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Clinical Neuroscience

NOVEMBER 30, 2020

Risk factors related to intracranial infections after transsphenoidal pituitary adenomectomy under endoscope

YIFAN Xu, YUXIN He, WU Xu, TIANYU Lu, WEIBANG Liang, WEI Jin

Background – Up to now, the risk factors related to intracranial infections after transsphenoidal pituitary adenomectomy remain controversial. Purpose – To analyze the risk factors related to intracranial infections after transsphenoidal pituitary adenomectomy under an endoscope, and to provide evidence for preventing and controlling the occurrence and development of infections. A total of 370 patients receiving endoscopic transsphenoidal pituitary adenomectomy in our hospital from January 2014 to October 2017 were selected. The risk factors related to postoperative intracranial infections were analyzed. The hospitalization lengths and expenditures of patients with and without intracranial infections were compared. Of the 370 patients, 18 underwent postoperative intracranial infections, with the infection rate of 4.86%. Intraoperative blood loss >120 mL, cerebrospinal leakage, diabetes, preoperative use of hormones, macroadenoma as well as surgical time >4 h all significantly increased the infection rate (P<0.05). Preoperative use of antibacterial agents prevented intracranial infection. Compared with patients without intracranial infections, the infected ones had significantly prolonged hospitalization length and increased expenditure (P<0.05). Discussion – It is of great clinical significance to analyze the risk factors related to intracranial infection after endoscopic transsphenoidal pituitary adenomectomy, aiming to prevent and to control the onset and progression of infection. Intracranial infections after endoscopic transsphenoidal pituitary adenomectomy were affected by many risk factors, also influencing the prognosis of patients and the economic burden.

Clinical Neuroscience

SEPTEMBER 30, 2020

A rare entity of acquired idiopathic generalised anhidrosis which has been successfully treated with pulse steroid therapy: Does the histopathology predict the treatment response?

ÖKTEM Özdemir Ece, ÇANKAYA Şeyda, UYKUR Burak Abdullah, ERDEN Simsek Nazan, YULUG Burak

Acquired idiopathic generalised anhidrosis is an uncommon sweating disorder characterized by loss of sweating in the absence of any neurologic, metabolic or sweat gland abnormalities. Although some possible immunological and structural mechanisms have been proposed for this rare entity, the definitive pathophysiology is still un­clear. Despite some successfully treated cases with systemic corticosteroid application, the dose and route of steroid application are controversial. Here, we present a 41-year-old man with lack of genera­lised sweating who has been successfully treated with high dose pulse intravenous prednisolone. We have discussed his clinical and histopathological findings as well as the treatment options in view of the current literature.

Clinical Neuroscience

MARCH 30, 2016

Cerebral amyloid angiopathy related inflammation: is susceptibility weighted imaging the clue for diagnosis?

CSÉCSEI Péter, KOMOLY Sámuel, SZAPÁRY László, BARSI Péter

Background - Cerebral amyloid angiopathy-related inflammation (CAA-ri) is characterized by various neurological symptoms such as gradually developing confusion, progressive cognitive decline, seizure or headaches; T2 hyperintensities on magnetic resonance imaging (MRI); and neuropathological evidence of cerebral amyloid angiopathy (CAA) and associated vascular or perivascular inflammation. Although histological confirmation is necessary for accurate diagnosis, in case of typical clinical features and neuroimaging, the diagnosis can be established without biopsy. Case summary - We present the case of a 57-year-old man with a history of hypertension who presented to the emer¬gency department 3-week history of progressive headache and a gradually developing altered mental status. On examination, he was found to have left sided weakness and decreased pscyhomotility. Routine clinical work-up (lab investigations, CT, cerebrospinal fluid analysis) did not show obvious diagnosis, so we performed an MRI. It raised the suspicion of CAA-ri which diagnosis was verified by neuroradiological evaluation. High dose steroid treatment was initiated. The patient rapidly responded to treatment, his focal neurological signs resolved. Control MRI after 1.5 months showed multiple haemorrhagic laesions in the field of previous inflammation which posteriorly supported the previous supposed work-diagnosis. Conclusions - Although histopathology is the gold standard for the diagnosis of cerebral amyloid angiopathy, the typical clinical presentation, good response to steroids and accurate neuroradiological criteria make biopsy unnecessary to diagnose CAA-ri.

