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A 27-year-old woman woke up with right hemiparesis and dysphasia (NIHSS 13). Normal CT scan.

Fig. 30.1
figure 1

DWI

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Fig. 30.2
figure 2

DSA

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Fig. 30.3
figure 3

CT

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FormalPara Questions
  1. 1.

    What are the findings on these images?

  2. 2.

    What are the possible aetiologies?

  3. 3.

    Why is the treatment urgent?

FormalPara Diagnosis

Acute ischaemic stroke in patient with hereditary haemorrhagic telangiectasia (HHT) and a pulmonary AVM

FormalPara Answers
  1. 1.

    MR ◘ Fig. 30.1 – left posterior lenticular restricted diffusion; DSA ◘ Fig. 30.2 – MCA occlusion by thrombus with collateral circulation via pial anastomoses from the ACA and anterior temporal branch of the MCA, two small bilateral temporal AVMs; thoracic CT ◘ Fig. 30.3 – pulmonary arteriovenous malformation.

  2. 2.

    Carotid dissection; carotid bulb atherosclerotic plaque (unlikely at this age); cardiac origin of the thrombus (patent foramen ovale, etc.).

  3. 3.

    Mechanical thrombectomy performed in an early phase will allow reperfusion of the ischaemic penumbra territory, reverting the neurological deficits; if the patient is treated in a late phase, the recanalisation will be futile, with no improvement of the neurological deficits and with an added risk of haemorrhagic transformation of the infarct.

Post-thrombectomy Images (◘ Figs. 30.4 and 30.5)

Fig. 30.4
figure 4

DSA, recanalised MCA

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Fig. 30.5
figure 5

CT, posterior lenticular infarct

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1 Comments

Hereditary haemorrhagic telangiectasia, also known as HHT or Osler-Weber-Rendu syndrome , is an autosomal dominant disorder with mucocutaneous telangiectasias and AVMs in visceral organs (primarily lungs, brain and liver) [1]. The most common clinical presentation is recurrent epistaxis from nasal mucosal telangiectasias. There is a significant lifetime risk of a brain abscess or stroke if a pulmonary AVM is present, as occurred with this patient. Pulmonary AVMs can be treated by embolisation or surgery with excellent results [2]. Most cerebral AVMs in HHT are low grade (Spetzler-Martin 1 or 2) and have a lower bleeding risk than sporadic AVMs . They can be treated with embolisation or radiosurgery, depending on the size and location of the AVM.