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COMMON SYMPTOMS OF UNCOMMON CEREBROVASCULAR DISORDERS: SOMETIMES IT REALLY
IS A ZEBRA.
Kim Page, NPUniversity of Rochester Medical Center
Rochester, NY
CEREBROVASCULAR DISORDERS
• Carotid and vertebral artery injuries
• Basilar artery disease
• Arteriovenous Fistulas
• Venous sinus thrombosis
• Cerebral small vessel diseases
• Vasculitis
• Central Retinal Artery Occlusion
CV TRAUMA: INJURIES TO THE CERVICAL ARTERIES
• Rare but associated with mortality rates 20-40%; permanent neurologic impairment 40-80%
• Symptoms frequently develop in a delayed fashion—important to have high index of suspicion based on mechanism of injury
• Biggest risk is thrombotic occlusion
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CAROTID & VERTEBRAL ARTERY DISSECTION
• Most common cause of stroke in young adults
• Extracranial ICA most common site
• More common in men than women• More common in Winter• Often goes undiagnosed
PATHOLOGY OF CERVICAL ARTERY DISSECTIONS
• Caused by an intimal tear
• blood dissects along the artery forming an intramural hematoma leading to thrombus
• Vessel occlusion
• Thrombus with subsequent distal emboli
• Pseudoaneurysm formation occurs when the dissection plain lies between the tunica media and adventitia (outer layer)
MECHANISM OF INJURY
• 80% preceded by trauma to the head or neck
• Hyperextension (as in sports, exercise)
• Rotational neck trauma
• Blunt trauma
• Vertebral artery injuries more common
• Direct compression of the neck
• Skull base injury
• Average time from trauma to symptoms is 2-3 days
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MECHANICAL CONTRIBUTING EVENTS
• MVC
• Fall
• Chiropractic manipulation
• Exercise–weight lifting, trampoline, impact sports
• Roller coasters
• Can be benign like shaving, yoga, massage, vomiting, coughing
• Can be spontaneous
POSSIBLE CONTRIBUTING MEDICAL COMORBIDITIES
• HTN
• Smoking
• Birth control pills
• FMD
• Ehlers-Danlos syndrome
• Marfan syndrome
• Atherosclerosis
• Migraine history
• infection
• Ipsilateral frontal/temporal headache and neck pain, facial pain
• Partial Horner’s syndrome (Miosis and ptosis without anhidrosis)
• Lower CN palsies (XII, IX, X)
• Diplopia
• Amaurosis fugax
• Ipsilateral Tinnitus (usually “whooshing” sound)
• Audible bruit
• Expanding hematoma/pulsatile neck mass/palpable thrill
• Sudden unilateral decrease in sensation or weakness
• Hypogeusia
• Mean age 47 years
PRESENTATION
CAROTID ARTERY DISSECTION
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WHAT YOU MIGHT SEE ON PHYSICAL EXAM
SIGNS TO LOOK FOR• Focal neurologic deficits/stroke
• Ptosis with miosis
• CN palsies
• Cervical bruit
• ”Seat belt sign”
• Signs of cervical spine injury
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VERTEBRAL ARTERY DISSECTION
PRESENTATION• Occipito-cervical pain
• Ipsilateral facial dysesthesia
• Vertigo
• Nausea and vomiting
• Dysarthria (CN IX & X)
• Ipsilateral hypogeusia or ageusia
• Diplopia or oscillopsia
• Ipsilateral hemiplegia
• Contralateral loss of pain and temperature
• Mean age 40.7 years
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EXAM
WHAT TO LOOK FOR• Dysmetria
• Limb or truncal ataxia
• Ipsilateral Horner’s
• Contralateral impairment of pain, thermal
• Ipsilateral impairment of fine touch and proprioception
• CN IX & X palsy
CEREBELLAR FINDINGS• Nystagmus
• Tongue deviation to the side of the injury
• Contralateral hemiparesis
• Ophthalmoplegia
• CTA, MRA
• Intimal flaps
• Pseudoaneurysm
• Stenosis or occlusion
• Ultrasound (higher false negatives with injuries closer to the skull base)
• DSA
IMAGING
APPROACH TO DIAGNOSIS
• Initial non contrast CT; if negative, then CTA
