing process and documented the residual clinical signs. Results - 35 children could be classified as having acute inflammatory demyelinating polyneuropathy (AIDP), 2 as having acute motor axonal neuropathy (AMAN) and 1 as Miller-Fisher syndrome. By those patients who at the very beginning did not show the characteristic clinical signs, electrophysiology helped in establishing the diagnosis. By one child spinal MRI with gadolinium supported our diagnosis. Those children, who lost their ambulation, got immunotherapy: intravenous immunoglobulin (IVIG) or plasmapheresis (PEX). Both method seemed to be effective. None of our patients died. All were cured. By five patients residual clinical symptoms could be found. Conclusion - The disease process, the relative incidence of each subtype of GBS is nearly similar to that in Western Europe and North America according to the literature. By the currently used immune therapy most of the pediatric patients recover fully within a short time.]