Vascular Disease and Intracranial Hemorrhage: Case Based Review. Disclosures

Transcription

1 Vascular Disease and Intracranial Hemorrhage: Case Based Review Mahmud Mossa-Basha, M.D. University of Washington School of Medicine Department of Radiology, Division of Neuroradiology Nothing to disclose. Disclosures 1

2 Case #1 All the following are likely etiologies for SAH except: Trauma Arteriovenous malformation Aneurysm Vasculitis Dissection Stroke Non-Contrast CT head Case #1 All the following are likely etiologies for diffuse SAH except: Trauma Arteriovenous malformation Aneurysm Vasculitis Dissection Stroke Non-Contrast CT head 2

3 Case #1 Intracranial Aneurysms Axial 3D TOF MRA: Left supraclinoid ICA aneurysm and basilar artery aneurysm. Case #1 Which of the following is true? Posterior communicating artery origin aneurysms can result in subdural hemorrhage Aneurysms rarely grow There is a higher rate of recurrence with clipped aneurysms as compared to coiled aneurysms Calcified aneurysms do not pose any additional treatment risk 3

4 Case #1 Which Aneurysm Ruptured? Case #1 Cerebral aneurysm Three types of cerebral aneurysms Saccular Fusiform Dissecting 4

5 Case #1 Saccular (Berry) Aneurysm True aneurysms: frequently arise at intracranial branch points, rounded 1-5% incidence on angiography Multiple aneurysms in 15-20% Age at presentation: years Most common presentation: SAH Typically Asx until rupture May cause cranial neuropathies, sz, headache, infarct Risk of rupture: 1-2% per year Case #1 Saccular Aneurysm Presentations: SAH Location: At bifurcations 30-35% ACOM 30-35% ICA/PCOM 20% MCA bifurcation 5% basilar Etiology Once thought to be congenital More likely hemodynamically induced degenerative vascular injury Other causes: trauma, infection, drug abuse, high flow states (AVM/AVF) Associated with connective tissue d/o, FMD and PCKD 5

6 Most common cause of death/morbidity in aneurysm rupture? Complications of vasospasm Case #1 Cerebral edema Brain herniation Hydrocephalus Hypotension Infection Case #2 Etiologies for stroke include all except? Plaque rupture/artery embolus Cardiac embolus Vasculitis Hypoperfusion Venous thrombosis Reversible Cerebral Vasoconstriction Syndrome (RCVS) Arachnoid Cyst DWI 6

7 Case #2 Differential Includes the following Except? Moyamoya Disease Atherosclerosis Vasculitis RCVS Vasospasm Case #2 Most Likely Diagnosis? Moyamoya Disease Atherosclerosis Vasculitis Reversible Cerebral Vasoconstriction Syndrome (RCVS) Vasospasm 7

8 Case #2 Most Likely Diagnosis? Moyamoya Disease Atherosclerosis Vasculitis Reversible Cerebral Vasoconstriction Syndrome (RCVS) Vasospasm Case #2 What is the correct treatment answer? Symptomatic Moyamoya disease should be first treated medically Reversible cerebral vasoconstriction syndrome is surgically managed Infectious vasculopathy should be treated with a trial of steroids for diagnosis First line management for stenotic atherosclerosis is surgical bypass Primary CNS vasculitis is treated with immunomodulators 8

9 Case #2 Vasculitis The Calabrese criteria for PACNS diagnosis: an acquired or otherwise unexplained neurological deficit angiographic or histopathologic features of angiitis within the CNS absence of another systemic disorder to explain these features Secondary: Any systemic vasculitis can affect the CNS Viral Bacterial Fungal Parasitic Systemic Connective Tissue diseases Miscellaneous Varicella HIV Hepatitis C CMV Lyme disease Syphilis TB Mycoplasma Bartonella Rickettsia Aspergillosis Mucormycosis Coccidiomycos is Candidosis Cysticercosi s Wegener s granulomatosis Churg-Strauss syndrome Behcet s disease Polyarteritis nodosa Henoch-Shonlein purpura Kawasaki disease Giant-cell arteritis Takayasu s arteritis Systemic lupus erythematosus Rheumatoid arthritis Sjogren's syndrome Dermatomyositis Mixed Connective Tissue disease Antiphospholipid antibody syndrome Hodgkin s lymphoma and NHL Neurosarcoidosis Inflammatory Bowel Disease Graft-versus-host disease Bacterial endocarditis Acute bacterial meningitis Drug-induced Case #2 Diagnosis Systemic vasculitis with cerebral manifestations diagnosis depends on serum tests and body tissue biopsies. Diagnosis of an isolated CNS manifestation can be more challenging. CSF studies Neuroimaging Definitive diagnosis may require brain biopsy 9

