Clinical Neuroscience

[A case history and diagnostical rewiev of primary cerebral angiitis]

CSÉPÁNY Tünde, KOLLÁR József, SIKULA Judit, MOLNÁR Mária, CSIBA László

MAY 10, 2005

Clinical Neuroscience - 2005;58(05-06)

[The authors present a case history of primary cerebral angiitis with four years of follow-up. The early diagnosis was based on typical clinical symptoms, brain MRI, intracerebral MRA and histology of sural nerve biopsy. Electroneurography suggested peripheral involvement, although the patient did not have clinical signs of peripheral neuropathy. Glucocorticoid and immunosuppressive treatment resulted in remission. The diagnostic difficulties of primary cerebral vasculitis are also summarized in the discussion.]

COMMENTS

0 comments

Further articles in this publication

Clinical Neuroscience

[Testing the administration of cannabinoids in neurological diseases]

Clinical Neuroscience

[Ferenc Obál Jr.]

BENEDEK György

Clinical Neuroscience

[CONGRESS CALENDAR]

Clinical Neuroscience

[Frontotemporal dementia - Part I History, prevalence, clinical forms]

GALARIOTIS Vasilis, BÓDI Nikoletta, JANKA Zoltán, KÁLMÁN János

[The authors report a comprehensive publication consisting of three parts going into the details of history, prevalence, clinical forms, differential diagnosis, genetics, molecular pathomechanism, pathology, clinical diagnosis and treatment of frontotemporal dementia (FTD). The first part of the present review deals with history, prevalence and clinical forms of FTD. The prototypical FTD with circumscribed atrophy was first described by Arnold Pick; Alois Alzheimer found the intraneural inclusions in the patients’ brain. Later it was recognised that many patients had neither the atrophy nor the cellular changes, but genetic mutations have been identified. Frontotemporal dementia is a degenerative condition with unknown etiology in the frontal and anterior temporal lobes of the brain. It is a progressive neurobehavioral syndrome characterized by early decline in social interpersonal conduct, early impairment in the regulation of personal conduct, early emotional blunting, and early loss of insight. There are no reliable epidemiological studies on the prevalence of FTD, but it is well-accepted that FTD is a common cause for dementia before the age of 65 (it constitues approximately five percent of all irreversible dementias). The nomenclature of the FTD has been confusing and continues to be. Three major clinical syndromes can be identified: 1 frontal variant FTD (dementia of frontal type) in which changes in social behavior and personality predominate, 2. in semantic dementia (progressive fluent aphasia) there is a breakdown in the conceptual database which underlies language production and comprehension, 3. in progressive nonfluent aphasia the phonologic and syntactic components of language are affected. The authors report two cases, which can point to clinical symptoms and forms, and mention the problems of the differential diagnosis and therapy.]

Clinical Neuroscience

[Primary prevention program of the Hungarian Spine Society - Part II Controlled prospective study of short term efficacy]

SOMHEGYI Annamária, TÓTH János, MAKSZIN Imre, GARDI Zsuzsa, FESZTHAMMER Artúrné, DARABOSNÉ Tim Irma, TÓTHNÉ Steinhausz Viktória, TÓTHNÉ Szabó Klára, VARGA Péter Pál

[Introduction - The primary prevention program of the Hungarian Spine Society is set around 12 test exercises that assess performance of postural muscles and then develops their strength and flexibility. By this the scheme aims to make the biomechanically correct use of the spinal column spontaneous. Subjects and methos - The application of the scheme in the frame of physical education in a primary school was investigated in a prospective controlled study in the school-year of 2001/2002. Participating children were aged 6 to 14. The intervention group (n=200) regularly did the posture correction exercises with their teacher's direction in physical education classes, while the control group (n=213) did not. The 12 test exercises of the posture correction scheme of the Hungarian Spine Society were tested by an independent physiotherapist at the beginning and at the end of the school-year in both groups. Results were analyzed by paired and unpaired t-tests and by a chi-square test. Results - At the end of the school-year the strength and flexibility of the postural muscles of the intervention group improved significantly compared both to their own performance (p<0.01) at the beginning of the school-year and to the control group. Test results of the control group were significantly (p<0.05) worse at the end of the school-year than their own results at beginning of the school-year, and were more significantly (p<0.01) worse than the test results of intervention group. Conclusion - The results of the controlled study confirm that regular use of the preventive exercises in physical education improves the strength and flexibility of postural muscles. In order to analyze the results concerning specific muscle groups or age groups a detailed study with age- and gender-matched controls will be necessary.]

All articles in the issue

Related contents

Hypertension and nephrology

[Functional and morphologic changes in patients with new-onset dyslipidemia after transplantation]

BORDA Bernadett, LENGYEL Csaba, VÁRKONYI Tamás, SZABÓ Viktor, SZEDERKÉNYI Edit, LÁZÁR György

[The principal risk factors for cardiovascular mortality after transplantation are hyperglycemia, hypertriglyceridemia, immunosuppressive therapy, obesity, and smoking. Among 115 patients, we assessed the risk factors for new-onset dyslipidemia, and their effects on the function and histopathology changes in the allografts one year after transplantation. Evaluating the risk factors and the initial recipient data, we observed a significant difference in age when comparing normal versus new-onset dyslipidemia patients (p=0.002). The difference in body mass index was significant one year after kidney transplantation when comparing normal with new-onset dyslipidemia patients (p=0.02). The trigliceride levels were significantly higher among those on cyclosporine- A than those on tacrolimus (3.02±1.51 mmol/l vs 2.15±1.57 mmol/l, p=0.004). The difference also proved to be significant for the total cholesterol level: 5.43±1.23 mmol/l versus 4.42±1.31 mmol/l respectively (p=0.001). In regard to allograft function there was no significant difference one year after transplantation between the normal and new-onset dyslipidemia patients. When assessing morphologic changes in the kidney, we observed significantly more frequent interstitial fibrosis/tubular atrophy among new-onset dyslipidemia than normal function patients. Disruption of lipid homeostasis is known to severely damage the allograft. Without timely recognition and treatment, these conditions may not only lead to irreversible damage in the allograft, but also increase cardiovascular risk.]

