Vascular Syndromes

The areas supplied by the most commonly affected arteries and the deficits after their occlusion are described in the following sections.

Cerebral Vascular Syndromes

The cerebral cortex is supplied by the following arteries.

The anterior cerebral artery: This artery and its branches supply blood to the medial and dorsal aspect of the frontal and parietal lobes of the cerebral hemisphere, including the postcentral gyrus (which is
concerned with the processing of sensory information from the contralat-eral leg) and precentral gyrus (from which motor control of the contralateral leg originates). The regions of the cerebral cortex supplied by the
anterior cerebral artery are shown in Figure 27-7 (see also Fig. 4-2).

FIGURE 27-7 The coronal section through cortex showing the territories supplied by the anterior cerebral (blue), middle cerebral (red), and posterior cerebral (green) arteries.

The middle cerebral artery: This artery and its branches supply blood to the precentral gyrus (primary motor area), premotor region, the somesthetic and auditory areas, and the integrative association areas of the
parietal lobe. The cerebral cortical areas supplied by the middle cerebral artery are shown in Figure 27-7 (see also Fig. 4-3).

The posterior cerebral artery: This artery supplies most of the midbrain and thalamus as well as the subtha-lamic nucleus (Fig. 27-7; see also Fig. 4-4). After this artery passes around the midbrain, three major
branches arising from it (i.e., anterior and posterior temporal and parieto-occipital branches) supply the temporal and inferior occipital lobes of the cortex (Fig. 4-4). A branch of the posterior cerebral artery, called the
calcarine artery (Fig. 4-4), supplies the primary visual cortex.
The following syndromes are caused by the interruption of the blood flow in the above-mentioned arteries.

Anterior Cerebral Artery Syndrome : is caused by interruption of blood flow in the trunk of one of the anterior cerebral arteries, which damages the precentral gyrus and results in contralateral paralysis
(contralateral hemiplegia), mainly of the leg. Occlusion of both anterior cerebral arteries produces bilateral paralysis, which is pronounced in the lower limbs. Ischemic damage to the postcentral gyrus results in
impaired sensation, mainly to the leg.

Middle Cerebral Artery Syndrme : is caused by occlusion of the main trunk of this artery, which results in contralateral hemiplegia predominantly in the upper extremities and face because the upper limb
region is represented in a l ateral position of the precentral gyrus.If the left hemisphere is involved, aphasia (disturbances in speech) may occur because the speech centers are located laterally in this hemisphere.

Posterior Cerebral Artery Syndrome: is caused by interruption of the calcarine branch of the posterior cerebral artery, which supplies both the upper and lower banks of the visual cortex, which results in
hemianopsia (see Figs. 4-4 and 16-10). CT scans taken 6 and 48 hours after the onset of a right homonymous hemianopsia, due to the embolization of the left posterior cerebral artery, are shown in Figure 27-2, A and
B, respectively. A subtle loss of gray-white contrast and marked hypodensity of gray and white matter in the infarcted left visual cortex are visible after 6 and 48 hours of the onset of hemianopsia, respectively. Other
symptoms are described below under "Webers Syndrome."

Midbrain Vascular Syndromes

Arterial supply of different regions in the midbrain is schematically shown in Figure 27-8. The posterior cerebral arteries arise at the terminal bifurcation of the basilar artery (See Fig. 4-1). Occlusion of branches
of the posterior cerebral arteries that supply the midbrain results in the following midbrain vascular syndromes.

FIGURE 27-8 Arterial supply of the midbrain (schematic representation). Important structures are shown on the left and the pattern of arterial blood supply is shown on the right side of each diagram. BP = basilar pons; CC = crus cerebri; DecSCP =
decussation of the superior cerebellar peduncle; IC = inferior colliculus; LGNu = Lateral geniculate nucleus; MGNu = Medial geniculate nucleus; ML = medial lemniscus; RNu = Red nucleus; SC = superior colliculus; SN = substantia nigra.

