Clinical Neuroscience

[Tolosa-Hunt syndrome]


JANUARY 30, 2011

Clinical Neuroscience - 2011;64(01-02)

[Both men and women are affected by the rare disease called Tolosa-Hunt syndrome. We don’t know exactly what causes it to evolve. It is usually put into the categories of either idiopathic inflammation or pseudotumor. Its patological feature is a non-specific inflammatory process with fibroblastic, lymphocytic, plasmocytic infiltration, which can be found, for the most part, in the wall of the sinus cavernosus. Granulocytic and giant-cell infiltrations have been described too. The possibility of autoimmune disease has also come up. In our current study we describe the case of a female patient who recovered with the help of a steroid therapy. Through examining her, we also found immunological alterations, which should urge us to thoroughly examine the further observations of this kind.]



Further articles in this publication

Clinical Neuroscience

[World of agression]

RAJNA Péter, HÁRDI István

Clinical Neuroscience

[Neurological complications of Fabry-disease]


[Background - Fabry-disease (FD) is a rare X-linked lysosomal storage disease. Deficiency of alpha-galactosidase A activity leads to the accumulation of neutral glycosphingolipids, primarily globotriaosylceramide (GL-3) in various tissues, particularly blood vessels, kidneys, myocardium and in ganglions of the peripheral and autonomic nervous system and causes diverse symptoms. The classical phenotype is seen in most males and rarely in females. In women, symptomes start later and the severity is milder. Both peripheral and central nervous system can be both affected. Objectives - Fabry-patiens and gene-carriers in the central region of Hungary are treated in the 2nd Pediatric Department, Semmelweis University. These patients are consulted by an interdisciplinary team. At present, four hemizygous male, four heterozygous female Fabry-patients and three asymptomatic heterozygous gene carriers are followed. Results - After reviewing the neurological complications of FD, we present clinical and neuroimaging data of our patients. Conclusion - We emphasize that neurologists should suspect the rare monogenic FD in the case of acroparaesthesia and heat-cold intolerance in childhood or adolescence; clinical signs of TIA/stroke or unexplained MRI alterations suggesting small vessel disease in young adults. Early diagnosis and introduction of enzyme replacement therapy (ERT) can halt or reverse progression.]

Clinical Neuroscience

[Paroxysmal kinesigenic dyskinesia]


[Paroxysmal kinesigenic dyskinesia (PKD) is a rare neurological disease and the diagnosis is based on case history and clinical features. Despite of simply diagnostic criteria, the recognition of the disease is sometimes delayed. The involuntary movements in PKD lead to anxiety, social isolation, trauma and worsens the quality of life. To establish the diagnosis many other paroxysmal syndromes have to be excluded. The disease responds to antiepileptic therapy well. The genetic background of the familiar cases is not known. Here we present a 19 year-old patient with PKD and review the current literature. Our patient’s events were triggered by sudden movements and last several seconds. His physical and neurological examinations were normal and responded well to carbamazepine therapy.]

Clinical Neuroscience

[Ageing and arithmetic performance - electrophysiological complexity-, and graph theoretical characteristics]

MOLNÁR Márk, BOHA Roland, BENYOVSZKY Máté, GAÁL Zsófia Anna, TÓTH Brigitta

[During the course of ageing the decline of cognitive performance, including attention and working memory processes - essential for arithmetic procedures - is well known. For the investigation of the neuronal mechanisms of these processes the application of methods capable of taking into account the high complexity of the nervous system, the role of nonlinear processes and network-properties of its constituents are necessary. As for the latter the recently realized small world network characteristics representing optimal conditions for information processing may be of particular importance. In the present study the spectral, complexity-, and network characteristics of the EEG recorded during performing an arithmetic task in a group of young (n=32, mean age 22.0 yrs) and elderly (n=19, mean age: 66.7 yrs) was analyzed. Heart rate and behavioral measures (number of mistakes, reaction time) were also investigated. The alpha2 band decreased in the young, while the delta band increased in the elderly in the task condition. The increased Omega-complexity observed in the elderly is probably caused by reduced interneuronal connectivity. “Small-world” network characteristics were found in the beta and delta bands although in the elderly the topology was closer to a random pattern. In the task condition the network features of the elderly subjects shifted more towards the small world pattern than those seen in the young indicating that for the elderly the mobilized effort for task completion was higher. In spite of this, the level of performance and the heart rate change observed in the elderly was lower than that seen in the young. The application of complexity-, and graph theoretical analysis appears to be a promising tool for the investigation of diseases of the nervous system characterized by diffuse pathology as in the case of as various types of dementias.]

