Clinical Approach to Brainstem Lesions

Maria Rosa Querol-Pascual

The brainstem consists of the midbrain, pons, and medulla. The cerebellum is attached to the
dorsal surface of the pons and upper medulla. The brainstem contains 9 of the 12 cranial nerves
and is crossed by ascending, descending, and cerebellar pathways and their nuclei as well as
the reticular formation. Numerous and rare crossed brainstem syndromes have been described
in recent years, many of them without clinical significance. The aim of this article is to provide
a brief clinical description of some conditions affecting the brainstem.
Semin Ultrasound CT MRI 31:220-229 © 2010 Elsevier Inc. All rights reserved.

T he brainstem is situated in the posterior cranial fossa and

is divided into 3 transverse regions. The most caudal is
the medulla, the middle or central part is the pons, and fina-
lly the most rostral portion is the midbrain or mesencepha-
lon. The brainstem is also organized longitudinally into 3
further regions: the roof or tectum, posteriorly, the basis or
basilar portion, anteriorly, and the tegmentum in the center.
Nine of the 12 cranial nerves are situated in the brainstem,
which is covered by the cerebellum and connected to it by the
cerebellar peduncles. The fourth ventricular cavity is located
between the tegmentum and the roof.1-5
The brainstem has 3 roles. The first is to provide transit
and procession nuclei for ascending and descending path-
ways that convey messages to and from the cerebrum, cere-
bellum, and spinal cord. The second is to play a part in
integrative functions, such as level of consciousness, the
sleep-wake cycle, muscle tone, posture, and respiratory and
cardiovascular control. The third relates to actions of the
cranial nerves, which are composed of sensory fibers termi-
nating in the brainstem nuclei and motor fibers originating in
the brainstem nuclei.1,6,7 Therefore, a small and single lesion
could produce severe and mixed deficits.2
Lesions of the brainstem may manifest as cerebellar, so-
matosensory, and motor symptoms as well as cranial nerve
dysfunction. The level of the lesion can usually be deter-
mined by the injured cranial nerve. The affected cranial nerve
or fascicles localize the lesion to the medulla, pons, or mid-
brain. Thus, if the glossopharyngeal, vagus, accessory, and
hypoglossal nerves (IX, X, XI, XII) are involved, the lesion lies
within the medulla; by contrast, if the cranial nerves affected
Neurology Department, Complejo Hospitalario Universitario de Badajoz,
Badajoz, Spain.
Address reprint requests to: Maria Rosa Querol-Pascual, Hospital Infanta
Cristina, Avda, De Elvas s/n, Badajoz, Spain. E-mail: rquerolp@
hotmail.com
are V, VI, VII, or VIIIl, the lesion is within the pons; and
finally, if the lesion is located in the midbrain, the cranial
nerves affected are III and IV.5,8
Most of these syndromes comprise ipsilateral cranial nerve
lesions and contralateral signs of long tract involvement, such as
hemiparesis or hemisensory or bilateral deficit. Other symptoms
that indicate brainstem disease include vertigo, unsteadiness of
gait or ataxia, dysarthria-clumsy Hand disease, blepharospasm,
hiccup, palatal myoclonus, respiratory dysfunction, a special
type of hallucinosis (peduncular hallucinosis), and conjugate
eye deviation towards hemiparesis (Table 1).8
Brainstem ischemia is the most frequent cause of acute
brainstem lesions, and most syndromes have been de-
scribed as a sequel of brainstem infarcts.9-10 Ischemic ver-
tebrobasilar strokes account for 23% of all first episodes of
ischemic brain strokes, and 48% of these affect the brain-
stem.11 Most ischemic brainstem strokes involve the pons
(27%), followed by the medulla (14%), and the midbrain
(7%).12-15 The most common mechanism of brainstem
stroke includes embolism and lipo hyalinosis.12 An arterial
dissection causes 20% to 30% of medullary strokes and
approximately 5% of mesencephalic strokes but is rather
rare in pontine strokes.14,16,17
Examples of nonvascular disorders that commonly affect
the brainstem include demyelinating disease (multiple scle-
rosis or acute disseminated encephalomyelitis), degenerative
disease, trauma, intraaxial neoplasms (such as brainstem gli-
omas/ependymomas), brainstem encephalitis (Bickerstaff’s
encephalitis), central pontine myelinolysis, Arnold–Chiari
malformations, and syringobulbia, which are multifocal and
unsuitable for topographic studies.18
Before describing the more common brainstem syn-
dromes, some anatomical features will be reviewed2,5:
● Descending motor tracts decussate in lower medulla;
● Ascending somatosensory tracts decussate in the middle
medulla.

