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Clinical Neuroscience

JANUARY 30, 2021

Electrophysiological investigation for autonomic dysfunction in patients with myasthenia gravis: A prospective study

NALBANTOGLU Mecbure, AKALIN Ali Mehmet, GUNDUZ Aysegul, KIZILTAN Meral

Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular transmission. Autonomic dysfunction is not a commonly known association with MG. We conducted this study to evaluate autonomic functions in MG & subgroups and to investigate the effects of acetylcholinesterase inhibitors. This study comprised 30 autoimmune MG patients and 30 healthy volunteers. Autonomic tests including sympathetic skin response (SSR) and R-R interval variation analysis (RRIV) was carried out. The tests were performed two times for patients who were under acetylcholinesterase inhibitors during the current assessment. The RRIV rise during hyperventilation was better (p=0.006) and Valsalva ratio (p=0.039) was lower in control group. The SSR amplitudes were lower thereafter drug intake (p=0.030). As much as time went by after drug administration prolonged SSR latencies were obtained (p=0.043).Valsalva ratio was lower in the AchR antibody negative group (p=0.033). The findings showed that both ocular/generalized MG patients have a subclinical parasympathetic abnormality prominent in the AchR antibody negative group and pyridostigmine has a peripheral sympathetic cholinergic noncumulative effect.

Clinical Neuroscience

JANUARY 30, 2020

Myasthenia gravis, Guillain-Barré syndrome, or both?

ERDOGAN Cagdas, TEKIN Selma, ÜNLÜTÜRK Zeynep, GEDIK Korkut Derya

Myasthenia gravis (MG) and Guillain-Barré syndrome (GBS) are autoimmune disorders that may cause weakness in the extremities. The coexistence of MG and GBS in the same patient has rarely been reported previously. A 52-year-old male presenting with ptosis of the left eye that worsened with fatigue, especially toward evening, was evaluated in our outpatient department. His acetylcholine receptor antibody results were positive, supporting the diagnosis of MG. His medical history revealed a post-infectious acute onset of weakness in four extremities, difficulty in swallowing and respiratory failure, which was compatible with a myasthenic crisis; however, his nerve conduction studies and albuminocytologic dissociation at the time were compatible with GBS. With this case report, we aimed to mention this rare coincidental state, discuss possible diagnoses and review all other similar cases in the literature with their main features.

Hypertension and nephrology

OCTOBER 20, 2013

[Therapeutic apheresis in pediatry]

TÚRI Sándor, BERECKI Csaba, HASZON Ibolya, PAPP Ferenc

[The possible mechanisms of therapeutic plasma mexchange: 1. the removal of circulatory plasma factor (anti Gbm disease, myasthenia gravis, Guillain Barré syndrome), 2. monoclonal antibody (Waldenström macroglobulinemia, myeloma protein), 3. circulatory immuncomplexes cryoglobulinaemia, myeloma protein, SLE), 4. alloantibody, 5. toxic factor, 6. replacement of a specific plasma factor, 7. a repear of the function of reticulo-endothelial system, 8. the removal of the inflammatory mediators, 9. the changes of the ratio of antigen-antibody which makes immuncomplexes more soluble, 10 stimulation of lymphocyte clones for supporting the cytotoxic therapy. Indications of emergency plasmapheresis: 1. Goodpasture syndrome with rapidly progressive glomerulonephritis and hemoptoe, 2. hyperviscosity syndrome, 3. TTP/HUS, 4. High level of factor VIII inhibitor, 5. respiratory insufficiency Guillain-Barré syndrome, 6. myasthenia gravis, 7. acute mushroom intoxication, or protein bound toxins. Further indications for plasmapheresis: 8. cryoglobulinemia, 9. other cases of rapidly progressive glomerulonephritis (when steroid+ cyclophosphamide are ineffective), 10. Wegener granulomatosis, 11. polyarteritis nodosa, 12. systemic lupus erythematosus (when steroid and cyclophosphamid therapy is not effective or associated with cerebral vasculitis, antiphospholipid syndrome combined with bleeding and thrombosis), 13. focal segmental glomerulosclerosis (resistant for therapy), 14. acute tubulointerstitial nephritis, 15. acute vascular rejection, 16. rheumatoid arthritis systemic type, 17. hypertrigliceridemia (≥25 mM), 18. thyreotoxic crisis, 19. acute necrotizing pancreatitis, 20. acute fulminant hepatitis, 21. paraquat intoxication, 22. snake bite (when antiserum is unavailable), 23. drug intoxication.]