10 Case #2 Imaging Findings Luminal Imaging (CTA, MRA, DSA): Multi-focal arterial stenoses with beaded appearance Nonspecific Luminal imaging may be normal DSA sensitivity between 50-90% for CNS vasculitis Non-contrast CT findings SAH and IPH Signs of acute and chronic ischemia Case #2 MRI Findings Conventional MRI most sensitive imaging exam for vasculitis. Negative MRI can essentially rule out vasculitis MRI abnormali es in % of vasculitis Findings of ischemia: DWI, T2 FLAIR, enhancement findings of different ages IPH and SAH: FLAIR and SWI MRI non-specific for the diagnosis of vasculitis DWI Behcet Vasculitis ADC 10

11 Case #2 MRI Findings Conventional MRI most sensitive imaging exam for vasculitis. Negative MRI can essentially rule out vasculitis MRI abnormali es in % of vasculitis Findings of ischemia: DWI, T2 FLAIR, enhancement findings of different ages IPH and SAH: FLAIR and SWI MRI non-specific for the diagnosis of vasculitis T1 POST T2 FLAIR Cerebral Vasculitis Tissue damage Ischemia and hemorrhage result from 1 : Hemorrhage Infarct 1. Vessel wall thickening, obstruction of the lumen vessel wall vessel wall inflammation inflammation 2. Thrombosis due to inflammatory cytokines at the endothelium 3. Vasospasm 4. Wall rupture and hemorrhage arterial rupture high-grade stenosis intraluminal thrombus 11

12 Case #2 Vessel Wall Imaging Findings T1 Pre-Contrast VWI T1 Post-Contrast VWI Circumferential, intense enhancement of the vessel wall. Case #3 What is the most likely diagnosis in this 20 year old female? Moyamoya disease Reversible Cerebral Vacoconstriction Syndrome Atherosclerosis Dissection Vasospasm R CCA Injection L CCA Injection 12

13 What is the most common pediatric/adult presentation of Moyamoya? Hemorrhage/Ischemia Case #3 Dementia/Ischemia Hemorrhage/Dementia Ischemia/Hemorrhage PRES/Dementia Infection/PRES Case #3 Moyamoya Vasculopathy Progressive steno-occlusive vascular disease affecting the carotid termini with development of extensive basal collaterals Moyamoya disease: Idiopathic disease, bimodal distribution (<10 yo and 40 s), most commonly in Japanese Moyamoya syndrome: Secondary process- Atherosclerosis, vasculitis, radiation, genetic disorders (sickle cell, Downs, NF-1, etc.) 13

14 Case #3 Moyamoya Presentation MMS more frequently seen in US populations Pediatric Ischemia as collaterals are not well developed Adults Hemorrhage relating to rupture of fragile collaterals Treatment Peds: Indirect ECA to MCA bypass Adults: Heterogeneous, but for symptomaticdirect ECA to MCA bypass Case #3 Imaging Findings MRI/CT Ischemia and intracranial hemorrhage Chronic ischemic changes/atrophy Loss of flow voids Luminal Imaging Occlusion of the carotid termini with extensive collaterals DWI SWI T2 ADC FLAIR 14

15 Case #3 Imaging Findings MRI/CT Ischemia and intracranial hemorrhage Chronic ischemic changes/atrophy Loss of flow voids Luminal Imaging Occlusion of the carotid termini with extensive collaterals Axial TOF MRA Coronal TOF MRA Case #4 All the following disease/treatment possibilities are correct except: Vasculitis- Immunosuppressive RCVS- Calcium channel blockers Moyamoya Disease- Aspirin Atherosclerosis- Statins/Anti-platelet DSA AP ICA DSA Lat ICA 15

16 Case #4 35 year old female presented with thunderclap headache, diagnosis? Vasculitis RCVS Moyamoya disease Aneurysm with rupture Atherosclerosis Dissection DSA AP ICA DSA Lat ICA Case #4 RCVS Presentation: Thunderclap headache that is intermittent Young and middle aged females Inciting etiology: SSRI, sympathomimetic agents, coitus, cold shower, eclampsia, post-partum, activity, alcohol, etc. Intracranial vasospasm that tends to resolve after 1-3 months 16

17 Case #4 RCVS- Imaging Findings MRI/CT Multifocal infarcts Subarachnoid and IP hemorrhage PRES-like appearance DWI ADC NC CT Case #4 RCVS- Imaging Findings Luminal Imaging Beaded arterial appearance Alternating segments of narrowing and dilatation Indistinguishable from vasculitis Findings will progressively improve on CTA/MRA/DSA Initial MRA Follow-up MRA 2 weeks later 17

