Acute Myelitis in a Patient with Vogt-Koyanagi-Harada Disease: Case Report and Review of the Literature
A 50-year-old Chinese Han woman presented with sudden onset of difficulty walking, numbness on the left side of the body, and difficulty with urination for 6 days. Twelve days prior to the presentation she had been diagnosed with incomplete VKH disease by an ophthalmologist based on blurred vision in both eyes, bilateral nontraumatic granulomatous iridocyclitis, retinal edema, and the presence of exudates. She had received corticosteroid treatment (500-mg intravenous methylprednisone for 3 days followed by 300-mg intravenous methylprednisone for 3 days and a tapering course of 80-mg prednisone for 6 days). Neurological manifestations (headache, tinnitus, difficulty climbing stairs, numbness on the left side of the body, and dysuria) emerged during the tapering of steroid treatment. She recalled a history of upper respiratory tract infection a month previously but denied any history of vaccination. Physical examinations revealed normal vital signs. There was no lymphadenopathy, or oral or genital ulcers. Her skin and hair showed no vitiligo, poliosis, or alopecia. Neurological examinations showed a normal mental status and cranial nerves except for a visual acuity of 20/200 bilaterally and papilledema of both eyes in a fundus examination. The strength of both upper and lower limbs was decreased. Her muscle tone was increased. Her deep tendon reflexes were hyperactive without clonus. The Babinski sign was present bilaterally, and her response to the finger-to-nose test was impaired. Her gait was slow and stiffly shuffling. Pinprick sensations were decreased in the left limbs, whereas position sense was preserved. No obvious sensory level was identified. Kernig's sign was absent. Routine laboratory evaluation showed all relevant values to be within normal limits. Screening for connective-tissue disease, including C reactive protein, rheumatic factor, antinuclear antibody, C-antineutrophil cytoplasmic autoantibody, anti-double-strand DNA, anti-Smith, anti-ribonucleoprotein, anti-Sjogren's syndrome A, and anti-Sjogren's syndrome B antibodies, produced normal or negative results. The erythrocyte sedimentation rate was 39 mm/hr. Cerebrospinal fluid (CSF) contained 128 WBCs/mm3 (normal <10/mm3) with 94% mononuclear cells and 4.83 g/L protein (normal 1.5-4.5 g/L). A CSF bacterial culture produced negative results. A nerve conduction study showed mild polyneuropathy, and visual evoked potentials were normal. A chest X-ray was normal. A brain MRI scan was normal with no callosal or significant periventricular lesions. Optical coherence tomography performed independently by two ophthalmologists revealed bilateral disc edema accompanied by serous retinal detachment (). Spinal-cord MRI () revealed a hyperintense signal in the T2-weighted sequence with significant gadolinium enhancement between the C1-3 vertebrae in the cervical cord. The patient was diagnosed as aseptic meningitis with acute myelitis complicating VKH disease, and was given pulse steroid therapy again (intravenous administration of 1000 mg of methylprednisolone for 3 days, 500-mg intravenous methylprednisolone for 10 days, 250-mg intravenous methylprednisolone for 10 days, and a tapering course of 60-mg prednisone over a 6-month period). After 4 months she was able to climb stairs without help. Her visual acuity recovered to 50/200 and her numbness and dysuria also improved significantly. At the 1-year follow-up she was back to her baseline overall condition and showed no recurrence of any visual or neurological symptoms.