Clinical Neuroscience

[Inclusion body myositis - a rarely recognized disorder]


MARCH 30, 2013

Clinical Neuroscience - 2013;66(03-04)

[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.]



Further articles in this publication

Clinical Neuroscience

[Antiepileptic drugs in treatment of epilepsy and follow up of their efficacy]

GYIMESI Csilla, BÓNÉ Beáta, TÓTH Márton, HORVÁTH Réka, KOMOLY Sámuel, JANSZKY József

[Epilepsy is one of the most common neurological diseases usually demanding long term treatment. The prime goal of therapy is to achieve seizure freedom with avoidance of side effects. Precise diagnosis is fundamental selecting the proper antiepileptic drug(s). In addition of wide-spectrum antiepileptics, selective syndrome-specific antiepileptic drugs are available. Pharmacological features of the new antiepileptics allow more personalized clinical use. Aim of this paper is to provide a comprehensive pragmatic review of therapeutic possibilities and recommendations currently accessible in Hungary.]

Clinical Neuroscience

[Effects of spinal cord stimulation on heart rate variability in patients with chronic pain]

KALMÁR Zsuzsanna, KOVÁCS Norbert, BALÁS István, PERLAKI Gábor, PLÓZER Enikõ, ORSI Gergely, ALTBACKER Anna, SCHWARCZ Attila, HEJJEL László, KOMOLY Sámuel, JANSZKY József

[Background - Spinal cord stimulation has become an established clinical option for treatment of refractory chronic pain and angina pectoris, but its precise mechanism of action is unclear. We investigated the effect of spinal cord stimulation (SCS) on heart rate variability (HRV) and evaluating its influence on the sympathetic/parasympathetic balance in chronic pain. Materials and purpose - Seven patients (three men, four women) with SCS due to chronic pain were included. The SCS was programmed in three different ways: (i) to stimulate at an amplitude known to generate paresthesias (ON-state), (ii) at a subliminal level (SUB state), or (iii) switched off (OFFstate). HRV analysis was based on 5-min segments of the consecutive normal RR intervals and was performed with custom software (Kubios HRV Analysis). Results - The mean heart rate was higher in ON state compared to SUB state (p=0.018) and the high-frequency component of the HRV was lower in ON compared to OFF period (p=0.043). Other HRV parameters values did not significantly differ during the three tested periods. Conclusion - Spinal cord stimulation in chronic pain seems to be accompanied by reduced parasympathetic tone, unlike SCS in angina pectoris where previous studies found a reduced cardiac sympathetic tone. Our study might lead to understand the mechanism of action of SCS We investigated a relatively small number of patients, which is the main limitation of our study. Thus, further studies with larger number of patients are required for validation of our results.]

Clinical Neuroscience

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


[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

[Efficacy of deep brain stimulation in our patients with Parkinson’s disease]

GERTRÚD Tamás, TAKÁTS Annamária, RADICS Péter, RÓZSA Ildikó, CSIBRI Éva, RUDAS Gábor, GOLOPENCZA Péter, ENTZ László, FABÓ Dániel, ERÕSS Loránd

[Background and purposes - In advanced Parkinson’s disease, medically refractory motor fluctuation or medically resistant tremor considerably affects quality of life. However, these symptoms can be mostly successfully treated by deep brain stimulation. We analyzed the efficacy of bilateral subthalamic stimulation in our patients with Parkinson’s disease. Methods - We assessed the clinical data of ten patients who have been treated in the Department of Neurology, Semmelweis University and have been operated in the National Institute of Neurosciences between 2008 and 2011. The Hoehn-Yahr scale score, the Unified Parkinson’s Disease Rating Scale score and the Parkinson’s Disease Questionnaire 39, as well as the dose of antiparkinson medication were documented prior to and one year after surgery. Results - Patient condition improved according to the Hoehn-Yahr scale, approximately by two stages. The dose of antiparkinson medication could be reduced by 63.4% (p=0.005) post operation. Unified Parkinson’s Disease Rating Scale scores decreased by 70.9% (p=0.005). 12 hours after medication withdrawal, execution of daily activity improved by 57.1% (p<0.01) and motor functions developed by 79.1% (p<0.01). Duration of dyskinesias decreased by 62.5% (p=0.018), duration of akinesia diminished by 87.5% (p=0.005). Quality of life rose by 41.6% (p<0.01). Neuropsychological tests detected improvement in verbal memory. Conclusion - With deep brain stimulation, the dosage of antiparkinson medication could be significantly reduced, with considerable improvements in motor function and quality of life. Although the number of patients is still low, good results have been established by careful patient selection, precise neurosurgical procedure and by appropriate programming and patient care.]

