Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0004134 (ataxia)
 15,886 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

Two cases of Vogt-Koyanagi-Harada disease with rare neurological complications were reported, which are one case with vestibular and cerebellar ataxia, and one with multiple cranial nerve palsies. The first case, a 32-year-old man, was admitted to our ophthalmologic department because of visual impairment. Two weeks later, he complained of gait disturbance. Limb and truncal ataxia, vestibular dysfunction and perceptive deafness were observed. Ophthalmologic examination revealed typical findings of Vogt-Koyanagi-Harada disease. CSF showed elevated protein and mononuclear pleocytosis. After treatment with oral steroid therapy, vestibular and cerebellar ataxia, and visual impairment improved. The second case, a 22-years-old man, showed the VIIth, VIIIth, IXth, and Xth cranial nerve palsies with visual impairment, and he was diagnosed to have cranial nerve palsies associated with Vogt-Koyanagi-Harada disease. After steroid therapy, there was a marked clinical improvement. Vogt-Koyanagi-Harada disease is an autoimmune disease against the melanocytes and involving tissues containing melanocytes such as uvea, meningea, skin and inner ear, etc.. Neurological complications except for meningitis and perceptive deafness have been rarely reported in Japan. Related to the present cases, possible pathogenesis was discussed.
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PMID:[Each one case of Vogt-Koyanagi-Harada disease with vestibular and cerebellar ataxia, and multiple cranial nerve palsies]. 266 96

We reported unique magnetic resonance imaging (MRI) findings of a 57-year-old Japanese man who was diagnosed as Vogt-Koyanagi-Harada disease. This patient presented with complaints of a transient severe headache followed by a bilateral loss of visual acuity and truncal ataxia. Magnetic resonance imaging revealed abnormal contrast enhancement of both the uveas and the cerebellar vermis corresponding to his neurological abnormalities. The distribution and the nature of the resolution of this unusual pattern of contrast enhancement suggested that these MRI findings might illustrate transient destruction of the blood brain barrier or vascular extravasations. Such events might be representative of pathophysiology involving the central nervous system that occurred in patients with Vogt-Koyanagi-Harada disease.
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PMID:[Vogt-Koyanagi-Harada disease with cerebellar lesions demonstrated on MRI: a case report]. 882 4

We report on a 28-year-old man with a 2-year history of Vogt-Koyanagi-Harada disease (VKH), who developed one-and-a-half syndrome, facial hypoesthesia, facial nerve palsy, hearing loss and limb ataxia on the right side. An MRI showed inflammatory lesions in the pons extending into the left middle cerebellar peduncle. Corticosteroid treatment successfully ameliorated his symptoms and lesions. This patient demonstrated the possible association of brainstem encephalitis with VKH.
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PMID:Vogt-Koyanagi-Harada disease associated with brainstem encephalitis. 1920 Nov 91

Background: Vogt-Koyanagi-Harada disease (VKHD)-like symptoms have previously been reported in 11 melanoma patients treated with immune checkpoint inhibitors. Materials & methods: We report a female patient with multilocular metastatic melanoma who was treated with nivolumab. Results: Following the first nivolumab dose, she experienced bilateral blurry vision, hearing loss, vertigo and ataxia. Ocular ultrasound was consistent with the diagnosis of uveitis. Audiography revealed severe bilateral sensorineural hearing loss. A high-dose corticosteroid regimen was initiated under which the patient developed generalized vitiligo. Abdominal and thoracic CT scans showed an almost complete response to nivolumab therapy. This patient fulfilled all criteria of VKHD which is characterized pathogenetically by an antimelanocytic autoimmune process. Conclusion: The present case showed an impressive response to antimelanoma immunotherapy. Based on these data, the occurrence of VKHD in melanoma patients appears to be a strong indicator for immune checkpoint inhibitor efficacy.
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PMID:Concurrent Vogt-Koyanagi-Harada disease and impressive response to immune checkpoint blockade in metastatic melanoma. 3230 86