Lege Artis Medicinae

JULY 20, 2019

[Severe polymyositis associated with multiplex pulmonary abscesses]

SZABÓ Katalin, VINCZE Anett, NAGY-VINCZE Melinda, DANKÓ Katalin, GRIGER Zoltán

[INTRODUCTION – Idiopathic inflammatory myopathies are heterogeneous autoimmune diseases characterized by immune mediated inflammation of the skeletal muscles. CASE REPORT – A case of a 62-year-old male patient with severe proximal muscle weakness, elevated creatine kinase and swallowing difficulity is presented. Electromyography showed myogenic pattern, thus probable polymyositis was diagnosed. Radiological examination has confirmed bilateral multiplex lung lesions, which were caused by the possibility of tumor, tuberculosis, vasculitis and abscess as well. The condition of the patient deteriorated, nasogastric feeding, high dose steroid treatment was initiated, which reduced the patient's creatinine kinase values, but muscle strength was not changed. Based on the results of various investigations, the condition of the patient was finally confirmed by the development of myositis, resulting dysphagia, chronic aspiration, and multiplex lung abscess. Antibiotic therapy, steroid treatment was continued and finally intravenous immunoglobulin treatment was administered. The condition of the patient gradually improved, the swallowing dysfunction disappeared, and the lung abscesses were resolved. As a result of physiotherapy and rehabilitation treatment, the patient could walk again. CONCLUSIONS – Nasogastric feeding is recommended to prevent aspiration in the case of myositis-associated dysphagia. In case of steroid refractory therapy, the use of intravenous immunoglobulin may be effective. ]

Hypertension and nephrology

OCTOBER 20, 2018

[Hungarian Vasculitis Registry – results of the first five years]

HARIS Ágnes, TISLÉR András, ONDRIK Zoltán, FILE Ibolya, MÁTYUS János, ZSARGÓ Eszter, DEÁK György, AMBRUS Csaba

[Launching the Hungarian Vasculitis Registry aimed to collect information about prevalence and outcome of our patients with ANCA-associated vasculitis, and treatment protocols of the disease. The on-line data collection has been developing dynamically since its initiation five years ago, presently 278 patients’ files are available. Patients’ mean age is 58.2±14.5 years, 62% are women; their disease is associated with c-ANCA positivity in 51% and p-ANCA in 49%. At diagnosis GFR was 24.6±21.6 ml/min/1,73 m2, that time 29%, during the total follow up 39% of the registered subjects needed dialysis. Renal replacement therapy could be discontinued in 23% of them. In cases with focal histological changes, also with upper respiratory tract and skin involvement dialysis was significantly less frequently necessary, which underlines the importance of early diagnosis. In induction therapy steroid was administered for 94% of the patients, 85% of them got cyclophosphamide, 59% was treated by plasmapheresis, 11% got rituximab. Maintenance treat ment contained steroid in 80%, per os cyclophosphamide in 23%, parenteral cyclophosphamide in 22%, furthermore 40% of the patients got azathioprin, 8 subjects got mycophenolate and 6 got methotrexate. Median follow up was 30 months (IQR 6-78), during which period 20% of the patients died, 5% got kidney transplantation, and 5% were lost to follow up. Median survival was 14.8 years, five years survival was 85%, and ten years survival was 70%. Long term survival in patients with c-ANCA vasculitis seemed better comparing to p-ANCA vasculitis, but when correcting by age this difference disappeared. Predictors of death were age and dialysis dependent renal failure. Relapses developed in 27% of patients, 28% of them presented in the first year, 21% suffered it after five years of care. Collected data by the Hungarian Vasculitis Registry shows our society’s successful professional activity. Our results are comparable to the published data in the literature, yet there are several areas in our care where further improvements are warranted in order to increase our patient’s survival and quality of life.]

