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Longitudinally extensive transverse myelitis: a rare association with common variable immunodeficiency
  1. Edwin Jabbari1,
  2. Charles Robert Marshall2,
  3. Hilary Longhurst3,
  4. Richard Sylvester4
  1. 1Department of Neurology, Homerton University Hospital NHS Trust, London, UK
  2. 2Department of Neurology, Barts and The London Hospital NHS Trust, London, UK
  3. 3Department of Immunology, Barts and The London NHS Trust, London, UK
  4. 4Homerton University Hospital NHS Trust, National Hospital of Neurology and Neurosurgery, UCH, London, UK
  1. Correspondence to Dr Richard Sylvester, Homerton University Hospital NHS Trust, National Hospital of Neurology and Neurosurgery, UCH, London, UK; richard.sylvester{at}nhs.net

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Introduction

Transverse myelitis requires careful investigation, as although many causes respond acutely to immunomodulation, the longer term management depends upon its precise cause. We describe a patient presenting acutely with a corticosteroid-responsive longitudinally extensive transverse myelitis and granulomatous lung lesions with a previous history of recurrent generalised lymphadenopathy, pyogenic infections and idiopathic thrombocytopenic purpura. We initially suspected an underlying primary autoimmune disorder. However, investigations showed granulomatous common variable immunodeficiency (gCVID) and following regular intravenous immunoglobulin, her symptoms did not recur. We discuss this relatively common immunodeficiency disease, frequently misdiagnosed as a systemic autoimmune disease (often sarcoidosis). We also discuss the range of neurological syndromes, including transverse myelitis, that may accompany common variable immunodeficiency (CVID).

Case report

A 41-year-old Caucasian woman presented with a 1-week history of progressive bilateral leg weakness, with urinary hesitancy and frequency. She had an intriguing past medical history of multiple episodes of corticosteroid-responsive widespread tender lymphadenopathy over 10 years, splenectomy for refractory idiopathic thrombocytopenic purpura in 2005, frequent respiratory tract infections and multiple buttock abscesses.

On examination at presentation, there was increased tone in both lower limbs with mild bilateral proximal weakness. Her reflexes were pathologically brisk in both legs and plantars were extensor. There were no sensory abnormalities. Upper limb and cranial nerve examinations were normal.

Routine blood tests were normal, including full blood count, renal function, liver function, clotting function and inflammatory markers. Her MR scan of brain was normal, but an MR scan of spine showed longitudinally extensive cord signal change between T2 and the conus medullaris (figure 1) with focal contrast enhancement at T6/7 (figure …

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