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Postpneumococcal Moyamoya syndrome case report and review of the postinfective cases

Federica Pinardi1, Andrea Stracciari, Luca Spinardi

  • 1Neurological Unit, S. Orsola-Malpighi University Hospital, Bologna, Italy. federicapinardi@gmail.com

BMJ Case Reports
|February 9, 2013
PubMed

Insights

Pneumococcal meningitis can lead to delayed Moyamoya syndrome (MMS), a rare cerebrovascular disease. This case highlights meningitis as a potential risk factor for developing MMS.

Area of Science:

  • Neurology
  • Vascular Neurology
  • Infectious Disease

Background:

  • Moyamoya syndrome (MMS) is a rare, progressive cerebrovascular disorder characterized by stenosis of the terminal internal carotid arteries and development of collateral vessels.
  • Postinfective vasculopathy, particularly following bacterial meningitis, is an under-recognized cause of MMS.
  • Pneumococcal meningitis is a significant cause of childhood bacterial meningitis and can lead to serious neurological sequelae.

Observation:

  • A 55-year-old man presented with delayed neurological deficits three months after pneumococcal meningitis.
  • Initial symptoms included headache, otalgia, and paresthesiae, followed by acute visual field defects, hemiplegia, hemineglect, and cognitive/behavioral changes.
  • Brain imaging revealed ischemic lesions in the occipital and frontal lobes, with cerebral angiography confirming Moyamoya vessel alterations.

Findings:

  • The patient's presentation and imaging findings were consistent with a delayed manifestation of postpneumococcal Moyamoya syndrome.
  • Cerebral CT and MRI-angiography demonstrated characteristic Moyamoya vessel changes, indicating stenosis and collateral formation.
  • Treatment involved antiplatelets, corticosteroids, and rehabilitation, with no relapses observed at six months follow-up.

Implications:

  • This case underscores meningitis as a potential risk factor for developing Moyamoya syndrome, even with a delayed presentation.
  • Early recognition and management of postinfective vasculopathy are crucial to prevent severe cerebrovascular events.
  • Further research is warranted to elucidate the mechanisms linking meningitis to Moyamoya syndrome and optimize treatment strategies.

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