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A difficult case
When is ‘idiopathic intracranial hypertension’ no longer idiopathic?
  1. Karen Suetterlin1,
  2. Nicholas Borg2,
  3. Harriet Joy3,
  4. Joanna K Lovett1,
  5. Boyd C P Ghosh1
  1. 1Department of Neurology, Wessex Neurological Centre, Southampton General Hospital, Southampton, Hampshire, UK
  2. 2Department of Neurosurgery, Wessex Neurological Centre, Southampton General Hospital, Southampton, Hampshire, UK
  3. 3Department of Neuroradiology, Wessex Neurological Centre, Southampton General Hospital, Southampton, Hampshire, UK
  1. Correspondence to Dr Karen Suetterlin, Department of Neurology, Wessex Neurological Centre, Southampton General Hospital, Southampton, Hampshire, SO16 6YD, UK; Karen.stevens{at}doctors.org.uk

https://doi.org/10.1136/practneurol-2013-000680

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    History

    A 23-year-old woman with idiopathic intracranial hypertension (IIH) presented with a recurrence of headache and visual disturbance. She had presented with similar symptoms 5 years earlier, when the diagnosis of IIH had been made, after magnetic resonance venography (MRV) had excluded cerebral venous sinus thrombosis (VST) (figure 1A,B). She had been asymptomatic for 18 months and had stopped her acetazolamide 6 months earlier.

    Figure 1

    Magnetic resonance venography (MRV). (A) MR Venogram at time of diagnosis with IIH in 2007: flow signal is seen in the transverse and sigmoid sinuses bilaterally, although they are asymmetrical, the right side being dominant. (B) MR Venogram 2007. Lack of flow signal in the distal transverse sinuses (arrows) is the characteristic appearance seen in IIH. (C) MR Venogram at time of diagnosis with VST in 2012: there is no flow signal in the left transverse and sigmoid sinuses. (D) MR Venogram 2012. The characteristic IIH appearance remains on the right (arrow).

    On examination, her visual acuity was 6/9 (right) and 6/15 (left). Her left visual field was constricted and there was bilateral papilloedema. Her cerebrospinal fluid (CSF) opening pressure was 80 cmH2O and we drained to a closing pressure of 22 cmH2O, resulting in complete resolution of both headache and visual disturbance. She was started on acetazolamide.

    Within 48 h, her headaches returned with a new left-sided lower motor neurone seventh nerve palsy. We organised MR imaging with venography and then repeated her lumbar puncture. The opening pressure was now 29 cmH2O and she reported postprocedure improvement in her headache. However, within 24 h, her headache recurred, she developed a right lower motor neurone seventh nerve palsy (figure 2), bilateral sixth nerve palsies, neck stiffness, vomiting and visual disturbance. Magnetic resonance venography (MRV) showed a VST affecting the left transverse and sigmoid sinuses (figure 1C) and …

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    Footnotes

    • The duties of the senior author were shared between the two authors JKL and BCPG.

    • Contributors The idea for the article came from discussions between Ashwin Pinto, Sean Slaght, Jonathan Frankel, Joanna Lovett and Boyd Ghosh. Literature search was performed by Karen Suetterlin and Nicholas Borg. The article was written by Karen Suetterlin with contribution from Nicholas Borg. Joanna Lovett and Boyd Ghosh edited the article. Harriet Joy provided radiology images and the radiology figure legend. Joanna Lovett was the consultant in charge of the case management with input from Ashwin Pinto. Boyd Ghosh and Joanna Lovett are the guarantors.

    • Competing interests None.

    • Patient consent Obtained.

    • Provenance and Peer Review Not commissioned. Externally peer reviewed. This paper was reviewed by Luke Bennetto, Bristol.

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