RESUMEN
INTRODUCTION: Central nervous system involvement is rare in Wegener granulomatosis. Stroke is the most common event suggestive of the disease. COMMENT: A 35-year-old woman, who was followed for rhinitis and mild asthma, described gradual decline of visual acuity in the right eye over two months, persistent nasal obstruction and fronto-orbital headache since a few weeks. She presented left hemiparesis due to a stroke, associated with exophthalmos and deficits of the optic nerve and abducens of the right eye. The otolaryngological examination found signs of crusty rhinitis and right nasal stenosis. The diagnosis of Wegener's granulomatosis was established on the basis of the clinical findings, radiological aspects and the presence of ANCA. The patient was treated by antiplatelet agents and high-dose corticosteroids associated with immunosuppressive drugs including cyclophosphamide in a monthly bolus. DISCUSSION: This case illustrates two of the three pathogenic mechanisms that may account for central nervous system involvement in Wegener granulomatosis: vasculitis, extension by contiguity of granulomatous tissue from the nasal cavity or sinuses, and in situ formation of a granuloma into the brain parenchyma or meninges.
Asunto(s)
Isquemia Encefálica/etiología , Granulomatosis con Poliangitis/diagnóstico , Enfermedades del Nervio Abducens/etiología , Adulto , Anticuerpos Anticitoplasma de Neutrófilos/sangre , Corteza Cerebral/irrigación sanguínea , Cuerpo Estriado/irrigación sanguínea , Ciclofosfamida/uso terapéutico , Exoftalmia/etiología , Femenino , Granulomatosis con Poliangitis/complicaciones , Granulomatosis con Poliangitis/tratamiento farmacológico , Granulomatosis con Poliangitis/inmunología , Humanos , Inmunosupresores/uso terapéutico , Imagen por Resonancia Magnética , Metilprednisolona/uso terapéutico , Obstrucción Nasal/etiología , Síndromes de Compresión Nerviosa/etiología , Enfermedades del Nervio Óptico/etiología , Paresia/etiologíaRESUMEN
INTRODUCTION: Horton's disease is the most common vasculitis of elder people. Several neurological complications are reported, but pachymeningitis is exceptional. OBSERVATION: A 71-year-old patient who presented headache, hyperesthesia of the scalp, weight loss with a biological inflammatory syndrome and meningeal thickening on MRI. The diagnosis of pachymeningitis related to Horton's disease was retained. The patient was treated by corticosteroids with a good clinical, biological and radiological course after 22 months. CONCLUSION: Horton's disease is a potential diagnosis in elderly persons with pachymeningitis and inflammatory syndrome.
Asunto(s)
Corticoesteroides/uso terapéutico , Arteritis de Células Gigantes/complicaciones , Anciano , Duramadre/patología , Femenino , Arteritis de Células Gigantes/tratamiento farmacológico , Cefalea/etiología , Humanos , Hiperestesia/tratamiento farmacológico , Hiperestesia/etiología , Inflamación/patología , Imagen por Resonancia Magnética , Meninges/anatomía & histología , Meninges/efectos de los fármacos , Meninges/patología , Meningitis/etiología , Cuero Cabelludo/fisiopatologíaRESUMEN
INTRODUCTION: Neurological manifestations of celiac disease are rare and polymorphic. Similar to lesions of the digestive tract, the standard treatment includes steroids. CASE REPORT: A 41-year-old woman, followed up for celiac disease resistant to gluten-free diet, developed rapidly spastic paraparesis, cerebellar syndrome, horizontal diplopia and decline of visual acuity. The diagnosis of neurological complications of celiac disease was established and the patient was treated with methylprednisolone, followed by oral prednisone. For 9 years, the patient's neurological status remained stabilized with a prednisone dose at 20mg per day. The patient relapsed when progressive reduction of prednisone dose was attempted; neurological and gastrointestinal signs worsening at 15mg per day; increasing the dose to 30mg improved the clinical status. DISCUSSION: The mechanism of onset of neurological disease remains unknown. Immunological, nutritional, toxic or metabolic factors could be involved. The positive response to corticosteroids observed in this patient suggest an immunological mechanism.