995
CLINICAL NOTE
Pure Trigeminal Motor Neuropathy: A Case Report
Yoon-Kyoo Kang, MD, PhD, Eun-Ha Lee, MD, Miriam Hwang, MD
ABSTRACT. Kang Y-K, Lee E-H, Hwang M. Pure trigemi-
nal motor neuropathy: a case report. Arch Phys Med Rehabil
2000;81:995-8.
Pure trigeminal motor neuropathy is a trigeminal motor
paralysis unaccompanied by signs of trigeminal sensory or
other cranial nerve involvement. We present a case of a
38-year-old man complaining of progressive weakness and
wasting of the right masticatory muscles after a bout of mild
cold symptoms. No sensory symptoms were present. History,
neurologic examination, electrophysiologic studies, and mag-
netic resonance imaging of the brain led to the diagnosis of pure
trigeminal motor neuropathy. The cause may have been an
autoimmune reaction to a viral infection.
Key Words: Trigeminal nerve; Neuropathy; Masticatory
muscles; Electrophysiology; Magnetic resonance imaging; Re-
habilitation.
r 2000 by the American Congress of Rehabilitation Medi-
cine and the American Academy of Physical Medicine and
Rehabilitation
T RIGEMINAL NEUROPATHY is commonly seen as a
prolonged disorder of sensation in the distribution of the
fifth cranial nerve. The problem is generally unilateral and
involves more than one division. Despite the many studies on
trigeminal neuropathy, few patients present with signs of
trigeminal motor impairment.1-3 Reports of pure trigeminal
motor neuropathy are even rarer.4-6 The following case is one of
a trigeminal motor neuropathy with sensory sparing in the
distribution of the fifth cranial nerve.
CASE REPORT
History
A 38-year-old man was referred for electrophysiologic
examination because of complaints of a sunken cheek and sense
of chewing weakness on the right side. He first noticed the
masticatory weakness several days after symptoms of a com-
mon cold, 7 months before his initial visit. He subsequently
recognized that the right temporal and cheek areas had sunk
progressively, without pain or sensory change on the affected
side. The patient denied any history of specific neurologic
disease affecting the peripheral nerves or exposure to any
neurotoxic chemicals. He also denied systemic symptoms, such
as weight loss, fever, or sweating, and his past history and
family history were noncontributory.
Examination
Examination revealed atrophy of the right temporal and
cheek areas (fig 1). Weakness of the jaw-closing muscles was
From the Department of Rehabilitation Medicine, Korea University Medical Center
Guro Hospital, Republic of Korea.
Submitted June 28, 1999. Accepted in revised form November 16, 1999.
The authors have chosen not to select a disclosure statement.
Reprint requests to Yoon-Kyoo Kang, MD, PhD, Department of Rehabilitation
Medicine, Korea University Medical Center Guro Hospital, 80 Guro-dong, Guro-gu,
Seoul 152-703, Republic of Korea.
0003-9993/00/8107-5707$3.00/0
doi:10.1053/apmr.2000.6273
confirmed with muscle testing: patient was asked to clench his
teeth together as tightly as possible while the examiner gave
resistance vertically downward.7 The jaw could not be opened
because of normal strength of the intact contralateral side.
However, the patient could not move the jaw against resistance
toward the left side. The jaw deviated toward the right side
when he opened his mouth fully.
The light and corneal reflexes were active bilaterally, and all
the senses, including pain, temperature, and pinprick on the
face, were normal. Movements of extraocular muscles and
facial expression muscles were intact and symmetric. Taste
sensation, swallowing function, voice, and tongue motions
were normal. No other signs indicated central or peripheral
nervous system involvement.
Laboratory Tests
All hematologic, serologic, and urine chemical laboratory
test results were normal except for an elevated erythrocyte
sedimentation rate of 30 mm/h. A basal skull x-ray revealed that
the longest diameter of the right foramen ovale was somewhat
longer than that of the left side. However, no destructive mass
lesion was found near the foramen ovale on brain computed
tomography (CT) or magnetic resonance imaging (MRI). CT
and T1-weighted MRI of the brain showed atrophy of the
muscles innervated by the right trigeminal motor nerve (ie, the
masseter, medial and lateral pterygoids, and temporalis muscles)
(fig 2). A small round retention cyst was detected in the right
maxillary sinus near the anterior wall. No space-occupying
lesions or degenerative changes were seen in the brain or the
brainstem, including the rostal pons, on MRI.