220 0887-2171/10/$-see front matter © 2010 Elsevier Inc. All rights reserved.
doi:10.1053/j.sult.2010.03.004
Clinical approach to brainstem lesions 221

● Spinothalamic tracts (information on temperature and

● ipsilateral ataxia of the arm and leg.

● contralateral alteration of pain and

pain) occupy a more lateral position in medulla and

temperature (arm, leg and rarely

● ipsilateral alteration of pain and

pons than the medial lemniscus (information on stere-

Cranial nerves; V, VII, IX, and XI

● ipsilateral Horner’s syndrome
Lateral Structures

ognosis and position).

Sensory nucleus of the Vth

●

temperature on the face.

Most ascending spinocerebellar tracts are ipsilateral; and
and Deficits
Spinocerebellar pathways

Spinothalamic pathways ● Effects of the cranial nerves and their nuclei are ipsilat-

Sympathetic pathway;
eral except for IVth cranial nerve.

We shall now discuss briefly some clinical and neuroradio-

logical characteristics of the most common syndromes asso-
ciated with lesions of certain parts of the brainstem.
trunk)

Brainstem Syndromes
Mesencephalic Syndromes
vibration and proprioception

Medial longitudinal fasciculus

● Contraleral weakness (arm

The mesencephalon is the most rostral portion of the brain-

cranial nerve; IIIrd, IVth,

stem. It is located between the forebrain and the hindbrain.

Medial Structures

Motor nucleus and nerve

● ipsilateral internuclear

● ipsilateral loss of the

The mesencephalon has 2 main divisions: the tectum and the

and Deficits

● contralateral loss of

tegmentum. The tectum (“roof”) is the dorsal surface of the

ophtalmoplegia
Medial lemniscus

midbrain, which is composed of 4 rounded eminences

(arm and leg)
Motor pathway

known as the corpora quadrigemina (quadrigeminal plate):

VIth, XIIth

the posterior pair called the inferior colliculi, which have an

and leg)

auditory function, and the anterior pair, called the superior

colliculi, which have a visual function. The ventrolateral por-
Table 1 Cranial Nerve Names and Main Functions, Medial and Lateral Structures, and Their Deficits

tion of the tegmentum is formed by the cerebral peduncles.

The tegmentum contains 3 colorful structures: the periaq-
ueductal gray matter, the substantia nigra, and the red
Facial sensation, muscles of mastication

nucleus. There are 2 ocular nuclei and nerves associated

with the mesencephalon; the third and fourth cranial
Eye movements, pupil constriction

nerves (Fig. 1).5,19

muscles, facial expression, taste,

Pharyngeal muscles, salivation

laryngeal/pharyngeal muscles
Main Function

Ventral Mesencephalon Lesions

A unilateral lesion located in the ventral portion of the mes-
lacrimation, salivation

Parasympathetics org;

encephalon could involve the cerebral peduncles (corticospi-

Hearing, equilibrium

nal and corticobulbar tracts). Damage of the anteromedial

Tongue movement

structures causes dysarthria or dysphagia and contralateral

Eye movements

Eye movements

facial and upper-extremity weakness without accompanying

Head turning

sensory disturbances. Fibers in the corticospinal tract are

Olfaction

somatotopically arranged, with the fibers destined to the arm

Vision

medially placed and those to the leg laterally located, with the
trunk fibers in between.20 We can observe signs of third-
nerve and Edinger-Westphal nuclei involvement manifest as
a deviation of the ipsilateral eye downwards and laterally
VIII, vestibulocochlear nerve

IX, glossopharyngeal nerve

with pupillary dilatation and ptosis of the lid.2,12

XI, spinal accessory nerve

Conversely, more lateral mesencephalic lesions cause con-

Cranial Nerve

XII, hypoglossal nerve

tralateral hemiparesis that predominantly affects the lower

VI, abducens nerve
V, trigeminal nerve
IV, trochear nerve

extremities, loss of the contralateral vibration and joint posi-

I, olfactory nerve

VII, facial nerve

X, vagus nerve
III, oculomotor

tion sense (medial leminisci), and loss of pain and tempera-

II, optic nerve

ture sensors in the trunk and extremities (ascending spino-

thalamic tracts).
Adapted from Burger et al.12

Choreoathetosis, contralateral ataxia, and internuclear

ophthalmoplegia are produced by involvement of the red
nuclei, superior cerebellar peduncles, and medial longitudi-
nal fasciculus, respectively.
Brainstem

