Occlusion syndrome

General

Internal carotid artery and its branches

  • Risk and extent of stroke is influenced by
    • Suddenness of occlusion
    • Location of occlusion
    • Presence of collateral circulation

Numbers

  • Acute ICA occlusion (all comers)
    • Results in 26–49% risk of stroke (18th century data)
      • Not all of these strokes are severe
  • 30-day risk of stroke recurrence after first stroke is
    • 3% at 30 days
    • 26% at 5 years.
  • Annual stroke risk with symptomatic ICA occlusion
    • 7% overall
    • 5.9% ipsilateral to the occlusion (mean follow-up= 45.5 mos)
      • Even with anticoagulation or antiplatelet drugs
  • St. Louis Carotid Occlusion Study
    • 2-year ipsilateral ischemic stroke rate in patients with symptomatic ICA occlusion is
      • 5% in patients with normal O2 extraction fraction (OEF) by PET scan
      • 26% in patients with increased OEF
  • Stroke risk is less when one includes asymptomatic ICA occlusions
    • (i.e., there are people walking around with ICA occlusion and no symptoms)
  • In patients presenting with ICA territory stroke or TIA, complete ICA occlusion is found in 10 - 15%

Worst-case scenario of total ICA occlusion with no a-comm or p-comm flow and no collateral rescue

  • Stroke in ACA and MCA territories
    • Deficitᵃ
      Complete (M1 Occlusion)
      Superior Division
      Inferior Division
      [CL] Weakness UE > LE
      X
      X
      [CL] Weakness of Lower Face
      X
      X
      [CL] Hemisensory Loss (UE & LE)
      X
      X
      [CL] Hemisensory Loss of Face (all modalities)
      X
      X
      [CL] Neglectᵇ
      X
      X
      [IL] Gaze Preference
      X
      [CL] Homonymous Hemianopsia
      X
      Xᶜ
      Receptive Aphasiaᵈ (Wernicke's area)
      X
      X
      Expressive Aphasiaᵈ (Broca's area)
      X
      X
      Gerstmann Syndrome: with dominant parietal lobe infarct
    • ᵃ[CL]: contralateral; [IL]: ipsilateral; ᵇwith involvement on side of nondominant hemisphere; ᶜplus [CL]: upper quadrantanopsia; ᵈwith involvement on the side of the dominant hemisphere. An “X” indicates that the deficit is present
  • Risk of stroke after TIA: 5% during the first month. 25% are estimated to develop a stroke within 2 years.
  • Diseases associated with Stroke
    • Disease
      Presentation
      Diagnostics
      Other
      Treatment
      Fibromuscular dysplasia (FMD)
      - Transient ischemic attack (TIA) or stroke/headache/vertigo/pulsating tinnitus
      - Stenosis/aneurysm /dissection
      - Affects also renal artery (causing hypertension and bruits)
      - "String of beads" appearance on angiogram
      - Irregularities in internal carotid artery (usually bilateral). Vertebral artery may also be affected
      1. Anticoagulation (usually aspirin)
      2. Angioplasty
      CADASIL (Cerebral Autosomal Dominant Arteriopathy with Sub-cortical Infarcts and Leukoencephalopathy)
      - Migraine headaches often with auras/TIA or stroke
      - Progressively: dementia/pseudobulbar palsy/ incontinence
      - Binswanger's-like lacunae but at an early age (lesions around basal ganglia, pons, periventricular white matter)
      - Familial. Chromosome 19 (Notch 3 gene)
      - Anticoagulants (with slightly increased risk of microhemorrhages)
      MELAS (Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like episodes)
      - Stroke (frequently occipital)
      - Multisystem disorder with childhood onset
      - Elevated arterial lactate and pyruvate - Mitochondrial DNA analysis
      - MRI: high T2 signal and cerebral atrophy - Muscle biopsy
      - Defective mitochondrial DNA (maternal inheritance)
      - Coenzyme Q10/Idebenone has been tried
      - Avoid valproic acid (impacts mitochondria)
      Prognosis: Progressive with poor prognosis
Stroke syndrome
Stroke syndrome
notion image

Location of occlusion

Oxford (Bamford) Stroke classification

Types
Vessels
Features
Total Anterior Circulation Stroke (TAC)
Large cortical stroke in MCA/ACA territory
1. Unilateral weakness (and/or sensory deficit) of face, arm, and leg 2. Homonymous hemianopia
3. Higher cerebral dysfunction (dysphasia, apraxia, astereognosis, reduced consciousness)
Partial Anterior Circulation Stroke (PAC)
Cortical stroke in MCA/ACA territory
Any two of the following:
1. Unilateral weakness (and/or sensory deficit) of face, arm, and leg 2. Homonymous hemianopia
3. Higher cerebral dysfunction (dysphasia, apraxia, agraphia)
Posterior Circulation Stroke
PCA and vertebrobasilar
Any one of the following:
1. Cerebellar or brainstem syndromes
2. Loss of consciousness
3. Isolated homonymous hemianopia
Lacunar Syndrome (LACS)
Small-vessel subcortical stroke
Any one of the following (without higher cerebral dysfunction):
1. Pure motor stroke
2. Pure sensory stroke
3. Mixed sensorimotor stroke
4. Ataxic hemiparesis

