Sir,

Uveitis has been described as a precursor to acute disseminated encephalomyelitis (ADEM) in adults.1 Here, we describe a case in a teenager. ADEM is a rare post-infectious encephalomyelitis, with characteristic MRI signs and subsequent recovery.2

Case report

A 17-year-old Caucasian girl, with past medical history of hypermetropia, mild amblyopia, squint surgery, and long-standing headaches, presented with an episode of pain, watering, and sensitivity to light in the left eye, as an emergency. Her best-corrected visual acuity was RE 6/5, LE 6/18. This was diagnosed as severe anterior uveitis and treated with topical steroids. After 10 days, she was found to have mild anterior chamber activity in the right eye as well. During her follow-up, she was also found to have moderate amount of vitritis, snowballs, and cystoid macular oedema (CMO) on OCT (Figure 1). No afferent pupil defect was found. A fundus fluorescein angiogram did not show any evidence of vasculitis (Figure 2).

Figure 1
figure 1

OCT of the left eye showing cystoid macular oedema.

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Figure 2
figure 2

FFA of the left eye, cystoid macular oedema, and no evidence of vasculitis.

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Four months after the onset of uveitis, she presented to the Emergency Department with an acute confusional state, vomiting, decreased consciousness, and floppy right arm. There was no preceding febrile illness, or history of vaccination before this episode. She was intubated, ventilated, commenced on cefotaxime, and transferred to the regional paediatric intensive care unit, where she was started on aciclovir. An urgent MRI of her brain was obtained, showing diffuse oedema and multiple areas of demyelination consistent with ADEM (Figures 3 and 4). A lumbar puncture showed no evidence of infection on culture or PCR for herpes simplex. CSF examination showed 0 WBC, 0 RBC, protein 0.31 g/l, glucose 5.5 mmol/l, and lactate 1.6 mmol/l. Oligoclonal bands were negative. In addition to Behcets and juvenile idiopathic arthritis (JIA), sarcoidosis was considered and serum ACE was 37 U/l, and autoantibody screen including dsDNA was normal. Only NMDA receptor antibodies were carried out and they were negative. Neuromyelitis optica antibodies (AQP4) and myelin oligodendrocyte glycoprotein (MOG) were not carried out. ESR was 19 mm/h.

Figure 3
figure 3

MRI showing high signal in the region of left internal capsule, explaining patient’s hemiparetic weakness.

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Figure 4
figure 4

MRI coronal view showing bi-hemispheric widespread white matter high signal change indicative of ADEM.

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Once infection was excluded, she was started on intravenous methylprednisolone followed by a reducing course of oral prednisolone. She was ventilated for 3 days and went on to make a full recovery. Visual acuity improved at this stage to 6/6 in the left eye, after resolution of CMO.

Comment

Multiple sclerosis is associated with ocular inflammatory disease.3 ADEM is a related, rare condition with diagnostic criteria of polysymptomatic onset encephalopathy, subsequent recovery, and specific MRI changes. Tsutsumi et al1 previously reported a 69-year-old woman who had recurrent uveitis preceding ADEM. Our case presented with intermediate uveitis preceding encephalopathy features typical of ADEM in a teenager. Uveitis resolution occurred after complete resolution of ADEM features.

Although rare, this diagnosis should be considered with neurological features, especially in the presence of headache,4 optic neuritis,5, 6 diplopia,5 optic disc haemorrhages,6 and uveitis.1