INTRODUCTIONLes amyloses sont un groupe hétérogène de maladies liées à un dépôt extracellulaire de protéines capables d'adopter une conformation fibrillaire anormale. Elles peuvent être héréditaires ou acquises, localisées ou disséminées, asymptomatiques ou au contraire de pronostic redoutable (1). Cette affection est appelée amylose par Virchow en 1854 en raison de ses propriétés tinctoriales après coloration par l'iode et l'acide sulfurique (2, 3). Les amyloses localisées sont rares et représentent moins de 10 % des amyloses et 0,17 à 1,5% des tumeurs bénignes du larynx (2, 3). RESULTATSMadame H. M âgée de 51 ans, sans antécédents pathologiques particuliers, ni notion de contage tuberculeux, sans notion de consommation alcoolo-tabagique, a consulté en urgence pour une dyspnée inspiratoire avec tirage sus sternal sans stridor ni cornage. A l'interrogatoire, on a retrouvé la notion de dysphonie permanente évoluant depuis une année avec apparition secondairement d'une dyspnée d'effort sans notion de syndrome infectieux ni d'altération de l'état général. La patiente a été mise sous corticoïdes par voie intraveineuse avec des aérosols et une bonne évolution clinique. Une laryngoscopie directe faite sous anesthésie générale a objectivé une infiltration inflammatoire avec un oedème des deux cordes vocales s'étendant vers la sous glotte, la trachée était d'aspect normal. Une biopsie a été réalisée. $La radiographie du thorax était normale. Une tomodensitométrie du larynx a été pratiquée mettant en évidence un aspect infiltrant des cordes vocales, s'étendant sur environ 8mm sous leur niveau et réduisant discrètement la fente glottique ( fig.1). L'examen anatomopathologique des biopsies laryngées a révélé au niveau du chorion muqueux une substance éosinophile anhiste craquelée se colorant en rouge par le rouge congo avec biréfringence dichroïque jaune vert en lumière polarisée évoquant l'aspect amyloïde. La patiente a été adressée au service de médecine interne où un complément d'exploration a été réalisé (bilan rénal, échographie rénale, ECG, radiographie du thorax) concluant à une amylose laryngée localisée mais la patiente a été perdue de vue au bout de 4 mois. DISCUSSIONLes amyloses sont des maladies liées au dépôt extracellulaire, dans différents organes, d'une substance amorphe, la substance amyloïde, constituée à partir de différents précur-seurs protéiques (1, 2). La substance amyloïde peut être une protéine normalement présente dans l'organisme mais présentant une mutation génique qui la rend " amyloïdogène " dans les formes hérédi-taires, ou une chaîne légère " l'immunoglobuline monoclona-J. TUN ORL -N°16 JUIN 2006
Endocrine involvement during the course of GPA is exceptional, and only a few sporadic observations were reported. Thus the exact meaning of endocrinopathies associated with this vasculitis is not yet well known. Fortuitous association or specific localization of vasculitis in the endocrine glands? The purpose of this minireview is to study the endocrine involvement in granulomatosis with polyangeiitis and to discuss its possible pathogenic mechanisms.
Introduction The cardiac localization of hydatid cyst (HC) is rare and little known. It is exceptionally primary and isolated. Myocardial ischemia remains an exceptional and unusual manifestation of this localization. We report the original observation of cardiac HC revealed by acute myocardial ischemia in a young subject. Observation A 35-year-old patient with no notable pathological history was admitted to our department via the emergency room for acute anterior chest pain. The somatic examination was without abnormalities as well as the baseline biological tests. The electrocardiogram demonstrated epicardial ischemia in the infero-lateral territory without other abnormalities. The cardiac troponin I was slightly elevated (32ng/l, N<19ng/l). Subsequent controls after 30min, 60min and 180min did not show significant variations. Subsequent investigations (CT, coro-CT, coronary angiography) led to the diagnosis of intramyocardial HC of the left ventricle, with no abnormalities of the coronary arteries. After surgical excision, the evolution was favorable with normalization of the electrocardiogram and the troponin Ic. Conclusion Cardiac localization of this parasitosis is exceptional and characterized by an important clinical polymorphism making its diagnosis a real challenge. It seems useful to discuss the diagnosis of cardiac HC in front of any unexplained electrocardiogram abnormality occurring in endemic zone.
Introduction Psychiatric manifestations are exceptional during hypothyroidism and are not always easy to diagnose, especially since the clinical signs of hypothyroidism can be confused with those of the depressive syndrome. We report an original observation of depression of the elderly revealing an isolated primary hypothyroidism. Observation A 78-year-old patient, followed for essential hypertension, well-balanced under monotherapy with calcium channel blockers, and without degenerative complications, was referred by her family doctor for depressive syndrome not improved by the specific treatment prescribed and correctly taken for six months. The clinical examination noted in particular macroglossia with dental impressions and dry and cracked skin. The biological assessment showed hypothyroidism with TSH at 28µmol/l. Thyroid ultrasound showed atrophy of the thyroid gland. The thyroid autoimmunity (anti-thyroglobulin and anti-thyroperoxidase antibodies) was negative. The rest of the biological tests were in the normal range. The diagnosis of Riedel's thyroiditis was retained and the patient was treated with levothyroxine in progressive doses until TSH normalization was achieved. The evolution on the psychiatric level was also favorable with disappearance of the signs of the depression and the anti-depressive treatment was discontinued. Conclusion Depression is rare, complicating only 4% of overt hypothyroidism and its diagnosis is not always easy, especially in the elderly. Hormone replacement therapy significantly improves the depressive syndrome and protects against cognitive decline. Thus, hypothyroidism screening seems appropriate for any depressive syndrome in the elderly.
Optic neuritis is a common manifestation of multiple sclerosis. It occurs in two thirds of patients at some point in the course of this disease and is usually unilateral, acute and often recurrent. However, optic neuritis can be the first manifestation of this demyelinating disease in 15 to 20% of cases.Bilateral, simultaneous and retrobulbar forms of optic neuritis inaugurating multiple sclerosis remain exceptional and unusual. They represent a real diagnostic challenge and require special attention from the clinician Herein we report the case of bilateral simultaneous and isolated retrobulbar optic neuritis inaugurating multiple sclerosis in a 24 year old woman.
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