Clinical Neuroscience

OCTOBER 05, 2013

[Characterization of CD4+ and CD8+ Tregs in a Hodgkin’s lymphoma patient presenting with myasthenia-like symptoms]

KRAUSZ Ludovic Tibor, MAJOR Zoltán Zsigmond, MURESANU Dafin Fior, CHELARU Eugen, NOCENTINI Giuseppe, RICCARDI Carlo

[The co-occurrence of Hodgkin’s lymphoma (HL) and myasthenia gravis (MG) is a rare phenomenon that is sometimes considered a paraneoplastic manifestation. There are a few documented cases in which myasthenia symptoms manifested only after the surgical removal of the tumor. However, the biological basis of this association is unknown. One hypothesis is that it derives from the infiltration of the residual thymic tissue by the developing tumor. In our case, the myasthenic symptoms led to the HL diagnosis. Our objective was to investigate the T cell phenotype in a HL patient presenting myasthenia-like symptoms. In patients with autoimmune disease, Tregs are usually decreased, but in some diseases, they appear to be increased. It has been speculated that this phenomenon may occur due to a homeostatic attempt by the immune system to control the expansion of auto-reactive effector cells. In the described patient the proportion of lymphoma infiltrating Tregs was high (more than 10% of CD4+ and 1.34% of CD8+ cells), suggesting that Tregs are increased in patients suffering from HL and eventually of myasthenia gravis. Treg involvement in HL is controversial and is currently under investigation. In this context, our data may contribute to a better understanding of the underlying mechanism of the link between HL and autoimmune phenomena.]

Clinical Neuroscience

MAY 30, 2013

[Congenital myasthenic syndromes and transient myasthenia gravis]

GAJDA Anna, SZABÓ Hajnalka, GERGEV Gyurgyinka, KARCAGI Veronika, SZABÓ Nóra, ENDREFFY Emőke, TÚRI Sándor, SZTRIHA László

[Hypotonia in the neonatal period and early infancy is a common clinical finding. It can be caused by various heterogeneous disorders of different origin which might lead to diagnostic difficulties. Disorders of the neuromuscular junction, such as congenital myasthenic syndromes and neonatal transient myasthenia gravis are among the aetiologies. We report on a case of congenital myasthenia caused by mutation in the long cytoplasmic loop of the epsilon subunit of the acetylcholine receptor and a neonate of a myasthenic mother diagnosed with transient myasthenia gravis.]

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.]

Clinical Neuroscience

MARCH 30, 2006

[THE USE OF INTRAVENOUS IMMUNGLOBULIN IN THE TREATMENT OF AUTOIMMUNE NEUROMUSCULAR DISEASES]

MOLNÁR Mária Judit

[Intravenous immunglobulin given in autoimmune neuromuscular disorders modulates the immune system by complex actions, including, 1. the modification of the expression and function of Fc receptors, 2. interference with the activation of the complement and the cytokine network, 3. neutralisation of antiidiotypic antibodies, 4. effects on the activation, differentiation and effector functions of the T and B cells. Controlled trials have shown that intravenous immunglobulin is effective as first-line therapy in patients with Guillain-Barré syndrome and multifocal motor neuropathy. In case of steroid resistance or coexisting diabetes mellitus, intravenous immunglobulin can be the first line therapy in chronic inflammatory demyelinating polyneuropathy as well. As an alternative therapy it can be a second-line choice in dermatomyositis, myasthenia gravis, Lambert-Eaton myasthenic syndrome, and stiff person syndrome. While it has a remarkably good safety record for long term administration the following side effects have been observed: headache, skin rash, thromboembolic events and renal tubular necrosis. In some disorders, the appropriate dose and frequency of infusions that maintain a satisfactory therapeutic response is well defined on the basis of data of evidencebased medicine, whereas in others it still remains to be defined. For the analysis of pharmacoeconomical aspects and the mechanism(s) of response differences in the same disease categories, further studies are necessary.]

