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1110
NEUROLOGIC/HEAD AND NECK IMAGING

Midbrain, Pons, and Medulla:

Anatomy and Syndromes
Sara Sciacca, MD, MRCS, FRCR
Jeremy Lynch, MRCS, FRCR The anatomy of the brainstem is complex. It contains numerous
Indran Davagnanam, FRCR cranial nerve nuclei and is traversed by multiple tracts between the
Robert Barker, MRCP, FRCR brain and spinal cord. Improved MRI resolution now allows the
radiologist to identify a higher level of anatomic detail, but an un-
Abbreviations: ADC = apparent diffusion co- derstanding of functional anatomy is crucial for correct interpreta-
efficient, FLAIR = fluid-attenuated inversion tion of disease. Brainstem syndromes are most commonly due to
recovery
occlusion of the posterior circulation or mass effect from intrinsic
RadioGraphics 2019; 39:1110–1125 space-occupying lesions. These syndromes can have subtle imag-
https://doi.org/10.1148/rg.2019180126 ing findings that may be missed by a radiologist unfamiliar with the
Content Codes: anatomy or typical manifesting features. This article presents the
developmental anatomy of the brainstem and discusses associated
From the Department of Neuroradiology,
National Hospital for Neurology and Neuro- pathologic syndromes. Congenital and acquired syndromes are
surgery, University College London Hospitals described and correlated with anatomic locations at imaging, with
NHS Foundation Trust, Queen Square, Lon-
don WC1N 3BG, England (S.S., J.L., I.D.); and
diagrams to provide a reference to aid in radiologic interpretation.
Department of Radiology, Frimley Health NHS ©
Foundation Trust, Frimley, England (R.B.). Re- RSNA, 2019 • radiographics.rsna.org
cipient of a Certificate of Merit award for an ed-
ucation exhibit at the 2017 RSNA Annual Meet-
ing. Received April 1, 2018; revision requested
July 9 and received August 7; accepted August
13. For this journal-based SA-CME activity, the Introduction
authors, editor, and reviewers have disclosed no The brainstem is the most inferior and primitive part of the brain,
relevant relationships. Address correspon-
dence to S.S. (e-mail: [email protected]). continuous caudally with the spinal cord and rostrally with the dien-
I.D. supported by the National Institute for cephalon (thalamus, hypothalamus, epithalamus, and subthalamus)
Health Research UCL/UCLH Biomedical Re- (1). The named parts, from cranial to caudal, comprise the midbrain
search Centre.
(mesencephalon), pons (metencephalon), and medulla oblongata
©
RSNA, 2019 (myelencephalon). Functions include regulation of the cardiac, respi-
ratory, and central nervous systems including consciousness and the
sleep cycle. It has connections to the cerebrum, basal ganglia, dien-
SA-CME LEARNING OBJECTIVES cephalon, cerebellum, and spinal cord. The brainstem also contains
After completing this journal-based SA-CME all of the cranial nerve nuclei other than the olfactory (I), optic (II),
activity, participants will be able to: and part of the accessory (XI) nerves.
■■Describe the developmental anatomy of
Brainstem syndromes are usually due to vascular occlusion of
the brainstem.
branches of the posterior circulation or mass effect secondary to
■■Correlate the anatomy of the major
brainstem tracts and nuclei with their
space-occupying lesions. Knowledge of the complex function and
physiologic function and identify them anatomy of this region is key to understanding the wide spectrum
at MRI. of disease. We describe brainstem development and structure with
■■List common brainstem syndromes, a focus on the imaging appearances of pathologic abnormalities to
correlate them with their anatomic loca- help the radiologist understand these conditions.
tions, and recognize their MRI findings.
See rsna.org/learning-center-rg.
RG  •  Volume 39  Number 4 Sciacca et al  1111

