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