Tag Archives: Vascular

Giant Cell Arteritis

Synonyms: a.k.a. cranial arteritis a.k.a. temporal arteritis: Diagnosis: Biopsy of the temporal artery within 7 days of starting steroids: With serial sectioning Or clinical features plus raised ESR or CRP plus response to steroids Pathology: Panarteritis: Intimal thickening and proliferation, Lymphocytic infiltration of media and adventitia, giant cells, Disruption of elastic lamina Chronic phase: intimal … Continue reading Giant Cell Arteritis

Takayasu’s Disease

Synonyms: a.k.a. aortic arch syndrome a.k.a. Takayasu’s arteritis: Diagnosis: Arteriography: Irregular vessel walls, stenosis, poststenotic dilation, aneurysms, occlusion, collaterals Usually smooth stenosis of the common carotid and the subclavian arteries Other imaging: Ultrasound: thickened intima layer CT: thickened enhancing walls MRA Treatment: Control the symptoms and inflammation before surgery: Prednisolone, Correct the anatomical defects: Angioplasty … Continue reading Takayasu’s Disease

Encephalofacial Angiomatosis, Sturge-Weber disease

Diagnosis: A combination of clinical findings and imaging Clinical features: Skin: Facial nevus ‘port wine stain’ ‘nevus flammeus’, may be absent. In the distribution of trigeminal nerve Eye: Congenital glaucoma, bupthalmus, cloudy enlarged cornea Findings on Investigations: CT: Calcification of the cortex Cortical atrophy MRI: Venous hemangioma of the meninges in the occiptal region. Underlying … Continue reading Encephalofacial Angiomatosis, Sturge-Weber disease

Isolated Facial palsy, CN VII

Upper motor neuron lesion: Diagnosis: This is a clinical diagnosis. The underlying cause is determined by investigations and clinical correlation. Clinical features: Sparing of the forehead muscles Asymmetry of face at rest or on movement: smile, puffing the cheeks and on wincing The palpebral fissure may be widened on the affected side Investigations to consider: … Continue reading Isolated Facial palsy, CN VII

Acquired Oculomotor Apraxia

Synonyms: a.k.a. Roth-Bielschowsky syndrome (supranuclear palsy of gaze due to corticotectal/corticotegmental tract lesions) Diagnosis: A form of supranuclear palsy diagnosed clinically and by isolating the underlying cause Clinical features: Inability to perform horizontal saccades Vertical eye movements may be intact Oculocephalic reflexes are intact Caloric testing shows slow deviation without saccades Optokinetic nystagmus remains without … Continue reading Acquired Oculomotor Apraxia

Cavernous Sinus Syndrome

Diagnosis: This is a clinical diagnosis. Dysfunction of cranial nerves III, IV, and VI, and the superior divisions of cranial nerve V. Investigations to consider: Blood tests: FBC, U&E, fasting Glucose Vasculitic screen, ESR, CRP, ANCA, ANA, ENA SPEP, ACE Tests for infections: Borrelia serology, HIV MRI pre and post contrast: Pituitary tumors, meningioma, metastasis … Continue reading Cavernous Sinus Syndrome

Internuclear Ophthalmoplegia (INO)

Diagnosis: This is a clinical diagnosis. The underlying cause is determined by investigations Clinical features: Diplopia Classic variant, posterior INO: On lateral gaze: Failure of adduction of the contralateral eye, nystagmus of the abducting eye Medial rectus is less weak on testing each eye separately Anterior INO variant: Divergent eyes bilaterally, paralysis of both medial … Continue reading Internuclear Ophthalmoplegia (INO)

Abducens (cranial nerve VI palsy)

Diagnosis: This is a clinical diagnosis Clinical features: Horizontal diplopia In neutral gaze, normal appearance or head is slightly turned to the unaffected side (compensation for unopposed medial rectus) On movement: Affected eye fails to abduct Investigations to consider: Blood tests: ESR: giant cell arteritis TFT, glucose ANCA: Wegner’s granulomatosus HbA1c ESR CT: Out rules … Continue reading Abducens (cranial nerve VI palsy)

Trochlear Nerve Palsy (Cranial IV palsy)

Diagnosis: This is a clinical diagnosis Clinical features: Rare in isolation In neutral gaze: slight head tilted contralateral to the weak superior oblique muscle (loss of in-torsion) Test with eye adducted and moved inferiorly. This is also the position of worse diplopia If bilateral: on horizontal gaze, the abducting eye drifts downwards (inferior rectus of … Continue reading Trochlear Nerve Palsy (Cranial IV palsy)

Oculomotor Palsy (cranial nerve III palsy)