The gray matter forms the nuclei of the cranial

TEACHING POINTS nerves, autonomic nuclei, olivary nuclei, nuclei of
■■ The midbrain connects the pons and cerebellum with the
forebrain and can be divided into a ventral part, the tegmen-
the pons and cerebellum, red nuclei, substantia
tum, and a dorsal part, the tectal or quadrigeminal plate. nigra, nuclei of the corpora quadrigemina, and
■■ The ventral midbrain is composed of the cerebral peduncles
reticular formation. The white matter consists of
(crus cerebri), which contain the pyramidal and corticopon- myelinated tracts connecting the cerebrum with
tine tracts. the spinal cord and various cranial nerve nuclei.
■■ The dorsal tegmentum is ventral to the cerebral aqueduct
and contains the nuclei of the oculomotor (III) and trochlear Midbrain
(IV) cranial nerves, white matter tracts, and gray matter in-
cluding the substantia nigra and red nucleus. The substantia
nigra functions in motor control and reward pathways. The
Anatomy
red nucleus, part of the extrapyramidal system, has roles in The midbrain connects the pons and cerebel-
motor coordination. lum with the forebrain and can be divided into
■■ The pons connects the brain to the cerebellum and can be a ventral part, the tegmentum, and a dorsal
divided into a ventral part and a dorsal tegmentum. The part, the tectal or quadrigeminal plate (Figs 2,
ventral part contains longitudinal fibers primarily from the 3). The cerebral aqueduct of Sylvius is located
corticospinal, corticobulbar, and corticopontine tracts. The
in the midbrain and joins the third and fourth
dorsal tegmentum contains the nuclei of the trigeminal (V),
abducens (VI), facial (VII), and vestibulocochlear (VIII) cranial
ventricles, surrounded by periaqueductal gray
nerves, in addition to white matter tracts including the medial matter (3). This gray matter extends from the
longitudinal fasciculus, medial lemniscus, lateral lemniscus, superior border of the midbrain along the pos-
spinothalamic tract, and central tegmental tract. This region terior aspect of the floor of the third ventricle to
also contains the trapezoid body, part of the auditory path-
the fourth ventricle and the superior medullary
way involved in the localization of sound.
velum. It functions in pain modulation, control of
■■ The medulla oblongata relays information from the spinal
cord to the brain and is composed of a ventral portion and
emotional responses including fear and anxiety,
a dorsal tegmentum. The ventral part includes the pyramids vocalization, and cardiovascular control.
and the olives. The inferior olivary nucleus is part of the ol- The ventral midbrain is composed of the cere-
ivocerebellar system and has functions in cerebellar motor bral peduncles (crus cerebri), which contain the
learning. The superior olivary nucleus has roles in perception pyramidal and corticopontine tracts. The pyrami-
of sound. The dorsal tegmentum contains the nuclei of the
glossopharyngeal (IX), vagus (X), accessory (XI), and hypo-
dal tracts control movements of the limbs, trunk,
glossal (XII) cranial nerves, as well as the continuation of the and cranial nerves. They comprise the cortico-
white matter tracts (medial longitudinal fasciculus, medial spinal and corticobulbar tracts. These connect
lemniscus, spinothalamic tract, central tegmental tract, spino- the cerebral cortex with the spinal cord and with
cerebellar tract). nonoculomotor cranial nerves, respectively. The
corticopontine tracts connect the cerebral cortex
to the pontine nuclei and then to the opposite
cerebellum, allowing coordination of planned
Developmental Classification motor functions.
Development of the brainstem begins in the 4th The dorsal tegmentum is ventral to the cere-
week of gestation when the rostral neural tube bral aqueduct and contains the nuclei of the ocu-
forms three primary brain vesicles (2). The most lomotor (III) and trochlear (IV) cranial nerves,
anterior vesicle constitutes the prosencephalon white matter tracts, and gray matter including the
or forebrain, the central vesicle forms the mes- substantia nigra and red nucleus. The substan-
encephalon or midbrain, and the most posterior tia nigra functions in motor control and reward
vesicle forms the rhombencephalon or hindbrain. pathways. The red nucleus, part of the extrapyra-
From the primary vesicles arise five second- midal system, has roles in motor coordination.
ary vesicles. The telencephalon and diencephalon The white matter tracts pass through the
originate from the prosencephalon, and the met- entire length of the brainstem and include the
encephalon and myelencephalon originate from medial longitudinal fasciculus, medial lemnis-
the rhombencephalon. The mesencephalon forms cus, lateral lemniscus, central tegmental tract,
the midbrain, the metencephalon forms the pons spinothalamic tract, and rubro-olivary tract. The
and cerebellum, and the myelencephalon forms medial longitudinal fasciculus is the main central
the medulla oblongata (Fig 1). connection for the oculomotor nerve and coordi-
The cavity within the neural tube develops into nates conjugate gaze. The medial lemniscus car-
the cerebral ventricles and the central canal of the ries proprioceptive, vibratory, and touch-pressure
spinal cord. This is filled initially with amniotic sense. The lateral lemniscus carries auditory in-
fluid and subsequently with cerebrospinal fluid. formation to the auditory cortex. The central teg-
The mesencephalon, metencephalon, and mental tract contains ascending fibers connecting
myelencephalon consist of gray and white matter. the rostral nucleus solitarius to the thalamus and
1112  July-August 2019 radiographics.rsna.org

Figure 1.  Embryonic development of the brain from the five secondary vesicles.

