Neurological Behcet’s Disease in an Adolescent: A Case Report

Background Behcet’s disease (BD) is an idiopathic multisystemic inflammatory disease. There are 2 primary sets of diagnostic criteria. The 1990 International Study Group criteria consists of recurrent oral ulcers at least 3 times in 12 months and 2 or more of the following other features: recurrent genital ulcers, eye lesions (uveitis, retinal vasculitis), skin lesions (erythema nodosum, pseudo folliculitis, papulopustular lesions, or acneiform nodules) or a positive pathergy test (skin papule 2 mm or more 24-48 hrs post-needle insertion).[1,2] The 2014 International Criteria for Behcet Disease assigns 2 points for the following: ocular lesions, genital aphthosis, and oral aphthosis and 1 point for each of the following: skin lesions, neurologic manifestations (central and peripheral), and vascular manifestations (arterial and/or venous thrombosis, phlebitis) and a bonus point for a positive pathergy test. Four points or greater is consistent with a diagnosis of Behcet’s disease.[2] The vasculitis of Behcet’s disease is unique in that it can affect vessels of all sizes and may consist of arterial and/or venous involvement.[1] While disease onset is more common in adults 30-40 years of age, it can present in childhood with a mean onset of 12 years old and a 4:1 male predominance.[1,2] Neurological involvement occurs in 5-10% of all BD cases with pediatric-onset neurological involvement being rare at ~4% of all cases of NB.[1] Case We present a case of a 14-year-old Somali male with a history of latent tuberculosis (TB) who presented with 2 episodes of conjunctivitis and left eye blurred vision. He was diagnosed with bilateral pan uveitis and right cystoid macular edema. Workup demonstrated positive HLA-B51 and Toxoplasmosis IgG antibody and he was started on sulfamethoxazole and trimethoprim. The previous TB QuantiFERON was positive with negative chest x-ray and TB skin test. Three months later, he presented with acute genital ulcers, lower extremity paresthesia, back pain, urinary retention, headache, and photophobia. The family mentioned he had recurrent episodes of oral ulcers for a year which was not revealed during earlier assessment despite questioning, although there was a language barrier. On exam, there was loss of sensation to bilateral legs with left foot clonus, hyperreflexia bilaterally, and punctate ulcerations to the scrotum. He had an acute altered level of consciousness, apnea, and a paroxysmal event thought to be a seizure treated with lorazepam and required intubation. Cerebral spinal fluid (CSF) showed elevated protein and white blood cell count. CSF, blood, and urine cultures were negative. Magnetic resonance imaging (MRI) of the brain demonstrated abnormal restricted diffusion with associated T2/FLAIR hyperintensity to bilateral hippocampi and left greater than right mesial temporal lobes with patchy enhancement on post-gadolinium images (Figure).[1,3] There was no leptomeningeal or pachymeningeal enhancement. Spine MRI demonstrated patchy long segment T2 signal abnormality of the central spinal cord (greater than 2/3 of the area from C7-L5 and from L5-T10) with minimal cord expansion, and patchy enhancement at T6. Findings raised the possibility of atypical non-herpetic viral encephalitis versus transverse myelitis, the images did not have a typical appearance for Behcet’s disease. Given the clinical features, radiographic findings, and HLA-B51 positivity, he was diagnosed with neurological Behcet’s (NB). Given the seizure, he was started on Levetiracetam for a 6-month course. He had significant neuropathic pain, and was treated with pregabalin and a ketamine infusion, which was eventually discontinued. He received IV methylprednisolone, infliximab, and transitioned to oral steroids. Prophylactic sulfamethoxazole and trimethoprim were continued given the history of toxoplasmosis. He did not require prophylaxis for TB. He was transferred to a rehabilitation hospital for ongoing rehabilitation and subsequently discharged home. Repeat imagining demonstrated resolution of the lesions after treatment. Informed written consent for this case report was obtained. Conclusion Behcet’s can present with neurological features secondary to parenchymal, spinal cord, or brain vasculature involvement. CVST and ICHTN are more common in pediatric populations.[3] This case is unique in that it illustrates that children can present with hippocampal parenchymal and spinal cord involvement. Physicians should consider NB in the differential diagnosis of patients presenting with altered level of consciousness or seizures. [1.] Uluduz D. Neurology 2011;77(21):1900-5. [2.] Borhani-Haghighi A. Multiple Sclerosis and Related Disorders 2020;39:101906. [3.] Saadoun D. Arthritis Rheum 2009;61(4):518-26.

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