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Hypoglossal Nerve Palsy: A Segmental Approach1: Damage
Hypoglossal Nerve Palsy: A Segmental Approach1: Damage
Hypoglossal Nerve Palsy: A Segmental Approach1: Damage
Palsy: A Segmental
Approach1
Elizabeth 0. Thompson, MB, BS2
Wendy R. K. 5’;noket MD
. INTRODUCTION
Palsy of the hypoglossal nerve is relatively uncommon. l)aniage to the 1 2th cranial
nerve (hypoglossal nerve or cranial nerve XII) l)rodtlces characteristic clinical manifes-
tations. Nerve dysfunction may occur in isolation; more commonly, it may he associat-
ed with complex palsy of the lower nerve. ‘I’o design the most efficient imaging strate-
gy in the presence of such symptoms, one must I)e familiar with the anatomy of the hy-
poglossal nerve and the diseases that affect the nerve throughout its course from the
medulla oblongata to the tongue.
I lr,)tA the l)caniicnr of Radiulugv. Medical (olkge t)f Virginia. M( \ Station. 1kx 6 I S. I 2(X) E Marshall St. Richnund. VA
23298. Prcsentt.-d as a scientific t.-xhihit at the 19’)2 RSNA scicittitIc asscinhly. RcccivcdJunc 25. 993: revision n.qticstt.-d
Scptcmh.r I itd rcccivcd April 28. 1994: acccl)tcd April 29 Addrtss reprint requests to W K KS.
. RSNA. 1994
939
Dysfunction of the hypoglossal nerve may be
a consequence of supranuclear, nuclear, or
infranuclear disease (1). The nuclear and
infranuclear hypoglossal nerve can be divided I CISTERNAL
into five segments: the medullary, cisternal, SKULL BASE
skull base, nasopharyngeal/oropharyngeal Ca-
rotid space, and sublingual segments. Such ana-
tomie segmental analysis allows the radiologist
to develop a systematic approach to a case of
hypoglossal nerve palsy. Knowledge of the
most common pathologic conditions occurring
in each segment will permit the radiologist to
produce a more sharply focused differential di-
agnosis. The segmental approach to the anat-
omy and pathologic conditions of the hypoglos-
sal nerve is used to clarify the differential diag-
nosis.
This article reviews the normal anatomy of
the hypoglossal nerve by using a segmental ap-
proach, describes the clinical manifestations of BRANCHES TO
STRAP MUSCLES HYPOGLOSSI
hypoglossal nerve palsy, and demonstrates the
gamut of pathologic conditions affecting the hy-
poglossal nerve in each of its five segments. Figure 1. Sagittal view of the path of the hypoglos-
sal nerve in the suprahyoid neck. ICA = internal ca-
. NORMAL ANATOMY rotid artery.
The hypoglossal nerve exits the medulla oblon-
gata, extends through the skull base, and
traverses the suprahyoid neck before ramifying
to supply the tongue musculature (Fig 1). The
fibers of the hypoglossal nerve arise in the hy-
poglossal nuclei, which extend through the me-
dulla oblongata in a paramedian location (Fig
2). This is the medullary segment. The fibers
course anteriorly, lateral to the medial lemnis-
cus, to exit the medulla in the preolivary sul-
cus. The rootlets of the hypoglossal nerve lie
posterolateral to the vertebral artery within the
premedullary cistern; this is the cisternal seg-
ment of the nerve (Fig 3). The rootlets then
merge to form the hypoglossal nerve within the
HYPOGLOSSAL EMINENCE
hypoglossal (anterior condylar) canal of the oc-
Figure 2. View through the medulla oblongata
shows the hypoglossal nucleus, the nerve rootlets in
the premedullary cistern, and the hvpoglossal canal.
Iv = fourth ventricle.
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Scientific Exhibit Volume 14 Number 5
Figure 3. (a) Axial view through the skull base.
Dots indicate the left hypoglossal nerve. (b) Axial
computed tomographic (CT) scan at the same
level shows the hypoglossal canals (arrowheads).
(c) Axial Ti-weighted magnetic resonance (MR)
image at the same level shows the hypoglossal
nerve roots (arrowheads) as they exit the medul-
la oblongata and pass through the premedullary
cistern toward the hypoglossal canal.
cipital hone; this is the skull base segment ternal carotid artery and internal jugular
(Fig 4). from the hypoglossal
Emerging ca- ‘eiIi, superficial to the vagus nerve (Fig 5).
nal, the hypoglossal nerve enters the na- At the level of the angle of the mandible, the
sopharyngeal carotid space. At this point, nerve deviates from the path of these other
the nerve lies deep to the internal jugular lower cranial nerves. It loops anteriorly
vein, internal carotid artery, and glossopha- around the root of the occipital artery, lying
ryngeal and vagus nerves. As the nerve pass- inferior to the posterior belly of the digastric
Cs intcriorly, it comes to lie between the in- muscle, where it becomes superficial (Fig
6). At the level of the hyoid hone, the nerve
CO55C5 the lingual artery and curves anteri-
orly to run along the surface of the hyoglos-
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Scientific Exhibit Volume 14 Number 5
Figures 5, 6. (5a) Line diagram at the level
of the nasophar)’ngeal carotid space. Arnw mdi-
cates the position of the hypoglossal nerve.