Lege Artis Medicinae

APRIL 20, 2005

[RARE ASSOCIATION OF HODGKIN’S LYMPHOMA, GRAVES’ DISEASE AND MYASTHENIA GRAVIS]

RESS Zsuzsa, MEKKEL Gabriella, ILLÉS Árpád

[INTRODUCTION - In some cases other diseases associate with Hodgkin’s lymphoma, when it is diagnosed or relapses. Association of Hodgkin’s lymphoma with Graves’ disease and myasthenia gravis in one patient has not yet been reported in the literature. CASE REPORT - We report on a young female patient who had suffered from Hodgkin’s lymphoma since 1996. He had received polychemotherapy and mantle field irradiation previously. After treatment, complete remission was stated in 2000. Then she was treated because of Graves’ disease. In 2001 she complained of dysarthria, dysphagia, ptosis and diplopia. Thorough examinations proved myasthenia gravis. Considering the progression plasmapheresis was administered several times with cyclophosphamide and intravenous immunglobulin, besides conservative therapy. Recently she is euthyroid state, Hodgkin’s disease is in remission and her only complaint is dysarthria. CONCLUSION - The importance of this case on one hand is the rare association of these diseases, on the other is that Graves’ disease and myasthenia gravis occurred during in the remission of Hodgkin’s disease. Causal relation is not unambiguous but the role of disturbed immunregulation caused by Hodgkin’s lymphoma or the irradiation of the neck region can also contribute to it. The pure coincidental occurrence of Hodgkin lymphoma, Graves’ disease and myasthenia gravis is highly unlikely.]

Clinical Neuroscience

JULY 10, 2004

[Myasthenia in a patient with sarcoidosis and schizophrenia (in English language)]

RÓZSA Csilla, KIS Gábor, KOMOLY Sámuel

[A 44-year-old male patient was hospitalised with paranoid schizophrenia in 1985. Depot neuroleptic treatment was started which successfully prevented further psychotic relapses for the next ten years. His myasthenia gravis started with bulbar signs in 1997 and the symptoms soon became generalized. The diagnosis of myasthenia gravis was confirmed by electromyography, by positive anticholinesterase test and by the detection of anti-acetylcholine receptor antibodies in the serum. Mediastinal CT examination showed enlarged hilar lymph nodes on the left but no thymic pathology was observed. Mediastinoscopy was performed and biopsies were obtained from the affected nodes. Histology revealed sarcoidosis. The patient suffered respiratory crisis following the thoracic intervention (in September 1998). Combined oral corticosteroid (64 mg methylprednisolone/e.o.d.) and azathioprine (150 mg/day) treatment regimen was initiated and complete remission took place in both the myasthenic symptoms and the sarcoidosis. The follow-up CT scans showed no mediastinal pathology (January 2000). During steroid treatment a transient psychotic relapse occured which was successfully managed by supplemental haloperidol medication added to his regular depot neuroleptics. The patient currently takes 150 mg/day azathioprine and receives 40 mg/month flupentixol depot im. His physical and mental status are stable and he has been completely symptome free in the last 24 months. The association of myasthenia gravis and sarcoidosis is very rare. To our best knowledge no case has been reported of a patient suffering from myasthenia gravis, sarcoidosis, and schizophrenia at the same time.]

Clinical Neuroscience

MARCH 15, 2004

[Neurological aspects of some sleep disorders]

SZŰCS Anna

[My aim is to examine the relation between some sleep disorders and neurological diseases; to analyse their mutual interactions in order to achieve new practical data for clinical use. In the theoretical part I summarise some main points of sleep physiology concentrating on the associations of sleep regulation and neurological diseases. In my examinations, besides clinical methods, the most important tools used are sleep analyses performed by polysomnography and MESAM IV as well as brain imaging methods. To assess clinical state of my stroke patients I utilised NIH Stroke Scale. I found pathological sleep apnoea frequency in more than half of the patients in any type (bleeding/infarction) of acute stroke. In a prospective study, sleep apnoea parameters remain permanent during 3 months in the ischaemic group; on the other hand, sleep apnoea improves during follow up after brain haemorrhages. I showed pathological sleep apnoea frequency in myasthenia gravis among male patients without daytime respiration complaint. I looked for the link between the mechanism of the sleep disorder and the underlying organic lesion in two cases. In this analyses I took into account the function of the affected structure in sleep regulation. I found a basal forebrain tumour, affecting sleep regulating centres underlying severe insomnia and I suggest a neurovascular compression of the lateral preoptic area of the hypothalamus being the reason of sleep related painful erection, a parasomnia of unknown origin.]