Superficial Siderosis MRI Brain

A known case of Sellar Supra sellar mass with right para sellar component causing encasement of right ICA on MRI. Here are his Axial T2*GRE images. 

Axial T2 *GRE images of brain reveals low signal intensity hemosiderin staining along tentorium, sylvian fissures and hemispheric cortical sulci suggestive of Superficial siderosis, not at all obvious on any other parenchymal sequences implies to highest sensitivity of GRE to blood degradation product.

Superficial Siderosis

A rare condition characterised by abnormal hemosiderin staining of sub arachonid space, may be diffuse or focal, commonly overlying cerebral and cerebellar convexity, basal cisterns, ventral surface of brain stem on T2*GRE, results from excessive and repetitive subarachonid bleed.
An associated staining along cranial nerves particularly i, ii and viii CNs.
May see an associated atrophy of cerebellar hemispheres and vermis, lepto meningeal thickening with enhancement.
CT usually normal may show faint hyperdense layering.
Differential diagnosis is none, it has a pathognomonic appearace on T2*GRE.

Superficial siderosis is not a final diagnosis but an important finding indicating a remote or recurrent intra cranial bleed in subarachnoid space. Further imaging evaluation should be directed towards source of bleeding like MR Angiography to rule out aneurysm or any other vascular malformation.
The issue is cause of bleed. In ~25% cases cause in not found.

Clinically common symptoms are ataxia, hearing loss, anosmia, dementia; in long standing cases adjacent brain parenchymal atrophy ensues with altered cognition.
Treatment directed towards finding and removing cause of bleeding. Iron chelating agents.

Reference: Teaching atlas of brain imaging: By Nancy J. Fischbein, William P. Dillon, A. James Barkovich : Dural and lepto meningeal processes, Case 65, page  231.

To see other cases of Superficial Siderosis :
Case 1: Click here
Case 2: Click here

Chronic Hypertensive Encephalopathy MRI

A 45 yo male with known long standing hypertension.

Now admitted with recent right sided weakness with altered cognition.

This MRI study of Brain shows:
Confluent bilateral fronto parietal peri ventricular white matter T2 hyper intensity.
Foci of intra parenchymal bleed in bilateral basal ganglia, micro bleeds in thalami and Pons on GRE.

Imaging diagnosis : Chronic Hypertensive Encephalopathy.


CHRONIC HYPERTENSIVE ENCEPHALOPATHY

Brain parenchymal changes due to long-standing effects of untreated or poorly treated systemic hypertension (HTN)

CHE causes vascular dementia.
CHE can be associated with Subcortical arterial and arteriolar leukoencephalopathy, Leukoaraiosis, Binswanger disease.

Imaging wise characterized by
1. Chronic lacunes and lacunar infarcts.
2. Parenchymal hemorrhages and microbleeds.
3. Diffuse white matter lesions.

Chronic lacunes and lacunar infarcts are due to small vessel occlusion and microbleeds. Sites of lacunae in order of decreasing frequency is lenticular nuclei, pons, thalamus, internal capsule and caudate nuclei.

Parenchymal hemorrhages and microbleeds as multiple hypointense foci on T2*GRE images.
T2* GRE has superior sensitivity in detecting old Hemorrhages.
Parenchymal hemorrhages are common especially in basal ganglia, thalami and Pons.

Diffuse white matter (WM) lesions are marked in fronto parietal peri ventricular white matter as confluent ill defined hypodensity on CT / hyper intensity on MRI T2 and FLAIR.

DW images may show recent lacunar foci with restricted diffusion.

DDs
Amyloid angiopathy
• Second most frequent cause of cerebral hemorrhage (next to atheromatosis), especially recurrent.
• Hemorrhages are most common in frontal and parietal lobes followed by deep central gray nuclei, corpus callosum, cerebellum and brainstem.
• Amyloid deposition within small and medium arteries of cerebral leptomeninges and cerebral cortex.
• Amyloidosis may also cause
Transient ischemic attacks (TIA), cerebral infarcts.
Binswanger type leukoencephalopathy.
Symptoms resembling cerebral pseudotumor.

CADASlL
• Nonarteriosclerotic, amyloid-negative hereditary angiopathy primarily affecting leptomeningeal and long perforating arteries of brain.
• Characteristic subcortical lacunar infarcts and leukoencephalopathy in young adults.
• Lesions found predominantly within centrum semiovale, thalamus, BG, and pons.
• Anterior temporal pole and external capsule lesions have high sensitivity and specificity for CADASIL.