Synonyms: a.k.a. III nerve palsy Diagnosis: This is a clinical diagnosis. The underlying cause requires investigations. Clinical features: In neutral gaze: The eye is looking down & out. There is complete ptosis. On looking downwards torsional (in-torsion) nystagmus of the eye occurs (due to intact IV nerve action) Note the presence of meiosis: Normal pupil … Continue reading Oculomotor Palsy (cranial nerve III palsy)

Horner Syndrome

Synonyms: a.k.a. occulosympathetic defect Diagnosis: This is a clinical diagnosis plus pharmacological tests on occasion Clinical features: Miosis, partial ptosis, anhydrosis, illusion of enophthalmos, red eye Pharmacological test: Cocaine eye drops 2% in both eyes, failure to dilate diagnoses Horner’s syndrome. Dilation on adding amfetamine (Paradrine 1% (hydroxyamphetamine, most common) or Pholedrine 5% (n-methyl derivative … Continue reading Horner Syndrome

Alien Limb Syndrome

Synonyms: a.k.a. alien hand syndrome Diagnosis: This is a clinical diagnosis Clinical features: Inability to recognise ones limb once visual cues are removed or autonomous movements that are involuntary Also may have; apraxia, bimanual coordination difficulty, lack of goal directed activities Localisation: anterior corpus callosum, frontal lobe Investigations to consider: MRI: corpus callosum lesions e.g. … Continue reading Alien Limb Syndrome

Balint’s Syndrome

Diagnosis: This is a clinical diagnosis Clinical features: Ocular apraxia/ sticky fixation: the inability to move the eyes volitionally Optic ataxia: inability to reach for a target under visual guidance in the absence of primary visual deficits, patients are able to reach under auditory guidance. Simultinagnosia Investigations to consider: MRI: bilateral parietooccipital lesions Consider testing … Continue reading Balint’s Syndrome


Diagnosis: Neuropsychological/Psychometric testing Or Clinical features The underlying etiology is determined by a combination of clinical features, neuropsychological testing, imaging and laboratory testing   Clinical features: This is an acquired persistent disorder where there is an impairment of the content of consciousness (intellectual function) with compromise in at least 2, e.g.: Memory Language i.e. aphasia … Continue reading Dementia

Raised Intracranial Pressure, raised ICP

This is an abnormality that can occur due to multiple potential causes. It is treated based on the underlying cause and severity. Diagnosis: Intracranial pressure monitoring: Ventriculostomy (external ventricular drain EVD) Implantable ICP monitor Lumbar drain Raised opening pressure on Lumbar puncture (e.g. in cases of pseudotumor cerebri or meningitis). This should not be performed … Continue reading Raised Intracranial Pressure, raised ICP

Cerebral Edema

This is an abnormality that can occur due to multiple potential causes. It is treated based on the underlying cause and severity. Diagnosis: CT: Hypodensity Local effacement of sulci and ventricles and cisterns Blurring of the grey-white interface MRI: DWI: Allows differentiation between vasogenic (no abnormal diffusion restriction) vs. cytotoxic (abnormal diffusion restriction) types of … Continue reading Cerebral Edema

Vascular Parkinsonism

Diagnosis: Clinical features plus supportive MRI and poor response to dopamine agonists Clinical features: Lower body Parkinsonism. Variable cognitive & behavioural deficits. May accompany vascular dementia Findings on Imaging: MRI: multiple subcortical infarcts +/-subcortical leukoaraiosis Treatment: Supportive care Address vascular risk factors Related articles: Approach to movement disorders, Idiopathic Parkinson disease, vascular dementia, ischemic stroke,

Vascular Dementia

Diagnosis: Vascular dementia VaD, NINDS AIREN criteria for probable disease: Dementia +evidence of cerebrovascular disease +a relationship between them Evidence of cerebrovascular disease: Clinical features: Exam showing evidence of previous Also, a triad of: short shuffling gait “marche au petit pas”, pseudobulbar palsy, pseudobulbar affect can occur. MRI (1st choice) or CT: Large vessel disease: Bilateral … Continue reading Vascular Dementia

Spinal Cord Infarction

Synonyms: a.k.a. anterior spinal artery syndrome: Clinical features: Back pain +sudden paraplegia with areflexia & urinary retention, loss of touch, temperature, Usually spares proprioception & vibration Findings in Investigations: +MRI: may be negative initially Hyperintensity in the spinal cord, slightly expanded spinal cord later on Note: T1-T4 & T5-T7 are the vascular boundary zones Investigations … Continue reading Spinal Cord Infarction

Postural orthostatic tachycardia syndrome (POTS)