18 Case #5 30 year old with parenchymal hemorrhage, what is the most likely diagnosis? Cerebral amyloid Ischemic infarct Anterior communicating aneurysm rupture Ruptured developmental venous anomaly Arteriovenous malformation Case #5 Which of the following syndromes is associated with AVM? Kearns-Sayre Syndrome Hereditary Hemorrhagic Telangiectasia Down s Syndrome Morquio Syndrome Kartagener Syndrome 18

19 Case #5 Cerebral AVM Congenital lesions Feeding artery(ies) Intervening nidus Draining vein(s) Case #5 Cerebral AVM Presentation Hemorrhage most common (42-72%) Seizures (25%) Headaches (15%) Focal neurologic deficit Steal phenomenon Pulsatile tinnitus Thought as congential lesions Associations with initial hemorrhage Deep brain location Deep venous drainage Associated aneurysms Posterior fossa Risk for repeat hemorrhage Prior hemorrhage 19

20 Case #5 Imaging Findings CTA/MRA Tangle of vessels with large feeding arteries and draining veins Catheter Angiography Tangle of vessels/nidus Feeding arteries Early appearance of draining veins Case #5 20

21 Case #5 Case #5 Imaging Findings NC CT Curvilinear or speckled calcifications Hyperattenuating serpentine vascular structures Hemorrhage Surrounding hypoattenuation MR Arterial, venous and nidal flow voids Perinidal T2 hyperintensity Enhancement on post-contrast MRI AVM may be compressed by hematoma May not be seen on any imaging modality early Repeat imaging once the hematoma has reduced in mass effect may be necessary 21

22 Case #6 What is the most likely diagnosis? Fibromuscular dysplasia Dissection Arteriovenous fistula Atherosclerosis Thromboembolism 22

23 Case #6 Arterial Dissection Subintimal Intimal tear: Collection of blood in subintimal space Complications: Hypoperfusion from luminal narrowing Embolic events Subadventitial Blood collects in subadventitial space Secondary to vasa vasorum rupture or extension of subintimal dissection Case #6 Luminal Imaging Findings DSA/CTA Very good at depicting subintimal dissections Double lumen appearance Stenosis of true lumen Not as good with subadventitial dissections Especially if do not result in luminal stenosis 23

24 Case #6 MRI Findings Sensitive for detection of thrombus within subintimal or subadventitial dissections T1/PD fat sat Crescentic hyperintensity adjacent to lumen Case #7 What is abnormal? Left cerebellum Suprasellar cistern Interpeduncular cistern Frontal lobe white matter Right MCA 24

25 Case #7 What is the diagnosis? Subarachnoid hemorrhage Subdural hemorrhage Hyperacute ischemic infarct Meningitis w/ vasculitis Moyamoya Disease Case #7 What is the diagnosis? Subarachnoid hemorrhage Subdural hemorrhage Hyperacute ischemic infarct Meningitis w/ vasculitis Moyamoya Disease 25

26 Case #7 3D TOF MRA Case #7 MRI Exam DWI TTP MR Perfusion Map 26

27 Case #7 DSA before and after thrombectomy Case #7 Thrombus in Stent Retrieval Device 27

28 Case #7 Hyperacute Infarct CT Hyperdense MCA Loss of gray-white distinction Loss of basal ganglia MRI DWI abnormality with matching ADC abnormality Decreased CBV/Prolonged transit time on MRP No T2 FLAIR parenchymal signal abn Hyperintense arteries (slow or no flow) Case #7 Subacute Infarct CT Increased mass effect/herniation Edema Sulcal effacement May see hemorrhagic conversion MRI Infarct enhancement Pseudonormalization of ADC T2/FLAIR signal abn T2 fogging effect 28

29 Case #7 Subacute Infarct CT Increased mass effect/herniation Edema Sulcal effacement May see hemorrhagic conversion MRI Infarct enhancement Pseudonormalization of ADC T2/FLAIR signal abn T2 fogging effect Case #7 Subacute Infarct CT Increased mass effect/herniation Edema Sulcal effacement May see hemorrhagic conversion MRI Infarct enhancement Pseudonormalization of ADC T2/FLAIR signal abn T2 fogging effect 29

30 Case #7 Chronic Infarct Volume loss Shrinkage of parenchyma and ventricular enlargement Loss of overlying cortex CSF attentuation/signal encephalomalacia with surrounding gliosis SWI/GRE: Hemosiderin deposition due to hemorrhage Thank you! 30