Electrodiagnostic Examination
Several electrodiagnostic studies were performed.a The tri-
geminal blink reflex to evaluate the afferent loop of the
ophthalmic branch was performed by stimulating the supraor-
bital nerve and recording the response at the orbicularis oculi
muscle. The test showed normal latencies and amplitudes
bilaterally (table 1).
The function of the proprioceptive afferent and efferent
pathways through muscle spindles of the masseter muscle, the
mesencephalic trigeminal nucleus, and motor neurons to extra-
fusal muscle fibers was assessed by means of the masseter
reflex study. Stimulation was given by tapping the jaw with an
electronic reflex hammer while the active recording electrodes
were placed over the masseter muscles bilaterally. This study
revealed a trivial response on the right side compared with a
normal response on the sound side (fig 3, table 1).
We performed a mental nerve–stimulated trigeminal somato-
sensory–evoked potential study, using standard techniques to
check the sensory division of the mandibular branch of the
trigeminal nerve. For the recording, the filter setting was 30 to
3,000Hz, with stimulation duration 0.2msec, sweep speed
10msec, and sensitivity 1µV/div. This study showed symmetri-
cal normal responses on both sides (table 1).
An auditory brainstem–evoked response study was per-
formed using standard techniques to evaluate the brainstem
auditory pathway function. For the test, filters were set at 150
and 3000Hz with rarefaction clicks at 11.1Hz and an intensity
of 75dB. Normal responses were found bilaterally (table 1).
Arch Phys Med Rehabil Vol 81, July 2000
996 PURE TRIGEMINAL MOTOR NEUROPATHY, Kang

Fig 2. Frontal view of the patient’s brain MRI. Note wasting and fat
replacement of the right jaw-closing muscles: (a) masseter, (b)
medial pterygoid, and (c) lateral pterygoid.

retention cyst in the right maxillary sinus was near the anterior
wall, so it was not considered to be related to the present
neuropathy.
The patient was diagnosed with pure trigeminal motor
neuropathy on the basis of neurologic, radiologic, and electro-
physiologic findings. No demonstrable causal factors could be
found through the laboratory or electrophysiologic examina-
tion. Thus, the cause may have to be classified as idiopathic.
However, we cannot rule out the postviral infection phenom-
enon as a possible predisposing factor after the antecedent bout
of a common cold.
Fig 1. Right temporal and cheek areas have a sunken appearance
from atrophy of the masticatory muscles.
Clinical Course
The patient was followed up periodically for the next 12
A monopolar needle electrode was used for the needle months. After the initial evaluation, atrophy of some of the
electromyography (EMG). Spontaneous activity at rest, recruit- mouth opening muscles, such as the anterior belly of the
ment patterns, and shapes of the voluntary motor unit potentials
of the masticatory muscles were documented. Profuse abnormal Table 1: Electrodiagnostic Findings
spontaneous activities (fibrillation potentials and positive sharp R1 Lat R2 Lat
waves) occurred in the right temporalis and masseter muscles. Blink reflex Stimulation Recording (msec) (msec)
No voluntary motor unit potentials were seen in these muscles.
R supra-orb R 9.5 31.3
Frontalis, nasalis, and orbicularis oris muscles innervated by the
L — 31.4
facial nerve; sternocleidomastoid and trapezius muscles inner-
L supra-orb R — 32.2
vated by the spinal accessory nerve; and tongue muscles
L 9.4 32.4
innervated by the hypoglossal nerve were also examined. All
had normal silence at rest and normal motor unit potentials on Masseter reflex Stimulation Lat (msec) Amplitude
volition.
Right 5.7 ⬍100µV
Diagnosis Left 5.3 ⬎1mV

The electrodiagnostic findings were most consistent with a Mental nerve SSEP Stimulation P1 Lat (msec)
pure trigeminal motor neuropathy unaccompanied by sensory
Right 18.7
abnormalities. The afferent pathways of the trigeminal nerve
Left 19.0
were regarded as intact when trigeminal somatosensory–
evoked potential and blink reflex studies were considered. On Auditory Brainstem–Evoked Response
the other hand, abnormal findings of the masseter reflex and Latency (msec) I II III IV V
needle EMG study indicated denervation signs of the efferent
motor pathway to masticatory muscles. R 1.75 2.33 3.91 5.11 5.37
Although laboratory study findings were inconclusive, MRI L 1.54 2.16 4.08 5.16 5.66
findings of atrophy of the trigeminal innervated muscles added Abbreviations: L, left; Lat, latency; R, right; SSEP, somatosensory–
diagnostic evidence to the electrophysiologic findings. The evoked potential; supra-orb, supraorbital nerve.

