young stroke peter humphrey walton centre for neurology and neurosurgery, liverpool
TRANSCRIPT
Young Stroke
Peter Humphrey
Walton Centre for Neurology and Neurosurgery, Liverpool
Young Stroke
• Cardiac 20%
• Dissection 20%
• Idiopathic 30%
• Rare - including venous
Incidence
• Cardiac Embolism 24%• Cervical Artery Dissection 24%• Small Vessel Disease 9%• Large Artery Atheroma 4%• Undetermined Aetiology 33%• Others 6%• 203pts Zurich and Bern Stroke Registries• 11% aged 16 - 45 1997-2002
Carotid and Vertebral Dissection
Dissection
• Headache/neck pain proceeding stroke
• Horner’s syndrome
• TIAs/stroke
• MRI T1-Axial neck
• MRA
Carotid Dissection
• Cranial Nerve Palsies (esp XII,IX,X)• Other cranial nerves eg III,V,VII etc• The Surgeon with Altered Taste-dysgeusia• Pulsatile Tinnitus, Objective Bruit• Scalp Tenderness• 60-75% TIAs/Stroke• Incidence 2.5 per100,000 per year• Recurrence Rate 1% pa
Intracranial Dissection
• More difficult to diagnose
• Loss of elastic lamina
• Present similar to extracranial dissection
• Occasionally present as SAH esp.vertebral
• Can affect any vessel
Vertebral Dissection
• Presents as ischaemic brain stem stroke eg Lat Medullary Syndrome
• Occipital ischaemia
• Spinal Cord ischaemia (high)
• Unilateral pain and arm root lesion(C5/6)
• Spinal Epidural Haematomas
• SAH especially if intracranial
Vertebral Dissection
• Headache/Neck Pain
• Can be in any of the 4 parts of the vertebral artery ; usually V2 and V3
• 10% spread intracranially (unlike carotid)
• Intracranial - haemorrhage is common;consider in aneurysm -ve SAH
• More likely to be multiple (25%)
Basilar Dissection
• Rare
• Rapid Coma
• SAH
• Ischaemic Brain Stem Syndrome
• Occasionally subacute
• Probable account for ectatic basilar artery syndromes
Trauma
• Recent Series of 177 cases
• Age 44yrs +/-11
• 74 (41.8%) history of trauma
• Most sports,manipulations,sneezing,RTAs
• Timing;median time from headache to other symptoms is 4 days
Management
• Risk stroke mainly in 1st month (occas late)• No randomised data• Extracranial dissection - most anticoagulate -
stroke risk is embolic or ischaemic for 6m.• Intracranial - consider risk of haemorrhage
especially if vertebral ;LP first• ??Antiplatelets if symptoms >1 month or just
Horners
Management of Dissection
• Aneurysm - 30% heal• Late Risk of Rupture v. low; ?? never• Occasionally acts as source of emboli or as
mass lesion• Observe; rarely need intervention• Dissection itself- 60-70% recanalise;
sometimes within days-little change after 6m
Venous Infarction
• Pre-disposing cause - Pill, pregnancy, infection
• Headache
• Venous infarction - haemorrhagic - fits
• Raised intracranial pressure- papilloedema
• Fluctuating Focal Neurological Deficit
• False Localising Signs eg VI n palsy
• CT/MRI/MRV
Venous Thrombosis - Causes
• Pill/Pregnancy/Puerperium/Endocrine
• Haematological eg Lupus,protein S/C etc
• Malignancy Local/Systemic
• Inflammatory eg Behcets,Inflammatory Bowel Disease,Sarcoid,Wegeners etc
• Infective Local/Systemic
Treatment/Prognosis
• Anticoagulate if at all possible- even if haemorrhage; except if just BIH
• 624pts 57% full recovery
• 22% mRS grade1
