A 68-year-old gentleman, presented to us with a history of blurring of vision for 45 days, severe holocranial headache with mild grade of evening rise in temperature for eight days, and acute loss of hearing in both ears for two days. The illness was acute in onset and progressive in nature. He had a severe holocranial headache, drowsiness and clouded sensorium at the time of presentation. He was a one-eyed patient with a history of loss of vision in the right eye status post-cataract surgery 28 years back. This time, he presented with blurring followed by a diminution of the vision of the left eye for more than a month, with associated redness and watering of the eyes. There was no history of any injury to the eye. He was an old case of COPD, poorly managed for ten years, and was repeatedly prescribed oral prednisolone for acute exacerbation of the same for the last six years. While taking the history of medications, he mentioned using oral steroids "as and when needed". The patient was compliant to his inhaled steroid medications and his last oral prednisolone consumption was six months prior to the onset of ophthalmological symptoms. He had a history of primary pulmonary tuberculosis (TB) and was treated 20 years back with a full course of anti-tubercular medications. On examination, the patient was acutely ill, delirious, and was not oriented to time, location, or person; but could execute basic commands with considerable psychomotor slowing. His vitals were within normal limits, and all his reflexes were normal. Other than hearing loss and visual deterioration, there were no focal neurological deficits. He had nuchal rigidity. He also had several hypo-pigmented macules on his back and trunk (Figure 1). Since the patient had redness and lacrimation in both eyes, ophthalmological consultation was sought. His best-corrected visual acuity on ophthalmological examination was no perception of light in the right eye and finger counting close to the face in the left eye. The right eye had a complete leucomatous corneal opacity, obscuring any view of the anterior segment, and the ultrasound revealed retinal detachment with diffuse choroid thickening. The left eye revealed a non-reacting semi-dilated pupil, a normal anterior chamber, and old keratic precipitates on the endothelium. The retinal examination of the left eye revealed a pale optic disc with peripapillary atrophy and depigmented choroid (due to chronic disease), giving the impression of a "sunset glow fundus" (white optic disc surrounded by orange-red colored choroid) (Figure 2).
Cerebrospinal fluid (CSF) examination revealed lymphocyte-predominant pleocytosis and raised protein (63 mg/dl). Cerebrospinal fluid adenosine deaminase (ADA) was 3.30 units/L (normal range 0-9 units/L). High-resolution computed tomography (HRCT) chest revealed bilateral para-septal and centrilobular emphysema with bullae formation predominantly in bilateral upper lobes. His spirometry was suggestive of an obstructive pattern. His magnetic resonance imaging (MRI) brain revealed multiple lacunar infarcts. His pure tone audiometry showed bilateral sensorineural hearing loss ranging from severe to profound (Figure 3). Due to acute encephalopathy, nuchal rigidity with CSF pleocytosis, diagnosis of meningoencephalitis was considered. However, when the ophthalmologic examination findings were taken into account, the granulomatous uveitis along with meningoencephalitis, auditory (sensory neural hearing loss), and skin involvement (vitiligo), rather than isolated meningitis, were strongly pointing towards VKHD. Other differentials were ruled out using proper investigations. Considering the imminent possibility of sight-threatening complications, the patient was immediately initiated on intravenous methylprednisolone, followed by oral tablet prednisolone with the plan for slow tapering in the next six months. He also received supportive management, like topical local application of tacrolimus, 1% ointment for hypo-pigmented patches, oral calcium, and vitamin D supplements. The patient responded to corticosteroids dramatically. His sensorium improved after three days of treatment; he became alert, could execute complex commands, and was oriented to time, place, and person. After one week, his left eye visual acuity improved to 6/36, and he reported subjective hearing improvement after two weeks. Even so, after two months of therapy, repeated pure tone audiometry revealed no objective signs of improvement. The patient was informed of the recurring nature and poor progression of the disease due to chronicity.