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Rare Causes of Stroke

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Rare Causes of Stroke Joint Annual Meeting SNG|SSN Basel, October 10th, 2012 Rare Causes of Stroke PD Dr Patrik Michel Neurology Service, CHUV Unité Cérébrovasculaire How rare are « rare » ischemic strokes ? N=2612 consecutive acute strokes 2003-2011 Rare causes PFO Missing data 4% Dissections 3% 3% 5% Cardioembolic Multiple 5% 30% Lacunar 13% 10% 13% 14% Atherosclerosis stenosis) Unknown «Likely athero» Modified TOAST classification, standardized workup Source: Michel & Eskandari, unpublished Rare stroke syndromes Overview 1. Vasculitis 2. Hypercoagulability and oncologic 3. Drug related stroke 4. Migraine, vasospasms, pregancy 5. Rare cardiac causes 6. Genetic diseases 7. Other non-inflammatory vasculopathies 8. Unusual causes of ICH Primary systemic vasculitides Giant cell ¾ Temporal arteritis ¾ 7DND\DVX¶V arteritis Necrotizing ¾ Polyarteritis nodosa ¾ Churg-Strauss syndrome Granulomatous ¾ :HJHQHU¶V granulomatosis ¾ Lymphomatoid granulomatosis With prominent eye involvement ¾ 6XVDF¶V syndrome ¾ &RJDQ¶V syndrome (also necrotizing) ¾ Vogt-Koyanagi-Harada syndrome (VKH) ¾ Eales¶UHWLQRSDWK\ ¾ Acute posterior multifocal placoid pigment epitheliopathy Quiz : 76 y.o. man 'RHVQ¶W see the doctor Now : acute pure left hemiparesis NIHSS fluctuating between 8 and 1 CT/CT-perfusion : normal Diagnosis: lacunar warning syndrome Æ Hyperacute CT: normal Æ IV thrombolysis at 2h25min. Acute CT-angiography : IPP2819339 76 yo man, lacunar warning syndrome Pre-thrombolysis CTA 2819339 Segmental narrowing both vertebrals CTA: A. Fumeaux Duplex and temporal arteritis : arterial wall thickening Duplex: L.Hirt Halo Segmental narrowing MRI and temporal arteritis : arterial wall thickening T1- temporal art. T1-Gado-ophtalmic Temporal artery biopsy IPP2819339 Histology: Dr I. Letanovic, CHUV Giant cell: Temporal arteritis Is rare below 60 years Continuum TA - PMR If stroke (about 3-7%) : ¾ Mainly from extracranial vertebral arteritis ¾ May occur after corticosteroid have been started Diagnosis : ¾ Sedimentation rate may be normal (15%) ¾ Temporal artery Duplex/CTA/MRA: moderately helpful ¾ Biopsy: Take long segment, consider contralateral temporal artery Corticosteroids GRQ¶W negativize biopsy for 7-14 days 1. Vasculitis as potential cause of stroke Primary systemic vasculitides Vasculitides secondary to systemic disease Isolated vasculitis of the CNS Giant cell: 7DND\DVX¶V arteritis Extracranial vaculitis: aortic arch and main arterial trunks, descending aorta and renal arteries Clinical: ¾ Pulselessness/claudication/paraesthesias upper extremities ¾ CNS: dizziness, headache, syncope ¾ Systemic symptoms ¾ Strokes (20-30%): ischaemic or haemorrhagic ¾ ESR in most, renal hypertension Diagnosis: major and minor criteria Takayasu Japanese J Ophtal 1905. Diagnostic criteria: Arend 1990 Quiz : 26 y.o. lady, good health History : since 1 month : ¾ ³6PRNH\´YLVLRQ ¾ Memory problems ¾ Unstable gait ¾ One episode of diplopia ¾ Mild posterior headache Exam: ¾ Attentional problems ¾ Mild bilateral corticospinal and cerebellar signs ¾ Left hypoaccousis Primary vasculitides with eye/ear