Clinical Neuroscience

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BANCZEROWSKI Péter

MARCH 30, 2013

Clinical Neuroscience - 2013;66(03-04)

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Clinical Neuroscience

[Ferenc Garzuly: In labyrinth of diagnostics: studies for doctors and medical students]

HORVÁTH Boldizsár

Clinical Neuroscience

[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.]

Clinical Neuroscience

[10 years, 600 monitoring sessions - our experience with the video EEG monitoring of children]

SIEGLER Zsuzsa, HEGYI Márta, JAKUS Rita, NEUWIRTH Magda, PARAICZ Éva, SZABÓ Léna, FOGARASI András

[Introduction- The only Hungarian video EEG laboratorywhere children of ages 0-18 can be continuously monitoredfor several days was opened 1 June 2001 at Department ofNeurology of Bethesda Children’s Hospital.Objectives- Summarizing our 10 years of experience withthe video EEG monitoring (VEM) of children and defining theplace of VEM in the treatment of childhood epilepsy inHungary.Patients and methods- We have processed data from 597monitoring sessions on 541 patients between June 1, 2001and 31 May, 2011 based on our database and the detailedsummaries of the procedures. Results- 509 patients were under the age of 18. The average length of the sessions was 3.1 days. We haveobserved habitual episodes or episodes in question in 477(80%) sessions. 241 (40%) sessions were requested with anepilepsy surgery indication, and 74 patients had 84 opera-tions. 356 (60%) were requested with a differential diagnosisindication, and 191 (53%) cases of epilepsy werediagnosed. We most commonly diagnosed symptomaticgeneralized epilepsy (57 cases). In 165 sessions the episodein question was not diagnosed as epilepsy. Among theparoxysmal episodes we have identified events ofpsychogenic origin, movement disorders, sleep disordersand behavioral disorders. Only 3% of the differential diag-nosis procedures brought no additional clinical information.Discussion- The diagnostic efficiency in our VEM laborato-ry is in accordance with the data found in the literature.Besides epilepsy surgery VEM is recommended if suspectedepileptic episodes occur and interictal epileptiform signs arenot present or are not in accordance with the symptoms, ifthere is no explanation for therapy resistance and if paroxys-mal episodes of non-epileptic origin are suspected but theycannot be identified based on the anamnesis. VEM is also helpful in diagnosing subtle seizures. The procedure hasnumerous additional benefits in patient care and in trainingthe parents and hospital staff. ]

Clinical Neuroscience

[Application of minimally invasive instrumental spine surgery technique in lumbal diseases of degenerative or traumatic origin]

SCHWARCZ Attila, KASÓ Gábor, BÜKI András, DÓCZI Tamás

[Paradigm change has recently taken place in spine surgery with the application of minimally invasive techniques. Minimally invasive techniques have several advantages over the open traditional techniques: less blood loss, preservation of spine muscle integrity, shorter hospitalization, early mobilization, reduced pain levels, lower risk of infection. The presented cases cover following lumbar pathologies: segmental spinal instability, LV-SI grade II. spondylolisthesis, degenerative spondylolisthesis, spine trauma. Unilateral or bilateral mini-open technique was employed in the degenerative cases, depending on symptoms and signes. If unilateral symptoms - pathology was identified, screws and rod were implanted percutaneously on the side contralateral to the pathology. The segmental fusion between vertebral bodies was always assured by a cage and autologous bone. The presented trauma case involved combined AO type A2 and B fractures. The anterior column was strengthened with vertebral body stents filled with bone cement, the posterior column was fixed with a percutaneously implanted screw rod system. Insertion of stents in the collapsed vertebra significantly increased the vertebral body height and also improved the stability of the spine. Minimally invasive spine surgery techniques appear more advantageous over the traditional open spine surgery that necessitates for large midline approaches.]

Clinical Neuroscience

[Péter Halász, Róbert Bódizs: Dynamic structure of NREM sleep]

BORBÉLY Alexander

[We spend four fifths of our sleep time in nonREM (NREM) sleep. The rather strange designation of this sleep state was a consequence of the fascination by rapid-eye-movement sleep (REM sleep) after its discovery in the middle of the last century.]

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[Health behaviour and cancer screening ]

DÖBRŐSSY Lajos, KOVÁCS Attila, CORNIDES Ágnes, BUDAI András

[The purpose of the paper is to find out: what influences the participation in cancer screening. For answer, concepts such as health, health consciousness, health culture, health behaviour have been taken into account, and the factors that have an influence on the health behaviour considered. It concludes that the participation in screening makes a part of the health behaviour which is a mandatory task for the health education to include screening into the health message. ]

Clinical Neuroscience

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SZOK Délia

Clinical Neuroscience

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KLIVÉNYI Péter

Clinical Neuroscience

[Apoptosis in focal brain ischaemia]

ZÁDOR Zsolt, LACZA Zsombor, BENYÓ Zoltán, HARKÁNY Tibor, HORTOBÁGYI Tibor

[Ischaemic stroke is one of the major causes of death and disability in the developed world. It is caused by focal impairment of cerebral blood flow. The subsequent ischaemic cell death is predominantly necrotic in nature. However, a therapeutically important characteristic is the delayed apoptotic cell demise in the border zone of the primary lesion core. Apoptosis is one of the most intensively studied field of current medical and biological research. The better understanding of its mechanism may provide novel and more effective ways of therapy in a wide range of diseases including ischemic stroke. The salient neurological features of focal brain ischaemia and the morphological signs of apoptotic and necrotic cell death are summarized. The mechanism of apoptosis is discussed. It is divided into an early genetic phase of decisionmaking followed by a cellular execution phase. The characteristics of the early shift in the finely tuned balance of proand antiapoptotic genes and their protein products, which is preceded by an inbalance in intracellular ionized calcium homeostasis, energy depletion and mitochondrial dysfunction is discussed. The crucial role of caspases in apoptosis is emphasized. The three possible pathways during the execution phase is described: the intrinsic- and extrinsic caspase activation cascade and the caspase-independent intracellular signal transduction route. The molecular mechanism of neural cell membrane damage in the execution phase is discussed and some examples of altered protein synthesis also known as message-selection are given. The important role of late reperfusion in the execution phase is emphasized. The possible targets of antiapoptotic therapeutic approaches and the results of experimental studies are presented as well as the perspectives of their use in human clinical care.]

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