Diagnosis: 15-45 year old, F>M, Orthostatic symptoms upon standing: Palpitations, light-headedness, blurred vision, anxiety, substernal chest pain, fatigue, and occasionally syncope +increase of heart rate >30 beats/min within 5 minutes standing or tilt up: usually HR =or >120 +without a significant drop in blood pressure Full autonomic nervous function testing: sensitive and specific for diagnosis … Continue reading Postural orthostatic tachycardia syndrome (POTS)

Carotid Sinus Syncope

Diagnosis: A type of neurally mediated syncope A clinical diagnosis. Occasionally carotid sinus massage is used. Carotid sinus massage: False positives occur i.e. if positive it is not necessarily the cause of syncope Observe contraindications & procedural care Must have negative results for other causes of syncope Positive if with 5 seconds of unilateral longitudinal … Continue reading Carotid Sinus Syncope

Simple Faints

Synonyms: a.k.a. neurally mediated syncope a.k.a. Vasovagal syncope a.k.a. neurocardiogenic syncope a.k.a. vasodepressor syncope: Clinical features: Syncope after emotional stimulus Syncope after Valsalva & Valsalva like: Cough, defaecation, Micturation, Deglutition, Hyperventilation Syncope after hemodynamic/orthostatic stress e.g. prolonged standing Preceded by autonomic activation: Cold sweat: piloerection, sweating & pallor (vasoconstriction) Nausea & epigastric discomfort (vagal activation) … Continue reading Simple Faints

Orthostatic Hypotension

Synonyms: a.k.a. postural hypotension Diagnosis: Definition: Drop in BP >30 mmHg systolic or >10 mmHg diastolic when changing from lying to standing (not sitting), after 2-5 minutes of standing Or ≥20 mm Hg (or ≥20%) drop in systolic pressure, either immediately or after 2 min of standing. If negative but symptomatic, do orthostatic stress test: … Continue reading Orthostatic Hypotension

Subclavian Steal Syndrome

Diagnosis: Clinical features plus imaging Clinical features: Difference in BP between arms Findings on Investigations: Vertebral ultrasound: Reversal of flow in vertebral artery Catheter angiography: Subclavian stenosis or occlusion With reversal of flow in vertebral artery Treatment: Treat only if symptomatic Angioplasty or stenting of subclavian artery Occlusion of vertebral artery Carotid subclavian bypass Related … Continue reading Subclavian Steal Syndrome

Lymphomatoid granulomatosis (LG)

Diagnosis: Suggested by MRI and confirmed by pathology (brain biopsy/resection) Pathology: A type of diffuse large B cell nonHodgkin’s lymphoma B lymphocytis, granuloma involving the vessels (angiocentric & destruction of vessels) & brain parenchyma. Reactive T cells, Histiocytes EBV positive B cells Infarcts Clinical features: Affects lungs & skin, CNS, Peripheral nervous system (cranial & … Continue reading Lymphomatoid granulomatosis (LG)

Intravascular B cell Lymphoma

Synonyms: a.k.a. neoplastic angioendotheliosis a.k.a. angiocentric lymphoma a.k.a. angiotropic malignant lymphoma Diagnosis: Suggested by MRI and angiography, and confirmed by pathology (CSF, brain biopsy) Clinical features: Encephalopathy, dementia, seizures Strokes, myelopathy Can affect any organ: most commonly CNS & Skin or fever of unknown origin Pathology: NonHodgkin lymphoma, B-cell Confined to blood vessels only, doesn’t … Continue reading Intravascular B cell Lymphoma

Dolichoectasia of intracranial arteries

Synonyms and related terms: Vertebrobasilar dolichoectasia, Vertebral artery dolichoectasia, basilar artery dolichoectasia, internal carotid artery dolichoectasia Diagnosis: Clinical: Asymptomatic, or nerve palsies e.g. XII, Associated with longstanding uncontrolled hypertension or family history CTA: Ectatic and elnongated intracranial arteries, may be confused with fusiform aneurysms, may be associated with aneurysm in the same patient, Typically in … Continue reading Dolichoectasia of intracranial arteries

Vertebral Artery Stenosis, Extracranial

This section refers to extracranial vertebral artery stenosis due to atherosclerotic disease Diagnosis: Must exclude other etiologies of stroke: including intracranial atherosclerotic disease Catheter angiography: Gold standard test Allows accurate determination of degree of stenosis and collateral pathways Allows determination of intracranial disease MRA CTA Ultrasound: is if insufficient sensitivity to exclude the diagnosis Treatment: … Continue reading Vertebral Artery Stenosis, Extracranial

