Abstract Background/Aims A 23-year-old female presented with several months of profound fatigue, progressive visual disturbance, headaches severe enough to require opioid analgesia, and tinnitus. Clinical examination revealed bilateral papilloedema with striking optic disc swelling, raised intracranial pressure (ICP), a petechial rash affecting the arms and legs, and widespread lymphadenopathy. She also reported recurrent oral ulceration and an atypical vulval lesion. The constellation of features prompted urgent multidisciplinary assessment. Methods Serial lumbar punctures confirmed persistently raised ICP, cerebrospinal fluid (CSF) lymphocytosis and rising protein, with negative xanthochromia. Neuroimaging excluded cerebral venous sinus thrombosis or mass lesion. She was treated initially as likely idiopathic intracranial hypertension with Acetazolamide and steroid. Laboratory investigations showed chronically raised erythrocyte sedimentation rate (ESR), elevated IgG, and negative connective tissue disease screening. Computed tomography (CT) imaging of neck, chest, abdomen and pelvis revealed extensive lymphadenopathy. The differential diagnoses considered included systemic autoinflammatory disease, low-grade lymphoproliferative disorder, and sarcoidosis. Rheumatology input was sought to evaluate for connective tissue disease or autoinflammatory syndromes such as Behçet’s disease, although these were felt unlikely (absence of typical genital ulceration / uveitis / skin changes / negative pathergy). Extended immunological and infectious disease work-up was therefore recommended. Results Treponema pallidum infection was confirmed with positive serum syphilis IgM, strongly positive Treponema pallidum haemagglutination assay (TPHA) and rapid plasma reagin (RPR) titre. CSF was also RPR and TPHA positive, consistent with neurosyphilis. The vulval lesion was considered a chancre. The patient was treated under the advice of the genitourinary medicine team with IV Benzylpenicillin and IV Methylprednisolone followed by a reducing course of oral Prednisolone, to treat the raised ICP and optic disc swelling. The patient’s vision and headache slowly improved with treatment. Conclusion The case highlights the importance of sending a broad infection screen when referred a case with unusual features, not typically fitting with a primary rheumatological disorder. The number of syphilis cases being diagnosed in the UK continues to rise - reasons for this may include changes in sexual practices and lack of awareness of the infection. Rheumatological manifestations of syphilis infection can include inflammatory arthritis, myositis and vasculitis. In addition, as illustrated in our case, it can lead to neurological presentations which have the potential to result in long term morbidity if not diagnosed and treated promptly. Disclosure M.J. Dolaghan: None. C. Donaghy: None.
Dolaghan et al. (Wed,) studied this question.