Clinical Neuroscience

NOVEMBER 30, 2017

Nerve conduction study and gastrocnemius H reflex response in rheumatoid arthritis

EMRE Ufuk, ORTANCIL Özgül, UNAL Aysun, KIRAN Sibel, SAPMAZ Perihan, ATASOY Tugrul

Objectives - We aimed to evaluate nerve conduction studies and gastrocnemius H reflex responses in rheumatoid arthritis (RA) patients and compared to the healthy adult subjects. Materials and methods - Twenty-six RA patients and twenty-two healthy adult subjects were included in the study. The nerve conduction study (NCS) findings and bilateral gastrocnemius H reflex responses were evaluated in all the groups. Age, gender, subcutaneous nodules, joint deformities, laboratory parameters, duration of disease, anti-rheumatic drug and steroid usage were recorded. Activity of disease was assessed using a 28-joint disease activity score (DAS28).The functional status was measured using the health assessment questionnaire (HAQ), pain intensity measured using a visual analog scale (VAS). Results - The rate of electroneuromyographic (ENMG) abnormalities was 73% in RA patients. The most common diagnosis was carpal tunnel syndrome (61.4%). There were no significant correlations between ENMG findings and clinical and laboratory features evaluated. Right H reflex latencies were statistically longer in RA patients (p=0.03). According to calculated cut-off levels, there were more subjects with longer H reflex latencies in RA patients. Conclusions - In this study, entrapment neuropathies were found common as independent identity from duration and severity of disease in RA patients. For H reflex latencies, cut-off values were longer in RA patients. It may provide information about the early neuropathic involvement of long peripheral nerves in RA patients. But this findings are needed to be supported by larger population study.

Hypertension and nephrology

DECEMBER 20, 2016

[New development in the pathogenesis, diagnosis and treatment of IgA nephropathy]

NAGY Judit, VAS Tibor, KOVÁCS Tibor

[IgA nephropathy is one of the leading cause of primary glomerulonephritis worldwide. IgA nephropathy is regarded as an immune mediated disease with a multi-hit pathogenesis starting with the production of poorly glycosylated IgA1 and glycan-specific IgG and IgA autoantibodies leading to the formation of IgA1 containing immune complexes. These immune complexes deposit in the glomerular mesangium followed by the onset of mesangioproliferative glomerulonephritis. The disease has variable clinical presentation and outcome. There is a need to identify patients who have the potential to progress to end-stage renal disease with the help of clinical, histological and biological markers. Treatment options for IgA nephropathy are largely based on opinion or weak evidence. It is true for the KDIGO Clinical Practice Guideline for Glomerulonephritis treatment recommendations containing low level of evidence for almost all recommendations related to IgA nephropathy. It is suggested to separate the patients into 3 groups on the basis of risk to progression and to give not-specific supportive treatment (especially angiotensin converting enzyme inhibitors or angiotensin receptor blocking agents) to all of them on the basis of the risk factors. We discuss the recommendations of the KDIGO Guideline about steroid and immunosuppressive treatment for moderate and high risk patients. Lastly, we provide our perspective on the existing other treatment options (tonsillectomy etc.) and on ongoing clinical trials.]