Vertebral Artery Stump Syndrome

Diagnosis: Ongoing ischemic events in the territory supplied by the vertebral artery despite occlusion of the vertebral artery Must be differentiated from intracranial atherosclerotic disease (ICAD), small vessel disease and other competing stroke mechanisms Pathophysiology: Emboli travel to the posterior circulation in the distribution of the affected vertebral artery via collaateral pathways. Treatment: Medical therapy: … Continue reading Vertebral Artery Stump Syndrome

Carotid Stump Syndrome

Diagnosis: Ongoing ischemic events in the territory supplied by the internal carotid artery despite occlusion of the internal carotid artery Must be differentiated from intracranial atherosclerotic disease (ICAD), small vessel disease and other competing stroke mechanisms Pathophysiology: Emboli travel to the ipsilateral brain or retina in the distribution of the affected internal carotid artery via … Continue reading Carotid Stump Syndrome

Ischemic Stroke with Patent Foramen Ovale

This section will summarize ischemic stroke with Patent foramen ovale (PFO) or atrial septal aneurysm Diagnosis: This Patent foramen ovale is associated with cryptogenic stroke, but is not the major cause of cryptogenic stroke. Consider this potential etiology in patients with non-lacunar stroke after investigations for intracranial atherosclerosis, extracranial atherosclerosis, atrial fibrillation (including long term … Continue reading Ischemic Stroke with Patent Foramen Ovale

Varicella Zoster Virus VZV vasculopathy

Diagnosis: Clinical: large artery or small vessel ischemic stroke, recurrent, protracted course. Rash may be absent or occur months before MRI, CT: large artery or small vessel ischemic stroke, typically at grey-white junction. Imaging is sensitive but not specific Anti-VZV IgG & VZV PCR: IgG is more sensitive for VZV vasculopathy, also reduced serum/CSF ratio … Continue reading Varicella Zoster Virus VZV vasculopathy

Subcortical Arteriosclerosis Encephalopathy

Now considered a subtype of Subcortical Vascular Dementia when cognitive dysfunction is present. It is the result of longstanding small vessel disease, particularly due to uncontrolled hypertension. Synonyms: Binswanger disease a.k.a. subcortical arteriosclerosis encephalopathy: Diagnosis: MRI T2/FLAIR: subcortical white matter hyperintensities in confluent areas (initially multifocal), more prominant periventricularly, usually with evidence of previous lacunar … Continue reading Subcortical Arteriosclerosis Encephalopathy

Primary Angiitis of the Central Nervous System

Synonyms: Primary angiitis of the central nervous system PACNS a.k.a. Granulomatous angiitis of the CNS GANS a.k.a. isolated CNS vasculitis Diagnosis: Brain +Meningeal biopsy: 1 cm wedge biopsy with meninges, grey & white matter Small or medium sized vessels. Parenchymal or meningeal Transmural destruction. Granulomatous (multinucleated cells) or lymphocytic infiltrate. Sometime macrophages. Intimal proliferation may … Continue reading Primary Angiitis of the Central Nervous System

Reversible Cerebral Vasoconstriction Syndrome

Synonyms: Reversible cerebral vasoconstriction syndrome RCVS a.k.a. Call-Fleming syndrome a.k.a. benign angiopathy of the CNS a.k.a. thunderclap headache with vasoconstriction Diagnosis: Clinical: Female >Male, thunderclap headache with out without neurological symptoms. Hemiplegia, visual field defects, May be postpartum +Imaging Findings on investigations: CT: infarcts usually posterior/watershed or hemorrhagic transformation, no SAH MRI: infarcts usually posterior/watershed … Continue reading Reversible Cerebral Vasoconstriction Syndrome

Cervical Artery Dissection

Synonyms: Cervical artery dissection: Internal Carotid artery dissection ICAD a.k.a. carotid dissection AND Vertebral artery dissection VAD Diagnosis: A combination of MRI dissection protocol and catheter angiography is best Findings on Investigations: MRI axial T1: False lumen= intermural hematoma (high signal, appears after 48hrs) compressing the true lumen (signal void). [274] Conventional spin echo MR: … Continue reading Cervical Artery Dissection

Radiation Vasculopathy

Diagnosis: MRI: Infarcts Enhancing mass lesion Cavernous marlformations Angiogram/Catheter angiography: Stenosis in MCA, ICA Can also cause accelerated atherosclerosis in the extracranial internal carotid artery Treatment: Depends on presentation: Enhancing mass, typically treated with steroids Infarcts are treated with antiplatelets, but data is limited Extracranial accelerated atherosclerosis is treat with antiplatelets, risk factor control and … Continue reading Radiation Vasculopathy