Webers Syndrome : is also called medial midbrain syndrome and results from occlusion of paramedian branches of the posterior cerebral artery causing unilateral damage to the ventral region of the midbrain. It is
characterized by ipsilateral paresis of adduction and vertical gaze, pupillary dilation (due to damage to the oculomotor nerve), and paresis of the contral-ateral face, arm, and leg (due to damage to the corticospinal
and corticobulbar tracts.

Claudes Syndrome

Unilateral damage to the tegmental region of the midbrain causes Claudes syndrome. It is characterized by ipsilateral oculomotor paresis (due to damage to the oculomotor nucleus) and contralateral ataxia and
tremor (due to damage to the superior cerebellar peduncle).

Parinauds Syndrome : is also called gaze palsy syndrome, sylvian aqueduct syndrome and midbrain syndrome. Dorsal midbrain lesions cause this syndrome, which is characterized by impaired upward vertical
gaze and loss of the pupillary light reflex (due to damage to the pretectal area.
Benedikts Syndrome is caused by combined lesions of the ventral and tegmental midbrain. It is characterized by oculomotor paresis (due to damage to the rootlets of the oculomotor nerve), ataxia, and weakness
(due to damage to the region of the red nucleus and superior cer-ebellar peduncle.

The superior cerebellar artery arises just caudal to the bifurcation of the basilar artery (see Fig. 4-1). This artery supplies the rostral pons, caudal midbrain, and the superior surface of the cerebellum (Fig. 27-8).
Occlusion of this artery results in the following midbrain syndrome.

Superior Cerebellar Artery Syndrome : syndrome include: (1) analgesia and thermoanesthesia on the ipsilateral side of the face due to damage to the nucleus and spinal tract of the trigeminal nerve; (2) ipsilateral
Horners syndrome, which is characterized by a triad of symptoms, including constriction of the ipsilateral pupil, drooping of the upper eye lid, and sinking in of the eyeball (in this syndrome, there is a disruption of
the descending sympathetic pathways that supply the eye, resulting in unopposed parasympathetic effects on the eye); (3) contralateral loss of sensations of pain and temperature due to damage to the spinothalamic
tract; and (4) ipsilateral limb and gait ataxia due to damage to the superior cerebellar peduncle and adjoining cerebellar efferents, respectively.

Pontine Vascular Syndromes

The arterial supply of different regions of the pons is shown in Figure 27-9. The arteries supplying the pons include paramedian and long circumferential branches of the basilar artery and branches of anterior
inferior and superior cerebellar arteries (see Fig. 4-1). The pontine paramedian arteries arise from the basilar artery and supply the medial portion of the caudal and rostral pons. The short circumferential arteries
supply a wedge-shaped area in the ventrolateral pons. The long circumferential arteries supply most of the tegmentum of the rostral and caudal pons and lateral portions of the mid-brain tegmentum. The anterior
inferior cerebellar artery (AICA) supplies the ventral surface of the cerebellum and lateral portions of the caudal pons. Occlusion of different arteries supplying the pons elicits the following syndromes

-------------Focal Vascular Syndromes of the midbrain, pons and medulla

Brainstem syndromes (Neuroanatomic features):

Physical findings take center stage: The most effective method to approach a diagnosis in a patient with suspected brainstem disorder is to organize the differential diagnosis around the
objective physical exam findings.
Descending motor tracts have not crossed
Ascending sensory tracts have already crossed caudally
The long tracts are in close relation with lower motor neurons of the CN nuclei
CN innervate structures of the head and neck ipsilaterally
A process affecting the brain-stem long tracts on one side causes clinical deficits on the contralateral side of the body.
CN dysfunction is ipsilateral to lesion and hemiparesis or hemisensory loss if contralateral (crossed hemiplegia)
Reticular activating system if affected can produce alterations in level of consciousness or coma.
The 4th ventricle and cerebral aqueduct lie nearby; if affected can lead to obstructive hydrocephalus.
Rostral to caudal localization, and medial to lateral level localization:

o CN III and IV, or vertical gaze abnormality = midbrain lesion

 o CN VI or VII, or horizontal gaze palsy = pontine lesion

o CN VIII = pontomedullary lesion

o CN IX, X, XI, or XII = medullary lesion.

o CN V, facial sensory abnormalities can occur with lesions anywhere from the pons to the cervical spinal cord.

