Clinical Neuroscience

[In memoriam György Fényes (1924-1998)]

EMED Alexander

JULY 22, 2009

Clinical Neuroscience - 2009;62(07-08)

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Clinical Neuroscience

[Expert opinion on opioid therapy of non tumorous pain]

EMBEY-ISZTIN Dezső

[Expert opinion on opioid therapy of non tumorous pain 2009;62(07-08)]

Clinical Neuroscience

[Dezső Embey-Isztin: Pain relieve]

KOMOLY Sámuel, NAGY Ferenc

[Dezső Embey-Isztin: Pain relieve 2009;62(07-08)]

Clinical Neuroscience

[Current medical aspects of pantethine (in English language)]

HORVÁTH Zoltán, VÉCSEI László

[Pantethine, the stable disulfide form of pantetheine, is the major precursor of coenzyme A, which plays a central role in the metabolism of lipids and carbohydrates. Coenzyme A is a cofactor in over 70 enzymatic pathways, including fatty acid oxidation, carbohydrate metabolism, pyruvate degradation, amino acid catabolism, haem synthesis, acetylcholine synthesis, phase II detoxification, acetylation, etc. Pantethine has beneficial effects in vascular disease, it able to decrease the hyperlipidaemia, moderate the platelet function and prevent the lipid-peroxidation. Moreover its neuro-endocrinological regulating role, its good influence on cataract and cystinosis are also proved. This molecule is a well-tolerated therapeutic agent; the frequency of its side-effect is very low and mild. Based on these preclinical and clinical data, it could be recommended using this compound as adjuvant therapy.]

Clinical Neuroscience

[Pompe’s disease - Part I - Pathogenesis and clinical features]

ILLÉS Zsolt, TRAUNINGER Anita

[Pompe’s disease is an ultra-orphan disease caused by the deficiency of lysosomal alpha-glucosidase. At present, it is the only inherited muscle disorder, which can be treated by replacement of the enzyme. According to the natural course, early infantile and late childhood-juvenile-adult cases are known. Respiratory insufficiency, cardiomyopathy, and muscle hypotonia are cardinal symptoms/signs in infantile Pompe’s disease, while cardiomyopathy is absent in adult-onset cases. CK levels are always elevated in the sera of infantile patients. Hip-girdle dystrophy and orthopnoe should alert suspicion in adult patients. Diagnosis is established by decreased activity of the enzyme or mutational analysis. Muscle biopsy can be misleading in adult cases due to absence of glycogen in the examined specimen. In this review, we also discuss our experiences obtained by the treatment of three patients.]

Clinical Neuroscience

[Acute disseminated encephalomyelitis in childhood]

LIPTAI Zoltán, ÚJHELYI Enikő, MIHÁLY Ilona, RUDAS Gábor, BARSI Péter

[Background and purpose - Acute disseminated encephalomyelitis is a rare inflammatory demyelinating disorder often preceded by infection or vaccination. The purpose of the study was the systematic analysis of clinical, radiological and microbiological profiles of children treated at Szent László Hospital, and the comparison of findings with literature data. Methods - Demographic, infectological, clinical, radiological, laboratory and virological data of patients treated and followed-up between 1-Jan-1998 and 30-June-2008 were reviewed and analysed. Results - 19 children met diagnostic criteria. Their mean age was 6.8 years. A prodromal illness - mostly febrile viral infection, upper respiratory infection or chickenpox - preceded neurological symptoms in 17 patients. All had polysymptomatic encephalopathy, 2 children had spinal symptoms. The cerebrospinal fluid was abnormal in all but one. A viral etiology was definite in 7 and probable in 8 cases. MRI disclosed white matter changes in 18, cortical and deep gray matter in 16, cerebellar in 6, brain stem in 14 and spinal cord changes in 2 cases. Repeat MRI performed mean 4 months later showed complete resolution in 6 and partial resolution in 11 patients. 13 patients received high-dose methylprednisolone, 2 of whom were also treated with plasma exchange and 1 with immunoglobulin. 9 children required mechanical ventilation. 2 patients died, 10 recovered without and 7 with sequelae. 2 patients developed further demyelinating events: multiple sclerosis and multiphasic disseminated encephalomyelitis, respectively. Conclusion - Clinical, radiological and follow-up results were similar to those published in literature however, triggering viruses were identified in a larger proportion of cases.]

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