Fibromuscular Dysplasia

Synonyms: Fibromuscular dysplasia FMD, Diagnosis: Catheter angiography: Affects: extracranial vessels e.g. carotid artery Dilated portions (larger than normal parent vessel) & stenotic portions +/-Pseudoaneurysm Type 1: ‘string if pearls’ beaded appearance Type 2: tubular stenosis (focal or multifocal) Type 3: limited to part of the arterial wall False negatives with MRA, CTA and ultrasound but … Continue reading Fibromuscular Dysplasia

Intracranial Atherosclerotic Disease

Synonyms: Intracranial atherosclerotic disease ICAD a.k.a. Intracranial stenosis & intracranial atherosclerosis Diagnosis: Catheter angiography: WASID intracranial stenosis criteria: 50% stenosis by WASID criteria: Measure the diameter of the residual lumen Measure the normal reference diameter (one of the following in descending order of preference): 1 Same artery proximal to the stenosis 2 Same artery distal … Continue reading Intracranial Atherosclerotic Disease

Carotid Artery Stenosis, Extracranial

This section discusses extracranial internal carotid artery stenosis due to atherosclerotic disease. Diagnosis: Catheter angiography DSA digital subtraction angiography: Gold standard test NASCET measurement: N= narrowest diameter of residual lumen D= diameter of ICA well beyond the bulb Percentage stenosis= (1-N/D) X 100 Useful for: Suspected Total occlusion, Difficult anatomy, Fibromuscular dysplasia, Dissection, Disconcordant tests, … Continue reading Carotid Artery Stenosis, Extracranial

Cardioembolic Stroke

Diagnosis: Ischemic stroke with cardiac source found on investigations. Treatment: No use for anticoagulation  if not cardioembolic [TOAST] Choice of anticoagulant: Apixaban Dabigatran Warfarin orally, goal INR= 3.0-4.0 monitor every 2 weeks. Start Warfarin therapy without bridging [retrospective review] Other options: edoxaban, rivaroxaban Anticoagulate if: Atrial fibrillation [EAFT] Prosthetic valve: If recurrent on anticoagulation add … Continue reading Cardioembolic Stroke

Small Vessel Disease

This section will cover ischemic stroke due to small vessel disease Synonyms: Microangiopathic stroke a.k.a. microangipathic infarct ~Lacunar infarct Diagnosis: Clinical lacunar/thalamic syndrome: +HTN or DM +negative cardiac evaluation +negative large artery disease evaluation Note that cardiac embolism and large artery disease can cause imaging features consistent with lacunar infarcts Extensive work up is still … Continue reading Small Vessel Disease

Transient Ischemic Attack

Diagnosis: Sudden neurological focal deficit of vascular origin lasting <24 hrs (usually lasts <1 hr), with normal diffusion weighted MRI (DWI). PWI: may show decreased perfusion. Investigations to Consider: Blood tests: FBC, Coagulation screen, Blood Glucose, Blood chemistry panel, Fasting: Cholesterol, Lipids, glucose ESR: vasculitides, giant cell arteritis. Consider Homocystein, vasculitic screen, thrombophilia screen Extracranial … Continue reading Transient Ischemic Attack

Cerebral Amyloid Angiopathy

Patholgy: Brain Biopsy: Involves the cortex & overlying meninges, sparing the deeper structures. Histology: Congophilic angiopathy: amyloid in medium & small sized arteries. Dyshoric angiopathy: amyloid leaking from capillaries. Media & advantitia infiltration. H&E: eosinophilic acellular amorphous material (media & adventitia). Sometimes double barrel vessels (loss of smooth muscle & preservation of endothelium). Congo red … Continue reading Cerebral Amyloid Angiopathy

Cavernous Malformation

Synonyms: a.k.a. Cavernous hemangioma a.k.a. Cavernous angioma a.k.a. cavernoma Pathology: Dilated thin walled veins, without intervening brain tissue, surrounding gliosis & hemosiderin, no elastin in the walls Diagnosis: MRI: TI, T2 & FLAIR: Heterogenous mass (blood at different ages) with high signal on TI, T2 & FLAIR, low signal rim (hemosiderin) on T2 & FLAIR … Continue reading Cavernous Malformation

Spinal Vascular Lesions

Classification: Neoplastic vascular lesions: Hemangioblastoma Cavernous hemangioma Spinal aneurysms, rare Spinal Arteriovenous fistula, AVF Extradural Intradural: a.k.a. Foix-Alajouanine Ventral intradural: A. Small shunt B. Medium shunt C. Large shunt Dorsal intradural: A. single feeder B. multiple feeders Spinal Arteriovenous malformation AVM: Extradural-intradural Intradural: Intramedullary: Compact Diffuse Conus medullaris     Spinal Arteriovenous Fistula, spinal-AVF: Spinal … Continue reading Spinal Vascular Lesions

