Clinical Neuroscience

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

KRAUSZ Ludovic Tibor1, MAJOR Zoltán Zsigmond1, MURESANU Dafin Fior2, CHELARU Eugen1, NOCENTINI Giuseppe3, RICCARDI Carlo3

OCTOBER 05, 2013

Clinical Neuroscience - 2013;66(09-10)

[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.]

AFFILIATIONS

  1. Department of Pharmacology, University of Medicine and Pharmacy ”Iuliu Hatieganu”, Cluj-Napoca, Romania
  2. Department of Neurology, University of Medicine and Pharmacy ”Iuliu Hatieganu”, Cluj-Napoca, Romania
  3. Department of Clinical and Experimental Medicine, University of Perugia, Italy

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Clinical Neuroscience

[Occurence and molecular pathology of low grade gliomas]

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[Background - The WHO grade I. and II. low-grade gliomas represent nearly the 15% of all primary brain tumors. These tumours contain clinically, hisologically and molecularly distinct tumor types. According to their histologic characteristic, grade II glial tumours are the diffuse astrocytoma, oligodendroglioma and oligoastrocytoma subgroups; the ependymal tumors are not included in this study. Methods - In our publication, we analysed the histological diagnosed glioma cases between 2007 and 2011 at our institution. Results - Low-grade gliomas were diagnosed in 127 cases (62 male / 65 female), and the mean ages were 39 years (±20.3). More than half of the cancers were localizated in the frontal lobe, and the second most frequent area was the temporal lobe. Finally, we comlete our report with an overview of major molecular pathways in low-grade gliomas.]

Clinical Neuroscience

[In memoriam Zoltán Várhegyi (1939. 01. 03-2012. 08. 10.)]

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Clinical Neuroscience

[EDITORIAL MESSAGE]

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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.

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

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[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.]

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[IMMUNE-MEDIATED NEUROLOGICAL DISORDERS]

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[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

Four cases of GABAB receptor encephalitis

SZŐTS Mónika, MORTEN Blaabjerg, KONDZIELLA Daniel, DIÓSZEGHY Péter, BAJZIK Gábor, BERKI Tímea, KÁLMÁN Endre, NAGY Ferenc, ILLÉS Zsolt

GABAB receptor (gamma-aminobutyric acid type B receptors - GABABR) encephalitis is a rare manifestation of autoimmune encephalitides. We report four cases - including the first two Hungarian patients - with some peculiar features. One patient developed subacute disorientation and almost complete loss of short-term memory, but no epilepsy. Without immunotherapy, his memory spontaneously improved up to mild cognitive impairment in six weeks. GABABR antibodies persisted in his serum, and 18 months later, FDG-PET detected abnormal mediastinal lymph nodes and small cell lung cancer (SCLC). Another patient had persistently decreased sodium content in the peripheral blood. In those three patients who died, CSF was abnormal, but CSF was not pathological in the patient, who spontaneously improved. Brain MRI indicated signal intensity changes in the medial temporal areas in three cases. SCLC was found in three patients. Only the patient, who spontaneously improved, survived for more than 24 months. In summary, our cases show that (i) GABABR encephalitis may develop without epilepsy; (ii) the severe short-term memory loss can spontaneously improve; (iii) persistent hyponatremia can be present in the blood; (iv) the patient with benign course without epilepsy and CSF abnormality survived; (v) spontaneously remitting encephalitis can precede SCLC by 1.5 year, which emphasizes that repeated search for cancer is of paramount importance even in cases with spontaneous improvement.

Clinical Neuroscience

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

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[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.]