• If CTA shows dissection,
• MRI/MRA and
• Referral to neurovascular specialist for DSA
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COMPLICATIONS
• Stroke
• Thrombus formation in the false lumen
• Hypoperfusion
• Severe vessel narrowing
• Occlusion of the dissected vessel
• Occlusion of a branch of the vessel by the intimal flap
• Pseudoaneurysm formation
TREATMENT
• Antiplatelets
• Systemic anticoagulation
• Surgical
• Stenting
• Bypass
• Dissections can take 3-6 months to heal
OUTCOMES
• Usually good
• Infarction
• Generally occurs in the first 2 weeks
• Majority have good long term outcome
• Pseudoaneurysms
• Rupture risk about 1%
• Causes SAH
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BASILAR ARTERY THROMBOSIS
• Symptoms may be stuttering and wax and wane over weeks to months
• Often mimic other neurologic syndromes like BPV, delirium, coma
CAUSES
• Large-artery atherosclerosis (most common)
• Embolism
• Penetrating small artery disease
• Dissection
• Cervical spine or skull base fracture
• Arteritis
• Aneurysms
PRESENTATION
• Disequilibrium
• Vertigo, nausea, vomiting
• Dysarthria –scanning or staccato speech
• Visual disurbances
• Headache
• Alternating paresthesias
• Tinnitus
• Dysphagia
• Drop attacks
• Ataxia and intention tremor
• Perioral numbness
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WHAT TO LOOK FOR ON EXAM
• Test for dysmetria
• Finger to nose, heel to shin
• EOMs—look for nystagmus
• Dysdiadochokinesia (slow alternating movements)
• Gait test—heel to toe, stand on one leg
• Pupil abnormalities
• Oculomotor signs
• Facial weakness, dysphonia, dysarthria, dysphagia
DIAGNOSIS AND TREATMENT
• MRI (assesses for acute stroke)
• CTA/MRA
• Thrombectomy
• tPA
• Antiplatelets
• Warfarin
ARTERIOVENOUS FISTULAS
Abnormal connection between and artery and vein
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DURAL ARTERIOVENOUS FISTULA
• Acquired lesions involving an intracranial venous sinus
• Most commonly present at age 40-60
• Fistula forms between the meningeal arteries and veins
• Commonly occurs in the cavernous sinus
CAUSES
• Traumatic head injury
• Infection
• Previous neurologic surgery (craniotomy)
• Tumors
• Progressive stenosis of a venous sinus
• Genetic risk factors
• Predisposition to vein thrombosis
• Benign meningiomas
PRESENTATION
• Highly variable and dependent upon location and anatomy of the lesion
• Pulsatile tinnitus most common
• Bruit (skull/mastoid process)
• Should raise a high suspicion for a vascular lesion
• Headache
• Visual disturbances
• Hydrocephalus may be present as a result of venous hypertension in the superior sagittal sinus which interferes with CSF absorption
• Intracranial venous hypertension can mimic
• Progressive dementia, bithalamic dementia
• Pseudotumor cerebri
• Parkinsonism
• Cervical myelopathy
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SPECIFIC PHYSICAL EXAM FINDINGS
• Bruit present
• Cranial neuropathies
• Signs of intracranial hypertension
• papilledema
DIAGNOSIS
• Catheter angiography is the best
• CT scan is not useful
• MRI/MRA is useful in both subjective (only the patient can hear it) and objective (bruit is present) tinnitus
• White matter edema=venous congestions
• Can see hydrocephalus if present
• May give information about the drainage
MANAGEMENT
• Minimally symptomatic dAVF are managed conservatively and expectantly
• Endovascular treatment
• Surgery to interrupt the draining vein
• Stereotactic radiosurgery for those who cannot undergo endovascular or surgical treatment
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CAROTID CAVERNOUS FISTULA
Abnormal connection between the carotidartery and/or its branches and a large vein called the cavernous sinus.