Capillary telangiectasia

Diagnosis: Clinical: Usually an incidental finding, very rarely [case reports] of bleeding. MRI: Pons ~3cm diameter T1 +contrast: Nodular enhancement after contrast Gradient echo GRE: hypointense, T2 isointense CT: invisible Angiography: Usually nothing is visible Pathology: Pons is the commonest location Microscopic Ectatic thin walled vascular channels (Capillaries) with interspersed normal appearing brain matter. Usually, … Continue reading Capillary telangiectasia

Sinus pericranii

Diagnosis: Clinical: Scalp mass that increases with valsalva or lying down & reduces with standing CT: Focal bone defect may be present Extracranial venous malformation that enhances heterogenously Cortical varix that strongly enhances CTV shows this well MRI: T1, T2: signal based on flow Contrast: Cortical Varix enhances well. Extracranial Venous malformation enhances heterogeneously MRV … Continue reading Sinus pericranii

Developmental Venous Anomaly

Synonyms: a.k.a. Venous malformations a.k.a. venous angioma Diagnosis: MRI: Linear structures with flow voids Dilated medullary veins: Around the ventricles, transcerebral course One large vein may drain into dural sinus or deep vein Enhance with contrast: caput medusae or umbrella shaped Features of associated cavernous malformation may be present Catheter Angiography: Normal arteries in size … Continue reading Developmental Venous Anomaly

Vein of Galen malformation

Synonyms: a.k.a. vein of Galen aneurysm a.k.a. Galenic varix   General points: This is a type of direct arteriovenous fistula AVF. It’s a misnomer because it involves the Median prosencephalic vein of Markowski. It is not an AVM because there is no nidus. It is not a dural arteriovenous fistula because it does not involve … Continue reading Vein of Galen malformation

Vertebral arteriovenous fistula

This section refers to Vertebral arteriovenous fistula (without dural involvement): Diagnosis: Clinical: Vertebrobasilar insufficiency, lower cranial nerve compression, neck pain, or asymptomatic Usually post trauma. Spontaneous fistula occur in fibromuscular dysplasia and neurofibromatosis. i.e. usually a complication of dissection Catheter angiography DSA: Arterial supply: Vertebral artery directly fills veinous plexus. Fistula point is usually at … Continue reading Vertebral arteriovenous fistula

Carotid Cavernous Fistula

Carotid cavernous fistulas are a type of dural arteriovenous fistula that involves the cavernous sinus. There are two types of carotid cavernous fistula (CCF): Direct carotid-cavernous fistula Indirect carotid-cavernous fistula   Classification (by catheter angiography): Barrow Type A i.e. direct CCF: direct ICA to Cavernous sinus shunt. i.e. no dural arterial branches are involved Type … Continue reading Carotid Cavernous Fistula

Dural Arteriovenous Fistula, Cranial

Clinical features: Wide range including: asymptomatic, pulsatile tinnitus, headache, cranial nerve palsy, cognitive deficits, hydrocephalus, focal deficits, seizures\ Pulsatile tinnitus, pulsatile exophthalmos Cranial neuropathy Encephalopathy, dementia, Parkinsonism In neonates/infants: heart failure Diagnosis: Catheter Angiogram: Use superselective catheterisation of 4 vessels, +external carotid Abnormal arteries and veins with shunting contained entirely within the dura (dural leaflets) … Continue reading Dural Arteriovenous Fistula, Cranial

Arteriovenous Malformations

Findings on Investigations: CT: Tangle of blood vessels: Serpentine or punctate high density on noncontrast Enhance with contrast Calcification may occur, hemorrhage may occur Hydrocephalus may occur with Vein of Galen malformation CTA: Arteries & draining veins are visible well, but angiography is still necessary MRI: Localises the lesion well. T1: curvilinear vessels T2: curvilinear … Continue reading Arteriovenous Malformations


Synonyms: Posterior communicating artery infundibulum: Diagnosis: CTA, MRA, catheter angiography: <3mm out pouching with an artery arising directly from the apex. Most commonly Posterior communicating artery Must be distinguished from very small aneurysms (=or<3mm) based on morphology

Cerebral Pseudoaneurysms

Subtypes: These can be Intracranial or extracranial Intracranial Pseudoaneurysm (Cerebral Pseudoaneurysm) Extracranial cervical artery pseudoaneursym (cervical pseudoaneurysm): Diagnosis: Focal dilation of a vessel wall that is not outlined by the layers of normal arterial wall. Usually, a hematoma that communicates with the vessel lumen. CT: Hematoma adjacent to vessel. Enhancing focus within a hematoma. CTA: … Continue reading Cerebral Pseudoaneurysms

