Idea Transcript
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
Dissections Multiple Lacunar
4%
5% 5%
data 3% 3%
30%
Cardioembolic
13% 13%
10% 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Michel & Eskandari, unpublished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 ¾ ¾ ¾ ¾
6XVDF¶V syndrome &RJDQ¶V syndrome (also necrotizing) Vogt-Koyanagi-Harada syndrome (VKH) Eales¶UHWLQRSDWK\ 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 :
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76 yo man, lacunar warning syndrome Pre-thrombolysis CTA
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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
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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 ¾ ¾ ¾ ¾
Pulselessness/claudication/paraesthesias upper extremities CNS: dizziness, headache, syncope Systemic symptoms Strokes (20-30%): ischaemic or haemorrhagic 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
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Indocyanide angiography Contrast leak
Fluoro-angiography Arterial narrowings 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 ¾ ¾ ¾
1) Prodromal (days): meningeal&neurologic manifestations 2) Uveitic (weeks): posterior-> anterior uvea 3) Convalescent (months) : hair/skin changes 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 ¾ ¾ ¾ ¾
SLE 6M|JUHQ¶V syndrome %HKoHW¶V disease Sarcoidosis Rheumatoid polyarthritis
¾ Scleroderma, MCTD ¾ Dermatomyositis ¾ Ulcerative colitis/&URKQ¶V disease
11 criteria. Stroke, venous thrombosis Dry eyes/mouth, ANA+ (-> SSA/SSB+) Oral/genital ulcers 3x/y, eye, skin Respiratory problems, liver, skin, uveitis 4 or 7 criteria (morning stiffness >1h, 3 joints, hand joints, rheumatoid nodules) ¾ Skin, multiples autoimmune ¾ Myopathy, dermatitis (extensor-side of joints/eye-lids) ¾ GI symptoms, malabsorption ¾ ¾ ¾ ¾ ¾
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
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61 y.o. woman : MRI
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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-, APLALumbar punctuer: ¾ 70 leukocytes (PMN)
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Angio: Dr JB Zerlauth, CHUV
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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
CTP 281 min.
2230097
IRM à 12 h
2005: massive right MCA stroke Æ ivthrombolysis 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