Long motor tracts tend to lie medial in the brain stem.

Long sensory tracts tend to lie laterally in the brain stem.
Somatic motor nuclei: CN III, IV, VI, XII.

Vertebrobasilar ischemia: Bilateral weakness, numbness, circumoral numbness. Dizziness, dysequilibirum, diplopia, dysarthria, dysphagia, nausea, and vomiting. Bilateral impaired vision.

Signs of midbrain dysfunction:

o CN III palsy - unilateral or bilateral pupillary dilation

o CN III and IV involvement - vertical gaze palsy

o Ataxia

o Crossed hemiplegia

o Flexor (decorticate) posturing

o Impaired consciousness

Signs of pontine dysfunction:

o Bilateral Babinski's signs

o Generalized weakness
 o Perioral numbness

o Facial tingling

o Bilateral upper or lower visual loss or blurring

o Irregular (apneustic) respiration

o Ocular bobbing

o Palatal myoclonus

o Abducens palsy or horizontal gaze palsy

o Bilateral small but reactive pupils

o Extensor posturing (decerebrate)

o Impaired consciousness

Signs of medullary dysfunction:

o Vertigo

o Ataxia

o Nystagmus

o Nausea, vomiting

o Respiratory arrest

o Autonomic instability
 o Hiccups.

Ocular Motor Syndromes

o Combined vertical gaze ophthalmoplegia: paresis of both upward and downward gaze. Both vertical saccades and pursuit are lost.

o Lesion is in rostral interstitial nucleus of the MLF (riMLF).

Cause: stroke in the rostral dorsal midbrain and PSP (vertical gaze limitation is an early finding). In CBD (vertical and horizontal gaze limitation is a late finding)

Etiology:

Gradual onset: In elderly, think PSP (DDx: Whipple disease. Look for oculomasticatory myorhythmia). Tumor (thalamic, pineal, mesencephalic), hdyrocephalus,
bulbar-onset ALS (TDP-43 positive inclusions), paraneoplastic brainstem encephalitis (Ma-2 antibodies); CJD

Abrupt onset: Stroke, Wernicke encephalopathy

Young patient: MS, lipid and lysosomal storage diseases (Niemann-Pick type C), Wilson disease, Kernicterus

DDX of combined vertical gaze ophthalmoplegia

o Associated with mental status changes caused by dysfunction of the reticular formation that lies adjacent to the vertical gaze generator in the rostral midbrain.

o Vertical Eye movements: Vertical saccades are controlled by cortical pathways descending to the rostral interstitial nucleus of the MLF at the junction between
midbrain and thalamus.

It comprises:

Nucleus raphe interpositus (RIP) = omnipause neurons

Rostral interstitial nucleus of the MLF (riMLF) = burst neurons

 Interstitial nucleus of Cajal (INC = neural integrator neurons)

o The brainstem control center for vertical gaze is located in the midbrain, just superior to the oculomotor nucleus.

o The rostral interstitial nucleus of MLF contains excitatory burst neurons for vertical and torsional saccade. It projects to the motor neurons innervating
the elevator muscles bilaterally in the case of upward gaze and mainly ipsilaterally in the case of downward gaze. The gaze-holding neural integrator for
vertical gaze is located in the interstitial nucleus of Cajal.

o Oculocephalic (doll's eye) maneuver is positive. VOR is present.

o Bell's phenomenon (reflex movement of the eyes up and out in response to forced eye closure) often is absent.

