Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Vogt-Koyanagi-Harada syndrome is a rare disease, which probably has a cell-mediated autoimmune pathogenesis, marked by ocular (anterior and/or posterior uveitis), dermatological (poliosis, canities, vitiligo) and neurological (meningo-encephalitis) disorders of variable severity in variable combinations. The clinical pattern in the case reported here showed severe neurological involvement (headache, ataxia and confusional state) followed by anteroposterior uveitis. Instrumental investigations (cerebrospinal fluid, VEPs, BAEPs, EEG, CT and MRI brainscans) confirmed the diagnosis. The response to cortisone therapy was excellent. We emphasize the importance of the neuroradiological investigations, because of their peculiarities, and review the reports of cases with marked meningo-encephalitic impairment, given the dearth of reports in the neurological journals.
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PMID:Vogt-Koyanagi-Harada syndrome: clinical and instrumental contribution. 195 5

We here report the case of a 27-year old patient, followed-up in our Department for treatment of chronic Vogt-Koyanagi-Harada disease ( VKH disease). Fundus examination showed depigmentation of the retinal pigment epithelium and of the choroid, appearing as a pseudotumoral peripapillary lesion. Vogt-Koyanagi-Harada disease is a multisystem disorder, characterized by bilateral granulomatous panuveitis with serous exudative multifocal retinal detachment. Pathophysiology of this disease is unknown, but an immunological cellular reaction against melanocytes of the skin, the meninges, the retina, the uvea, the cochlea and the labyrinth is suspected. This disease mainly occurs in young subjects from the Far East as well as in pigmented subjects. Ocular involvement is often associated with neurological (meningeal stiffness, headache, sometimes associated with focal deficit and erebrospinal fluid (CSF) pleocytosis), auditory ( perceptive deafness) and cutaneous (vitiligo, poliosis, alopecia and canities) manifestations. It usually evolves in three phases: a prodromal phase mainly characterized by neurological signs, an acute uveitic phase, a chronic phase of convalescence characterized by choroidal and tegument depigmentation or a phase of recurrence during which subretinal neovessels and subretinal fibrosis may appear. Scarrings manifest during the chronic phase of VKH disease, which is dominated by diffuse depigmentation of the fundus of the eye, scars due to nummular chorioretinal atrophy, wheals due to diffuse depigmentation, macular scar remodeling. Pseudotumoral appearance is rare and atypical during the chronic phase of VKH disease. Treatment is based on intravenous corticosteroids followed by a cycle of oral therapy. Patient should be early treated with massive and prolonged therapy to improve prognosis.
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PMID:[A rare case of ocular scarrings in a patient with Vogt-Koyanagi-Harada disease]. 2972 Nov 43