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.]

All articles in the issue

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Clinical Neuroscience

TLR4 (Toll-like receptor-4) expression and frontal-cingulate volumes in schizophrenia

LI Hua, KÉRI Szabolcs

Evidence suggests that pathogen-associated pattern recognition receptors (Toll-like receptors, TLRs) are implicated in the pathophysiology of schizophrenia. TLRs are important in both peripheral immune responses and neuronal plasticity. However, the relationship between peripheral TLR expression and regional brain volumes is unknown in schizophrenia. We therefore assessed 30 drug-naïve, first-episode patients with schizophrenia. TLR4+/TLR1+ monocytes were measured using flow-cytometry. High resolution magnetic resonance images (T1 MRI) were obtained and analyzed with FreeSurfer. Results revealed significant negative correlations between the percentage of TLR4+ monocytes, mean fluorescent intensities, and brain volumes in frontal and anterior cingulate regions. The measures of TLR1+ monocytes did not show significant relationships with regional brain volumes. These results raise the possibility that abnormal TLR-activation is associated with decreased brain volumes in schizophrenia.

Clinical Neuroscience

Could red cell distribution width and mean platelet volume be a predictor for lumbar disc hernias?


Background - Lumbar disc herniation (LDH) causes major disabilities worldwide. Several studies in the literature had reported the correlation between radiculopathy and inflammatory markers. Mean platelet volume (MPV), red cell distribution width (RDW) and neutrophil to lymphocyte (N/L) ratio are parameters of hemogram which have been found to be associated with inflammatory conditions. Purpose - Since inflammation has an important role in lumbar disc hernias, and RDW, MPV and N/L ratio are also known to be in correlation with inflammation, we have investigated these parameters of the patients with lumbar disc hernias and compared them with the results of the healthy subjects. Methods - Our study group was composed of patients with lumbar disc hernia, whereas the control group was consisted of healthy volunteers whom visited our outpatient clinics for a routine check-up. Patient characteristics and hemogram parameters of the study cohort were obtained from computerized database system of our institution. SPSS software (SPSS 15.0 for Windows, Chicago, IL, USA) was used for the analysis. Results - There was no significant difference between study and control groups in terms of WBC, neutrophil count, lymphocyte count, neu\lym ratio, Hb, Htc, MCV, and PLT levels (all p>0.05). RDW was significantly increased in study group [15.6 (12.3-22.5)] when compared to control group [14.5(11.9-16.3)] (p=0.004). And MPV in the study group [9.25 (6.38-14.5)] was also significantly increased in comparison to the control subjects [8.8 (6-10.1)] (p=0.013). Discussion - In this retrospective study, we found that, RDW and MPV values in hemograms were increased in patients with lumbar disc herniation when compared to the control group. Conclusions - We suggest that, elevated RDW and MPV may help physicians in decision taking to order radiological imagings in patients with symptoms which can be associated with possible LDH diagnosis. However, for the sake of precision, prospective studies with larger populations are needed.

Lege Artis Medicinae


KISS Róbert Gábor, BÉRES Bernát János

[Nitric oxide is a key molecule of the human body. Since its discovery, a library of books and papers have been published on its physiological and pathophysiological role. It is involved in almost all pathological processes. In healthy individuals nitric oxide plays a crucial role in the vascular regulation by protecting against atherosclerosis, myocardial infarction and stroke. In the central nervous system, nitric oxide in its function as a neurotransmitter is responsible for synaptic plasticity, long-term potentiation, memory and a number of neuroendocrine control mechanisms. Furthermore, during inflammation and host defense, macrophages and neutrophils produce nitric oxide that has antibacterial, antiviral, and tumour cell killing activity. In pathologic conditions, however, nitric oxide reacts with superoxide anion to form peroxynitrite that damages the enzymes of the mitochondrial respiratory chain, superoxide-dismutase, reduced glutathion and activates or inactivates signalling molecules. During ischaemia- reperfusion, nitric oxide and peroxynitrite contribute to nitrative/nitrosative stress, DNAfragmentation and consequent polyADP-ribosepolymerase- 1 enzyme activation both in coronary thrombosis and ischaemic stroke.]

