The Vogt-Koyanagi-Harada (VKH) syndrome, a disease of unknown origin, characterized by inflammation of the uveal tract, retina and meninges, was observed in a patient with Hodgkin's disease (HD) who had been treated with chemotherapy and radiotherapy. Ten years later the patient developed a cluster of neurological symptoms and signs (fever, pain, bilateral hypoacousia, dizziness, uveitis and psychiatric disorder) which were initially interpreted as a relapse of HD. The diagnosis of VKH syndrome was thereafter suspected from the clinical findings, response to therapy and further evolution. During the follow-up (14 months) the patient showed a varying course; hypoacousia remained unaltered while uveitis recurred and only a partial improvement was obtained by immunosuppressive therapy.

A case of Vogt-Koyanagi-Harada syndrome in a patient affected by Hodgkin's disease.

Les uvéites: aspects étiologiques

Uvéite à forme pseudosarcoïdosique révélant un lymphome cérébral primitif

Primary CNS lymphomas (PCNSL), until recently representing about 1% of all brain tumors, show dramatically increased incidence both in high-risk groups (immunocompromised, AIDS) and in the general population. They are extranodal diffuse non-Hodgkin's lymphomas, the morphology and classification of which are identical to those of systemic lymphomas, although PCNSL show different biological behavior and diagnosis according to the New Working Formulation and updated Kiel classification may be difficult. The majority are large B cell variants of high-grade malignancy; low-grade subtypes and T cell lymphomas are rare. Sixty per cent occur in the supratentorial space (hemispheres, periventricular) and 12% in the posterior fossa; 30% are multiple (50%-70% in AIDS). PCNSL show a male preponderance with a peak incidence in the 5th-7th decade (3rd-4th in AIDS). The duration of diffuse or focal clinical symptoms averages 1-2 months. Computed tomography and magnetic resonance imaging scans show single or multiple or diffuse, often typical lesions. Diagnosis is achieved by evaluation of stereotactic biopsy material or cerebrospinal fluid cytology using immunocytological markers. Current therapy in immunocompetent patients, radiation plus corticosteroids and pre- or postradiation polychemotherapy, shows response rates of 85% with a median survival of 17-44 months, a prognosis similar to that for glioblastoma. Meningeal PCNSL is treated with intrathecal methotrexate or cytosine arabinoside. Transliquoral seeding of PCNSL is frequent, distant metastases occurring in 6%-8%. Therapy of AIDS-related PCNSL makes use of radiation and corticosteroids, and rarely of chemotherapy. The pathogenesis of PCNSL is unknown, but Epstein-Barr virus may be a contributory factor.

Primary central nervous system lymphomas--an update.

Uveitis and cerebral lymphoma

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Uveitis and cerebral lymphoma