Lateral mesencephalic lesions can affect descending sym-

Midbrain

Medulla

pathetic tracts, producing Horner’s syndrome. If the medial

Pons

geniculate bodies are involved, auditory dysfunction could

occur as well.21
222 M.R. Querol-Pascual

Figure 1 Cranial nerve nuclei. (A) View of the cranial nerve nuclei that settle at the brainstem. It represents the location and
the direction of their axons. (B) dorsal view of the brainstem and cranial nerve nuclei location. Corpora quadrigeminal
(asterisks); lateral and medial geniculate bodies (arrows). The nuclei of cranial nerves III to XII lie in the brainstem, just in
front of the floor of the fourth ventricle and the Sylvian aqueduct. Four are located in the medulla oblongata (IXth, Xth, XIth,
XIIth); 4 in the pons (Vth, VIth, VIIth, and VIIIth) and 4 above the pons (3rd, 4th are in the midbrain). The 1st (olfactory) and
the 2nd (optic) are above the brainstem. The 4 motor nuclei that are in the midline of the brainstem are those that divides
equally into 12, that is, 3, 4, 6, and 12. They emerge through the anterior midline surface, except the IVth, which exits from
the dorsal part of the midbrain after its decussation in the superior medullary velum. Although the Vth, VIIth, and IXth cranial
nerve nuclei have motor function, they also have sensory components and do not divide evenly into 12. Thus, they are not
pure motor nuclei and, on this basis, they are not located medially. The VIIIth cranial nerve nucleus (entirely sensory) and
XIth (pure motor, which does not divide equally into 12), are located laterally.42 Salivatory and lacrimal nuclei. (Courtesy of
M. Ángeles Fernández-Gil.) (Color version of figure is available online.)

There are 3 classic eponyms describing these syn-
dromes10,12,20,22,23:
1. Weber’s syndrome involves the third nerve and cere-
bral peduncle, causing ipsilateral third nerve palsy with
contralateral hemiparesis (Fig. 2).
2. Benedikt’s syndrome involves the red nucleus, causing
ipsilateral third nerve palsy and contralateral chorea,
tremor or athetosis (Fig. 3).
3. Claude’s syndrome involves the superior cerebellar pe-
duncle, causing third-nerve palsy and contralateral
ataxia.
Dorsal Mesencephalic Lesions
Dorsal mesencephalic lesions produce mainly neuro-oph-
thalmologic abnormalities and are most often seen with hy-
drocephalus or tumors of the pineal region.6,20 These lesions
cause dysfunction of the IVth cranial nerve (trochlear), which
produces diplopia, slight elevation of the eye, vertical gaze
abnormalities from periaqueductal gray matter, and tinnitus
or auditory alterations from inferior colliculi.24 Bilateral mes-
encephalic lesions damaging the reticular formation can re-
sult in consciousness disturbances.2,12,14
There are 2 syndromes associated with mesencephalic le-
sions:
1. Parinaud’s syndrome, characterized by loss of up-
ward gaze, large and irregular pupils, eyelid retrac-
tion, convergence nystagmus and loss of accommo-
dation.
2. Top of the basilar syndrome, characterized by pupillary
and visual disorders, vertical gaze palsy, delirium and
hallucinosis, sensory deficits, and motor deficits. This
syndrome can result from giant basilar artery tip aneu-
rysms, vasculitis, or as a complication of cerebral an-
giography.20,25 Table 212 summarizes the mesence-
phalic syndromes.
Clinical approach to brainstem lesions 223

Figure 2 An 85-year-old patient exhibiting sudden right 3d cranial nerve palsy and light loss of strength in left
extremities (Weber’s syndrome). (A, B) Axial fluid-attenuated inversion recovery (FLAIR) and (C) coronal spin echo
(SE) T2-weighted images show a hyperintense area with imprecise margins affecting the right thalamus just next to the
wall of the 3rd ventricle (arrowheads) and the paramedian and anterior right region of mesencephalon (arrows)
consistent with ischemic insult. (D, E) Axial isotropic diffusion-weighted magnetic resonance images and (E, F)
Apparent diffusion coefficient maps show the typical imaging features of acute ischemic infarcts: hyperintensity on
isotropic images and hypointensity on Apparent diffusion coefficient maps (arrows).

