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Paediatric neuro-Behçet’s disease presenting with optic nerve head swelling
  1. SANDIP MITRA,
  2. R L KOUL
  1. Department of Ophthalmology, Sultan Qaboos University Hospital, PO Box 38, Alkhod, Muscat 123, Sultanate of Oman
  1. Dr Mitra.

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Editor,—Involvement of the central nervous system in Behçet’s disease (BD) occurs in approximately 10–49%1 of patients and the frequency of ocular manifestations is believed to be in the range of 28–80%.2 Neuropapillitis has been reported very rarely as a manifestation of Behçet’s disease.3 As far as we know, there has been no report of paediatric onset neuropapillitis in BD. Here we report a case of neuropapillitis in a 10 year old girl.

CASE REPORT

A 10 year old Omani girl presented in August 1998 with complaint of right sided headache and blurring of vision in the right eye for 10 days and complete loss of vision in the same eye for 5 days. There was no history of vomiting, loss of consciousness, convulsion, or any other neurological deficit. On admission, examination revealed total vision loss in the right eye. The vision was 20/20 in the left eye. Examination of right eye showed relative afferent pupillary defect and fundus examination revealed blurred disc margins with no apparent cup. There was a 2 dioptre elevation of the disc (Fig1).

Figure 1

The right eye fundus picture shows blurred disc margins with full cup and elevation of 2 disc diameters.

Visual evoked potentials (VEP) and brainstem auditory evoked potentials (BAEP) on the right side were prolonged in latency and poorly formed. Magnetic resonance imaging of the brain revealed left sided temporoparietal lesion suggestive of inflammation/demyelination. The child was started on methylprednisolone 500 mg intravenously in two doses followed by 15 mg/day for 2 weeks. Vision returned on day 2 with perception of hand movements, finger counting day 4, and complete recovery on day 8. VEP was repeated and showed normal response. She was admitted in September 1995 with complaint of headache, diplopia, and visual blurring. She had meningeal signs, left lateral rectus palsy, and bilateral papilloedema. Cerebrospinal fluid (CSF) examination showed opening pressure of 210 mm H2O, closing pressure of 140 mm H2O, with CSF protein 0.36 (0.15–0.50 g/l), glucose 3.8 (2.2–3.9 mmol/l). White blood cells (WBC) 23, 60% lymphocytes, 40% polymorphs, RBC 10, and no organisms. Chestx ray was normal, Mantoux test was negative, blood culture and CSF had no growth after 48 hours. The clinical picture was suggestive of aseptic meningitis with raised intracranial pressure. The child was treated with systemic steroids over 2 weeks and improved with complete recovery of the sixth nerve palsy and resolution of papilloedema. In November 1995, she presented with left supranuclear facial nerve palsy and left hemiparesis, with hemihypoaesthesia. There was no papilloedema. CT showed right side thalamic and posterior internal capsule infarct. There was complete recovery after 1 month. During follow up the child had aphthous ulcer and skin rash. Skin biopsy showed prominent lymphocytic infiltration of the vessels in the dermis consistent with vasculitis, possibly Behçet’s disease.

COMMENT

Neuro-Behçet’s disease is an uncommon presentation in childhood.4 This patient had four episodes of neurological involvement and a skin rash once. Initial manifestation was raised intracranial pressure with aseptic meningitis-like picture and no underlying cause was found. The child recovered with steroids (prednisolone) and diuretics (acetazolamide), which are recommended in the severe form of Behçet’s disease.2 Later the child had a cerebrovascular accident that also recovered with steroids. There was no underlying cause found. It was on follow up, when the child had aphthous ulcer and skin rash, that diagnosis of Behçet’s disease (neuro-Behçet’s) was made. The fourth time (last admission) she presented with loss of vision in right eye and investigations—for example, VEP, suggested neuropapillitis.5 The diagnosis of Behçet’s disease was made on two major criteria oral aphthous ulcers, eye involvement and minor criteria of skin biopsy suggestive of lymphocytic vasculitis,6 CNS involvement and abnormal changes in the magnetic resonance imaging (MRI).5 Colvardet al7 reviewed the ocular manifestations of BD in 32 patients. Inflammatory involvement of the optic nerve in the form of papillitis was reported in their three patients. Our case here is the first reported case of inflammatory optic nerve involvement in the paediatric age group, and clinically resembles multiple sclerosis (MS) as reported in adults by Kansuet al.4 Multiple sclerosis is rare at this age and the presentation of aseptic meningitis, papilloedema, skin lesion, and aphthous ulcer, with MRI showing a single patch of demyelination, excludes this possibility. The MRI of the optic nerve was normal despite abnormal VEP in our case. Unless the patient presents with clear symptoms, the diagnosis of Behçet’s disease is difficult initially. The diagnosis in children is more difficult as the disease is uncommon and usually the diagnosis is made after several systemic presentations and the exclusion of other common diseases.

References

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