The cavernous sinus is located behind the eye and receives blood from brain, orbit, and pituitary gland.
• Communication between cavernous segment of the ICA and cavernous sinus due to a defect in the wall of the ICA (usually a single hole 2-6 mm)
• Usually caused by some type of trauma
• Can be iatrogenic as a result of surgery or neurointerventional procedure
• Endoscopic sinus surgery
• Transphenoidal pituitary surgery
• Balloon rhizotomy
• Pipeline Flow Diversion for cavernous aneurysm
• High flow
TWO TYPES OF CCFDIRECT
• Connection between the cavernous sinus and meningeal branches of the ICA, ECA or both
• Mostly women in their 60s and 70s
• Slight propensity to be on the left
• Risk factors
• HTN, atherosclerotic vascular disease, pregnancy, Ehlers-Danlos syndrome, minor trauma
• Slow flow
INDIRECT
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PRESENTATION
• Influenced by the size and type of fistula, location, flow rate, drainage route
• Ocular – can be subtle
• Chemosis, proptosis, eye discomfort
• Cranial nerve palsy (6, 3 and rarely 7)
• Elevated IOP
• Diplopia
• As fistula worsens, periorbital or retrobulbar discomfort, facial pain
• Pulsatile Tinnitus
• Cranial neuropathies
• Facial or ocular motor nerve paresis
• Hemi-facial spasm, TN
PHYSICAL EXAM
• Examine eyes
• Proptosis, chemosis, eyelid edema, ocular pulsations, bruit
• Look for facial pain in trigeminal nerve distribution
• Diplopia
• Optic disc swelling
• Intra-retinal hemorrhage
DIAGNOSIS AND TREATMENT
• Catheter angiogram is the definitive test
• If a fistula is suspected non-invasive imaging:
• CT, CTA
• MRI with contrast concentrated on the cavernous sinus
• MRA
• Specify you suspect a fistula when ordering
• Neuro-ophthalmology referral
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TREATMENT
• Treatment is generally endovascular and VERY safe
• Fistula closed (keeping the artery open) with glue or coils
• Accessing the vein through the orbit
• Carotid sacrifice
SPINAL FISTULAS
• Usually starts asymptomatic
• Cord swelling gradually develops
• Problems with leg weakness, back pain, leg pain and bowel and bladder
• Causes generally unknown
• Usually identified by MRI which will show large veins surrounding the cord
• DSA for definitive diagnosis
TREATMENT
• Endovascular embolization
• Surgical
• Outcomes are usually very good
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VENOUS SINUS THROMBOSIS
• Clinical symptoms and signs vary
• Affects all age groups
• Large sinuses (eg, superior sagittal sinus) most frequently involved
• Venous system has extensive collaterals and compensates early
• Systemic inflammatory diseases and coagulation disorders are common causes
• Oral BCP
• Continuing process; thrombus progresses over time
PRECIPITATING FACTORS
• Connective tissue diseases
• Granulomatous or inflammatory disorders and malignancies
• 6 weeks post partum
• Oral contraception use
• Prothrombotic conditions
• FacorV Leiden
• Protein C and S deficiency
PRESENTATION
• Symptoms usually appear gradually
• Headache (70-90%)
• Papilledema
• Focal deficits
• More common in women
• Seizure
• Coma
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WHAT TO LOOK FOR ONPHYSICAL EXAM
DIAGNOSIS
• MRI with venography (MRV) or CTV
• DSA
• LP to rule out meningitis, SAH, IICH
• Tests to evaluate for the underlying cause:
• Infection
• Head injury
• Malignancies
TREATMENT
• IV heparin
• Local thrombolysis if patient decompensates
• Oral anticoagulation 3-6 months
• Favorable prognosis
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CEREBRAL SMALL VESSEL DISEASE
• Diseases affecting the small arteries, arterioles, venules and capillaries of the brain