Pontine Syndromes This vascular distribution is classified in 5 main clinic-topo-

The pons extends from the pontomedullary junction cau-
dally to the pontomesencephalic junction rostrally. The dor-
sal part of the pons is referred to as the tegmentum, and the
ventral portion is referred to as the basis pontis. The fourth
ventricle separates the pontine tegmentum from the cerebel-
lum. The pontine tegmentum contains important nuclei and
pathways (pontine reticular formation). The basis pontis
contains the corticospinal and corticobulbar tracts, cranial
nerve nuclei, and transverse cerebellar fibers.6,19,20 In the
pons there are important structures involved in several func-
tions. Cranial nerve nuclei are associated with ocular move-
ments, facial expression, mastication, salivation, equilibrium,
and audition.5 Fiber tracts, such as the paramedian pontine re-
ticular formation, the medial longitudinal fasciculus, the medial
lemniscus, the ventral spinocerebellar, spinothalamic, lateral
tectospinal, rubrospinal, and corticopontocerebellar tracts, au-
ditory connections and the middle cerebellar peduncle.
The main arteries that provide the blood supply to the
pons are branches of vertebral artery and basilar artery.6,12,19
graphic patterns: anteromedial, anterolateral, tegmental, uni-
lateral, and multiple pontine infarcts.12,13
Anteromedial/Anterolateral Syndromes
Lesions at this level can produce some of following symp-
toms, depending on the tract that is damaged. The tracts and
possible damage are listed to follow:
1. Corticobulbare tracts: involved in movement of the
eyes, face, pharynx, and tongue and produce dysar-
thria, dysphagia, or facial palsy.
2. Cortipontocerebellar tracts: ataxia or pathologic laugh-
ter associated with paresis constitutes the ataxic-hemi-
paresis syndrome; ataxia or pathologic laughter in con-
junction with dysarthria constitutes the dysarthria
clumsy-Hand disease.12,26
3. Corticospinal tracts: pure hemiplegia or hemiparesis
involving the face, the arm, and the leg are the most
common presentations of anteromedial syndromes.12
In addition, pure sensory syndromes or associated mo-
224 M.R. Querol-Pascual

Figure 3 A 75-year-old man with unilateral left IIIrd cranial nerve palsy and contralateral ataxia (Benedikt’s syndrome). (A, B)
Axial SE T2-weighted images show a high-intensity lesion that affects the midbrain in its medial and anterior region
(anteromedial or paramedian syndrome) consistent with ischemic infarct. The lesion involves the left 3rd cranial nerve
nucleus and tract as it travels near the red nucleus (black arrow). The lesion of the red nucleus interrupts fibers from the
opposite cerebellar hemisphere, which reach the red nucleus via the superior cerebellar peduncles that decussate in the
anterior midbrain (white arrow). (C) coronal FLAIR image shows the left midbrain ischemic lesion (arrowhead), as well as
the signal changes in juxta-ventricular white matter related to small vessel angiopathy typical in the elderly patient (arrows).

tor lesions are further manifestations of anteromedial
pontine syndrome.12,27
4. Medial lemnisci: these are responsible for vibration,
proprioception, and deep sensation from the contralat-
eral extremities.
5. Nuclei or fibers of cranial nerve VII: responsible for
ipsilateral facial palsy.
6. Fascicles of cranial nerve VI: responsible for ipsilateral
sixth nerve palsy producing diplopia, which is accen-
tuated when the patient looks toward the lesion.
7. Medial longitudinal fasciculus: responsible for internu-
clear ophthalmoplegia, which produces disconjugate
lateral gaze.
The medial zones of the corticospinal tract and the medial
leminsci transmit fibers to the upper extremities and the
more lateral fibers affect the lower extremities. In addition,
the lateral zones of the medial lemnisci predominantly
contain fibers to the lower extremities. The most severe
weakness and loss of position sense in the lower extremi-
ties could be produced by the involvement of the antero-
lateral region.12
Three syndromes are associated with these zones:
● Raymond’s syndrome, which is ipsilateral paresis of the
VI nerve and contralateral hemiparesis;
● Millar-Gubler’s syndrome, which is an ipsilateral palsy
of both the VI and VII cranial nerves and contralateral
hemiparesis; and
● Cheiro-oral syndrome, which is sensory loss in the peri-
oral region and contralateral hand.
Lateral Syndromes
Lateral syndrome lesions can produce the following problems:
● Contralateral ataxia, by damage to the inferior and mid-
dle cerebellar peduncles and pontocerebellar fibers;
Clinical approach to brainstem lesions 225

