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Superficial siderosis in cerebral amyloid angiopathy.

Wani NA, Kosar TL, Rawa AA, Qayum AK - Ann Indian Acad Neurol (2011)

View Article: PubMed Central - PubMed

Affiliation: Department of Radiodiagnosis and Imaging, Sher-I- Kashmir Institute of Medical Sciences, Srinagar, Jammu & Kashmir, India.

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Intracerebral hemorrhages in CAA are typically superficial in cortical and subcortical distribution sparing deep gray matter... Bleeding results from weakening of the vessel walls due to deposited amyloid protein in media... According to Boston criteria a probable diagnosis of CAA is made in elderly patients with at least two acute or chronic lobar hemorrhagic lesions without any other definite cause of intracerebral hemorrhage like prior trauma, ischemic stroke, CNS tumor, vascular malformation, or bleeding diathesis... MRI using GRE sequence has increased sensitivity for detecting intracerebral bleed based on the susceptibility effect caused by hemosiderin... Paramagnetic effect by hemosiderin in microhemorrhages causes variation in local magnetic field and local reduction of T2*, which results in signal loss on GRE images... GRE sequence also enhances detection of superficial siderosis, which may be located close to cortical hemorrhages in more advanced CAA... Striking gyriform appearance of superficial siderosis with numerous adjacent corticosubcortical signal voids due to microhemorrhages were more prominent on SWI as compared to conventional T2W images in our case... This identification of superficial siderosis, enhanced by GRE imaging, may further facilitate the non-invasive imaging diagnosis of CAA with MRI... Identification of CAA with evidence of extensive lobar microhemorrhages and superficial siderosis on MRI as in our case, make it extremely necessary to decide about the judicious use of anticoagulation and antiplatelet therapy for preventing ischemic events prevalent in this age group... MRI can be used for monitoring the progress... We conclude that presence of superficial siderosis may be related to severity of CAA... Recognition of superficial siderosis on MRI may provide further support to imaging diagnosis of CAA, which is enhanced by using SWI.

No MeSH data available.


(a) T2 weighted axial MR image showing gyriform hypointense signal intensity foci along the surface of frontal and parietal cerebral convexities of both cerebral hemispheres (downward arrows). Small rounded signal void dots are seen in the cortices (upward arrows) with diffuse hyperintense signal intensity in the white matter (leftward arrows), (b) Axial T2 W fluid attenuated inversion recovery (FLAIR) image through the supraventricular level shows diffuse hyperintense signal intensity in the centrum semiovale bilaterally (upward arrows); small subcortical signal void foci are also seen (right and leftward arrows), (c) Susceptibility weighted (SWI) axial MR image through cerebral convexities above the ventricular level showing prominent gyriform (upward arrows) and innumerable small rounded signal voids (leftward arrow) in bilateral cerebral hemispheres.
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Figure 0001: (a) T2 weighted axial MR image showing gyriform hypointense signal intensity foci along the surface of frontal and parietal cerebral convexities of both cerebral hemispheres (downward arrows). Small rounded signal void dots are seen in the cortices (upward arrows) with diffuse hyperintense signal intensity in the white matter (leftward arrows), (b) Axial T2 W fluid attenuated inversion recovery (FLAIR) image through the supraventricular level shows diffuse hyperintense signal intensity in the centrum semiovale bilaterally (upward arrows); small subcortical signal void foci are also seen (right and leftward arrows), (c) Susceptibility weighted (SWI) axial MR image through cerebral convexities above the ventricular level showing prominent gyriform (upward arrows) and innumerable small rounded signal voids (leftward arrow) in bilateral cerebral hemispheres.

Mentions: A 70-year-old non-alcoholic, non-diabetic, and non-smoker right hand dominant man presented with a 5 year history of dementia with progressive aphasia. On examination, blood pressure was observed to be 140/80 mm Hg; neurological examination was normal whereas cognitively he had impairment of memory and language in the form of non-confluent aphasia. The erythrocyte sedimentation rate, C-reactive protein, lupus anticoagulant, and anticardiolipin antibodies; and prothrombin and partial thromboplastin times were all within normal limits. Magnetic resonance imaging (MRI) examination of brain was performed with a 1.5 tesla MR imager using T1 weighted (T1 W) and T2 W sequences in various planes; T2 W fluid attenuation inversion recovery (T2 W FLAIR) and susceptibility weighted imaging (SWI) sequences were also performed. MR imaging with conventional T1 W and T2 W sequences revealed generalised atrophy of brain. T2 W and FLAIR images showed diffuse and symmetric confluent hyperintense signal intensity in the periventricular white matter of both cerebral hemispheres [Figures 1]. T2 W axial MR images showed innumerable small (up to 1 cm in diameter) subcortical signal void foci in the bilateral cerebral hemispheres. Thin gyriform hypointensities were seen on T2W images in the cerebral convexities of frontal and parietal regions of both hemispheres [Figure 1]. Small rounded subcortical and gyriform convexity hypointense signal intensities on T2W images became more prominent and extensive on SWI suggesting these to be hemosiderin deposits. Basal ganglia, thalami, and internal capsule were all normal. Clinical features and MRI findings were compatible with a diagnosis of probable cerebral amyloid angiopathy (CAA) with leukoencephalopathy and superficial siderosis.