Figure 2.  Left: Axial T2-weighted MR image (3 T) at the level of the midbrain. Right: Ex vivo axial T2-weighted
image (9.4 T) of the midbrain. CN3 = cranial nerve III.

cortical taste area. The spinothalamic tract carries Syndromes

touch, pain, and temperature information. The
rubro-olivary tract consists of descending fibers Weber Syndrome.—Weber syndrome is caused
from the parvocellular red nucleus projecting to by infarction of the oculomotor nucleus and
the inferior olivary nucleus. cerebral peduncle in the ventromedial midbrain
The tectum is located dorsal to the cerebral from occlusion of the paramedian branches of the
aqueduct and has two paired eminences on its basilar artery or posterior cerebral artery (Fig 4)
posterior surface: the superior and inferior collic- (1,4). Patients present with ipsilateral oculomotor
uli. The superior colliculi have roles in preliminary nerve palsy (inferolateral eye deviation, diplopia,
visual processing and control of eye movements. ptosis, afferent pupillary defect) and contralateral
The inferior colliculi have functions in auditory hemiplegia or hemiparesis owing to infarction
processing, projecting the inputs from the brain- of the corticospinal and corticobulbar tracts in
stem nuclei to the medial geniculate nucleus of the the crus cerebri. If the substantia nigra is also in-
thalamus, which in turn relays auditory informa- volved, the syndrome manifests with contralateral
tion to the primary auditory cortex. parkinsonian rigidity.
The vertebrobasilar system supplies the mid-
brain via perforating branches from the basilar, Benedikt Syndrome.—Benedikt syndrome (para-
superior cerebellar, and posterior cerebral arteries. median midbrain syndrome) is an infarct of the
RG  •  Volume 39  Number 4 Sciacca et al  1113

Figure 5.  Axial T2-weighted image (3 T) at the level of the

midbrain shows the area involved in Benedikt syndrome (blue).

Figure 3.  Top: Labeled anatomy of the midbrain. MLF = me-

dial longitudinal fasciculus. Bottom: Axial T2-weighted image
(3 T) of the midbrain.

Figure 6.  Axial T2-weighted image (3 T) at the level of the

midbrain shows the area involved in Claude syndrome (blue).

Claude Syndrome.—Claude syndrome is caused

by infarction of the dorsomedial aspect of the
midbrain due to occlusion of the small perforat-
ing branches of the posterior cerebral artery sup-
plying the medial aspect of the red nucleus with
the rubrodentate fibers, the third cranial nerve
nucleus, and the superior cerebellar peduncle
(Fig 6). Patients present with ipsilateral oculomo-
tor nerve palsy, incoordination, and contralateral
upper and lower limb cerebellar hemiataxia (1).
Figure 4.  Axial T2-weighted image (3 T) at the level of the
midbrain shows the area involved in Weber syndrome (blue). Parinaud Syndrome.—Parinaud syndrome (dorsal
midbrain syndrome) is caused by compression
of the tectal plate near the level of the superior
colliculus from a space-occupying lesion located
tegmentum of the midbrain due to occlusion of in the posterior commissure or pineal region (Figs
branches of the posterior cerebral artery supply- 7, 8). It classically causes the triad of upward gaze
ing the fascicles of the oculomotor nerve and red palsy (often manifesting as diplopia), pupillary
nucleus (Fig 5). Patients typically present with light-near dissociation (the pupil is poorly reac-
ipsilateral oculomotor nerve palsy, crossed hemi- tive to light but constricts with convergence), and
ataxia, incoordination, and chorea (1,5). convergence-retraction nystagmus (1,6).
1114  July-August 2019 radiographics.rsna.org

Figure 7.  Axial T2-weighted image (3

T) at the level of the midbrain shows
the area involved in Parinaud syn-
drome (blue).