Arch Phys Med Rehabil Vol 81, July 2000

 PURE TRIGEMINAL MOTOR NEUROPATHY, Kang
Fig 3. Masseter reflexes elic-
ited by electrical hammer. The
response was trivial on the
right side.
digastric and mylohyoid muscles on the mouth floor, was
noticed. However, there was still no change in neurologic
symptoms or signs reflecting any other abnormalities of the
central or peripheral nervous system, including the trigeminal
sensory nerve. The patient did not mention any change in his
general condition or sense of well-being. No medication or
operative management was required because the symptoms and
signs were already stable at his first visit.
Four years afterward, the patient reported a little improve-
ment in chewing power. Minimal muscle contraction was
palpable in the right temporal fossa. Follow-up masseter reflex
study showed an amplitude of 159µV on the affected side, an
improvement from the previous examination result of an
amplitude around 50µV. Needle EMG examination of the
masseter and temporalis muscles, which initially demonstrated
no motor unit potentials, showed decreased insertional activi-
ties with a single long durational motor unit potential on
volition. We consider the patient’s condition to be benign in
light of the clinical course.
DISCUSSION
Pure trigeminal motor neuropathy is a trigeminal motor
paralysis unaccompanied by trigeminal sensory signs or other
cranial nerve involvement. It is rarely reported, and histologic
evidence of a lesion has not yet been reported. Even in the
idiopathic trigeminal neuropathy, weakness of the muscles
innervated by the trigeminal motor nerve is relatively rare.8 The
etiology of the pure trigeminal motor neuropathy remains
obscure. Suggested causes include neurofibromatosis,4 viral
infection,5 multiple sclerosis,9 trauma,10 or unknown factors.5,6
The lesions producing trigeminal motor nerve symptoms
may occur anywhere along the protracted course of the fifth
cranial nerve from its nucleus in the brainstem to its distal facial
ramifications to the masticatory muscles. The trigeminal nerve
has four brainstem nuclei: the main sensory nucleus, the spinal
nucleus, the motor nucleus, and the mesencephalic nucleus,
which is involved in proprioception. The motor nucleus of the
trigeminal nerve is located medial to the principal sensory
nucleus in the rostal pons. The large sensory and smaller motor
roots exit via the lateral pons as a common trunk and the motor
root remains inferior to the sensory root through the course in
the preganglionic segment and the trigeminal ganglion. The
trigeminal ganglion lies in Meckel’s cave, and distal to the
ganglion, the trigeminal nerve trifurcates into its three principal
997
branches, the ophthalmic (V1 ), maxillary (V2 ), and mandibular
nerves (V3 ). The mandibular nerve runs along the skull base
laterally and exits through the foramen ovale. The motor root
bypasses the trigeminal ganglion altogether, joining V3 as it
exits the skull base through the foramen ovale. As V3 exits the
skull base, it enters the nasopharyngeal masticator space. It then
divides into several sensory branches with the principal ones
including the buccal, auriculotemporal, inferior alveolar, and
lingual nerves. The sensory branches of V3 supply sensation to
the lower third of the face, the tongue, the floor of mouth, and
the jaw.11
In addition to the sensory branches, the peripheral extensions
of the motor nucleus that fuse with V3 and make up the motor
division of the trigeminal nerve have two major branches, the
masticator nerve and the mylohyoid nerve. The masticator
nerve supplies motor innervation to the masseter, temporalis,
and medial and lateral pterygoid muscles, and the mylohyoid
nerve supplies the mylohyoid and anterior belly of the digastric
muscles. The medial pterygoid muscle is innervated by a small
nerve that originated slightly below the foramen ovale, the
lateral pterygoid muscle is innervated by the buccal nerve, the
temporalis muscle is innervated by the anterior and posterior
deep temporal nerves, and the masseter muscle is innervated by
the masseteric nerve.8 All these branches that supply the
jaw-closing muscles originate before the mandibular nerve
divides into its terminal mixed nerves.12 This patient showed
atrophy on MRI and denervation of all the jaw-closing muscles;
therefore, anatomically or clinically the possible lesion site
must be located at or proximal to the foramen ovale.