Table 2 Midbrain Syndromes

Midbrain Syndromes Structures Affected Signs and Symptoms
Anteromedial Corticospinal tract Contralateral
Corticobulbar tract ● Hemiparesia/hemiplegia
Red nucleus ● Dysartria
Decussation of superior cerebellar ● Choreoathetosis/tremor
peduncle ● Ataxia
Cranial nerve 3 fascicle Ipsilateral
● IIIrd nerve palsy
Anterolateral Corticospinal Contralateral
Medial spinothalamic tract ● Hemiparesia/hemiplegia
Portions of superior cerebellar peduncle ● sensory loss (pain and temperature) in extremities
● Ataxia
Lateral Descending sympathetic fibers Horner’s syndrome contralateral
Lateral spinothalamic tract ● Sensory loss (pain and temperature) in extremities
Dorsal Superior/inferior colliculi ● Vertical gaze palsies
Posterior commissure ● Loss of pupillary light reflex/loss of accommodation
Cranial nerve 3 nucleus Ipsilateral
Cranial nerve 4 nucleus ● IIIrd nerve palsy
Contralateral
● Superior oblique weakness
Bilateral Midbrain ● Vertical gaze palsies and cranial nerve 3 palsy
Thalamus ● Sensory finding and inattention
Medial temporal lobes ● Memory disturbances and aphasias
Occipital lobes ● Visual deficits
Adapted from Burger et al.12

● Loss of pain and temperature sensation in the contralat- Tegmental

eral extremities and trunk caused by damage from the
lateral spin thalamic tract excluding the face.
● Tinnitus and disturbances in hearing from lesions affect-
ing the lateral leminsci.
● Ipsilateral paresis of the face from the facial nuclei and
fascicles involvement.
● Ipsilateral motor deficits of muscles of mastication and
loss of ipsilateral corneal reflex from lesions of the tri-
geminal complex.
● VIth cranial nerve palsy.
Dorsolateral Syndromes
The characteristic clinical picture associated to these lesions
often comprises the following:
● Reduced auditory acuity and sound localization from
lesions of the lateral lemniscus or cochlear nucleus.
● Ataxia from lesions involving the superior cerebellar pe-
duncles.
● Parkinsonian symptoms from the loci cerulei.
● Ipsilateral jaw jerks from the mesencephalic nuclei of
the Vth cranial nerve.
There 3 syndromes associated with dorsolateral lesions:
● Marie-Foix syndrome, or ataxia, contralateral hemiparesis,
and contralateral hypoesthesia to pain and temperature.
● Foville syndrome, or ipsilateral horizontal gaze paresis, ip-
silateral facial palsy, and contralateral hemiparesis.
● Raymond-Cestan-Chenais syndrome, which includes
ataxia, contralateral loss of facial and body sensation,
and contralateral hemiparesis.
Lesions at the tegmental level are very unusual and consist of
disturbances of consciousness, severe ataxia, skewed devia-
tion of the eyes, VIth cranial nerve palsy, one and a half
syndrome, and vertigo.13,17,28,29
Bilateral Lesion
The most characteristic clinical symptomatology is the
pseudobulbar syndrome and the “locked-in syndrome.”13
The structures involved on both sides are the motor tracts
(corticospinal, corticobulbar, corticopontine), the fascicles of
the abducens nerves, paramedian pontine reticular formation
(horizontal gaze center), and reticular formation.12,13 Because
of this damage, the patient is unable to move (quadriplegia),
to speak (aphonia), and experiences bilateral palsy of the
facial nerve and horizontal gaze paresis. Consciousness may
be affected initially, although it is recovered later. If the spino-
thalamic tract is affected, there is loss of pain and temperature in
the trunk and extremities but not in the face. The patient can
communicate through vertical movements of the eyes and
blinking of the eyelids. This bilateral lesion in the basal portion
of the lower pons is known as “locked-in syndrome.”2 Table 312
summarizes the pons syndromes.
Medullary Syndromes
Medial Medullary Syndrome
Déjerine’s syndrome, a rare clinical entity, usually is pro-
duced by distal occlusion of the vertebral artery or the upper
portion of the anterior spinal artery.30,31 Lesions of the pyra-
midal tract in the medial medulla result in contralateral arm
and leg paresis, and sometimes (50% of cases) the contralat-
226 M.R. Querol-Pascual