Lege Artis Medicinae

[Up-to-date management of systemic sclerosis]

SZŰCS Gabriella

[Systemic sclerosis is a chronic autoimmune disease characterized by three major features: widespread fibrosis in the skin and internal organs, a non-inflammatory small vessel obliterative vasculopathy and immunological activation with disease-specific autoantibodies. It is necessary to take a systematic approach to the diagnosis and evaluation of each case in order to provide appropriate treatment. Disease-modifying approaches can be classified according to the underlying pathogenic process. Thus vascular therapies include agents used for Raynaud’s phenomenon, critical digital ischaemia and organ-based vascular complications such as scleroderma renal crisis and pulmonary hypertension. Immunosuppressive drugs are used in lung involvement or rapid skin progression. The results of different anti-fibrotic therapies are controversial. Finally in managing organ-based manifestations and complications a multidisciplinary approach to the therapy is useful with patient education as an integral component of successful management.]

Lege Artis Medicinae

[Autoimmune pancreatitis in Hungary: a national multicenter study]

CZAKÓ László, GYÖKERES Tibor, TAKÁCS Tamás, TOPA Lajos, SAHIN Péter, DUBRAVCSIK Zsolt, SZEPES Attila, PAP Ákos, FÖLDESI Imre, TISZLAVICZ László, WITTMANN Tibor

[BACKGROUND - Autoimmune pancreatitis (AIP) is an increasingly recognised, special form of chronic pancreatitis, which greatly differs from other forms of chronic pancreatitis. Most papers on this condition have been published in Japan. METHODS - In our multicenter study, we aim to present the characteristics (demographics, clinical symptoms, laboratory and morphological findings, extrapancreatic symptoms, response to therapy, remission) of the first 13 Hungarian cases of AIP. RESULTS - The mean age at presentation was 44.2 years (range: 19-74); 54% of patients were women. New-onset mild abdominal pain (77%), weight loss (38%) and jaundice (31%) were the most common symptoms, with ulcerative colitis as the most frequent (38%) extrapancreatic manifestation. Diffuse pancreatic swelling was seen in 7 patients (54%), and a focal mass in 5 (38%). Pancreatic duct strictures were present in all patients. Serum immunoglobulin-G4 level was elevated in 71% of the patients in whom it was measured. All percutaneous core biopsies (4 patients) and surgical specimens (2 patients), and 2 of the 4 biopsies of the papilla of Vater revealed hystological findings typical for AIP: periductal, diffuse lymphoplasmacytic infiltration, marked interstitial fibrosis, and obliterative phlebitis. Immunostaining revealed IgG4-positive plasma cells in 57% of the patients in whom it was examined. Granulocytic epithelial lesions (GEL) were detected in 3 patients. These patients were younger (mean age 34 years), 66% were women and 6% had ulcerative colitis, whereas the mean age of patients without GEL was 65 years, and the majority of them were men. Steroid treatment resulted in remission of the symptoms in all patients. Because of suspicion of pancreatic tumour, 2 patients with focal AIP underwent partial pancreatectomy. Symptoms relapsed in one patient, but an increased dose of steroid resulted in a remission, which was maintained by azathioprine therapy. CONCLUSIONS - In our first Hungarian cases, we have confirmed previously reported characteristics of AIP. AIP with GEL is frequent among our patients; this condition is more frequent among women and younger patients, and is often associated with ulcerative colitis. If AIP is suspected, the performance of percutaneous biopsy is highly recommended. The therapeutic response to steroid therapy was excellent.]

Lege Artis Medicinae

[THE ROLE OF REGISTRY ANALYSIS IN THE FORMING OF THERAPEUTIC GUIDELINES IN RENAL TRANSPLANTATION]

PERNER Ferenc, HERCZEG Balázs, SZENOHRADSZKY Pál, ASZTALOS László, KALMÁR-NAGY Károly, JÁRAY Jenő

[The authors assess the two main outcomes of the immunosuppressive therapy after renal transplantation: graft and patient survival. According to their view, evidence from randomised clinical trials results can be well complemented by the several unique transplant registries and outcome research based upon these databases. The comparison of evidence from these two sources addresses the question of achievable outcome under ideal (controlled) versus real life conditions. Based on a systematic review of the relevant clinical trials and registries it can be stated, that in the case of some immunosuppressants (mycophenolate mofetil vs azathioprine, microemulsified cyclosporin vs cyclosporin and tacrolimus vs cyclosporin) the improvement in the intermediate outcome can lead to improved graft and patient survival, while in the case of other drugs no significant difference in hard endpoints were detected (tacrolimus vs microemulsified cyclosporin). Evidence on graft and patient survival differences could not be derived from traditional randomised clinical trials, only from transplant registries. For the sake of improved evidence based therapeutic guidelines in renal transplantation, authors call for further development of the Hungarian transplant registry.]