C H A P T E R 95 
Brain and Cranial Nerve Disorders
This chapter discusses cranial nerve (CN) problems, cerebral
venous thrombosis (CVT), and multiple sclerosis (MS)—neuro-
logic disorders that often provide diagnostic and therapeutic
challenges in the emergency department (ED) setting (Table 95.1).
TRIGEMINAL NEURALGIA (CRANIAL NERVE V)
Principles
Trigeminal neuralgia, or tic douloureux, is a syndrome featuring
painful paroxysms in one or more distributions of the trigeminal
nerve (CN V). Trigeminal neuralgia is more common in women
than in men, with a female-to-male ratio of 2 : 1. Affected persons
typically are between 50 and 69 years old, and symptoms occur
more frequently on the right side of the face.
Trigeminal neuralgia is an idiopathic disorder, although evi-
dence points to vascular compression of the trigeminal nerve root
in many cases. This compression commonly is caused by a tortu-
ous arterial or venous loop in the posterior fossa, an arteriovenous
malformation, or rarely a tumor. Although structural lesions are
not found in all patients, surgical case series report up to 90% of
cases having vascular compression of the trigeminal nerve root.
Clinical Features
Trigeminal neuralgia is manifested with unilateral facial pain,
which is typically characterized as lancinating paroxysms of
pain in the lips, teeth, gums, or chin. The pain is commonly associ-
ated with physical triggers, such as chewing, brushing the teeth,
shaving, washing or touching the affected area of the face, swal-
lowing, or exposure to hot or cold temperature in the affected
area. The maxillary and mandibular divisions of the trigeminal
nerve are most commonly involved; rarely, the ophthalmic divi-
sion alone is involved. Patients tend to experience the pain
in clustered episodes that last a few seconds to several minutes.
The attacks can occur during the day or night but rarely arise
during sleep.
The physical examination in patients with facial pain includes
an evaluation of the sinuses, teeth, and the CNs. Given the loca-
tion of the pain, the ears must be examined for signs of otitis, the
teeth and face examined for evidence of odontogenic infection
(such as, facial swelling or gingival fullness), and the skin and
scalp examined for the painful vesicular eruption of zoster. Puru-
lent drainage from the sinus indicates sinusitis as a possible
underlying cause requiring treatment before continued pursuit of
a diagnosis of trigeminal neuralgia.
Differential Diagnosis
Other painful facial conditions that are considered in patients
with facial pain include odontogenic infections, sinus disease,
otitis media, acute glaucoma, temporomandibular joint disease,
and herpes zoster. Although the temporal components of the
pain in these conditions are not similar to the sudden onset,
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Brian A. Stettler
lancinating pain of trigeminal neuralgia, the distribution is similar
and these diagnoses should be considered before anchoring on a
diagnosis of trigeminal neuralgia. Two percent to 4% of patients
with trigeminal neuralgia also have MS, which should be consid-
ered in patients who present with neurologic findings that do not
fit a specific pattern.
Diagnostic Strategies
Patients with normal findings on the head and neck examination
and no neurologic deficits who have episodic, unilateral facial
pain associated with nonpainful triggers are likely to have trigemi-
nal neuralgia. The presence of a neurologic deficit should
prompt suspicion of a structural lesion, such as aneurysm, tumor,
or other intracranial lesion (eg, from MS). Patients with a
neurologic deficit require urgent imaging studies, typically mag-
netic resonance imaging (MRI), to rule out a mass or vascular
abnormality.