• Reduced cerebral blood flow, impaired autoregulation, increased BBB permeability
• Leading cause of cognitive decline and functional loss in elderly patients
• Most common forms are cerebral amyloid angiopathy and hypertension related SVD
Small vessels comprise 2 components:
• Leptomeninges vasoganglion (derived from the subarachnoid space covering) and the convex surface of the brain
• Perforating arteries from ACA, MCA and PCA
HYPERTENSIVE SVD
• Age related, most common in elderly
• Exacerbated by HTN, DM
• Vessels become elongated, tortuous and inflexible due to wall damage
• Autoregulation is impaired leading to reduced CBF and chronic hypoperfusion
• Acute occlusion of the arterial lumen leads to acute ischemia and lacunar infarcts
• Severe stenosis and hypoperfusion causes incomplete ischemia seen as white matter hyperintensities on imaging
• Often asymptomatic
• Progressive accumulation of amyloid proteins in small vessels
• Present in almost all elderly with dementia and 65-85% of those without
• Can lead to cognitive dementia, ICH or other transient neurologic events
• ICH usually spontaneous, usually occurring in patients >60 years
• In patients with ICH, 25-40% have recurrence, highest in the first year, and are associated with a high mortality rate
• Common in elderly with Alzheimer disease and Down syndrome
• Severity is age related
CEREBRAL AMYLOID ANGIOPATHY
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PRESENTATION
• Cognitive impairment
• >40% of those presenting with ICH have some degree of dementia
• Cognitive changes can precede ICH
• Can present with progressive dementia with rapid decline over days or weeks
• Perceptual speed, language skills and episodic memory impaired
• Intracranial hemorrhage
• Headache
• Pain generally in the area of the hemorrhage
• Vomiting
TRANSIENT FOCAL NEUROLOGIC EVENTS
“Amyloid Spells”
• Transient events similar to TIAs
• Spreading focal weakness, paresthesiaslasting several minutes
• Symptoms spread smoothly over contiguous body parts
• May be a prodrome for hemorrhage
• Avoid antithrombotics!
Distinguished from TIAs by
• smooth spread of symptoms
• lack of large vessel disease
• presence of microhemorrhages on MRI (GRE)
DIAGNOSTIC TESTING
• Head CT—usually find hemorrhages in the frontal and parietal lobes involving the cortex and subcortical white matter
• MRI—microhemorrhages, evidence of multiple large and small petechial cortical and subcortical hemorrhages, siderosis
• DSA—not always abnormal
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TREATMENT
• Basically untreatable
• Manage the ICH if present
• Hematoma evacuation
• Hemorrhage prevention
• Avoid AC, BP control
• In inflammatory forms, immunosuppressive therapy
• Referral to a stroke neurologist is highly suggested
GENETIC TYPES OF SMALL VESSEL DISEASECADASIL
(CEREBRAL AUTOSOMAL DOMINANT ARTERIOPATHY WITHSUBCORTICAL INFARCTS AND LEUKOENCEPHALOPATHY)
• Inherited condition that causes stroke and other impairments
• Progressive dementia
• Mood disorders
• Migraine
• Recurrent subcortical cerebral infarcts
PRESENTATION & COURSE
• Tetrad
• Dementia
• Psychiatric disturbances
• Apathy, major depression
• Migraine with aura that begins in young adulthood
• Recurrent strokes – most common
• Later followed by recurrent TIAs then eventually strokes
• Progressive cognitive impairment
• Frontal lobe impairment, memory impairment with intact language
• Late symptoms include gait apraxia, pseudobulbar palsy, urinary incontinence
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PHYSICAL FEATURES
• Variable degrees of weakness
• Variable degrees of sensory deficit