Jannetta and Robbins13 reported five cases of trigeminal
neuropathy of idiopathic variety. Among the five cases, they
discovered microvascular loops of superior cerebellar artery
compressing the trigeminal nerve at the root entry zone in four
cases. They suggested that in persons with persistent idiopathic
trigeminal neuropathy, with or without intractable pain, retro-
mastoid craniectomy with microvascular decompression of the
fifth nerve could be a therapeutic alternative.
Central lesions causing fifth cranial symptoms were also
reviewed,13 and these include multiple sclerosis, glioma, stroke,
metastasis, cavernous angioma with hemorrhage, and syringo-
hydrobulbia. In the present case, those lesions were ruled out by
the brain MRI study.
Hutchins and associates14 found clinical findings to be
extremely inaccurate in localizing the lesion and difficult to
Arch Phys Med Rehabil Vol 81, July 2000
998 PURE TRIGEMINAL MOTOR NEUROPATHY, Kang
correlate with radiologic findings in trigeminal neuropathy.
They therefore set up an MRI protocol to evaluate trigeminal
neuropathy, which included (1) a sagittal T1-weighted 800/30
(time to repetition/time to spin echo) for a localizing scan, (2)
an axial T2-weighted 2000/30,80 for a brainstem and central
projection scan, and (3) an axial and coronal T1-weighted
800/30 scan from mid-pons, including orbit and maxillary sinus
for cisternal, skull base, and extracranial V1, V2, and proximal
V3 scans. Because of the frequency of clinically occult perineu-
ral tumor spread, they recommended that all patients with
trigeminal nerve symptoms should undergo MRI according to
this protocol. Our patient’s MRI results did not show an
anatomic lesion other than atrophy, but lesions that are difficult
to detect by an imaging study, such as entrapment of the nerve,
may be present.
Cruccu and coworkers12 said that facial hemiatrophy involv-
ing deep tissue such as fat, muscles, ligaments, cartilage, and
bone might lead to stretching, angulation, or compression,
followed by focal demyelination of the masticatory nerves.
Because of their anatomical relationship—they turn sharply
around bone crests and finally run a narrow course between the
bone and their own target muscles—these nerves may suffer
from entrapment.
The peripheral motor neurons are different from the sensory
neurons in various aspects. Although estimating antiganglioside
antibody to GM1 ganglioside may help physicians diagnose
multifocal motor neuropathy with conduction block in patients
with pure motor neuropathy, it helps few patients who have
other conditions.15 Engel and colleagues16 showed that motor
nerve fascicles had significantly higher choline acetylase activ-
ity than did sensory nerves. Motor nerves contain a higher
proportion of axons with strong acetylcholinesterase activity,
and the overall intensity of staining of motor nerves is greater
than that of sensory nerves.17,18 Taylor and coworkers19 found
that the high titer of immunoglobulin M antibodies against
monosialo GM1 occurred only in the patients with pure motor
neuropathy. Synaptobrevin, a membrane protein of synaptic
vesicles, plays a key role in exocytosis. Li et al20 reported that
synaptobrevin I is present predominantly in motor neurons,
whereas synaptobrevin II is present in adrenergic and sensory
neurons in cases of lumbar sympathectomy, ventral rhizotomy,
and double-labeling studies. These differences may account for
the different susceptibility of motor and sensory neurons.
We thoroughly evaluated our patient through history and
clinical, laboratory, electrophysiologic, and radiologic examina-
tions. The motor branch of the trigeminal nerve was damaged,
but sensory nerve function was preserved clinically and electro-
physiologically. The cause of the neuropathy remains obscure,
but was possibly a post–viral-infection autoimmune response
that predominantly affected the motor division of the trigeminal
nerve because it is less susceptible than the sensory neurons.
Other possible causes, such as malignancy or other mass
lesions, have been ruled out clinically in follow-up observation.
Arch Phys Med Rehabil Vol 81, July 2000
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Supplier
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