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CASE REPORT
Spontaneous intracranial hypotension associated with pachymeningeal enhancement in a patient with systemic lupus erythematosus (SLE): an extremely rare presenting feature
  1. Shrivarthan Radhakrishnan1,
  2. Deepanjali Surendran2,
  3. Deepak Barathi3 and
  4. Chanaveerappa Bammigatti4
  1. 1 Medicine, Jawaharlal Institute of Postgraduate Medical Education & Research (JIPMER), Puducherry, Pondicherry, India
  2. 2 Medicine, JIPMER, Puducherry, India
  3. 3 Radio-diagnosis, Jawaharlal Institute of Medical Education & Research, Pondicherry, India
  4. 4 Medicine, Jawaharlal Institute of Post Graduate Medical Education & Research (JIPMER), Puducherry, Pondicherry, India
  1. Correspondence to Dr Chanaveerappa Bammigatti, bammigatti{at}yahoo.com

Abstract

A 16-years-old girl presented to our institution with history of severe bilateral headache for 5 days associated with vomiting. She also had fever for 2 months without any localising symptoms and skin lesions for 1 month. Examination revealed erythematous rash over bridge of nose and ear lobes, ulcer over hard palate and tenderness of small joints of both hands. Systemic examination was unremarkable except for bilateral papilloedema. Investigations revealed anaemia, leucopenia and elevated erythrocyte sedimentation rate. Cranial imaging revealed diffuse pachymeningeal enhancement with subdural effusion. Lumbar puncture revealed no abnormal findings in cerebrospinal fluid except low opening pressure. Antinuclear antibody was 4+ with anti-Sm antibody positive. She was diagnosed to have spontaneous intracranial hypotension associated with pachymeningeal enhancement secondary to systemic lupus erythematosus. She showed dramatic improvement with steroid and azathioprine therapy. She continues to be asymptomatic after 2 years of follow-up.

  • headache (including migraines)
  • systemic lupus erythematosus
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Footnotes

  • Patient consent for publication Obtained.

  • Contributors SR has managed the patient in the ward and prepared the initial draft of the manuscript. DS has helped in the management of the patient in the ward and manuscript preparation. DB is responsible for radiological investigation of this patient and also has contributed for the manuscript preparation. CB is responsible for overall management of the patient including follow-up, manuscript preparation and submission.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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