Figure 8.  Parinaud syndrome. Sagittal (a) and axial (b) contrast-enhanced T1-weighted images show a
pineal germinoma, which effaces the cerebral aqueduct and compresses the tectal plate.

Nothnagel Syndrome.—Nothnagel syndrome (VII), and vestibulocochlear (VIII) cranial nerves,

is caused by a mass or infarct of the superior in addition to white matter tracts including the
cerebellar peduncle from extension of a lesion medial longitudinal fasciculus, medial lemnis-
within the quadrigeminal plate (Fig 9). It causes cus, lateral lemniscus, spinothalamic tract, and
unilateral or bilateral oculomotor nerve paralysis central tegmental tract. This region also contains
and ipsilateral cerebellar ataxia (7). the trapezoid body, part of the auditory pathway
involved in the localization of sound.
Pons The pons is supplied by medial branches of
superior cerebellar arteries, perforating branches
Anatomy of the basilar artery, and anterior inferior cer-
The pons connects the brain to the cerebellum ebellar arteries (variably supplying the lower and
and can be divided into a ventral part and a lateral pons).
dorsal tegmentum (Figs 10, 11) (8). The ventral
part contains longitudinal fibers primarily from Syndromes
the corticospinal, corticobulbar, and corticopon-
tine tracts. The dorsal tegmentum contains the Marie-Foix Syndrome.—Marie-Foix syndrome
nuclei of the trigeminal (V), abducens (VI), facial (lateral pontine syndrome) is caused by infarction
RG  •  Volume 39  Number 4 Sciacca et al  1115

Figure 9.  Coronal T1-weighted

(top left), sagittal T1-weighted
(right), and axial T2-weighted
(bottom left) images (3 T) at the
level of the midbrain show the
area involved in Nothnagel syn-
drome (blue).

Figure 10.  Axial T2-weighted image (3 T) at the level of the pons. V = cranial nerve V, VI = cranial nerve VI,
VII = cranial nerve VII, VIII = cranial nerve VIII.

of the lateral pons and middle cerebellar pedun- Foville Syndrome.—Foville syndrome (inferior
cle from occlusion of perforating branches of the medial pontine syndrome) is due to an infarct of
basilar and anterior inferior cerebellar arteries. the pons involving the corticospinal tract, medial
The corticospinal, spinothalamic, and cerebel- lemniscus, medial longitudinal fasciculus, parame-
lar tracts and the facial and vestibulo­cochlear dian reticular formation, and nuclei of the abdu-
cranial nerve nuclei are affected (Fig 12). Clini- cens and facial nerves (Figs 13, 14). Patients pre­
cal findings include contralateral hemiplegia or sent with contralateral hemiparesis or hemiplegia,
hemiparesis, ipsilateral impairment of pain and hemi–sensory loss, ipsilateral facial nerve palsy,
temperature sensation, limb ataxia, facial paraly- and conjugate gaze palsy with inability to look to
sis, hearing loss, vertigo, and nystagmus (9). the side of the lesion with diplopia (10,11).
1116  July-August 2019 radiographics.rsna.org

Figure 12.  Axial T2-weighted image (3 T) of the pons shows

the area involved in Marie-Foix syndrome (gold).

Figure 11.  Top: Labeled anatomy of the pons. Bottom: Axial

T2-weighted image (3 T) of the pons. V = cranial nerve V.

