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  • 1990-1994  (2)
  • 1990  (2)
  • 1
    ISSN: 1432-1459
    Keywords: Myasthenia gravis ; Multicentre follow-up study
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A multicentre retrospective study was carried out on the characteristics and course of myasthenia gravis (MG) in Italy. Data from 1152 patients, fairly representative of the myasthenic population seeking medical advice, were analysed for diagnostic criteria, clinical aspects and therapeutic approaches. Mean follow-up was 4.9 years. The disease was correctly diagnosed within 2 years of the onset in 80% of cases. Onset of symptoms peaked in the second and third decade in females and fell between 20 and 59 years in males. At first observation 87% of the patients had generalized MG. Maximal worsening was observed within 3 years in 77% of patients. At the last follow-up, 35% of cases were symptom-free (pharmacological remission 24%, remission without treatment 11%). The more severe the disease at the first observation and at the maximal worsening of symptoms, the lower was the proportion of remissions. Steroids were given in 54% and immunosuppressants in 18%. Thymectomy was performed in 72%, mostly in women, younger than age 40, and with generalized MG. Thymectomy seemed to improve the course of the disease, mostly in patients operated on shortly after diagnosis and those with generalized mild-to-moderate disease and with a normally involuted thymus. MG was lethal in 4% of patients, principally men, older than 40, in grade 3 or worse at first observation, with a short history of disease, and with thymona.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Neurological sciences 11 (1990), S. 609-613 
    ISSN: 1590-3478
    Keywords: HIV infection ; muscle biopsy ; rod body myopathy ; light and electron microscopy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Sommario Gli AA riportano il caso di un soggetto di 35 anni omosessuale con una debolezza muscolare progressiva prossimale ai 4 arti ad impronta miopatica. L’EMG era di tipo miogeno; il CPK era lievemente aumentato; l’esame del liquor evidenziava un danno di barriera e positività degli anticorpi HIV. La biopsia muscolare evidenziava un quadro miopatico, con aspetti infiammatori, che comprendeva: marcata variabilità del diametro delle fibre, fibre necrotiche e fagocitosi, nuclei interni ed infiltrati linfo-istiocitari. Lo studio istochimico e istoenzimatico evidenziava compromissione di entrambi i tipi di fibre con modificazione delle attività ossidative nelle fibre in degenerazione. La microscopia elettronica mostrava un quadro di degenerazione miofibrillare con la presenza di caratteristici “rod bodies” nel 30% delle fibre. La stretta bra indicare l’esistenza di una specifica miopatia strutturale associata con l’AIDS.
    Notes: Abstract A 35 year old homosexual man showed clinical features of myopathy, with progressive muscular weakness of proximal muscles. EMG demonstrated a myopathic pattern; serum CPK was mildly elevated and CSF examination revealed antibodies to HIV and a blood-brain barrier damage. An open biopsy of the quadriceps femoris muscle showed myopathic changes with inflammatory features including a marked variation in fiber size, necrotic fibers and phagocytosis, a profusion of internal nuclei. Fiber type analysis with myosin ATPase reaction revealed that myopathic changes involved both fiber types. Changes in the oxidative enzyme activities were also observed in the degenerating muscle fibers. Electron microscopy showed patterns of myofibrillar degeneration and characteristic rod bodies in 30% of fibers. The close resemblance of the present morphological results with those recently observed in some HIV antibody positive men seems to indicate the existence of a specific structural myopathy associated with AIDS.
    Type of Medium: Electronic Resource
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