Table 3 Pontine Syndromes

Pontine Syndromes Structures Affected Signs and Symptoms
Anteromedial Corticospinal tract Contralateral
Corticopontine tract ● hemiparesis/hemiplegia
Corticobulbar tract ● ataxia or pathologic laughter
Cranial facial nerve fascicle ● dysarthria, dysphagia
Cranial abducens nerve fascicle Ipsilateral
Parmedian pontine reticular formation ● facial weakness
Medial longitudinal fasciculus ● lateral rectus palsy
● horizontal gaze palsy
Others symptoms
● internuclear ophthalmoplegia
Anterolateral Corticospinal tract Contralateral
Spinothalamic tract ● hemiparesis/hemiplegia
● ataxia
● contralateral numbness
Lateral/dorsolateral Lateral corticospinal tract Contralateral
Spinothalamic tract ● hemiparesis leg > arm
Spinal nucleus/tract of cranial nerve 5 ● contralateral numbness
Cranial nerve 7 fasciculus/nucleus Ipsilateral
Cranial nerve 8 ● facial numbness
Cerebellum ● facial weakness
● hearing loss
● ataxia
Bilateral Corticobulbar tract ● aphonia/dysphagia
Corticospinal tract ● quadriplegia
Paramedian pontine reticular formation ● bilateral horizontal gaze paresis
Cranial facial nerve fascicle/nucleus ● bilateral facial weakness
Reticular formation ● transient disturbances of
consciousness
Adapted from Burger et al.12

eral face can be affected. Often the hypoglossal nerve is dam-
aged, producing ipsilateral tongue weakness. This weakness
may a person’s position sense of position, stereognosis, and
vibratory perception in contralateral extremities, although
pain and temperature may be preserved. Occasionally, nys-
tagmus or skew deviation of the eyes is produced by injury of
medial longitudinal fasciculus.2,12,30-32
Lateral Medullary Syndrome
The lateral syndrome is the most common in the medulla
oblongata (Fig. 4). This is known as Wallenberg’s syndrome,
a relatively common brainstem infarct, and according to the
findings of Marx10 is the only crossed syndrome with clinical
importance. Vertebral artery thrombosis is the most common
cause (67%) and isolated involvement of the PICA is less
common (10%).8,32,33 Spontaneous dissection of the verte-
bral artery is a common cause.34
The most characteristic symptoms of the lateral medullary
syndrome are2,8,12,20,35,36:
● Ipsilateral ataxia produced by involvement of the infe-
rior cerebellar peduncle, restiform body, or dorsal
spinocerebellar tract.
● Vertigo caused by involvement of the vestibular nu-
clei.
● Hypalgesia and thermoanesthesia in ipsilateral face
caused by involvement of the spinothalamic tract and
nucleus of cranial nerve V.
● Involvement of the sympathetic pathways, which produces
Horner’s syndrome (ptosis, myosis, and anhydrosis).
● Headache, especially a unilateral headache localized to
the upper posterior cervical region, is common with the
lateral medullary syndrome.20
● Damage to the nucleus ambiguous and fibers of the va-
gus nerve cause paralysis of the ipsilateral palate, phar-
ynx, and larynx, producing dysphagia, dysarthria, di-
minished gag reflex, and hoarseness.
● In some case palatal myoclonus is produced by dysfunc-
tion of the inferior olive.
● Finally, there may be horizontal or vertical nystagmus
(vestibular nuclei) and skew deviation of the eyes with
diplopia (medial longitudinal fasciculus).37
Other Hemimedullary Syndromes
A very uncommon combination of the 2 major syndromes
occurs as bilateral medial medullary, hemimedullary, and
bilateral medullary syndrome:
Bilateral medial medullary syndrome. Flaccid quadriplegia
sparing the face, bilateral loss of deep sensation, hypoglossal
nerve palsy and respiratory failure38,39
Clinical approach to brainstem lesions 227