Dementias
• Alzheimer dementia
Parietal and temporal cortical atrophy
Volume loss in hippocampi, entorhinal cortex
Often co-existing microvascular disease, WM hyperintensities
• Multi-infarct dementia
o Hyperintense lesions on T2WI and focal atrophy suggestive of chronic infarcts.

Pseudoxanthoma elasticum
• Subcortical leukoencephalopathy
• Dementia, multiple strokes with hypertension

Antiphospholipid antibody syndrome
• Early stroke, recurrent arterial and venous thromboses
• Spontaneous fetal loss, thrombocytopenia
• Infarcts of various sizes and T2 hyperintense WM foci.

Neuropsychiatric systemic lupus
erythematosus (NPSLE)
• Most common: Small multifocal WM lesions
• Cortical atrophy, ventricular dilation
• Periventricular and diffuse WM changes
• Infarcts, hemorrhage, multifocal gray matter lesions.

Other vasculitides
• Primary angiitis of CNS, granulomatous angiitis
• Polyarteritis nodosa, Behcet disease
• Syphilis, Sjogren syndrome

Pathology 
- Chronic HTN  irreversible structural changes in small arterial vessels of cerebral parenchyma leading to WM changes and lacunar infarctions.
- Elevated blood pressure (BP)  hyalinosis and sclerosis in walls of small intraparenchymal arterioles predisposition to thrombotic occlusion.

Gross Pathologic and microscopic features
- Demyelination of periventricular and central WM.
- Multiple lacunae and infarctions.
- Parenchymal hemorrhage.

Clinical Presentation
- Most common signs/symptoms : Memory loss (various features of dementia), Motor disorders, pseudobulbar syndrome.
- Stepwise or gradual progression of mental deterioration, acute strokes, lacunar syndrome, subacute onset of focal, pseudobulbar and extrapyramidal signs and seizures.

Age and Gender 
- Incidence increases with age.
- HTN more prevalent in men than women.

Treatment
- Long-term control of Blood pressure.

Superficial Siderosis MRI T2*GRE Brain

Axial T2 *GRE images of brain reveals low signal intensity hemosiderin staining on surface of parietal lobes marked on right side suggestive of Superficial siderosis, not at all obvious on any other sequence. Axial FLAIR images in corresponding region normal. 

Superficial Siderosis

A rare condition characterised by abnormal hemosiderin staining of sub arachonid space, may be diffuse or focal, commonly overlying cerebral and cerebellar convexity, basal cisterns, ventral surface of brain stem on T2*GRE, results from excessive and repetitive subarachonid bleed.
An associated staining along cranial nerves particularly i, ii and viii CNs.
May see an associated atrophy of cerebellar hemispheres and vermis, lepto meningeal thickening with enhancement.
CT usually normal may show faint hyperdense layering.
Differential diagnosis is none, it has a pathognomonic appearace on T2*GRE.

Superficial siderosis is not a final diagnosis but an important finding indicating a remote or recurrent intra cranial bleed in subarachnoid space. Further imaging evaluation should be directed towards source of bleeding like MR Angiography to rule out aneurysm or any other vascular malformation.
The issue is cause of bleed. In ~25% cases cause in not found.

Clinically common symptoms are ataxia, hearing loss, anosmia, dementia; in long standing cases adjacent brain parenchymal atrophy ensues with altered cognition.
Treatment directed towards finding and removing cause of bleeding. Iron chelating agents.

Reference: Teaching atlas of brain imaging: By Nancy J. Fischbein, William P. Dillon, A. James Barkovich : Dural and lepto meningeal processes, Case 65, page  231.

To see another case of Superficial siderosis :
Case 1 : Click here
Case 2 : Click here

Vertebral artery thrombosis

On MR Angio, non visualization of a vessel or a part of vessel needs careful interpretation as it can be due to many reasons right from thrombotic occlusion of vessel, absent vessel as a part of normal anatomical variation or a technical error while doing post processing of raw images of 3D TOF sequences by using add vessel technique.
So diagnosis of vessel thrombosis or passing it off as a normal anatomical vessel should not be solely based on MR Angiography findings.
The diagnostic dilemma occur more so when there is no infarct in corresponding vascular territory.
This problem is most common with vertebral where one of the vertebral is not visualised. Opposite vertebral continues as basilar. In such cases we can take help of other routine parenchymal sequences like FLAIR and T2*GRE.