o Skew deviation (vertical misalignment of the eyes) may occur.

o Convergence is absent

o Loss of pupillary response to light occurs.

o Laboratory and diagnostic evaluation: CT head, MRI of brain/stem (look for lesion in brainstem and inferior to floor of 3rd ventricle); LP, syphilis serology, ESR, ANA. For
Whipple: small bowel bx (PCR), CSF Tropheryma whippelii PCR.

o Upgaze Paresis (Dorsal Midbrain or Parinaud syndrome)

o Downgaze Paresis

o Patient has difficulty looking down as in writing, reading, walking down the stairs.

o Loss of downward saccades and sometimes even pursuit. Vertical oculocephalic maneuver is intact or have limitations.

o Loss of convergence

o Gaze-evoked upbeat nystagmus in upward gaze.

 o Perform forced ductions in young patients to check for congenital restrictive limitation.

o Site of lesion for downgaze paresis is bilateral involvement of the lateral portions of the riMLF (rostra-interstital MLF)

o DDX: ischemic stroke (acute onset), PSP (slow, progressive in elderly) and Whipple disease

o Investigations: CT, MRI. Lesions inferior to the floor of 3rd ventricle (rostral mesodiencephalic junction).

o Internuclear Ophthalmoplegia

o Horizontal Gaze Paresis.

o Patients complain

Focal Vascular Syndromes of the midbrain, pons and medulla

Brainstem syndromes (Neuroanatomic features):
Physical findings take center stage: The most effective method to approach a diagnosis in a patient with suspected brainstem disorder is to organize the differential diagnosis around the
objective physical exam findings.
Descending motor tracts have not crossed
Ascending sensory tracts have already crossed caudally
The long tracts are in close relation with lower motor neurons of the CN nuclei
CN innervate structures of the head and neck ipsilaterally
A process affecting the brain-stem long tracts on one side causes clinical deficits on the contralateral side of the body.
CN dysfunction is ipsilateral to lesion and hemiparesis or hemisensory loss if contralateral (crossed hemiplegia)
Reticular activating system if affected can produce alterations in level of consciousness or coma.
The 4th ventricle and cerebral aqueduct lie nearby; if affected can lead to obstructive hydrocephalus.
Rostral to caudal localization, and medial to lateral level localization:
 o CN III and IV, or vertical gaze abnormality = midbrain lesion

o CN VI or VII, or horizontal gaze palsy = pontine lesion

o CN VIII = pontomedullary lesion

o CN IX, X, XI, or XII = medullary lesion.

o CN V, facial sensory abnormalities can occur with lesions anywhere from the pons to the cervical spinal cord.

Long motor tracts tend to lie medial in the brain stem.

Long sensory tracts tend to lie laterally in the brain stem.
Somatic motor nuclei: CN III, IV, VI, XII.

Vertebrobasilar ischemia: Bilateral weakness, numbness, circumoral numbness. Dizziness, dysequilibirum, diplopia, dysarthria, dysphagia, nausea, and vomiting. Bilateral impaired vision.

Signs of midbrain dysfunction:

o CN III palsy - unilateral or bilateral pupillary dilation

o CN III and IV involvement - vertical gaze palsy

o Ataxia

o Crossed hemiplegia

o Flexor (decorticate) posturing

o Impaired consciousness

Signs of pontine dysfunction:

o Bilateral Babinski's signs

 o Generalized weakness

o Perioral numbness

o Facial tingling

o Bilateral upper or lower visual loss or blurring

o Irregular (apneustic) respiration

o Ocular bobbing

o Palatal myoclonus

o Abducens palsy or horizontal gaze palsy

o Bilateral small but reactive pupils

o Extensor posturing (decerebrate)

o Impaired consciousness

Signs of medullary dysfunction:

o Vertigo

o Ataxia

o Nystagmus

o Nausea, vomiting

o Respiratory arrest
 o Autonomic instability

o Hiccups.