(5b, 5c) Axial contrast material-enhanced CT
(5b) and axial TI-weighted MR (5c) images at the
same level as in 5a. The hypoglossal nerve (ar-
rowheads) passes around the r(x)t of the occipital
artery, then lies anterior to the vessels of the ca-
rotid space. (6a) Line diagram through the carot-
id space. The posterior bellies of the digastric
(D), lateral pterygoid (1.7). and masseter (Al) mEls-
cks are identified. I)ot indicates the position of
the hypoglossal nerve. (6b) Coronal 1 I -weighted
MR image at the same level shows the left inter-
nal carotid artery (arrowheads). At this level, the
hypoglossal nerve lies deep to the posterior belly
of the digastric muscle.
Sa.
sus muscle, deep to the mylohyoid sling. This The hypoglossal nerve supplies motor inner-
segment of the nerve lies within the sublingual vation to the intrinsic and extrinsic muscles of
space (Figs 7, 8). As it passes anteriorly, the the tongue. The ansa hypoglossi contributes
nerve lies on the surface of the genioglossus motor innervation to the infrahyoid strap mus-
muscle before penetrating that muscle. des. The ansa hypoglossi is made up of fibers
from C-i that course through the carotid space
with the hypoglossal nerve.
Common Pathol ogic Conditions Affe cting Each Segment of th e Hypoglossal Nerve
U RADIOLOGIC DIAGNOSIS OF PALSY ma, may simulate a nitss within the tongue.
OF CRANIAL NERVE XII Disease within the sublingual space may
t1nilaterLl atrophy of the tongue musculature is also masquerade LA5 atrophy of the tongue, par-
the most important radiologic feature of hypo- ticularly in the case of fatty lesions such as
glossal nerve palsy (Fig 9) (2-4). Once identi- lipomas and dermoid tumors. These lesions lie
fled, atrophy should pronipt a thorough assess- adjacent to the intrinsic tongue muscles, lead-
ment of the VJflOUS segments of the nerve as it ing to muscle displacement rather than fatty re-
travels from the medulla oblongata to the placement.
tongue. Iii sonic cases, changes may occur When a latient has OhVK)tIs palsy of the by-
within the tongue musculature in the setting of poglossal nerve or tongue atrophy is identified
acute paresis of the nerve. The muscles may un- at imaging, systematic evaluation of the seg-
dergo frttv change and, if associated with ede- merits of the hypoglossal nerve should be per-
formed. The most cOnImOIi diseases affecting
each segment are discussed below and are list-
eti in the Table.
Figure 13. Vertebrobasilar dolichoectasia in a 58-year-old man with a spontaneous intracranial hem-
orrhage. He had an unexplained left hypoglossal nerve palsy. Axial (a) and coronal (b) Ti-weighted
MR images demonstrate marked ectasia of the left vertebral and basilar arteries (arrows), leading to
considerable compression of the medulla oblongata (11 in a).
. Cisternal Segment
Lying in the premedullary cistern, the rootlets
of the hypoglossal nerve may be affected by a
variety of pathologic processes. The vertebral
arteries lie close to the nerve rootlets, which
may be compressed by a vertebral aneurysm or
by dolichoectasia (Figs 13, 14) (8). Direct cx-
tension of neoplasms of the skull base, such as
C. chordoma of the clivus or meningioma of the
foramen magnum (Fig 1 5), may lead to nerve
dysfunction. Basal meningitis, especially tuber-
plete paralysis of the tongue. When the corti- culous meningitis, or subarachnoid hemor-
cospinal tract is involved, contralateral spastic
herniplegia develops in association with flaccid
paralysis of the hypoglossal nerve.
rhage with exudation and organization can the rootlets of the hypoglossal nerve (Fig 16)
compromise the nerve. An abnormally located (9). Displacement of the medulla oblongata
odontoid process, such as that associated with into or through the foranien magnum, as a con-
rheumatoid arthritis, may impinge on or stretch sequence of trauma or the Arnold-Chiari mal-
formation, may also lead to nerve dysfunction.
a. b.
Figure 18. Condylar fracture in a I 5-year-cild boy who was involved in a high-speed motor vehicle
LCCi(k1it. On eXLfl1iflItiOfl, his tongue was deviated to the left. Axial CT images through the skull has-
at (lifferent levels show a linear fracture (arrowheads) involving the left occipital condyle and the hy-
poglossal (IIill.
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Scientific Exhibit Volume 14 Number 5
a. b.
Figure 21. Nasopharyngeal carcinoma in a 61-year-old man with isolated hypoglossal nerve palsy. (a) Axial
contrast-enhanced CT scan reveals a large soft-tissue mass extending posteriorly from the nasopharynx, with cvi-
dence of hone destruction. (b) CT scan obtained with a bone window shows destruction of the left skull base
and hypoglossal canal (large arrowheads). The margins of the right hypoglossal canal are well visualized (small
arrowheads).
