Clinical Neuroscience

[Tolosa-Hunt syndrome]

PÉTERFI Anna, ZÁDORI Péter, SÜTŐ Gábor, HORVÁTH Gyula, KOPA János

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.]

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[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.]

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[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.]

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[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.]

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