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Background <p>Moyamoya Disease (MMD) is a rare, progressive cerebrovascular disorder characterised by stenosis of the terminal internal carotid arteries and development of fragile collaterals, “moyamoya” vessels, predisposing to ischaemic and haemorrhagic stroke. Haemorrhagic manifestations are more frequent in Asian populations, and the impact of concomitant vertebrobasilar (VB) involvement is insufficiently established. This case highlights the complex clinical presentation of MMD with involvement of both the anterior and posterior circulations, particularly the VB system, which is rare in MMD.</p> Case Presentation <p>A 31-year-old woman with childhood-onset seizures and no other comorbidities presented with a seven-day history of headache, altered sensorium and generalised tonic-clonic seizures accompanied by abnormal limb movements and facial deviation. Non-Contrast Computed Tomography (NCCT) of the head revealed pan-ventricular haemorrhage with secondary hydrocephalus and a chronic infarct in the left frontal lobe. Computed Tomographic Angiography (CTA) and Magnetic Resonance Angiography (MRA) demonstrated multifocal narrowing of intracranial arteries involving both anterior and posterior circulations, with marked narrowing of the supraclinoid internal carotid, middle cerebral, vertebral, and basilar arteries and extensive collateralisation, consistent with bilateral MMD (Suzuki stage 3). Brain MRI showed an additional acute infarct in the splenium of the corpus callosum. The patient was managed conservatively with antiepileptics, blood pressure optimisation, and supportive care, without the placement of an external ventricular drain. Serial NCCT scans demonstrated progressive resolution of intraventricular haemorrhage (IVH) and hydrocephalus, with complete clearance by day 20; she was discharged neurologically stable and seizure‑free and remained seizure‑free at 1-month follow‑up, with planned surgical revascularisation.</p> Conclusion <p>This case illustrates the coexistence of haemorrhagic and ischaemic manifestations in bilateral MMD with extensive VB involvement, presenting as pan‑ventricular IVH and acute splenial infarction. It underscores the need for comprehensive evaluation of both the anterior and posterior circulations in MMD, careful control of blood pressure and seizures, and early consideration of revascularisation to optimise long‑term stroke prevention.</p>

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Pan‑ventricular Haemorrhage and Acute Splenial Infarct in Bilateral Moyamoya Disease with Vertebrobasilar Involvement: A Case Report and Brief Literature Review

  • 期刊论文

  • 发表日期：

    2026年6月24日

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  • Rahul Dev,
  • Rajnish Kumar Arora,
  • Bheru Dan Charan

摘要

Background

Moyamoya Disease (MMD) is a rare, progressive cerebrovascular disorder characterised by stenosis of the terminal internal carotid arteries and development of fragile collaterals, “moyamoya” vessels, predisposing to ischaemic and haemorrhagic stroke. Haemorrhagic manifestations are more frequent in Asian populations, and the impact of concomitant vertebrobasilar (VB) involvement is insufficiently established. This case highlights the complex clinical presentation of MMD with involvement of both the anterior and posterior circulations, particularly the VB system, which is rare in MMD.

Case Presentation

A 31-year-old woman with childhood-onset seizures and no other comorbidities presented with a seven-day history of headache, altered sensorium and generalised tonic-clonic seizures accompanied by abnormal limb movements and facial deviation. Non-Contrast Computed Tomography (NCCT) of the head revealed pan-ventricular haemorrhage with secondary hydrocephalus and a chronic infarct in the left frontal lobe. Computed Tomographic Angiography (CTA) and Magnetic Resonance Angiography (MRA) demonstrated multifocal narrowing of intracranial arteries involving both anterior and posterior circulations, with marked narrowing of the supraclinoid internal carotid, middle cerebral, vertebral, and basilar arteries and extensive collateralisation, consistent with bilateral MMD (Suzuki stage 3). Brain MRI showed an additional acute infarct in the splenium of the corpus callosum. The patient was managed conservatively with antiepileptics, blood pressure optimisation, and supportive care, without the placement of an external ventricular drain. Serial NCCT scans demonstrated progressive resolution of intraventricular haemorrhage (IVH) and hydrocephalus, with complete clearance by day 20; she was discharged neurologically stable and seizure‑free and remained seizure‑free at 1-month follow‑up, with planned surgical revascularisation.

Conclusion

This case illustrates the coexistence of haemorrhagic and ischaemic manifestations in bilateral MMD with extensive VB involvement, presenting as pan‑ventricular IVH and acute splenial infarction. It underscores the need for comprehensive evaluation of both the anterior and posterior circulations in MMD, careful control of blood pressure and seizures, and early consideration of revascularisation to optimise long‑term stroke prevention.