Management
The medical treatment of choice for trigeminal neuralgia is
carbamazepine. The mechanism of action of anticonvulsant
therapy for trigeminal neuralgia is unclear, but carbamazepine
appears to be an effective and well-tolerated treatment. The initial
dosage is 100 mg twice daily, increased to three times daily after
1 week. The dose may then be increased by 100 mg/day, up to
a maximum of 1200 mg/day. A complete blood count and liver
function studies are performed periodically in patients who are
taking carbamazepine to monitor for hematologic and hepatic side
effects. Additional agents that have been used for treatment of tri-
geminal neuralgia include phenytoin, baclofen, valproate sodium,
lamotrigine, gabapentin, and levetiracetam. None has been shown
to be more effective than carbamazepine. Several recent studies
have investigated the use of onabotulinum toxin A for refractory
pain management in trigeminal neuralgia. Although the studies
are small, there are two randomized controlled trials that have
shown decreased pain scores and increased quality of life with
this therapy when combined with conventional medications.1,2
Both peripheral and central surgical interventions for trigemi-
nal neuralgia are management options for difficult cases. Periph-
eral strategies include medication injection and cryotherapy
techniques designed to temporarily block or permanently ablate
branches of the peripheral trigeminal nerve. Although these
procedures are relatively effective initially, recurrence is common.
Central procedures include percutaneous destruction of the tri-
geminal ganglion, although these procedures carry the risk of
corneal anesthesia, oculomotor paresis, or masticatory weakness.3
Open surgical management includes microvascular decompres-
sion of the nerve with or without partial ablation. Although pain
relief is achieved in 80% to 95% of patients, the surgery is associ-
ated with the risk of complications. Gamma knife radiosurgery, a
minimally invasive, precision-directed stereotactic radiosurgery,
has shown good outcomes.4
1289
1290 PART III  Medicine and Surgery  |  SECTION Seven  Neurology
TABLE 95.1 
The Cranial Nerves: Normal Function and Pathologic Considerations
CLINICAL FUNCTION RELEVANT
CRANIAL NERVE TO EMERGENCY MEDICINE PATHOLOGIC FEATURES
CN I: Olfactory nerve Sense of smell
CN II: Optic nerve Vision
CN III: Oculomotor nerve Extra oculomotor function via
motor fibers to levator
palpebrae, superior rectus,
medial rectus, inferior rectus,
inferior oblique muscles
Pupillary constriction via
parasympathetic fibers to
constrictor pupillae and ciliary
muscles
CN IV: Trochlear nerve Motor supply to the superior
oblique muscle
CN V: Trigeminal nerve Motor supply to muscles of
mastication and to tensor
tympani
Sensory to face, scalp, oral cavity
(including tongue and teeth)
CN VI: Abducens nerve Motor supply to the lateral rectus
muscle
CN VII: Facial nerve Motor supply to muscles of facial
expression
Parasympathetic stimulation of the
lacrimal, submandibular, and
sublingual glands
Sensation to the ear canal and
tympanic membrane
CN VIII: Vestibulocochlear Hearing and balance
nerve
CN IX: Glossopharyngeal General sensation to posterior
nerve third of tongue
Taste for posterior third of tongue
Motor supply to the
stylopharyngeus
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Unilateral anosmia
Unilateral vision loss
Ptosis caused by loss of levator
palpebrae function
Eye deviated laterally and down
Diplopia
Dilated, nonreactive pupil
Loss of accommodation
Inability to move eye downward and
laterally
Diplopia
Patients tilt head toward unaffected
eye to overcome inward rotation
of affected eye
Partial facial anesthesia
Episodic, lancinating facial pain
associated with benign triggers,
such as chewing, brushing teeth,
light touch
Inability to move affected eye
laterally
Diplopia on attempting lateral gaze
Hemifacial paresis:
Lower motor neuron lesion leaves
entire side of face paralyzed
Upper motor neuron lesion leaves
forehead musculature functioning
Abnormal taste