Locked-in Syndrome.—Locked-in syndrome

(pseudocoma) is caused by an insult to the
pyramidal bundles of the ventral brainstem with
paralysis of all voluntary and respiratory muscles,
often necessitating mechanical ventilation (Figs
15, 16). Patients may be awake and conscious Figure 13.  Axial T2-weighted image (3 T) of the pons shows
with preserved cognitive function but de-effer- the area involved in Foville syndrome (blue).
ented and unable to speak or perform limb or fa-
cial movements, with a condition resembling the
vegetative state or akinetic mutism. The oculomo- Millard-Gubler syndrome is caused by a lesion
tor nerve is typically spared, so eye opening and to the ventral aspect of the caudal pons involv-
vertical eye movements may be preserved. The ing the corticospinal tract and the abducens and
facial and abducens nerves are paralyzed from facial nerves. The manifestation is characterized
impairment of corticobulbar tracts (12–16). by ipsilateral facial paresis with loss of the corneal
reflex and contralateral hemiplegia, isotropism,
Ventral Pontine Syndromes.—A ventral pon- and diplopia that is worsened when the patient
tine insult may result in Raymond and Millard- looks toward the side of the lesion (18–20).
Gubler syndromes. Raymond syndrome (al-
ternating abducens hemiplegia) is caused by a Facial Colliculus Syndrome.—This syndrome is
unilateral lesion of the ventromedial pons affect- due to a lesion of the facial colliculus (located
ing the ipsilateral abducens nerve fascicles and on the pontine tegmentum on the floor of the
corticospinal tract but sparing the facial nerve fourth ventricle) and causes impairment of the
(Figs 17, 18). Patients present with lateral gaze medial longitudinal fasciculus, the abducens
weakness from ipsilateral lateral rectus paresis nerve, and the genu fibers of the facial nerve
and contralateral hemiplegia (17). (Figs 19, 20). It results in a lower-motor-neuron
RG  •  Volume 39  Number 4 Sciacca et al  1117

Figure 14.  Foville syndrome. Axial T2-weighted image (a), axial diffusion-weighted image (b), axial apparent diffusion coefficient
(ADC) map (c), and coronal fluid-attenuated inversion-recovery (FLAIR) image (d) show signal intensity change and restricted diffu-
sion in the right medial pontine region, indicative of infarction.

Figure 15.  Sagittal T1-weighted image (3 T) at the level of the pons shows the area involved
in locked-in syndrome (orange).
1118  July-August 2019 radiographics.rsna.org

Figure 16.  Locked-in syndrome. (a–c) Axial T2-weighted im-

age (a), diffusion-weighted image (b), and ADC map (c) show
a large area of signal intensity change and restricted diffusion
in the pons indicating infarction, with further foci of ischemia
in the cerebellar hemispheres. (d) Axial MR angiogram shows
marked attenuation of the basilar artery, indicating near-total
occlusion.

facial nerve palsy proximal to the geniculate

ganglion and hence causes loss of taste sensation
in the anterior two-thirds of the tongue, hyper-
acusis, diplopia, and horizontal conjugate gaze
palsy (21,22).

Medulla Oblongata

Anatomy
The medulla oblongata relays information from
the spinal cord to the brain and is composed of
a ventral portion and a dorsal tegmentum (Figs
21, 22). The ventral part includes the pyramids
and the olives (23). The inferior olivary nucleus is
part of the olivocerebellar system and has func-
tions in cerebellar motor learning. The superior Figure 17.  Axial T2-weighted image (3 T) of the pons shows
olivary nucleus has roles in perception of sound. the area involved in ventral pontine syndromes (orange).
The dorsal tegmentum contains the nuclei of
the glossopharyngeal (IX), vagus (X), accessory
(XI), and hypoglossal (XII) cranial nerves, as cus, spinothalamic tract, central tegmental tract,
well as the continuation of the white matter tracts spinocerebellar tract). The medulla is supplied by
(medial longitudinal fasciculus, medial lemnis- the anterior spinal artery, penetrating branches
RG  •  Volume 39  Number 4 Sciacca et al  1119

Figure 18.  Raymond syndrome. Axial T2-weighted image (a), axial ADC map (b), and sagittal T2-weighted image (c) show
signal intensity change and facilitated diffusion involving both sides of the pons at the level of the superior and middle cerebel-
lar peduncles, indicating established infarction. An incidental second remote border zone infarct is evident in the left cerebellar
hemisphere.

Figure 19.  Axial T2-weighted image (3 T) of the pons shows

the area involved in facial colliculus syndrome.

from the vertebral artery, and the posterior infe-

rior cerebellar artery.

Syndromes

Wallenberg Syndrome.—Wallenberg syndrome

(lateral medullary syndrome) is caused by an
insult to the lateral medulla, usually from an
infarction of the posterior inferior cerebellar
artery (24). The inferior cerebellar peduncle,
vestibular nucleus, spinal trigeminal nucleus,
and nucleus ambiguus are typically affected
(Figs 23, 24).
Clinical features include vestibulocerebellar
symptoms such as vertigo, falling toward the
side of the lesion, diplopia, multidirectional nys-
tagmus, ipsilateral Horner syndrome, hiccups, Figure 20.  Facial colliculus syndrome. Axial
T2-weighted image (a) and diffusion-weighted
contralateral body loss of pain and temperature image (b) show signal intensity change and re-
sensation, hoarseness, dysphonia, dysphagia, stricted diffusion involving the left facial collicu-
dysarthria, and decreased gag reflex. lus, consistent with acute infarction.
1120  July-August 2019 radiographics.rsna.org

Figure 21.  Axial T2-weighted

image (3 T) at the level of the
medulla. V = cranial nerve V, IX =
cranial nerve IX, X = cranial
nerve X, XII = cranial nerve XII.