Ocular Motor Syndromes

o Combined vertical gaze ophthalmoplegia: paresis of both upward and downward gaze. Both vertical saccades and pursuit are lost.

o Lesion is in rostral interstitial nucleus of the MLF (riMLF).

Cause: stroke in the rostral dorsal midbrain and PSP (vertical gaze limitation is an early finding). In CBD (vertical and horizontal gaze limitation is a late finding)

Etiology:

Gradual onset: In elderly, think PSP (DDx: Whipple disease. Look for oculomasticatory myorhythmia). Tumor (thalamic, pineal, mesencephalic), hdyrocephalus,
bulbar-onset ALS (TDP-43 positive inclusions), paraneoplastic brainstem encephalitis (Ma-2 antibodies); CJD

Abrupt onset: Stroke, Wernicke encephalopathy

Young patient: MS, lipid and lysosomal storage diseases (Niemann-Pick type C), Wilson disease, Kernicterus

DDX of combined vertical gaze ophthalmoplegia

o Associated with mental status changes caused by dysfunction of the reticular formation that lies adjacent to the vertical gaze generator in the rostral midbrain.

o Vertical Eye movements: Vertical saccades are controlled by cortical pathways descending to the rostral interstitial nucleus of the MLF at the junction between
midbrain and thalamus.

It comprises:

Nucleus raphe interpositus (RIP) = omnipause neurons

 Rostral interstitial nucleus of the MLF (riMLF) = burst neurons

Interstitial nucleus of Cajal (INC = neural integrator neurons)

o The brainstem control center for vertical gaze is located in the midbrain, just superior to the oculomotor nucleus.

o The rostral interstitial nucleus of MLF contains excitatory burst neurons for vertical and torsional saccade. It projects to the motor neurons innervating
the elevator muscles bilaterally in the case of upward gaze and mainly ipsilaterally in the case of downward gaze. The gaze-holding neural integrator for
vertical gaze is located in the interstitial nucleus of Cajal.

o Oculocephalic (doll's eye) maneuver is positive. VOR is present.

o Bell's phenomenon (reflex movement of the eyes up and out in response to forced eye closure) often is absent.

o Skew deviation (vertical misalignment of the eyes) may occur.

o Convergence is absent

o Loss of pupillary response to light occurs.

o Laboratory and diagnostic evaluation: CT head, MRI of brain/stem (look for lesion in brainstem and inferior to floor of 3rd ventricle); LP, syphilis serology, ESR, ANA. For
Whipple: small bowel bx (PCR), CSF Tropheryma whippelii PCR.

o Upgaze Paresis (Dorsal Midbrain or Parinaud syndrome)

o Downgaze Paresis

o Patient has difficulty looking down as in writing, reading, walking down the stairs.

o Loss of downward saccades and sometimes even pursuit. Vertical oculocephalic maneuver is intact or have limitations.

o Loss of convergence
 o Gaze-evoked upbeat nystagmus in upward gaze.

o Perform forced ductions in young patients to check for congenital restrictive limitation.

o Site of lesion for downgaze paresis is bilateral involvement of the lateral portions of the riMLF (rostra-interstital MLF)

o DDX: ischemic stroke (acute onset), PSP (slow, progressive in elderly) and Whipple disease

o Investigations: CT, MRI. Lesions inferior to the floor of 3rd ventricle (rostral mesodiencephalic junction).

o Internuclear Ophthalmoplegia

o Horizontal Gaze Paresis.

o Patients complain of inability to see or to look to the side.

o Diplopia does not occur as supranuclear gaze pareses are conjugate.

o Lesions of PPRF results in loss of saccades and pursuit towards the affected side (right PPRF if affected > gaze paresis to right, or patient is unable to direct gaze to right).

o Full eye movements can be demonstrated by the oculocephalic maneuver (VOR).

o Lesions of CN VI nucleus results in horizontal gaze paresis that cannot be overcome by VOR (oculocephalic maneuver).