Sensory deficit around ear
Intolerance to sudden loud noises
Unilateral hearing loss
Tinnitus
Vertigo, unsteadiness
Clinical pathology referable to the
nerve in isolation is very rare
Occasionally painful paroxysms
beginning in the throat and
radiating down the side of the
neck in front of the ear but
behind the mandible
POSSIBLE CAUSES
Trauma: Skull fracture or shear injury
interrupting olfactory fibers traversing the
cribriform plate
Tumor: Frontal lobe masses compressing the
nerve
Trauma: Traumatic optic neuropathy
Tumor: Orbital compressive lesion
Inflammatory: Optic neuritis (MS)
Ischemic: Ischemic optic neuropathy
Trauma: Herniation of the temporal lobe
through the tentorial opening, causing
compression and stretch injury to the
nerve
Ischemic: Especially in diabetes; microvascular
ischemic injury to nerve causes
extraocular muscle paralysis but usually is
papillary sparing (often painful)
Vascular: Intracranial aneurysms may press on
the nerve, leading to dysfunction
Myasthenia gravis can lead to atraumatic
ocular muscle palsy
Trauma is the most common cause of nerve
dysfunction
Trauma: Facial bone fracture may injure one
section, leading to area of facial
anesthesia
Tic douloureux
Tumor: Lesions in the cerebellopontine angle
Any lesion, vascular or otherwise, in the
cavernous sinus may compress nerve
Elevated ICP: Because of its position and long
intracranial length, increased ICP from
any cause may lead to injury and
dysfunction of the nerve
Lower motor neuron:
Infection (viral): The likely cause of Bell’s palsy
Lyme disease: The most common cause of
bilateral CN VII palsy in areas where Lyme
disease is endemic  
Bacterial infection extending from otitis
media
Upper motor neuron: Stroke, tumor
Tumor: Acoustic neuroma
Mimics Ménière’s disease, perilymphatic
fistula
Brainstem lesion
Glossopharyngeal neuralgia

TABLE 95.1 
The Cranial Nerves: Normal Function and Pathologic Considerations—cont’d
CLINICAL FUNCTION RELEVANT
CRANIAL NERVE TO EMERGENCY MEDICINE PATHOLOGIC FEATURES
CN X: Vagus nerve Motor to striated muscles and
muscles of the pharynx, larynx,
and tensor (veli) palatini
Motor to smooth muscles and
glands of the pharynx, larynx,
thoracic and abdominal viscera
Sensory from larynx, trachea,
esophagus, thoracic and
abdominal viscera
CN XI: Spinal accessory Motor supply to the
nerve sternocleidomastoid and
trapezius muscles
CN XII: Hypoglossal Motor supply to the intrinsic and
nerve extrinsic muscles of the tongue
CN, Cranial nerve; ICP, intracranial pressure; MS, multiple sclerosis.
Disposition
Patients with newly suspected trigeminal neuralgia should be
referred for specialty evaluation. Coordination with a neurologist
will help in therapeutic decision making. Patients with an estab-
lished diagnosis who present with uncontrolled pain refractory to
maximum pharmacologic management may require a neurosur-
gical consultation for an operative intervention.
FACIAL NERVE PARALYSIS (CRANIAL NERVE VII)
Principles
Peripheral paralysis has no geographic, gender, or race predilec-
tion; pregnancy is a risk factor.5 The facial nerve (CN VII)
innervates the muscles of facial expression and the muscles of the
scalp and external ear in addition to the buccinator, platysma,
stapedius, stylohyoid, and posterior belly of the digastric muscles.
The sensory portion of the nerve supplies the anterior two-thirds
of the tongue with taste and sensation to portions of the external
auditory meatus, soft palate, and adjacent pharynx. The parasym-
pathetic portion supplies secretomotor fibers for the submandibu-
lar, sublingual, lacrimal, nasal, and palatine glands.
The nerve originates from the pontomedullary junction of
the brainstem and enters the internal auditory meatus with CN
VIII. Within the temporal bone, the facial nerve has four major
branches: the greater and lesser superficial petrosal nerves, the
nerve to the stapedius muscle, and the chorda tympani. The facial
nerve exits the temporal bone at the stylomastoid foramen and
then enters the parotid gland, where it divides to supply the
muscles of facial expression.
