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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Clinical Neuroscience

[Special concerns in medical care of severely demented patients suffering from Alzheimer disease]

RAJNA Péter

[While the expected life-time increases dementias will show a pandemic nature. Author analyses the special medical and social concerns of severe states of Alzheimer disease. Having introduced the epidemiologic, diagnostic data and forms of palliatíve therapies he concentrates on the hitherto unsolved problems of patients and caregivers. He belongs to the experts identifying the Alzheimer diseases as a forced aging process. Accepted the theory of L. Hayflick he evaluates the aging process in frame of an evolution model. He states that for aged human beings having already performed the task of reproduction further conditions of living circumstances and even that of death are no more programmed. This hypothesis may have a very negative content at the first sense. But the author rather considers the uncertainty of aging a positive message. He establishes that the occurrence of degenerative processes connecting the aging must be limited by preventive interventions far more efficient than the present possibilities. Concerning these steps we are still on the level of recommendations for preserving our general health and data of relevant science are quite contradictory. Although criteria of “healthy", “qualified” or “assumable” aging are getting defined, we still do not know exactly how we should live optimally and what should we do for this in different epochs of our life. Somatic and psychological parameters are lacking which would be able to signalize the actual level of the aging process in a given person. We, physicians, also do not have exact opinion on the level of severity of dementias at which the patient already lost his her features of personality and individuality on an irreversible way. We have no idea on the phase when the ratio of joy and suffer falls below the value of 1.0 because we still have no tool for its measure. The author pointed the necessity of acceptance of a new preventive attitude and application of new methods in the medical care of the forced or pathological aging, instead of the present approaches based on the “wait and treat” attitude. Because of the intensive increase of the patient population the identification and care of patients can be efficient with a much more involvement of the basic medical network. Financial background can be improved by new theoretical bases of criteria of the palliative treatments. But the direct professional programs can be planned after solution of the existing moral and social dilemmas.]

Clinical Neuroscience

[EDITORIAL COMMENT]

SZOK Délia

Lege Artis Medicinae

[MAGNETIC RESONANCE IMAGING IN MUSCULOSKELETAL DIAGNOSTICS]

MESTER Ádám

[The aim of the author is present a practical MRI guideline for partners clinicians working in general or specialist patient care related to musculoskeletal diseases. The evidence based diagnostic decision-making process requires specific and realistic expectations related to MRI in daily practice. The focus message of the article is that the more exact and specific clinical question arrives, the more accurate and precise answer of the radiologist is achieved. The importance of detailed clinical information based optimal planning is emphasized versus “general” studies based on poor clinical information. It is not less important for the referring physician to prepare and inform the patient prior to the MRI study, with the exclusion criteria and contrast agent application all explained. Very few technical details are presented, only as little as possible to understand the clinically relevant properties of the imaging process.]

Clinical Neuroscience

[Editorial comment]

KLIVÉNYI Péter

Lege Artis Medicinae

[Members of the editorial and advisory board and the redaction of the LAM, 1990-2010]