Figure 4 A 58-year-old patient with vertigo and difficulty in walking. The patient demonstrated left Horner’s syndrome
(descending autonomic fibers), right loss of pain and temperature sensation, ataxia, left IXth and Xth cranial nerve
nuclei impairment, left facial dysesthesias, vertigo, and hiccups (Wallemberg’s syndrome). (A, B) Axial SE T2-weighted
and (C) coronal FLAIR images reveal a diagonal band-shape of high intensity of signal involving the posterolateral left
side of the medulla oblongata (arrows) consistent with ischemic lesion. Note the imprecise borders and the correspon-
dence with the posterolateral vascular territory.

Hemimedullary lesions. These may result in contralateral
hemiparesis, contralateral hemisensory loss, ipsilateral Hor-
ner’s syndrome, ipsilateral ataxia, ipsilateral facial sensory
loss, ipsilateral tongue paresis, dysarthria, nausea, and vom-
iting.12,40 There are 3 eponyms associated with medulla le-
sions. Of these, Reinhold syndrome is a hemimedullary syn-
drome. The other 2 syndromes, Babinski-Nageotte and
Cestan-Chenais syndromes, are intermediolateral syndromes
of the medulla with all (Babinski-Nageotte) or nearly all (Ces-
tan-Chenais) features of the lateral Wallenberg syndrome and
the hemiparesis of the medial medullary syndrome.41 Table 412
summarizes the medullary syndromes.
Summary
The rule of 4 suggested by Gates42 is a simple method devel-
oped to diagnose and localize where damage has occurred in
the brainstem (Table 4).
The principles of the rule of 4 are:
1. There are 4 structures in the midline beginning
with (M);
X the motor pathway: contralateral weakness,
X the medial leminscus (ie, contralateral loss of vib-
ration and propioception in the arm and leg),
X the medial longitudinal fasciculus (ie, ipsilateral in-
ternuclear opthalmoplegia),
X the motor nuclear nerve (ie, ipsilateral loss of af-
fected cranial nerve).
2. There are 4 structures laterally beginning with (S);
X the spinocebellar pathway,
X the spinothalamic pathway (contralateral alteration
of pain and temperature in the arm, leg, and rarely
trunk),
X The sensory nucleus of the Vth (ie, ipsilateral alter-
ation of pain and temperature on the face),
228 M.R. Querol-Pascual

Table 4 Medullary Syndromes

Medullary Syndromes Structures Affected Signs and Symptoms
Medial medullary Corticospinal tract Contralateral
Medial lemniscus ● Arm and leg hemiparesis
Cranial nerve XII Nucleus/fascicle ● Sensory loss (vibratory and positional)
Extremities
Ipsilateral
● Tongue paresis
Lateral medullary Lateral spinothalamic tract Contralateral
Spinal trigeminal nucleus/tract ● Arm/trunk/leg numbness
Inferior cerebellar peduncle Ipsilateral
Sympathetic fibers ● Absent corneal reflex/
Nucleus ambiguous ● facial numbness
Vestibular nuclei ● Ataxia
● Horner syndrome
Other symptoms
● Dysphagia, dysarthria, hoarse voice
● Vertigo, nausea, and vomiting
Hemimedullary syndrome Both structures (medial and lateral) Contralateral
● Arm and leg hemiparesis
● Sensory loss (vibratory and positional)
extremities
● Arm/trunk/leg numbness
Ipsilateral
● Absent corneal reflex/facial numbness
● Ataxia
● Horner’s syndrome
● Tongue paresis
Other symptoms
● Dysphagia, dysarthria, hoarse voice
● Vertigo, nausea, and vomiting
Adapted from Burger et al.12

X the sympathetic pathway (ie, Horner’s ipsilateral
syndrome).
3. There are 4 nerves in the medulla, 4 in the pons, and 4
above the pons (2 in midbrain).
4. The 4 motor nuclei that are in the midline are those that
divide equally into 12 except 1 and 2, that is, 3, 4, 6,
and 12. The others, 5, 7, 9, and 11, are in the lateral
brainstem.
Uncited References
This section consists of references that are included in the
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