Carotid artery dissection

  • Presentation
      • Acute retro-orbital pain + Horner syndrome
      • Reduced conscious level
      • Contralateral homonymous hemianopia
      • Contralateral hemiplegia
      • Contralateral hemisensory disturbance
      • Gaze palsy- eyes deviate towards lesion
      • Partial Horner's syndrome
      • Global aphasia if dominant
      • Outcome depends on collateral blood supply
      notion image

Anterior cerebral artery

  • A1 syndrome
    • Rare due to collateral supply
  • Distal occlusion
    • Contralateral leg weakness, sensory loss +incontinence
      • Leg is in the medial surface of the cerebrum
  • Proximal bilateral occlusion
    • Paraplegia, sensory loss, incontinence, grasp and snout and palmomental reflex
  • Bilateral frontal lobe infarction- akinetic mutism

Posterior cerebral artery

  • Unilateral occipital lobe infarction → homonymous hemianopsia with macular sparing (visual cortex of the macula receives dual blood supply from MCA and PCA)
  • Balint syndrome
  • Cortical blindness (Anton syndrome)
  • Weber syndrome
  • Alexia without agraphia
  • Thalamic pain syndrome (Dejerine-Roussy syndrome)

MCA strokes

  • Complete MCA syndrome
    • Contralateral hemiplegia
    • Contralateral hemianaesthesia
    • Contralateral homonymous hemianopia
    • Gaze preference to ipsilateral side
    • If dominant-global aphasia
    • If non dominant- hemispatial neglect, anasognosia and constructional apraxia
  • Partial MCA syndromes
    • MI- occlusion lenticulostriate branches-
      • If ischaemia of internal capsule pure contralateral motor or pure sensory motor stroke
      • If ischaemia of putamen, pallidus predominantly parkinsonian features
    • M2- occlusion
      • If inferior division
        • Dominant: Wernickes dysphasia without weakness and contralateral superior quadrantinopia
        • Non- dominant: hemispatial neglect, spatial agnosia without weakness
      • If superior division
        • Brachial syndrome
        • Frontal opercular syndrome- Broca's dysphasia with facial weakness or a combination of both

Recurrent medial striate artery (of Heubner)

  • Supplies
    • Head of caudate
    • Anteroinferior internal capsule
  • Causes
    • Expressive aphasia AND
    • Mild hemiparesis
      • UE> LE
      • Proximal muscles weaker than distal

Anterior choroidal artery (AChA) syndrome

  • Supplies
    • Visual system
      • Inferior optic chiasm
      • Posterior portion of optic tract
      • Optic radiation
      • Lateral geniculate body
    • Temporal lobe
      • Uncus
      • Parahippocampal gyrus
      • Amygdala
    • Choroid plexus
      • Temporal horn
      • Atrium
    • Basal ganglia
      • Globus pallidus medial
      • Tail of caudate
      • Internal capsule (genu)
    • Diencephalon
      • Subthalamus
      • Thalamus
        • Lateral ventrolateral nucleus
        • Lateral ventro-anterior nucleus
    • Midbrain
      • Middle 1/3 of cerebral peduncle
      • Upper red nucleus
      • Substantia nigra
  • Complete form: Triad (mnemonic: 3 H’s)
    • CL Hemiplegia
      • Corticospinal tract
    • CL hemisensory loss (to light touch and pinprick; usually transient)
      • Spinothalamic tract
    • CL Homonymous hemianopsia
      • Optic tract
  • Incomplete form: are more common.
  • Aetiology
    • Small vessel disease
  • CT or MRI usually shows infarct in
    • Posterior limb of IC (just above temporal horn of lateral vent), AND
    • White matter posterior and lateral to it.
  • Occlusion is usually tolerated fairly well, and ligation of this artery was actually utilized in treatment of Parkinsonism sometimes without ill effect but internal capsule infarct occurred in ≈ 15%.

Artery of Percheron

  • Bilateral thalamic and mesencephalic infarctions

Posterior circulation (see brainstem)

  • Vertebral artery
    • Medial medullary syndrome (Dejerine syndrome)
    • Lateral medullary syndrome (Wallenberg syndrome)
  • Basilar artery
  • AICA: lateral pontine syndrome (Marie-Foix syndrome)
  • PICA: Sometimes lateral medullary (Wallenberg) syndrome
  • SCA: infarction of superior cerebellar vermis and superior cerebellum
  • Anterior spinal artery

Lacunar strokes

Definition

  • Small infarcts in deep noncortical cerebrum or brainstem

Mechanism

  • Due to
    • Fibrinoid necrosis or Lipohyalinosis → thickening of the tunica media
    • Atheroma plaques on the parent vessel occluding the origins of the penetrating small vessels

Location in decreasing frequency

  • Putamen
  • Caudate
  • Thalamus
  • Pons
  • Internal capsule (IC)
  • Convolutional white matter

Size of infarcts

  • Ranges from 3–20mm (CT detects larger ones; better sensitivity in white matter).
  • Small (3–7mm) multiple lacunes may be due to lipohyalinosis (vasculopathy due to HTN) of arteries < 200 microns (may also be cause of many ICHs);
    • This vasculopathy is indicative of small vessel disease, unlikely to be prevented by carotid endarterectomy.