involvement &RJDQ¶V syndrome 6XVDF¶V syndrome Vogt-Koyanagi-Harada syndrome Eales retinopathy Acute posterior multifocal placoid pigment epitheliopathy 26 y.o. lady: MRI T2W Diffusion (DTI) FLAIR 6XVDF¶VV\QGURPHUHWLQDOILQGLQJV 68753 68753 Indocyanide angiography Fluoro-angiography Arterial narrowings Contrast leak Venous obstructions Courtesy : Dr JA Pournaras, Lausanne eye hospital Susac's syndrome Retino-cochleo-cerebral arteriolopathy Triade: ¾ Small cortical and subcortical strokes ¾ Retinal branch occlusion ¾ Infarctions of cochlea (Æ neurosensory hearing loss) Monophasic disease with or without preceeding specific infections Differential Dx: multiple sclerosis Susac Neurology 1979 and 2003; Barker JNNP 1999 Cogan's syndrome Triade: ¾ CNS-vasculitis ¾ Keratitis ¾ Deafness Rare: ¾ Headaches, encephalopathy, lymphocytic meningitis, encephalopathy, seizures ¾ Sinus vein thrombosis ¾ Peripheral or cranial neuropathy ¾ Aortitis, aortic insufficiency ¾ GI hemorrhage, hepatospleno, lympadenopathy DG Cogan 1945 Vogt-Koyanagi-Harada syndrome (VKH) Triade: ¾ CNS&meningeal symptoms ¾ Bilateral uveitis ¾ Hair changes (poliosis, alopecia) Other neurological manifestations (rare) ¾ Encephalopathy, coma ¾ Seizures ¾ Cranial nerve palsy (V-VIII) 4 stages: ¾ 1) Prodromal (days): meningeal&neurologic manifestations ¾ 2) Uveitic (weeks): posterior-> anterior uvea ¾ 3) Convalescent (months) : hair/skin changes ¾ 4) Chronic recurrent stage Vogt 1906, Koyanagi 1929, Harada 1926 Vasculitides secondary to systemic disease Systemic Lupus erythemtodes (SLE) 6M|JUHQ¶V syndrome %HKoHW¶V disease Sarcoidosis Rheumtoid polyarthritis Scleroderma Mixed connective tissue disease Dermatomyositis Ulcerative colitis/&URKQ¶V disease Systemic lupus erythematodes Mechanisms of stroke Ischaemic ¾ Libman-Sachs endocarditis ¾ Hypercoagulability: antiphopholipids, cytokines ? ¾ Cerebral vasculopathy/vasculitis Haemorrhagic Cerebral venous thrombosis ¾ Hypercoagulability: antiphopholipids Other vasculitides secondary to systemic disease ¾ SLE ¾ 11 criteria. Stroke, venous thrombosis ¾ 6M|JUHQ¶V syndrome ¾ Dry eyes/mouth, ANA+ (-> SSA/SSB+) ¾ %HKoHW¶V disease ¾ Oral/genital ulcers 3x/y, eye, skin ¾ Sarcoidosis ¾ Respiratory problems, liver, skin, uveitis ¾ Rheumatoid polyarthritis ¾ 4 or 7 criteria (morning stiffness >1h, 3 joints, hand joints, rheumatoid nodules) ¾ Scleroderma, MCTD ¾ Skin, multiples autoimmune ¾ Dermatomyositis ¾ Myopathy, dermatitis (extensor-side of ¾ Ulcerative colitis/&URKQ¶V joints/eye-lids) disease ¾ GI symptoms, malabsorption Most can develop have CNS and/or PNS vasculitis Most can have other immunological disorders affecting near-neurological structures (autoimmune meningitis, uveitis, nerve compression, myositis) Quiz : 61 y.o. old woman Hypertension, smoking Controlled Basedow Recent removal of a skin kyste (neck) Now : 3 days of fluctuating confusion, aphasia? On exam: ¾ Inattentive, sometimes crying ¾ Fluent aphasia, major apraxia ¾ Mild right corticospinal signs IPP507240 61 y.o. woman : MRI 507240 DWI T1-Gadolinium 61 y.o. woman : work-up Blood normal CRP and sed. rate normal ETT & ETO : normal Ophta : normal retina No cancer Homocysteine-, APLA- Lumbar punctuer: ¾ 70 leukocytes (PMN) IPP507240 Angio: Dr JB Zerlauth, CHUV IPP507240 Isolated (primary) vasculitis of the CNS Rare, difficult diagnosis Presentation: very variable ! Recurrences/progression of ¾ Cognitive problems/confusion ¾ Focal symptoms and signs ¾ Headaches Diagnosis: no gold standard ¾ Multiple lesions on MRI (80%), leptomeningeal enhancement (60%) ¾ Abnormal LP (50%-90%) ¾ Segmental narrowing on DS-angiography (50-80%, DDx: spasms, emboli) ¾ Brain/meningeal biopsy (80%) ¾ Retinal fluorescein angiography ¾ Rule out endocarditis, toxics/drugs, malignancy etc. Infectious and postinfectious vasculitis/vasculopathy Meningovascular syphilis Neuroborreliosis (Lyme disease) Tuberculosis and mycosis Bacterial meningitis with strokes Neurocysticercosis VZV-related; CMV and herpes related Chlamydia pneumoniae/Mycoplasma HIV: hetereogenous mechanisms Hepatitis C and mixed cryoglobulinemia Meningovascular syphilis Any vessel affected, often cognitive problems Diagnosis ¾ Clinical & ¾ Pleocytosis on LP & ¾ Elevated IgG or IgM CSF-index for treponema IgG-CSF/IgG-Serum Albumine-CSF/Albumine-Serum Mechanisms of stroke in symphilis: ¾ Meningeal vasculitis ¾ Marantic endocarditis ¾ Aortic dissection VZV related vasculopathy Adults: 2-6 weeks after shingles mainly of the trigeminal nerve Children: 1-3 months after varicella = chickenpox Pathogenesis: controversial (infectious vs. immunologic) Clinical ¾ Ipsilateral deep or superficial MCA-stroke ¾ Rarer: disseminated CNS-vasculopathy (mainly if immunosuppressed) ¾ Very rare: optic nerve ischaemia, retinal necrosis 2. Hypercoagulable causes of stroke Antiphospholipid antibody syndrome Hyperhomocysteinaemia Hyperviscosity syndromes Disseminated intravascular coagulation (DIC) and Moschcowitz syndrome (thrombotic thrombocytopenic purpura) Disorders of the coagulation cascade Mixed cryoglobulinemia (hepatitis C) Paraneoplastic (intestinal, lung, gynecologic) Quiz: 30 y.o. man 2003: minor right MCA stroke. PFO & ASIA, smoking, cholesterol Æ PFO-closure 2230097 2230097 CTP 281 min. IRM à 12 h 2005: massive right MCA stroke Æ iv- thrombolysis at ¶ No more R-L shunt, still cholesterol, smoking &73j¶T-occlusion carotid No atherosclerosis Hyperhomocysteinaemia Causes ¾ Genetic: MTHFR (rarely cystathionine-beta-synthase or methionine synthase deficiency* (skin-Bx!) ¾ Genetic childhood form: also marfanoid features, mental retardation, ectopia lentis ¾ Nutrition/malabsorption: B6, B12 and folic acid deficiency Clinical: ¾ Linear increase of stroke and coronary risk with serum levels ¾ Incresed risk of venous thrombosis Treatment (folic acid, B6, B12): ¾ No clear benefits for stroke prevention ¾ Individual cases may benefit* Vitamin stroke trials: Toole/VISP: JAMA 2004; VITATOP, Lancet Neurol 2010 *Novy, Thromb Haemostas 2010 Antiphospolipid antibody syndrome (APLS) Diagnostic criteria (1 clinical & 1 laboratory) Clinical criteria ¾ Vascular thrombosis (arterial or venous, any tissue or organ) ¾ Otherwise unexplained abortus 1 death fetus >10th week, or 1 premeature birth <34th week, or 3 consectutive spontaneous abortions <10th week Laboratory criteria: on 2 occasions 3 months apart: ¾ Lupus antcoagulant antibodies, or
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