Figure 23.  Axial T2-weighted image (3 T) of the medulla

shows the area involved in Wallenberg syndrome (green).
CN5 = cranial nerve V.

Dejerine Syndrome.—Dejerine syndrome (me-

dial medullary syndrome) is caused by an infarct
of the medial medulla affecting the hypoglossal
nerve nucleus from occlusion of small perforating
branches from the vertebral or proximal basilar
artery (Fig 25) or occlusion of the anterior spinal
artery. The syndrome causes contralateral weak-
ness and hemi–sensory deficit with ipsilateral
hypoglossal palsy (25).

Babinski-Nageotte Syndrome.—Babinski-
Nageotte syndrome (hemimedullary syndrome)
occurs with medial and lateral medullary infarc-
Figure 22.  Top: Labeled anatomy of the medulla. IV ven-
tricle = fourth ventricle. Bottom: Axial T2-weighted image (3 tion from occlusion of the intracranial part of the
T) of the medulla. vertebral artery (Figs 26, 27). The syndrome con-
sists of a combination of medial and lateral medul-
lary symptoms, with ipsilateral cerebellar ataxia,
sensory deficits of the face, Horner syndrome, and
contralateral hemiplegia and hemianesthesia (26).
RG  •  Volume 39  Number 4 Sciacca et al  1121

Figure 24.  Wallenberg syndrome. Axial diffusion-weighted images (a, c) and T2-weighted image (b) show signal intensity change
and restricted diffusion in the right lateral medulla and absence of flow void in the right vertebral artery on the T2-weighted image,
consistent with acute lateral medullary infarction due to right vertebral dissection.

rubral tract or central tegmental tract (between

the red nucleus and ipsilateral inferior olivary
nucleus) (Fig 29). The patient presents with
palatal myoclonus, which can be associated with
cerebellar or brainstem dysfunction (27).

Developmental Anomalies
Developmental abnormalities of the brainstem
are rare conditions characterized by absence or
abnormal development of brainstem structures.
They may be challenging to diagnose at MRI.

Möbius Syndrome
Figure 25.  Axial T2-weighted image (3 T) of the medulla Möbius syndrome (congenital facial diplegia
shows the area involved in Dejerine syndrome (green).
syndrome) is a rare congenital condition char-
acterized by absence or underdevelopment of
the abducens and facial nerve nuclei. Brainstem
hypoplasia is seen, with flattening of the floor
of the fourth ventricle as a result of absence of
the facial colliculus (Fig 30). It is characterized
clinically by unilateral or bilateral facial paralysis
with an expressionless face and inability to close
the mouth or eyes (28,29).

Axonal Guidance Disorders

This group of diseases have abnormal white mat-
ter tracts, typically failure to cross the midline or
presence of ectopic tracts. They can sometimes
be suspected on structural MR images but are
Figure 26.  Axial T2-weighted image (3 T) of the medulla better appreciated at diffusion tensor imaging
shows the area involved in Babinski-Nageotte syndrome (DTI) and fiber tractography. Common patho-
(green). CN5 = cranial nerve V. logic conditions include horizontal gaze palsy and
progressive scoliosis (Fig 31), Joubert syndrome,
and pontine tegmental cap dysplasia (30).
Hypertrophic Olivary Degeneration.—This is a
rare condition characterized by a lesion located Miscellaneous Conditions
in the triangle of Guillain and Mollaret, formed
by the red nucleus, inferior olivary nucleus, and Multiple System Atrophy
contralateral dentate nucleus (Fig 28). Hyper- Multiple system atrophy (MSA) is a sporadic
trophy of the inferior olivary nucleus is caused neurodegenerative disease characterized by
by transsynaptic degeneration of the dentato- varying degrees of cerebellar ataxia, autonomic
1122  July-August 2019 radiographics.rsna.org

Figure 27.  Axial susceptibility-

weighted (a) and T2-weighted (b)
images show bleeding from a
right inferior medullary caver-
noma, resulting in Babinski-Na-
geotte syndrome.

