Abstract

We report a 52-year old female who initially presented with episodes of vertigo, headache and diplopia. Vestibular migraine or Meniere's disease was diagnosed. Two-years later she reported sensory symptoms in her upper limbs. Normal investigations led to a functional diagnosis being considered. However she worsened with an acute-onset sensorineural hearing loss and diplopia. Examination revealed evidence of brainstem signs and ataxia. MRI showed an enhancing right dorsal pontine lesion, CSF was normal. She was diagnosed with primary CNS angiitis and managed with steroids and mycophenolate. Improvement was noted but on tapering steroid use symptoms worsened and new left dorsal pontine and medullary enhancing lesions seen on MRI. Serology was negative for aquaporin-4-antibodies and anti-MOG antibodies and CSF negative for oligoclonal bands. Chronic Lymphocytic Pontine Perivascular Enhancement Responsiveness to Steroids (CLIPPERS) is tabled as the unifying diagnosis. Symptoms and MRI appearances are characteristic as is steroid responsiveness with relapse on dose tapering. Diagnosis is challenging with the expanding phenotype of immune mediated CNS disorders. Accuracy is vital given the steroid responsiveness however management remains challenging with rituximab now being used to reduce corticosteroid dose. Reports suggest that timely intervention leads to better outcomes making the wider recognition of this syndrome important [1] Pittock SJ, et al. 2010 [2] Dudesek A, et al. 2014.