Blister Like Pseudoaneurysm

Synonyms: Blood blister-like aneurysm a.k.a. blister-like pseudoaneurysm a.k.a. trunk aneurysm Pathology: Atherosclerosis with ulceration & hematoma A small defect in the vessel wall covered only with overlying fibrous tissue Atherosclerosis in parent vessel Diagnosis: Catheter Angiography: Small lateral wall bulge (asymmetric i.e. only one side of the lateral wall), hemispherical in shape Usually <10mm May … Continue reading Blister Like Pseudoaneurysm

Cerebral Aneurysms, Fusiform

Subtypes: There are two main subtypes: Non-atherosclerotic fusiform cerebral aneurysms (some authors include dissecting aneurysms here) Atherosclerotic fusiform cerebral aneurysms These are different from saccular cerebral aneurysms These are discussed in the two sections below: Non-Atherosclerotic fusiform cerebral aneurysms: Diagnosis of non-atherosclerotic fusiform aneurysms: Catheter Angiography: Long segment fusiform dilatation in absence of atherosclerosis, +ectatic … Continue reading Cerebral Aneurysms, Fusiform

Cerebral Aneurysms, Saccular

Please see other articles for fusiform cerebral aneurysms Synonyms: Saccular Intracranial aneurysms, Saccular Cerebral aneurysms, saccular aneurysms Diagnosis: Catheter angiography is the gold standard MRA and CTA can also be used as good screening tests Clinical features: Asymptomatic SAH signs and symptoms Occulomotor nerve palsy Trigeminal neuralgia Visual field cut (optic nerve compression) Cavernous sinus … Continue reading Cerebral Aneurysms, Saccular

Superficial Siderosis

 Synonyms: Central nervous system siderosis: Diagnosis: MRI: T2: dark outlining similar to GRE of all affected structures T2 gradient echo GRE: dark outlining to cerebellum, cerebellar folia, VIII cranial nerve & meninges & rest of the brain or spinal cord FLAIR: dark outlining similar to GRE of all affected structures T1: high signal on CNS … Continue reading Superficial Siderosis

Perimesenchphalic Subarachnoid Hemorrhage

Diagnosis: CT or MRI: blood in CSF space around the midbrain i.e. peri-mesencephalic. Usually in prepontine cistern or quadrageminal cistern. +Catheter Angiography: that excludes any cause of SAH. Especially posterior circulation aneurysm. +absence of trauma DDx. Aneurysmal subarachnoid hemorrhage due to ruptured posterior  inferior cerebellar artery (PICA) aneurysms 7% of Posterior circulation aneurysms may present … Continue reading Perimesenchphalic Subarachnoid Hemorrhage

Posterior Reversible Leukoencephalopathy Syndrome PRES

Synonyms: Reversible posterior leukoencephalopathy syndrome RPLS Diagnosis: CT: Hypodensity in affected areas, MRI is better. MRI: T2: Hyperintensity in the occipital and sometimes parietal areas bilaterally. Involves the white matter and sometimes the grey matter. Other affected areas; basal ganglia, cerebellum, brain stem, frontal lobes. Areas usually spared: calcarine cortex, paramedian occipital lobe DWI: hyperintense … Continue reading Posterior Reversible Leukoencephalopathy Syndrome PRES

Degos disease

Synonyms: Malignant atrophic papulosis Diagnosis: Clinical: Skin, brain & bowel vasculitis Skin: initially red papules then, umbilicated papules, +white centre & telangiectatic rim Bowel: GI bleed, bowel perforation MRI: Infarcts T2 hyperintensities, +small hemorrhagic areas T1 +GAD: dural enhancement may occur Cather Angiogram/angiography: Vasculitis features Peripheral aneurysms Also stenosis in GI arteries e.g. coeliac artery

Eales Disease

Diagnosis: Clinical: Retinal hemorrhages, retinal vasculitis, stroke Fundoscopy/slit lamp exam: Bilateral +Vascular sheathing (thin white lines along either side of the vessel) +Peripheral nonperfusion +Neovascularisation Microaneurysms, retinal hemorrhages, chorioretinal scars, tortuous vessels, Branch retinal vein occlusion Macular oedema Vitreous hemorrhages & debris, Fluorescein angiography: Leakage of dye in areas of vascular sheathing MRI: White matter … Continue reading Eales Disease

