Abstract

Background and PurposeThe usual neurologic manifestations of Vogt-Koyanagi-Harada (VKH) disease include aseptic meningitis and headaches. We performed the present study to review all unusual neurologic manifestations reported in VKH disease to summarize them.MethodsA literature search was performed in the English language on Scopus and Medline via PubMed from 1946 to July 31, 2021, by using the following terms: “Vogt Koyanagi Harada disease” OR “VKH disease” AND “Brain” OR “Spinal cord” OR “CNS” OR “Central nervous system” OR “Neurologic” OR “Peripheral nervous system” OR “Polyneuropathies. Our inclusion criteria were unusual neurologic manifestations of VKH disease.ResultsOur literature search yielded 417 total articles (PubMed = 334, Scopus = 83) from which 32 studies comprising 43 patients (22 men and 21 women, of which 62.8% were younger than 50 years) were included in this systematic literature review. Regarding the study design, all studies were case reports and published between 1981 and 2021. CNS involvement was the most reported (93%) in VKH disease. Peripheral nervous system involvement represents 7% of cases. The cerebral lesions were parenchymal inflammatory lesions in the white matter or posterior fossa with or no contrast enhancement (16.3%), leptomeningitis (9.3%), pachymeningitis (7%), meningoencephalitis (2.3%), ischemic stroke (4.6%), hemorrhagic stroke (2.3%), transient ischemic attack (2.3%), and hydrocephalus (2.3%). The optic nerve lesions were anterior ischemic optic neuropathy (20.9%) and optic neuritis (9.3%). Concerning spinal cord lesion, it was mainly myelitis (14%).ConclusionThis systematic literature review provides a summary of the different unusual neurologic manifestations reported in VKH disease.

Highlights

  • Background and PurposeThe usual neurologic manifestations of Vogt-Koyanagi-Harada (VKH) disease include aseptic meningitis and headaches

  • The literature search was performed by using the following terms: “Vogt Koyanagi Harada disease” OR “VKH disease” AND “Brain” OR “Spinal cord” OR “CNS” OR “Central nervous system” OR “Neurologic” OR “Peripheral nervous system” OR “Polyneuropathies. The search was conducted in the English language

  • In the present systematic literature review (SLR), we found that unusual neurologic manifestations of VKH disease are rare, and all reported studies are case reports

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Summary

Introduction

Background and PurposeThe usual neurologic manifestations of Vogt-Koyanagi-Harada (VKH) disease include aseptic meningitis and headaches. Vogt-Koyanagi-Harada (VKH) disease is characterized by bilateral ocular involvement associated with extraocular manifestations such as neurological (related to aseptic meningitis: headache, neck and back stiffness), auditory (tinnitus, hearing loss, and vertigo), and integumentary (alopecia, poliosis, and vitiligo) [1]. VKH disease is a rare multisystemic autoimmune disease, mediated by T cells directed against melanocytes strongly present in the eye (choroids), inner ear, meninges, and the integumentary system [2, 3]. This disease affects mainly patients aged between 20 and 50 years, females (with a female/male ratio of 2:1), Asians, Native Americans, and Hispanics [2]. Infectious agents such as Epstein-Barr virus and cytomegalovirus are the mains environmental triggers reported [7, 8]

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