Clinical Neuroscience

[Thymic lesions in myasthenia gravis]

SZOBOR Albert1, SZEGEDY László1

MAY 01, 1967

Clinical Neuroscience - 1967;20(05)

[After a brief review of the literature on myasthenia gravis and thymic lesions, the authors address the issue based on their patient records. The significance of lymphoid and epithelial thymic hyperplasia in relation to myasthenia; the prevalence of mixed hyperplasia; the characteristics of thymic tumours; the relatively benign nature of tumours with malignant tissue structure.]

AFFILIATIONS

  1. Budapesti Orvostudományi Egyetem Psychiatriai Klinikája

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Clinical Neuroscience

[Statistical processing of 15 years of ES material from the Psychiatric Clinic of the Budapest Medical University]

ORVOSI Kara

[In the struggle for the cure of mental illnesses, for the health of the human mind, one of the central issues was therapeutic optimism, therapeutic activitas. The sick person and society as a whole expect the best possible restitution from the doctor, and the doctor's duty as a practitioner is to take responsibility, to take the lead, to apply the most modern methods of treatment. ]

Clinical Neuroscience

[Schizophrenia or schizophrenias]

DRIETOMSZKY Jenő, BRUNECKER Györgyi

[In analysing the development of the concept of "schizophrenia", both in terms of its professional history and its theoretical basis, authors adopt Nyirő's position that "...we cannot speak of schizophrenia, only of schizophrenics..." In the sense of Sternberg's concept, they narrow down their position by stating that a nosological approach and Leonhardian taxonomy are essential requirements for a substantive answer to the question.]

Clinical Neuroscience

[Resuscitation and neuropsychiatric therapy experiences with electrostimulator]

FORNÁDI Ferenc, BARTOS Valéria, ZSOMBÓK György, KAFFKA Károly

[The authors describe their joint research with Nyirő on the therapeutic value of transcerebral electrostimulation. Their experience: 1. electrostimulation is essential not only in the treatment of respiratory paralysis following electrotrauma, but also in the prevention and treatment of certain complications of psychiatric so-called major somatic treatments; 2. it is also a new treatment option for hormone-refractory hypothalamic syndromes, such as amenorrhoea, using more mitigated current parameters. ]

Clinical Neuroscience

[The problems of the psychoanalytical distinguishing between typical and atypical types of drunkenness]

HUSZÁR Ilona, IRÁNYI Jenőné

[In their communication, the authors deal with the differentiation of acute, typhoidal intoxication states from atypical (pathological) intoxication states, emphasising the sociological importance of the differentiation and defining the legal basis for the differentiation. The work of Nyirő on the subject is discussed in detail, followed by a discussion of the symptoms of atypical intoxication and their diagnostic significance. They also analyse all the external and internal factors that may play a role in the development of atypical drunkenness. It is considered possible to distinguish between the two forms of intoxication only by examining all the factors in each case in context, but it is stressed that a sharp dividing line cannot always be drawn. ]

Clinical Neuroscience

[The role of exogenous factors in psychiatric disorders ]

IVÁN László, TAKÁCS László, PETHŐ Bertalan

[Authors discuss the psychopathological significance of isolation or deprivation. They draw attention to these specific exogenous factors and stress the importance of a multidimensional approach in their interpretation. Using both the literature and their own clinical experience, they aim to outline certain conclusions on this topic. ]

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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

Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge

TOLVAJ Balázs, HAHN Katalin, NAGY Zsuzsanna, VADVÁRI Árpád, CSOMOR Judit, GELPI Ellen, ILLÉS Zsolt, GARZULY Ferenc

Background and aims – Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports – Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion – Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.

Clinical Neuroscience

[Diagnosis of multiple sclerosis: A review of the 2017 revisions of the McDonald criteria]

CSÉPÁNY Tünde

[The revolutionary progress of research in neuroimmu­nology has led to the introduction of disease modifying therapies in multiple sclerosis at the end of the last century. The International Panel on Diagnosis of Multiple Sclerosis originally proposed the 2001 McDonald criteria to facilitate the diagnosis of MS in patients with the first objective neurological symptom(s) suggesting demyelinating event, when magnetic resonance imaging is integrated with clinical and other paraclinical diagnostic methods. New terms have been introduced to substitute clinical information by MRI: dissemination in space - indicating a multifocal central demyelinating process and dissemination in time - indicating the development of new CNS lesions over time. The criteria for diagnosis of Multiple Sclerosis have continuously evolved, they were modified in 2005 and 2010 allowing for an earlier and more accurate diagnosis of MS over time, and they provided the most up-to-date guidance for clinicians and researchers. The last recommended revisions relied entirely on available evidence, and not on expert opinion thereby reducing the risk of the misdiagnosis. The 2017 McDonald criteria continue to apply primarily to patients experiencing a typical, clinically isolated syndrome. In this review, we provide an overview of the recent 2017 revisions to the criteria of dissemination in space and time with the importance of the presence of CSF-specific oligoclonal bands; keeping fully in mind that there is no better explanation for symptoms than diagnosis of MS. In the future, validation of the 2017 McDonald criteria will be needed in diverse populations. Further investigations are required on the value of new MRI approaches, on optic nerve involvement, on evoked potential and optical coherence tomography, in order to assess their possible contribution to diagnostic criteria.]

Clinical Neuroscience

Myasthenia gravis, Guillain-Barré syndrome, or both?

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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.

Clinical Neuroscience

[Role of peginterferon-β-1a in the therapy of multiplex sclerosis]

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[The subcutaneous peginterferon-b-1a is recently introduced in the therapy of relapsing-remitting multiplex sclerosis (RRMS) patients. Pegylation of IFN b-1a improved pharmacodynamic and pharmacokinetic properties, resulting in, increased biologic activity and a longer half-life. The efficacy of peginterferon-b-1a was proved by the ADVANCE study - a 2-year Phase 3, multicenter, randomized, double-blind study with a 1-year placebocontrolled period evaluating the efficacy and safety of subcutaneous peginterferon-b-1a administered every 2 or 4 weeks in patients with RRMS. Peginterferon-b-1a efficacy was maintained during the two years, with greater effects observed with every 2 week versus every 4 week dosing. Annualized relapse rate and confirmed disability progression was reduced comparing with patients on delayed treatment. Patients treated with continuous peginterferon-b-1a had fewer new or newly enlarging T2 lesions over 2 years than patients in the delayed treatment group. Adverse events were consistent with the known profiles of IFN b therapies in MS. The most commonly reported adverse events were injection site erythema, influenza-like illness. The less frequent administration is associated with fewer flu-like adverse events, which may improve patients’ compliance and adherence. Peginter-feron-b-1a could be an effective and safe treatment option for RRMS patients.]