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Idiopathic intracranial hypertension as the initial presentation of systemic lupus erythematosus

Chandrasekharan Rajasekharan1, Sanu Watson Renjith, Ali Marzook

  • 1Department of Internal Medicine, Medical College Hospital, Thiruvananthapuram, Kerala, India. drcrajasekharan@yahoo.com

BMJ Case Reports
|February 5, 2013
PubMed

Insights

Idiopathic intracranial hypertension (IIH) can be a rare initial presentation of systemic lupus erythematosus (SLE). Prompt diagnosis and corticosteroid treatment led to rapid symptom recovery in a young patient.

Area of Science:

  • Neurology
  • Rheumatology
  • Pediatrics

Background:

  • Idiopathic intracranial hypertension (IIH) is characterized by elevated intracranial pressure without a clear cause.
  • Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with diverse clinical manifestations.

Observation:

  • A 14-year-old female presented with headache, visual disturbances, fever, and skin lesions.
  • Neurological examination revealed papilledema and extensor plantar responses, indicative of raised intracranial pressure.
  • Cerebrospinal fluid analysis showed high opening pressure, while neuroimaging confirmed increased intracranial tension.

Findings:

  • Initial diagnosis of IIH was made, but symptoms persisted despite treatment.
  • High titers of antinuclear antibodies (ANA) and anti-double-stranded DNA (dsDNA) antibodies confirmed SLE.
  • The patient was diagnosed with SLE presenting as IIH.

Implications:

  • This case highlights the importance of considering autoimmune conditions like SLE in the differential diagnosis of IIH, especially in young patients with atypical symptoms.
  • Early recognition and management of SLE with corticosteroids resulted in a dramatic clinical improvement.
  • The rarity of IIH as the primary manifestation of SLE underscores the need for comprehensive diagnostic approaches.

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