• 7.5%mRS grade2
• 5.1%mRS grade3-5
• 8.3% died
• 2.2% Recurrence (FU16m); 10% seizures
Behcets
• Arterial ischaemia
• Venous thrombosis
• Inflammatory
• Venous Treatment - anticoagulant and steroids
• Few causes of arterial and venous isch. - behcets, antiphospholipid syndrome
SLE/Antiphospholipid Syndrome
• Age• Miscarriage/DVT/Livido-reticularis• Migraine• Myelopathy• Dementia• Endocarditis (Leibmann Sachs)• IgG - anticardiolipin antibody• Thrombo-embolic/not arteritic usually
Haematological Causes of Stroke
• Polycythaemia
• Thrombocythaemia
• Antiphospholipid Syndrome
• PNH
• Sickel Cell Disease
• Inherited Thrombophilias (usually venous)
• Thrombocytopenias
• 36yrs Nurse Zimbabwe HIV -ve
• 5 x Loss of use R arm/leg few mins
• Loss of speech x1
• ESR 111: vasculitic screen -ve : MRI-N
• LP normal except strongly +ve PCR to varicella zoster : other infection screen -ve
• Hep ABC -ve: MRA : Rpt HIV
Isolated Angiitis of the CNS
• Any age/Subacute onset
• Stroke
• Encephalopathy
• Headache
• EEG/csf/Angiography
• Brain biopsy
• Steroids & immunosuppression - cyclophos.
Brain Biopsy
• 61 pts• 36% Primary Angiitis• 38% Alternative Diagnosis• 25% Nondiagnostic• 10% Lymphoma• 12% Infective Enceph. eg CJD,PML,CMV• 5% Brain Abscess• Whipple’s disease - 1 case in Walton Centre
Drugs
• Warfarin/Aspirin
• Alcohol
• Amphetamines
• Heroin
• Cocaine
• Sympathomimetics (Over the Counter) eg phenylpropanolamine
Genetic Causes of Stroke
• Cerebral Autosomal Dominant Arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL)
• MELAS
• Hereditary Connective Tissue Disorders
• Fabrys
• Venous Infarction
• Homocystinuria
CADASIL
• Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leucoencephalopathy
• Migraine (beware)
• Dementia
• Pseudobulbar palsy
• Familial Chromosome 19
Fabry Disease
• 721 pts acute stroke
• 18-55yrs
• ά galactosidase activity
• 28 (3.9%) reduced activity
• 39% Painful Neuropathy
• 25% Angiokeratoma
• 50% proteinuria
Other Causes
• Radiotherapy
• Migraine
• Fibromuscular Dysplasia
• Moya Moya
• Non metastatic effect of malignancy
• Infection eg neurosyphilis
Clinical History
• Detailed description of vascular symptoms
• Arteriosclerotic risk factors
• Systemic disease
• Head injury
• DVT/miscarriage/skin
• Joints/mouth ulcers/genital ulcers
• Drugs/HIV
History (cont)
• Detailed Family History
• Previous DXR to head or neck
• Sexual Orientation
• Background
• Underlying background illness
• Chest Pain and Stroke - never synchronous?
• Frequent TIAs /Strokes -for no reason????
Investigations
• FBC U+E Gluc Chol ECG
• Doppler/Duplex Ultrasound
• CT/MR Head
• MRA/CTA
• Echo TTE/TOE
• Thrombophilia screen:Vasculitic screen:LP: HIV:TPHA :Angiography:Brain Biopsy
Investigation (cont.)
• Haemoglobin Electrophoresis
• Lupus anticoagulant/anticardiolipin Ab
• Drug screen
• Genetic testing for CADASIL, MELAS,Fabry’s
• Homocysteine screen
• Muscle / Skin Biopsy
Summary- Young Stroke
• Cardiac
• Dissection
• Venous Thrombosis
• Other causes all rare
• Need for detailed history and investigation
• Rare need for Brain Biopsy especially if inflammatory process - immunosuppression can be lifesaving