Hypertension and nephrology

APRIL 10, 2016

[Hungarian virus research and NASA – nephrology aspects]

RADÓ János

[After the occurrence of varicella viruses remain in a latent condition in the ganglions, but could be reactivated from here causing the disease of herpes zoster. In the years of 1960, we described a herpes zoster „house epidemic” where only the steroid treated patients were infected. Varicella zoster virus was identified by virological methods. Also in a steroid treated patient fatal meningoencephalitis was caused by the generalized herpes zoster. The VZ infection was obviously potentiated by the steroid. Our publications about the interaction between the VZ virus and steroid treatment was echoed – among others – by an editorial of four leading medical journals. Investigators of a NASA medical group also cited our articles. They found during and after spaceflight that in the astronauts symptomless reactivation of the VZ virus, EBV and CMV occurred which was contributed to the stress induced hypercortisolemia. Today we see more worries in the prognosis and outlook in certain cases of the herpes zoster than before. One reason of that is the high number of newly recognized complications. Recently also several new pathway of pathomechanisms has been explored, which led to serious risks. In addition, it turned out that in certain disorders as the artheritis temporalis, where today antivirus antibiotic is the first choice drug, instead of steroid administered alone in the past, inducing further progression in the basic disease and sometimes fatal complications when given too long. Nephrological patients are at special risk in the presence of chronic renal disease, high age and associated diabetes mellitus. The risk may even increase after an otherwise successful renal transplantation in response to the administration of steroids and other compounds. Fortunately in the meantime a vaccine was developed against the VZ virus, studied in large populations and found to be very effective. It probably will be a benediction to the old people with chronic renal disease, after transplantation as well as in others suffering from high risk diseases.]

Clinical Oncology

FEBRUARY 10, 2015

[Oncological management of gastro-entero-pancreatic neuroendocrine neoplasias]

PETRÁNYI Ágota, UHLYARIK Andrea, RÁCZ Károly, BODOKY György

[Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are unusual and relatively rare neoplasms. They characteristically synthetize, store and secrete a variety of peptides and neuroamines, which can lead to development of disctinct clinical syndromes. Clinical symptoms and presentations vary depending on the location and hormones produced by the tumor. The diagnosis of NETs is established by histological examination and the immunohistochemical detection of general neuroendocrine markers, such as chromogranin A (CgA) and synaptophysin. An update of the WHO classifi cation has resulted in a new classifi cation dividing neuroendocrine neoplasms into neuroendocrine tumors (NETs) including G1 (Ki67 index ≤2%) and G2 (Ki67 index 3-20%) tumors and neuroendocrine carcinomas (NECs) with Ki67 index >20%, G3. The different available therapeutic approaches, including surgery, liver-directed ablative therapies, peptide receptor radionuclide therapy, and systemic hormonal, cytotoxic or targeted therapy, are discussed in this overview.]

Lege Artis Medicinae

NOVEMBER 03, 2015

[Sudden death of a patient with purpura - post mortem recognized eosinophilic granulomatosis with polyangiitis]

DOBREAN Noémi, HAJNAL-PAPP Rozália, TUSA Magdolna, OROJÁN Iván, CSERNI Gábor

[INTRODUCTION - Systemic diseases may sometimes be challenging because physicians do not think about synthesizing the parts to a single entity. CASE REPORT - A 49-year-old asthmatic female was admitted to hospital for the investigation of her cutaneous symptoms suggestive of vasculitis associated with diffuse joint complaints. The chest X-ray raised the possibility of pneumonia or neoplastic disease. Following an episode of chest pain relieved by a non-steroidal anti-inflammatory drug, she suddenly died. Her previous history included restrictive cardiomyopathy, insufficiency of both atrioventricular valves and long dating eosinophilia. Autopsy revealed a partly granulomatous eosinophilic inflammatory process in several organs, including the heart, the lungs, the kidneys, the colon and the pituitary gland. Retrospective collection of unknown anamnestic features and symptoms made possible to unify the pieces of information and symptoms to a single entity, the Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis, EGPA). CONCLUSIONS - Bronchial asthma seldom leads to death. It can rarely be part of the Churg-Strauss syndrome, of which the manifestation may be related to the administration of leukotriene antagonists also used in the presented case. These drugs may allow the withdrawal of systemic steroid therapy which is beneficial not only in the treatment of asthma but also of the syndrome. Lowering the dose of steroids may promote the development of the full blown pattern of the latter.]