Susac’s syndrome

Synonyms: Retinocochleocerebral vasculopathy Diagnosis: Clinical: Encephalopathy (including psychosis), monocular vision loss, hearing loss Headache Branch retinal artery occlusion MRI: T2 hyperintensity in basal ganglia, thalamus, white matter (almost always involving corpus callosum). May involve brainstem & cerebellum T1 +contrast: leptomeningeal enhancement & parenchymal enhancement Ophthalmic fluorescein angiography: Retinal vasculitis: narrowing, occlusion, vessel wall hyperfluorescence, contrast … Continue reading Susac’s syndrome

Alagille syndrome

Synonyms: Arteriohepatic dysplasia Diagnosis: Genetics: Autosomal dominant JAG1 gene Chr. 20p, jagged1 Clinical features and investigations: Syndrome of involvement of Liver: LFTs: cholestasis, Ultrasound, MRCP: intrahepatic bile duct paucity +other systems including: Heart: coarctation of the aorta, aortic aneurysm Eyes: anterior chamber defects, posterior embryotoxon Face: frontal bossing, deep set eyes, bulbos nose tip, pointed … Continue reading Alagille syndrome

Cerebral Autosomal Dominant Arteriopathy and Subacute Infarcts with Leukoencephalopathy

Cerebral autosomal dominant arteriopathy and subacute infarcts with leukoencephalopathy (CADASIL) Diagnosis: Genetic testing: Notch 3 gene mutation on chromosome 19. Clinical including family history plus biopsy (skin or brain) +MRI: Diffuse white matter lesions including frontal lobes, temporal lobes (especially anteriorly), insula, deep & periventricular white matter. T2 hyperintense T1 hypointense Pathology: Skin biopsy: Electron … Continue reading Cerebral Autosomal Dominant Arteriopathy and Subacute Infarcts with Leukoencephalopathy

Postpartum Cerebral Angiopathy

Synonyms: Postpartum cerebral vasculopathy: Diagnosis: There are two forms: Idiopathic postpartum angiopathy a.k.a. Call-Fleming postpartum angiopathy Secondary postpartum cerebral angiopathy: Due to bromocriptine (used for lactation suppression), ergot alkaloids (used for control postpartum hemorrhage), sympathomemimetics, nasal decongestants Clinical features: Headache, seizure, encephalopathy within 1 month of delivery Normotensive (normal BP), no proteinuria MRI: In the … Continue reading Postpartum Cerebral Angiopathy

Cerebral Venous Sinus Thrombosis

Synonyms: Cerebral venous thrombosis CVT or cerebral venous sinus thrombosis/dural sinus thrombosis CVST and also Cortical cerebral venous thrombosis:   Diagnosis: By neuroimaging including CT venography, MR venography or catheter angiography revealing thrombus in the cerebral venous sinuses. May be clinically symptomatic or asymptomatic   Pathology: Any combination of the following may be involved: Venous … Continue reading Cerebral Venous Sinus Thrombosis

Moyamoya disease

Synonyms: Moyamoya disease a.k.a. idiopathic progressive arteriopathy of childhood a.k.a. spontaneous occlusion of circle of Willis: Diagnosis: Moyamoya syndrome is an angipathic pattern, not a specific disease. It has primary & secondary causes Moyamoya disease referes to the idiopathic condition that represents primary moyamoya syndrome   Clinical features: Bimodal age of onset: children ~5 years … Continue reading Moyamoya disease

Subarachnoid Hemorrhage

Synonyms: Subarachnoid haemorrhage Diagnosis: Findings on Investigations: Diagnosis is established by neuroimaging (CT or MRI) or by lumbar puncture Non-contrast CT: Demonstrates subarachnoid hemorrhage in the acute phase. May be negative, but is positive in the majority of cases. If CT is negative LP is indicated. Blood in CSF spaces: sulci & cisterns. May also … Continue reading Subarachnoid Hemorrhage

Intracerebral Hemorrhage

This section will discuss intracerebral hemorrhage; the commonest type of hemorrhagic stroke. Hemorrhagic stroke is a broad category of stroke and includes intracerebral hemorrhage and subarachnoid hemorrhage. Together these two conditions represent 13-20% of stroke cases with the rest being due to ischemic stroke. Intracerebral hemorrhage is more common than subarachnoid hemorrhage with the later … Continue reading Intracerebral Hemorrhage

Ischemic Stroke

Ischemic stroke is the commonest form of stroke. It represents infarction of the brain or spinal cord due to interruption of blood supply. There are many causes including embolism of material such as thrombus, cholesterol or rarely other material. Other mechanisms include thrombosis and miscellaneous causes of interruption of blood flow. The symptoms vary depending … Continue reading Ischemic Stroke