Superficial siderosis in cerebral amyloid angiopathy.

Wani NA, Kosar TL, Rawa AA, Qayum AK - Ann Indian Acad Neurol (2011)

(a) T2 weighted axial MR image showing gyriform hypointense signal intensity foci along the surface of frontal and parietal cerebral convexities of both cerebral hemispheres (downward arrows). Small rounded signal void dots are seen in the cortices (upward arrows) with diffuse hyperintense signal intensity in the white matter (leftward arrows), (b) Axial T2 W fluid attenuated inversion recovery (FLAIR) image through the supraventricular level shows diffuse hyperintense signal intensity in the centrum semiovale bilaterally (upward arrows); small subcortical signal void foci are also seen (right and leftward arrows), (c) Susceptibility weighted (SWI) axial MR image through cerebral convexities above the ventricular level showing prominent gyriform (upward arrows) and innumerable small rounded signal voids (leftward arrow) in bilateral cerebral hemispheres.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3108084&req=5

Figure 0001: (a) T2 weighted axial MR image showing gyriform hypointense signal intensity foci along the surface of frontal and parietal cerebral convexities of both cerebral hemispheres (downward arrows). Small rounded signal void dots are seen in the cortices (upward arrows) with diffuse hyperintense signal intensity in the white matter (leftward arrows), (b) Axial T2 W fluid attenuated inversion recovery (FLAIR) image through the supraventricular level shows diffuse hyperintense signal intensity in the centrum semiovale bilaterally (upward arrows); small subcortical signal void foci are also seen (right and leftward arrows), (c) Susceptibility weighted (SWI) axial MR image through cerebral convexities above the ventricular level showing prominent gyriform (upward arrows) and innumerable small rounded signal voids (leftward arrow) in bilateral cerebral hemispheres.
Mentions: A 70-year-old non-alcoholic, non-diabetic, and non-smoker right hand dominant man presented with a 5 year history of dementia with progressive aphasia. On examination, blood pressure was observed to be 140/80 mm Hg; neurological examination was normal whereas cognitively he had impairment of memory and language in the form of non-confluent aphasia. The erythrocyte sedimentation rate, C-reactive protein, lupus anticoagulant, and anticardiolipin antibodies; and prothrombin and partial thromboplastin times were all within normal limits. Magnetic resonance imaging (MRI) examination of brain was performed with a 1.5 tesla MR imager using T1 weighted (T1 W) and T2 W sequences in various planes; T2 W fluid attenuation inversion recovery (T2 W FLAIR) and susceptibility weighted imaging (SWI) sequences were also performed. MR imaging with conventional T1 W and T2 W sequences revealed generalised atrophy of brain. T2 W and FLAIR images showed diffuse and symmetric confluent hyperintense signal intensity in the periventricular white matter of both cerebral hemispheres [Figures 1]. T2 W axial MR images showed innumerable small (up to 1 cm in diameter) subcortical signal void foci in the bilateral cerebral hemispheres. Thin gyriform hypointensities were seen on T2W images in the cerebral convexities of frontal and parietal regions of both hemispheres [Figure 1]. Small rounded subcortical and gyriform convexity hypointense signal intensities on T2W images became more prominent and extensive on SWI suggesting these to be hemosiderin deposits. Basal ganglia, thalami, and internal capsule were all normal. Clinical features and MRI findings were compatible with a diagnosis of probable cerebral amyloid angiopathy (CAA) with leukoencephalopathy and superficial siderosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Radiodiagnosis and Imaging, Sher-I- Kashmir Institute of Medical Sciences, Srinagar, Jammu & Kashmir, India.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Intracerebral hemorrhages in CAA are typically superficial in cortical and subcortical distribution sparing deep gray matter... Bleeding results from weakening of the vessel walls due to deposited amyloid protein in media... According to Boston criteria a probable diagnosis of CAA is made in elderly patients with at least two acute or chronic lobar hemorrhagic lesions without any other definite cause of intracerebral hemorrhage like prior trauma, ischemic stroke, CNS tumor, vascular malformation, or bleeding diathesis... MRI using GRE sequence has increased sensitivity for detecting intracerebral bleed based on the susceptibility effect caused by hemosiderin... Paramagnetic effect by hemosiderin in microhemorrhages causes variation in local magnetic field and local reduction of T2*, which results in signal loss on GRE images... GRE sequence also enhances detection of superficial siderosis, which may be located close to cortical hemorrhages in more advanced CAA... Striking gyriform appearance of superficial siderosis with numerous adjacent corticosubcortical signal voids due to microhemorrhages were more prominent on SWI as compared to conventional T2W images in our case... This identification of superficial siderosis, enhanced by GRE imaging, may further facilitate the non-invasive imaging diagnosis of CAA with MRI... Identification of CAA with evidence of extensive lobar microhemorrhages and superficial siderosis on MRI as in our case, make it extremely necessary to decide about the judicious use of anticoagulation and antiplatelet therapy for preventing ischemic events prevalent in this age group... MRI can be used for monitoring the progress... We conclude that presence of superficial siderosis may be related to severity of CAA... Recognition of superficial siderosis on MRI may provide further support to imaging diagnosis of CAA, which is enhanced by using SWI.

No MeSH data available.