In above case, thrombosed distal most portion of right intra cranial vertebral near formation of basilar show a focal flow loss on MR Angio, the corresponding portion show an abnormal low signal on T2*GRE and high signal on FLAIR implies to thrombus. 

Role of T2*GRE

T2* pronounced "T 2 star"
GRE stands for GRadient Echo sequence.


Due to its clinical implications this has became a non  omissible sequence in our stroke protocol.
Paramagnetic effect of substances like deoxyhemoglobin, methemoglobin and hemosiderin give rise to T2* based contrast, which is used to depict hemorrhage, calcification and iron deposition in various tissues and lesions.
I won't go in much technical details of the sequence like flip angle, TR TE and all.

Clinical applications of T2*GRE sequence:

Vessel thrombosis:

On left side serpigenous or cord like low signal intensity in the region of right parietal cortical sulci going towards superior sagittal sinus are the para sagittal cortical veins which are thrombosed depicted only on this GRE sequence where ax FLAIR study of this patient was showing only a focal vasogenic odema in right parietal sub cortical white matter. MR Venogram particularly the superior sagittal sinus was normal which is expected as it depicts only the dural venous sinuses.


On right side thrombosed right vertebral show abnormal high signal on FLAIR,  low signal on T2*GRE due to thrombus in it. Corresponding portion of right vertebral show a focal flow loss on MR Angio.




Infarct with hemorrhage : Arterial infarcts with hemorrhagic transformation or hemorrhagic venous infarcts will show low signal intensity areas of bleed in the region of infarct.

On right side ax FLAIR image show a sub acute right MCA inferior division terriory infarct. Hemorrhagic transformation seen as an area of low signal intensity on T2*GRE.




Axonal shearing injury and petechial bleed.

A case of head injury with poor Glass glow coma scale. On admission CT brain normal. MRI Ax FLAIR study show focal T2 hyperintensity in the region of corpus callosum with low signal intensity hemosiderin staining on GRE. Multiple punctate low signal intenisity foci in left frontal sub cortical white matter attributed petechial bleeds as a part of axonal shearing injury.

Microbleeds seen as multiple punctate low signal intensity foci in bilateral cerebral cortical white matter. Platelet aggregation inhibitor drugs to be used cautiously in this patients.









Vascular malformation, high flow vs slow flow: 
On left side T2 w image show multiple serpiginous flow voids clustered in right temporo parietal lobe suggestive of a vascular malformation. High signal on T2*GRE supports a high flow vascular malformation like AVM.

Bleed in a lesion : 
This is a case of multiple parenchymal metastasis with fluid fluid levels. In that the dependent portion show low signal on GRE attributed to a sedimentation of blood degradation product supports hemorrhagic metastasis.



Calcification in a lesion: 
Evaluation of calcification in a neoplastic lesion like meningioma, craniopharyngioma or an infective lesion like Granuloma.

Gliosis secondary to a chronic resolved hematoma vs infarct:

In this case a focal Gliotic cavity seen in left corona radiata can be a chronic resolved hematoma or chronic infarct. Low signal intensity hemosiderin staining along the lesion on GRE goes in favor of a chronic resolved hematoma.









Lesions with hemosiderin staining: Hemosiderin staining is pathognomic of certain leisons or pathologies.

On right side a bubbly heterogeneously T2 hyper intense Cavernoma with typical low signal intensity hemosiderin staining on GRE.

On right side a case of superficial siderosis with low signal intensity hemosiderin staining along tentorium.

Arterio Venous Malformation (AVM)

A young female consulted in OPD for seizures.

This non contrast CT study of Brain shows mild asymmetry of cerebral hemispheres and lateral ventricles, right side hemisphere is larger in volume than left. 

Right lateral ventricle is smaller than left (Rules out Hemi megaencephaly).

Again multiple nodular densities along the ependymal lining of wall of right lateral ventricle which needed further evaluation.

MRI study of brain with MR Angiography of brain

Ax T2, T2*GRE with Non contrast 3D TOF brain Angio

 

This MRI study shows:
Multiple serpigenous T2 flow voids clustered in Right temporo parietal lobe suggestive of vascular malformation that to high flow malformation as signals are hyper intense on T2*GRE.
Imaging diagnosis : Arterio venous malformation (AVM).
Right MCA, ACAs and PCAs are the feeder on MR Angio.
Draining channels appears to be superior sagittal sinus and deep cerebral veins.

Related post : AVM