o Quite often an associated ipsilateral facial palsy is seen due to involvement of the fascicle of the CN VII coursing over the CN VI nucleus.

o Site of lesion for horizontal gaze paresis is PPRF, CN VI nucleus, frontopontine tract, mesencephalic reticular formation
Focal Vascular Syndromes of Medulla
Region Syndrome Vascular Supply Anatomical Clinical features
Structures
Medial medulla Dejerine Occlusion of anterior Hypglossal nucleus Ipsilateral CN XII, LMN weakness,
syndrome - spinal artery or and exiting CN XII atrophy, and fibrillation of the half of
alternating paramedian fascicles tongue - deviation of the protruded
hypoglossal branches of vertebral tongue towards the atrophic side.
hemiplegia artery.

Contralateral hemiparesis without facial

Pyramidal tract involvement, UMN (weakness,
hyperactive reflexes, Babinski's sign,
clonus, and spasticity).

Contralateral vibration and

Medial lemniscus proprioception sense and discriminative
touch.

.
Lateral medulla is Lateral Occlusion of the Inferior cerebellar Ipsilateral limb ataxia (lateropulsion) Ipsilateral paralysis of palate, pharynx,
the most medullary vertebral artery or peduncle (restiform and vocal cords, resulting in dysarthria,
common and syndrome less frequently, the body) hoarseness, dysphagia (due to damage
important (Wallenberg's) medial branch of the Vertigo, nausea of the nucleus ambiguus and issuing
syndrome of syndrome PICA. Vestibular nuclei fibers of glossopharyngeal and vagus
ICVA occlusion, Superior, middle, or nerves; vertigo, nausea, vomiting
and often missed inferior medullary Ipsilateral loss of pain and temperature (damage to vestibular nuclei) Ipsilateral
artery Spinal nucleus of the sensation in face - common. loss of pain and temperature sensation of
trigeminal nerve and face, ipsilateral Horner's
Vertebral thrombosis and its descending Corneal reflex is reduced in the syndrome, ipsilateral ataxia
is common cause. spinal tract ipsilateral eye. and dysmetria, and loss of pain and
temperature sensitivity over the
Damage is in the Loss of pain and temperature sensation contralateral torso and limbs.
dorsolateral medulla in the contralateral half of the body.
and inferior
cerebellar peduncle. Spinothalamic tract Dysphagia, hoarseness, and ipsilateral
 facial paresis are more common in
patients with lesions in the rostral
medulla.
Ipsilateral Horner's syndrome - late
finding Gait ataxia, vertigo, and nystagmus are
more common in patients with caudal
Descending medullary lesions.
sympathetic fibers
from the
hypothalamus

Hoarseness, dysphagia, and dysarthria

due to ipsilateral paralysis of palate,
vocal cord (loss of gag reflex)

Nucleus ambiguus Hiccuping that is of uncertain cause but

(CN 9, 10) issuing usually attributed to involvement of the
fibers respiratory center in the reticular
formation of the medulla.

Ipsilateral decreased taste

Nucleus solitarius Olivocerebellar climbing fibers to

cerebellum
Ocular lateropulsion

Babinski-Nageotte syndrome: Also known as medullary tegmental paralysis is a combined lateral and medial medullary syndrome. Lesion is at the pontomedullary
junction.
 o Ipsilateral Horner's syndrome (autonomic sympathetic fibers)

o Ipsilateral weakness of the soft palate, pharynx larynx (nucleus ambiguus); tongue (hypoglossal nucleus); loss of taste in the posterior third of the tongue
(nucleus soltarius); cerebellar ataxia (restiform body) and nystagmus (vestibular nuclei); and contralateral hemiparesis (pyramid) and hemianesthesia
(medial lemniscus).

Hemiplegia cruciata: Crossed motor hemiparesis; paralysis of ipsi arm & contralat leg due to lower medullary lesion compromising crossed fibers to arm &
uncrossed fibers to leg.