Figure 28.  Axial T2-weighted and coronal T1-weighted images (3 T) show the triangle of Guillain and Mollaret, which
is involved in hypertrophic olivary degeneration.

Figure 29.  Axial T2-weighted images at the level of the medulla (a) and pons (b) and FLAIR image at the level of the medulla (c)
show right hypertrophic olivary degeneration due to previous pontine bleeding.
RG  •  Volume 39  Number 4 Sciacca et al  1123

Figure 30.  Möbius syndrome. Axial (a, b) and sagittal (c) T2-weighted images at the level of the Meckel cave show absence of the
abducens and facial nerves.

Figure 31.  Horizontal gaze palsy and scoliosis. (a) Diffusion tensor image shows absent superior cerebellar decussation and absent
decussation of somatosensory and corticospinal tracts. (b–e) Axial T2-weighted (b, d), axial inversion-recovery (c), and sagittal T1-
weighted (e) images show a small pons with an absent facial colliculus and butterfly configuration of the medulla with a midline cleft.
(f) Radiograph shows lumbar scoliosis.
1124  July-August 2019 radiographics.rsna.org

Figure 32.  MSA-C. (a) Sagittal

T1-weighted image shows cerebel-
lar atrophy. (b) Axial T2-weighted
image shows cross-shaped hyper-
intensity through the pons (hot
cross bun sign), representing selec-
tive degeneration of pontocerebel-
lar tracts.

Figure 33.  Axial FLAIR images show increased signal intensity in the dorsomedial thalami (a), mammillary bodies and periaqueduc-
tal area (b), and tectal plate (c), typical of Wernicke encephalopathy.

dysfunction, parkinsonism, and corticospinal dys- natremia (33). At MRI, there is symmetric signal
function. In the MSA-C form, there is predomi- intensity abnormality in the pons, basal ganglia,
nance of cerebellar symptoms with olivoponto- midbrain, and subcortical white matter, with hy-
cerebellar atrophy. MRI shows T2 hyperintensity pointensity on T1-weighted images and hyperin-
in the pontocerebellar tracts, resulting in a “hot tensity on T2-weighted/FLAIR images. Restricted
cross bun” sign in the pons and disproportion- diffusion is usually the earliest sign, seen in the
ate atrophy of the cerebellum and brainstem (31) lower pons within 24 hours of the onset of quad-
(Fig 32). riplegia. Enhancement after administration of
gadolinium contrast material is typically absent.
Wernicke Encephalopathy
Wernicke encephalopathy is due to thiamine (vi- Progressive Supranuclear Palsy
tamin B1) deficiency and is typically seen in cases This neurodegenerative disease usually manifests
of alcohol abuse. MRI shows areas of symmetric after the 6th decade and progresses to death
increased T2/FLAIR signal intensity involving within 10–20 years (34). It is characterized by
the mammillary bodies, dorsomedial thalami, parkinsonism, supranuclear vertical gaze palsy,
tectal plate, and periaqueductal area and around decreased cognition and speech, and instability
the third ventricle (Fig 33). Patients present with and falls.
changes in consciousness, ataxia, and ocular Imaging classically reveals midbrain atrophy
dysfunction (32). resulting in loss of convexity of the brainstem,
giving the appearance of a hummingbird in the
Osmotic Demyelination Syndrome sagittal plane (hummingbird sign). A reduction
This is an acute demyelination caused by osmotic in the anteroposterior diameter of the midbrain
changes, typically after rapid correction of hypo- at the level of the superior colliculus may give a
RG  •  Volume 39  Number 4 Sciacca et al  1125

“Mickey Mouse” appearance in the axial plane 16. León-Carrión J, van Eeckhout P, Domínguez-Morales MR,
Pérez-Santamaría FJ. The locked-in syndrome: a syndrome
(Mickey Mouse sign). T2 lesions may be found in looking for a therapy. Brain Inj 2002;16(7):571–582.
the pontine tegmentum, tectum of the midbrain, 17. Satake M, Kira J, Yamada T, Kobayashi T. Raymond
and inferior olivary nucleus. syndrome (alternating abducent hemiplegia) caused by a
small haematoma at the medial pontomedullary junction.
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