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June 16, 2015; 84 (24) Video NeuroImages

Cerebral proliferative angiopathy

Rare case of seizures, bone erosion, and pulsatile head bump

Geeta Anjum Khwaja, Vikram Bohra, Ashish Duggal, Abhilekh Srivastava, Neera Chaudhry
First published June 15, 2015, DOI: https://doi.org/10.1212/WNL.0000000000001686
Geeta Anjum Khwaja
From GB Pant Hospital, New Delhi, India.
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Vikram Bohra
From GB Pant Hospital, New Delhi, India.
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Ashish Duggal
From GB Pant Hospital, New Delhi, India.
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Abhilekh Srivastava
From GB Pant Hospital, New Delhi, India.
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Neera Chaudhry
From GB Pant Hospital, New Delhi, India.
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Citation
Cerebral proliferative angiopathy
Rare case of seizures, bone erosion, and pulsatile head bump
Geeta Anjum Khwaja, Vikram Bohra, Ashish Duggal, Abhilekh Srivastava, Neera Chaudhry
Neurology Jun 2015, 84 (24) 2462-2463; DOI: 10.1212/WNL.0000000000001686

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A 22-year-old man presented with a 5-year history of focal seizures and a pulsatile bump on the forehead (figure, A; video on the Neurology® Web site at Neurology.org). He had subtle right hemiparesis with facial palsy, Mini-Mental State Examination score of 22/30, and audible bruit on forehead swelling, suggesting cerebral proliferative angiopathy (CPA). This condition differs from arteriovenous malformation in in that hemorrhages are uncommon; it has a typically large size, with normal brain parenchyma between vascular spaces (figure, B).1 There are multiple arterial feeders (figure, C) with prominent draining veins (figure, D).1,2 Embolization or surgical resections are not usually possible with a CPA diagnosis.1

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Figure MRI and CT angiogram of cerebral proliferative angiopathy with pulsatile forehead swelling

(A) Pulsatile swelling on the forehead. (B) T2-weighted axial image shows multiple flow voids in the left frontal and parietal area with intervening normal brain tissue and pressure on ipsilateral lateral ventricle. (C) CT angiogram shows extensive abnormal vascular network with multiple feeders including anterior and middle cerebral arteries. (D) Contrast MRI shows prominent sinuses and draining veins communicating with the forehead swelling.

Footnotes

  • Supplemental data at Neurology.org

  • Author contributions: Dr. Geeta Anjum Khwaja: drafting the manuscript, study concept, supervision and coordination. Dr. Vikram Bohra: drafting the manuscript, study design, literature search. Dr. Ashish Duggal: interpretation of data, acquisition of data. Dr. Abhilekh Srivastava: interpretation of data, acquisition of data. Dr. Neera Chaudhry: interpretation of data, study supervision.

  • Study funding: No targeted funding reported.

  • Disclosure: The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.

  • © 2015 American Academy of Neurology

References

  1. 1.↵
    1. Lasajaunias PL,
    2. Landrieu P,
    3. Rodesch G,
    4. et al
    . Cerebral proliferative angiopathy: clinical and angiographic description of an entity different from cerebral AVMs. Stroke 2008;39:878–885.
    OpenUrlAbstract/FREE Full Text
  2. 2.↵
    1. Doria-Netto HL,
    2. deSouza-Filho AM,
    3. Doria-Netto RH,
    4. et al
    . Cerebral proliferative angiopathy. Arq Neuropsiquiatr 2010;68:300–302.
    OpenUrlPubMed
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