Cerebral amyloid angiopathy and lobar haemorrhage

Case contributed by RMH Neuropathology

Presentation

Acute onset left hemiplegia

Patient Data

Age: 65 years
Gender: Male

There is a large right parietal/temporo/occipital/temporal haematoma measuring approximately 5.4cm x 3.7m with a large amount of surrounding oedema. There is significant mass-effect, characterised by right cortical sulcal effacement, compression of the right lateral and third ventricles, right uncal herniation and 8mm of leftward midline shift. Temporal horn dilatation is consistent with early hydrocephalus.

CTA COW (not shown) 

The extracranial vessels, circle of willis and vertebrobasilar system opacify normally.  No evidence of aneurysm, vascular malformation, dissection or significant stenosis. 

The patient deteriorated clinically and was taken to the operating theatre and the clot removed. No lesion or abnormal vessels were identified. Nonetheless the patient proceeded to the MRI scanner the next day. 

Right parietal craniotomy and evacuation of the right temporoparietal haematoma  with evacuation of much of the haematoma. There is persistent gyral swelling and sulcal effacement throughout the right temporal, parietal and occipital lobes with mild compression of the right lateral ventricle but no midline shift.   Foci of deep and periventricular white matter hyperintensity likely represents chronic small vessel ischaemic change. 

Poorly seen due to motion degradation, are a number of small punctate regions of signal drop out on EPI suggestive of microhaemorrhages. They are peripeheral in distribution. 

No enhancing lesion seen within the region of the haematoma. The overlying dura is slightly thickened and enhancing in keeping with the recent postoperative state. 

MRA (not shown) demonstrates no malformation, aneurysm, stenosis or other vascular abnormality. 

Conclusion: 

Location and presence of microhaemorrhages suggests a lobar haemorrhage secondary to cerebral amyloid angiopathy. 

Histology

Modality: Pathology

MICROSCOPIC DESCRIPTION: The section shows fragments of blood clot consisting predominantly of red blood cells with a small amount of fibrin.  At one edge there are several small calibre blood vessels.  These show thickening and hyalinisation of their walls.  Immunohistochemistry shows strong staining for A-beta protein within vessel walls.  The features are of amyloid angiopathy.  No evidence of tumour is seen.

FINAL DIAGNOSIS: A-beta amyloid angiopathy with haemorrhage.

Case Discussion

Lobar haemorrhages, when not due to an underlying mass or vascular lesion, are usually seen in the elderly in the setting of cerebral amyloid angiopathy (in this case confirmed on histology). 

Cerebral amyloid angiopathy can be divided according to the causative protein, of which there are many:

  • sporadic
    • A-beta (APP) - ApoE e4 e2
  • familial
    • A-beta (APP)
      • Dutch
      • Italian
      • Flemish
      • Swedish
    • cystatin C (ACys) - Icelandic  (20-30 years)
    • transthyretin (ATTR): meningovascular amyloidosis
    • gelsolin (AGel): finnish
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Case Information

rID: 24877
Case created: 17th Sep 2013
Last edited: 1st Mar 2016
Inclusion in quiz mode: Included

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