• Gait apraxia
• Pseudobulbar palsy
• Parkinsonism/movement disorders
• Psychomotor retardation
• Apathy
• Depressed affect
• Psychosis
REVERSIBLE CEREBRAL VASOCONSTRICTION SYNDROME
(RCVS)
• Group of disorders characterized by reversible multifocal narrowing of the cerebral arteries
• Associated with abrupt, severe headaches with or without seizures or neurologic deficits
• Constriction of cerebral arteries
• Predominately affects women mean age 42-44 years
• Is reversible with recovery around 3 months
• Can lead to stroke and hemorrhage
PRESENTATION
• Sudden, intense headache +/- nausea, vomiting, photosensitivity
• Frequently have triggers
• Orgasm, physical exertion, acute stressful or emotional situations, valsalva
• Symptoms related to ischemia from the vasoconstriction:
• Seizures
• Visual changes
• Stroke symptoms
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CAUSES/RISK FACTORS/ASSOCIATED CONDITIONS
• Drugs—cocaine
• Alcohol, especially binge drinking
• Anti-depressants -- SSRI
• Nasal decongestants and other vasoconstrictive drugs
• Nicotine patches
• Vasoactive tumors (pheochromocytoma, carcinoid)
• Hypercalcemia
• Head trauma
• Pregnancy
TESTING
• MRI
• Vasogenic edema
• Sulcal hyperintensities on FLAIR
• CTA/MRA/DSA
• multifocal segmental cerebral artery vasoconstriction
• Blood/urine toxicology
• Diagnosis is usually made based on clinical presentation (thunderclap headaches) and angiographic studies
TREATMENT
• Depends upon the severity—may resolve without treatment
• Migraine may be treated with aspirin, Depakote
• Calcium channel blocker
• Verapamil
• Magnesium
• STOP any drugs that may be associated!
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VASCULITIS
• Inflammation of the blood vessels
• Causes walls of the vessels to weaken, stretch, thicken, scar--> narrow and burst
• More common in people with autoimmune disorders (lupus, RA) or infectious (Hep B, C)
• Causes
• Immune system attacks blood vessels by mistake
• Frequently unknown
• Infection, allergic reaction, lymphoma, leukemia can trigger the immune system
AFFECTS OF VASCULITIS ON THE NERVOUS SYSTEM
• HA
• Aneurysm
• Thrombosis
• Confusion, dementia
• Abnormal sensation/loss of sensation
• Muscle weakness, paralysis
• Pain
• cerebral edema
• Visual problems
• Seizures
• Aphasia
• Cerebral edema
DIAGNOSIS
• Difficult to diagnose
• Difficult to distinguish from non-inflammatory cause of vasoconstriction
• Inflammatory labs
• CSF
• Brain biopsy
• CT, MRI, DSA, US
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TREATMENT
• Immune suppression (steroids)
• Supportive care
• Prevention
CENTRAL RETINAL ARTERY OCCLUSION (CRAO)
• Acute stroke of the eye
• Risk factors
• HTN, DM, CAD, Coronary artery disease, TIAs or CVAs, smoking
• Family h/o vascular disease, proatherogenic states
• Increased IOP, optic nerve head drusen, pre-retinal arterial loops
PRESENTATION
• Sudden, painless monocular vision loss
• Unilateral
• History of atherosclerotic disease and/or risk factors
• HTN, atrial fibrillation
• Immediate ophthalmology consult
• Acute: attempt to restore perfusion to the CRA
• Orbital massage
• Decrease IOP
• Acetazolamide, mannitol
• Vasodilators
• Pentoxifylline, nitroglycerine, isosorbide
• Prevent secondary complications to the eye
• Prevent other vascular events in the future
MANAGEMENT
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FINAL THOUGHTS
• History is VERY, VERY, VERY important
• A good neurologic exam is critical to picking up subtle (and not so subtle!) findings
• If you suspect a vascular issue, initiate a referral to stroke neurology
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