-5 _1, A
. V
‘ . -P
25a. 25b.
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Scientific Exhibit Volume 14 Number 5
Figures 23-25. (23) Hodgkin disease in a 54-year-old man with a short history of difficulty in swallowing. On
examination, he had complex neuropathy of the lower cranial nerves, including the hypoglossal nerve. (a) Axi-
al Ti-weighted MR image shows a large mass (arrowheads) extending to involve the retropharyngeal, preverte-
bral, and carotid spaces. (b) Coronal Ti-weighted MR image reveals the superior extension of the tumor, with
fliaITOV replacement in the right occipital condyle. The normal marrow of the left occipital condyle is indicat-
ed by a dot. The carotid artery (arrowheads) is displaced laterally. (24) Glomus vagale in a 47-yearuld man with
a mass in the right neck and isolated right hypoglossal nerve palsy. (a) Axial contrast-enhanced CT scan shows
an enhancing, rounded soft-tissue mass in the oropharyngeal carotid space (arrows). (b) Coronal Ti-weighted
MR iniage shows numerous flow voids (arrowheads) within the lesion. (25) Vagal schwannoma in a 32-year-old
man with a 2-month history of hoarseness. On examination, he had paralysis of the left vocal cord and left hy-
poglossal nerve palsy. (a) Axial Ti-weighted MR image demonstrates a rounded tumor (7) in the left carotid
space, lateral to the hypoglossal canal. (b) Coronal Ti-weighted MR image shows the tumor (7) extending infe-
riorly iflto the nasopharyngeal carotid space.
. NopharyngeaUOropharyngeal
Carotid Space Segment
Hypogbossal nerve injury within the carotid
space may result from a range of disorders, ma-
lignant disease being the most common. This
includes both primary and nodal squamous cell
carcinoma, lymphoma (Fig 23), salivary gland
malignancies, and soft-tissue sarcomas. Extra-
nodal metastatic disease from distant primary
sites is also encountered, as are benign lesions
such as lipomas, paragangliomas (Fig 24), and
tumors of neural origin (Fig 25). Vascular dis-
ease in the neck can lead to dysfunction of the
hypoglossal nerve. The nerve lies close to the
vessels of the carotid space. As a result, ectasia
and aneurysms of the carotid artery, as well as
arterial dissection (Fig 26) and jugular thrombo-
Figure 26. Carotid dissection in a 43-year-old man sis, can compress the nerve and lead to palsy
with a history of minor neck trauma and recent on- (14- 16). Iatrogenic causes include jugular
set of tongue weakness. Axial T 1-weighted MR im- venous puncture and complicated carotid end-
age reveals high signal intensity surrounding the in- arterectomy (17). Rarely, transient hypogbossal
ternal carotid arterv on the left (arrowheads), with nerve palsy may complicate a traumatic dcliv-
diminution of the vessel lumen. T2-weighted images
cry (18). Other abnormalities affecting the hy-
( not shown) Ll5() demonstrated high signal intensity
in the vessel wall, consistent with extracellular
methemoglobin. (Courtesy of \X”illiam Kelly, MI),
I)avid (;rtnt LISAF Medical Center, Travis Air Force
Base, (:alif.)
a. b.
Figure 28. Infection in a 25-year-old woman with
sepsis after a tonsillectomy. She developed multiple
lower cranial nerve palsies on the right side, fol-
lowed by left hemiparesis. (a) Axial Ti-weighted MR
image demonstrates infection involving the right
prevertebral, retropharyngeal, and nasopharyngeal
carotid spaces. (b) Contrast-enhanced Ti-weighted
MR image at a higher level shows signal intensity
typical of fluid within an abscess cavity (A). Enhance-
ment of the carotid arterial wall (arrowheads) and
marked luminal narrowing are noted. (c) Gradient-
echo MR image demonstrates absent flow in the yes-
sels of the carotid space on the right side (compare
the normal flow voids Earrowsi in the vessels on the
left side).
C.
. Sublingual Segment
Malignant tumors are the most common cause
of hypogbossal nerve paralysis in this region. Lo-
cally invasive squamous cell carcinoma is most
often seen (Fig 29), arising from the base or the
oral portion of the tongue. Salivary gland tu-
mors occur less commonly but may also lead to
rr;
palsy of the 1 2th cranial nerve. Odontogenic ab-
scesses involving the sublingual space may also
affect the distal portions of the nerve (Fig 30).
i1 -.
Temporary palsy of the hypoglossal nerve after
Figure 29. Carcinoma of the tongue in a 62-year- extraction of the third molar and after tonsillec-
old man who was found to have a tongue mass by
torny has been reported (20).
his dentist. Axial contrast-enhanced CT scan shows a
large soft-tissue mass (arrowheads). The mass shows
some peripheral enhancement and crosses the mid-
line.