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  • 1
    ISSN: 1432-1459
    Keywords: Key words Blepharospasm ; Dystonia ; Parkinsonism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract It has been suggested that a lesion in the dopaminergic neurons of the substantia nigra pars compacta combined with eye irritation is involved in the pathophysiology of idiopathic blepharospasm. If so, these patients might be prone to develop Parkinson’s disease or a parkinsonian syndrome. We therefore conducted a validated questionnaire-based follow-up study to estimate (a) the frequency of local eye disorders at onset and (b) frequency of development of parkinsonian symptoms in blepharospasm patients. Ninety-nine patients previously diagnosed with idiopathic blepharospasm were sent a two-part questionnaire to assess parkinsonian and other symptoms associated with their condition. The average period of follow-up was 12.7 years, ranging from 3 to 26 years, with an average age at onset of 53.5 years. Sixty-two patients reported other ocular symptoms prior to or at the onset of blepharospasm, and therefore ocular disease may act as a trigger to produce blepharospasm in those already predisposed. Only two patients had developed a score on the parkinsonism rating scale indicating possible Parkinson’s disease, but clinical examination confirmed this not to be the case. If a lesion in the dopaminergic neurons is involved in blepharospasm, it would appear to be relatively minor (and non-progressive), since patients with idiopathic blepharospasm do not seem prone to develop parkinsonian symptoms.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1569-8041
    Keywords: EBER-1 ; EBNA3C ; Epstein-Barr virus ; Hodgkin's disease ; LMP-1
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Background:Though Epstein–Barr virus (EBV) has been linkedto classical Hodgkin's disease (cHD), only a proportion of cHD cases showEBV-association. While there has been evidence to suggest a crucial role forEBV in the early phase of cHD evolution, we are unclear whether continuedpresence of EBV is essential for the maintenance of the neoplasm. We haveaddressed this issue by investigating the EBV-association in paired samplesof cHD obtained at presentation and relapse. Materials and methods:We have analysed lymph node biopsy samplesfrom 23 cases of cHD at presentation and relapse. In situ hybridization with EBER-1 probe and immunostaining with anti latentmembrane protein-1 (LMP-1) antibody was performed on the paraffin sections.PCR for EBNA 3C gene was performed for EBV subtype analysis on the DNAextracted from paraffin sections. Results:EBV-association was documented in both the presentationand relapse samples of 14 cases. One case showed loss of EBV-association atrelapse. PCR analysable DNA was obtained from both presentation and relapsesamples in four of the EBV-associated cases. The EBNA 3C amplimer correspondedto type A strain of EBV in all the samples. Conclusion:Loss of EBV-association between presentation andrelapse seen in one case implies a hit and run mechanism in EBV-inducedlymphomagenesis. Though EBV may be essential early in the evolution of cHD,it may not be required for maintenance of the neoplastic clone. Our study alsobrings out a speculation that a proportion of EBV-negative cHD could representloss of EBV in the tumour prior to clinical presentation. Alternatively, anunidentified lymphotropic virus other than EBV might play a pathogenetic rolein EBV-negative cHD.
    Type of Medium: Electronic Resource
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