Acute Myelitis in a Patient with Vogt-Koyanagi-Harada Disease: Case Report and Review of the Literature

Abstract

BackgroundVogt-Koyanagi-Harada (VKH) disease is characterized by bilateral granulomatous uveitis with neurologic, auditory, and dermatologic manifestations. However, acute myelitis complicating VKH disease has rarely been reported.Case ReportA 50-year-old Chinese Han woman presented with difficulty walking, numbness on the left side of the body, and difficulty with urination. The patient was diagnosed with incomplete VKH disease and received corticosteroid treatment prior to the neurological presentation. Acute myelitis was diagnosed based on both clinical and spinal-cord MRI findings.ConclusionsClinicians should consider acute myelitis as a rare possible neurological manifestation in VKH disease patients, and early systemic administration of corticosteroids will suppress the acute inflammatory process and prevent recurrences. This report raises the possibility that VKH disease and acute myelitis share common pathogenic pathways.

Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes

“…CSF pleocytosis is observed in more than 80 % of cases, with a predominance of lymphomononuclear cells, which may be present until the eighth week of the onset of the disease [ 45 ]. Serious meningeal-encephalic manifestations and focal neurological signs (i.e.cranial neuropathies, hemiparesis, aphasia, acute transverse myelitis and ciliary ganglionitis) were also reported [ 4 , 58 , 71 ].…”

Delayed diagnosis of myelitis in a patient with Vogt-Koyanagi-Harada disease: a case report

“…After treatment with corticosteroid, although the motor weakness in the left lower extremity did not recover, the right lower extremity showed good recovery. Corticosteroid therapy is the treatment of choice for myelitis, 5 as it has anti-inflammatory and immunosuppressive properties, 9 and reduction of inflammation by corticosteroids may prevent damage to the spinal cord. Therefore, before the spinal cord is significantly damaged, early treatment with corticosteroids should be initiated to achieve a good therapeutic response.…”

“…In 2013, Gu et al. 5 reported the case of a 50-year-old female patient with VKH disease with a sudden onset of motor weakness in the lower extremity (the degree of motor weakness was not described), numbness on the left side of the body, and difficulty in urination for 6 days previously due to acute myelitis. She was administered intravenous methylprednisolone at 1 g/day for 3 days, 500 mg/day for 10 days, 250 mg/day for 10 days, and oral prednisolone for 6 months.…”

“… 3 There are few reports about the occurrence of myelitis in patients with VKH disease. 4 – 6 Myelitis is an inflammatory disorder of the spinal cord. Patients with myelitis show diffuse immune derangement in the spinal cord and spinal fluid.…”

Immune-Mediated Disorders Affecting the Spinal Cord and the Spine

“…The disease can result in bilateral uveitis, alopecia, poliosis, vitiligo, and deafness with transverse myelitis being a rare manifestation of this disease. Treatment is with steroids [ 35 ].…”