Abstract
A case of idiopathic hypereosinophilic syndrome (HES) is reported. The disease started at the age of 31, with polyneuropathic-like symptoms and disorders of the gastrointestinal tract. Hypereosinophilia and leukocytosis were observed. Instrumental investigation of the digestive tract showed esophagitis, a peptic ulceration, signs of chronic rectocolitis. Charcot-Leyden crystals were present in feces. Peripheral nervous system changes were seen with EMG and nerve conduction studies. Muscle biopsy findings were in agreement with the electrophysiological data and showed inflammatory abnormalities. Qualitative histology and teased nerve fiber studies of sural nerve indicated axonal degeneration. Protein substances derived from eosinophils degranulation may account for the disturbances observed in various systems and organs.
Sommario
Viene descritto un caso di sindrome ipereosinofila idiopatica (HES). La malattia esordiva all'età di 31 anni con una sintomatologia simil-polineuropatica e disturbi dell'apparato gastrointestinale. Erano presenti ipereosinofilia e leucocitosi. Le indagini strumentali sull'apparato digestivo mostravano esofagite, ulcera peptica, segni di rettocolite cronica. Erano presenti cristalli di Charcot-Leyden nelle feci. All'EMG e agli studi di conduzione nervosa erano presenti alterazioni del sistema nervoso periferico. La biopsia muscolare era in accordo con i dati elettrofisiologici e mostrava anomalie di natura infiammatoria. L'istologia qualitativa ed il teasing delle fibre del nervo surale erano indicative di degenerazione assonale.
Alcune sostanze proteiche derivate dalla degranulazione degli eosinofili possono essere responsabili dei disturbi osservati in vari sistemi ed organi.
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References
Chusid M.J., Dale D.C., West B.C., Wolf S.M.:The hypereosinophilic syndrome: analysis of fourteen cases with review of the literature. Medicine, 54, 1–28, 1975.
Mercadanti M., Cavalieri F., Ferraccioli G.F., Ambanelli U.:Sindrome ipereosinofila idiopatica: attualità clinico-terapeutiche. Minerva medica, 76/39, 1781–1790, 1985.
Behse F., Buchtal F., Carlsen F., Kuappeis G.C.:Hereditary neuropathy with liability pressure palsies. Brain, 95, 777–794, 1972.
Behse F., Buchtal F., Carlsen F., Kuappeis G.C.:Endoneural space and its consituents in the sural nerve of patients with polyneuropathy. Brain, 97, 773–784, 1974.
Wichman A., Buchtal F., Pezeshkpour G.H., Fauci A.S.:Peripheral neuropathy in hypereosinophilic syndrome. Neurology, 35, 1140–1145, 1985.
Moore P.M., Harley J.B., Fauci A.S.:Neurologic dysfunction in the idiophatic hypereosinophilic syndrome. Ann. Int. Med. 102/1, 109–114, 1985.
Monaco S., Baggio F., Tezzon F., Huber R. et al:Peripheral neuropathy associated with hypereosinophilic syndrome. Acta Neurol. 8 (XLI), 2, 117–118, 1986.
Serratrice G., Pellissier J.F., Lachard A. et al.:Localized myositis with eosinophilia (Fren)-Myosite localisee avec eosinophilie. Presse Med., 1985, 14/9, 533–535.
Gleich G.J., Friges E., Loegering D.A., Wasson D.L., Steinmuller L.:Cytotoxic properties of the eosinophil major basic protein. J. Immunol., 123, 2925–2927, 1979.
Ackerman S.J., Loegering D.A., Venge P. et al:Distinctive cationic proteins of the human eosinophil granule: major basic protein, eosinophil cationic protein and eosinophil-derived neurotoxin. J. Immunol. 131, 2977–2982, 1983.
Gordon M.H.:Studies of the aetiology of lymphadenoma. Bristol, UK: John Wright and Sons Ltd, 7–76, 1932.
Durak D.T., Dale D.C., West B.C., Wolf S.M.:Purification of human eosinophil-derived neurotoxin. Proc. Nat. Acad. Sci. USA, 78, 5165–5169, 1981.
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Marolda, M., Orefice, G., Barbieri, F. et al. The idiopathic hypereosinophilic syndrome. Clinical, electrophysiological and histological study of a case. Ital J Neuro Sci 10, 79–84 (1989). https://doi.org/10.1007/BF02333876
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DOI: https://doi.org/10.1007/BF02333876