Hypertension and nephrology

[Nutritional status of hemodialysis patients, and the role of dietician in the complex care of renal patients]

POLNER Kálmán, KOVÁCS Lívia, HARIS Ágnes

[In chronic renal failure severe cardiovascular complications develop, which are the cause of death in 50% of the patients. According to recent results, behind the accelerated atherosclerosis, malnutrition and inflammation, developing in patients with chronic renal failure, play significant role. Malnutrition and inflammation show close relationship to the serum albumin level, which is an independent predictor of mortality. Authors studied the nutritional parameters of 94 chronically hemodialysis patients. Patients had been dialysed for more than three months, for 3×4-4.5 hours weekly. Among them 36% had diabetes. According to BMI (body mass index) 42.5% of the patients was normally nourished (20-24 kg/m2), 11.7% of them had malnutrition, 28.7% was overweight, and 17.1% was mildly or moderately obese. Subjective Global Assessment (SGA), calculated by dietician, revealed, that 47.9% of the patients has normal nutritional condition, all the others had some degree of malnutrition. Serum albumin level showed close correlation with the nutritional status, also with an inflammatory marker, the CRP. Only 63.8% of the patients had higher than 40g/l serum albumin. Those, who had higher than 10 mg/l CRP value, had significantly lower serum albumin (38.7±3.4 g/l), comparing to the albumin of the patients, whose CRP was below 10 mg/l (40.5±3.9 g/l, p=0.04). Comparing anthropometrical data, there was no significant difference between diabetic and non-diabetic patients. Grouping patients by their ages, the malnutrition, defined by SGA scores and by serum albumin level, was significantly worse in patients older than 80 years, than in the younger than 50 years old subjects, which signals increased risk of mortality of the elderly patients. A case presentation demonstrates, that malnutrition can be diagnosed at early stage by appropriate nutritional assessment, and it can be corrected by timely and satisfactory energy- and nutrient-substitution, in severe cases by specially prepared nutritional supplements, and thereby the patient’s severe cardiovascular risk can be ameliorated. The successful treatment of hemodialysis patients requires change in medical practice, and close cooperation between physicians and dieticians.]

Clinical Neuroscience

Inflammatory biomarkers in benign paroxysmal positional vertigo: A Turkey case-control study


Objective - Benign paroxysmal positional vertigo (BPPV) is the most common cause of recurrent vertigo. Inflammation is a hypothetic etiological factor in BPPV. The aim of this study was to evaluate inflammatory biomarker levels in BPPV patients and compare these with the healthy subjects. Materials and methods - This prospective case-control study was conducted with 114 newly diagnosed BPPV patients and age- and sex- matched 83 healthy subjects. The laboratory investigations included serum hemogram, full biochemistry profiles, vitamin levels, thyroid hormone profiles, high sensitivity C-reactive protein (hsCRP) and erythrocyte sedimentation rate (ESR). Neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and monocyte to HDL-cholesterol ratio (MHR) values were calculated and compared between the patients and healthy subjects. Results - The mean age was 39.1 ± 12.4 years for patients, and 37.0 ± 11.9 for controls. Vitamin B12, hematocrit (Hct), creatinine, urea, and fT4 values, lymphocyte, total bilirubin, direct bilirubin and indirect bilirubin levels were significantly lower in BPPV patients (p ˂ 0.05), while HDL, SGOT, and ESR values were significantly higher. In the BPPV patients the mean NLR, PLR, and mean platelet volume (MPV) values were significantly higher than in the control subjects. Neutrophil, platelet, monocyte, MHR, and CRP values were similar in both groups (p ˃ 0.05). Conclusion - Our result suggests that NLR, PLR, MPV, ESR, and bilirubin levels should be taken into account as potential biomarkers of BPPV. As they are inexpensive parameters and widely available, they can be used in clinical practice for prediction of BPPV. However, further large-scale studies are required to confirm this relationship.