Clinical Neuroscience

[Organization of rehabilitation of people with disabilities as a consequence of neurological conditions in Hungary]

FAZEKAS Gábor, DÉNES Zoltán, FÁY Veronika, URBÁN Edina, SZÉL István

[The history of rehabilitation of people with disabilities as a consequence of neurological conditions goes back to more than four decades in Hungary. The authors describe its history, how this service is organized nowadays, questions of specialization, scientific activity and quality improvement. They emphasise: any form of neurorehabilitation service (special neurorehabilitation department or programme of a multiprofile rehabilitation unit) must meet the same criteria. Quality assurance will be provided by a new accreditation system.]

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Clinical Neuroscience

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Delirium is a syndrome frequently encountered in intensive care and associated with a poor prognosis. Intensive care delirium is mostly based on general and palliative intensive care data in the literature. In this study, we aimed to investigate the incidence of delirium in coronary intensive care unit (CICU), related factors, its relationship with inhospital and follow up prognosis, incidence of age-related delirium and its effect on outcomes. This study was conducted with patients hospitalized in CICU of a tertiary university hospital between 01 August 2017 and 01 August 2018. Files of all patients were examined in details, and demographic, clinic and laboratory parameters were recorded. Patients confirmed with psychiatry consultation were included in the groups of patients who developed delirium. Patients were divided into groups with and without delirium developed, and baseline features, inhospital and follow up prognoses were investigated. In addition, patients were divided into four groups as <65 years old, 65-75 yo, 75-84 yo and> 85 yo, and the incidence of delirium, related factors and prognoses were compared among these groups. A total of 1108 patients (mean age: 64.4 ± 13.9 years; 66% men) who were followed in the intensive care unit with variable indications were included in the study. Of all patients 11.1% developed delirium in the CICU. Patients who developed delirium were older, comorbidities were more frequent, and these patients showed increased inflammation findings, and significant increase in inhospital mortality compared to those who did not develop delirium (p<0.05). At median 9-month follow up period, rehospitalization, reinfarction, cognitive dysfunction, initiation of psychiatric therapy and mortality were significantly higher in the delirium group (p<0.05). When patients who developed delirium were divided into four groups by age and analyzed, incidence of delirium and mortality rate in delirium group were significantly increased by age (p<0.05). Development of delirium in coronary intensive care unit is associated with increased inhospital and follow up morbidity and mortality. Delirium is more commonly seen in geriatric patients and those with comorbidity, and is associated with a poorer prognosis. High-risk patients should be more carefully monitored for the risk of delirium.

Clinical Neuroscience

Autonomic nervous system may be affected after carpal tunnel syndrome surgery: A possible mechanism for persistence of symptoms after surgery

ONDER Burcu, KELES Yavuz Betul

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Clinical Neuroscience

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Clinical Neuroscience

[Interdisciplinary approach of vestibular system impairment]


[In the first part of this review the definition of vertigo/dizziness was discussed. The major difference between the two signs is the exsistence of the direction, which is specific for vertigo. Dizziness is a frequent complaint in the clinical practice. Its frequency is increasing with advance of age, to intimate the play of declining cognitive process in the pathogenesis of its. The popular health significance of vertigo is in the rowing number of the patients. The onset of the most cases with acute vertigo appears between secundums and minutes so the patients will be provided in circumstances of emergency department. First of all three form schould be take into account: neuronitis vestibularis, benign paroxysmal positional vertigo and Meniere syndrome. Without tipical periferal signs of vertigo, central cause should be searched, principally stroke (lysis possibility). The differential diagnose of the different dizzeness/vertigo forms according to the elapsed time of the onset or congenital and acquired nystagmus was created in tables. The recommendations of the therapy of acute and chronic dizziness/ vertigo syndroms are, lack of results of evidence based trials doubtful. The more often used drugs based on clinical trials are discussed as vinpocetine, betahistine and piracetam. The in vitro and in vivo data suggest that the last molecule is eligible to use both in periferal and central type of vertigo syndroms.]