Lacunar strokes is virtually excluded by

  • Aphasia
  • Apractagnosia
  • Sensorimotor stroke
  • Monoplegia
  • Homonymous hemianopsia (HH)
  • Severe isolated memory impairment
  • Stupor
  • Coma
  • LOC
  • Seizures

L’etat lacunaire

  • Multiple lacunes → chronic progressive neuro decline with one or more episodes of hemiparesis;
  • Causes
    • Invalidism
    • Dysarthria
    • Small-step gait (marche á petits pas),
    • Imbalance,
    • Incontinence,
    • Pseudobulbar signs,
    • Dementia.
  • Can be mis-diagnosed as NPH (unrecognized originally).

Lacunar syndromes

      Syndrome
      Site
      Vessel
      Symptoms
      Pure motor
      Post limb internal capsule
      Lenticulostriate
      Weakness contralateral face, arm, leg, dysarthria
      Pure sensory
      VPL thalamus
      Thalamogeniculate
      Numbness contralateral face and limbs
      Dysarthria
      Dorsal pons
      Basilar perforator
      Dysarthria, weakness of face and tongue, clumsy contralateral arm
      Ataxic hemiparesis
      Ventral pons
      Basilar perforator
      Mild hemiparesis with marked ataxia
      Dysarthria + facial weakness
      Anterior limb internal capsule
      Lenticulostriate
      Dysarthria, dysphagia, mutism with mild facial weakness
  • Pure sensory stroke or TIA
    • Most common lacunar manifestation
    • Isolated unilateral numbness of face, arm, and leg.
    • Only 10% of TIA go on to stroke.
    • Lacune in sensory (posteroventral) thalamus → CT detection is poor.
    • Dejerine-Roussy= rare thalamic pain syndrome that may develop late
  • Pure motor hemiparesis (PMH)
    • 2nd most common lacunar manifestation
    • Pure unilateral motor deficit of face, arm, and leg without sensory deficit, HH, etc.
    • Lacune in
      • Posterior limb of IC,
      • Lower basilar pons where corticospinal (CS) tracts coalesce,
      • Mid-cerebral peduncle (rare)
  • Ataxic hemiparesis
    • Contralateral PMH+ cerebellar ataxia of affected limbs (if they can move).
    • Lacune in
      • Basilar pons at junction of upper third and lower two–thirds
    • Clinical features
      • Dysarthria,
      • Nystagmus
      • Unidirectional toppling
      • Differential severity in face, arm, and leg possible because Corticospinal fibers are dispersed by nuclei in the pons (unlike compact pyramids and peduncle)
    • Variant: dysarthria-clumsy hand syndrome
      • Lesion in same location or genu of IC.
      • May be mimicked by a cortical infarct, but latter will have numb lips
  • Pure Motor Hemiparesis sparing the face
    • Lacune in
      • Medullary pyramid;
    • Clinical features
      • At onset, there may be vertigo and nystagmus (approaching lateral medullary syndrome)
    • Variant: thalamic dementia
      • Central region of one thalamus + adjacent subthalamus
      • Abulia, memory impairment+ partial Horner (miosis + anhydrosis)
  • Mesencephalothalamic syndrome
    • Aka
      • “Top o’ the basilar syndrome.”
    • Usually caused by embolus.
    • Infarct typically butterfly shaped and bilateral
    • Involving rostral brainstem and cerebral hemisphere regions fed by the distal basilar artery.
    • Clinical
      • III palsy,
      • Parinaud’s syndrome & abulia,
      • May have amnesia, hallucinations and somnolence, usually without significant motor dysfunction
  • Weber’s syndrome
    • Clinical features
      • CN3 palsy AND
      • Contralateral Pure Motor Hemiparesis (no sensory loss).
    • Due to
      • Occlusion of interpeduncular branches of basilar artery
      • Aneurysm of
        • Basilar bifurcation or
        • BA-SCA junction
    • Central midbrain infarction → disrupting cerebral peduncle and CN3 fascicles.
  • Pure Motor Hemiparesis with crossed VI palsy
    • Lacune in paramedian inferior pons
  • Cerebellar ataxia with crossed III palsy (Claude syndrome)
    • Lacune in dentatorubral tract (superior cerebellar peduncle)
  • Hemiballism
    • Due to
      • Infarct OR
      • Hemorrhage
    • Located in subthalamic semilunar nucleus of Luys
  • Lateral medullary syndrome
  • Locked-in syndrome
    • Bilateral PMH from infarct at IC, pons, pyramid or (rarely) cerebral peduncles