Clinical Features
The medical history in patients with facial paralysis focuses on
onset of the paralysis, concentrating on timing and rapidity of
onset and associated signs and symptoms. The most common
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CHAPTER 95  Brain and Cranial Nerve Disorders 1291
POSSIBLE CAUSES
Unilateral loss of palatal elevation: Brainstem lesion
Patients complain that on Injury to the recurrent laryngeal nerve during
drinking liquids, the fluid refluxes surgery
through the nose
Unilateral vocal cord paralysis:
Hoarse voice
Downward and lateral rotation of Trauma to the nerve
the scapula and shoulder drop
Tongue deviations: Stroke or tumor can cause upper motor
Upper motor neuron lesion causes neuron lesion
the tongue to deviate toward the Amyotrophic lateral sclerosis can cause
opposite side bilateral lower motor neuron lesion with
Lower motor neuron lesion causes atrophy
the tongue to deviate toward the Metastatic disease to the skull base may
side of the lesion, and the involve the nerve
affected side atrophies over time
causes of a CN VII palsy are Bell’s palsy, Ramsey Hunt syndrome,
Lyme disease, and bacterial infections of the middle ear, mastoid,
or external auditory canal.
Bell’s Palsy
Bell’s palsy, also commonly called idiopathic facial paralysis, is
postulated but not confirmed to have a viral cause. It is character-
ized by an abrupt onset of a lower motor neuron paresis that
typically develops over 72 hours and can progress during 1 to 7
days to complete paralysis. A prodromal viral-like illness is
described by 60% of patients. Symptoms and signs frequently
associated with the facial paresis include ear pain, perception of
sensory change on the involved side of the face, decreased tearing,
overflow of tears on the cheek (epiphora), abnormally acute
hearing (hyperacusis), and impairment or perversion of taste
(dysgeusia).
To make the diagnosis of Bell’s palsy, both the upper and lower
facial muscles must be involved. If only lower face involvement
can be elicited, there should be a suspicion for a central lesion,
such as a cerebral infarct or neoplasm. Lid closure is assessed by
asking the patient blink rapidly; if the patient is unable to fully
close the eye, a diagnosis of Bell’s palsy suspected.
Ramsey Hunt Syndrome
Ramsay Hunt syndrome (herpes zoster oticus) is characterized by
unilateral facial paralysis, a herpetiform vesicular eruption, and
vestibulocochlear dysfunction. The vesicular eruption, which may
follow the facial paralysis by a few days, may occur on the pinna,
external auditory canal, tympanic membrane, soft palate, oral
cavity, face, and neck as far down as the shoulder. The pain is
considerably more severe than that associated with Bell’s palsy,
and it frequently is out of proportion to physical findings. In
addition, outcomes are worse than with Bell’s palsy, with a lower
incidence of complete facial recovery and the possibility of senso-
rineural hearing loss.
1292 PART III  Medicine and Surgery  |  SECTION Seven  Neurology
Lyme Disease
Systemic symptoms or bilateral facial paresis, especially in endemic
areas, should raise the possibility of Lyme disease. Lyme disease is
the most frequent vector-borne infection in the United States. It
is caused by the spirochete Borrelia burgdorferi and is spread by
the bite of Ixodes genus ticks. Neurologic manifestations can arise
in any phase of the disease, and facial palsy accounts for up to
50% of the neurologic presentations. In regions in which Lyme
disease is endemic, it has been shown to be the leading cause of
facial paralysis in children.
Bilateral facial nerve paralysis is rare but can occur with sys-
temic infections. The two diseases most commonly associated
with bilateral simultaneous onset of facial paralysis are Lyme
disease and infectious mononucleosis. Bilateral facial paralysis
should be considered to be a manifestation of Lyme disease until
further testing excludes this diagnosis. The evaluation and treat-
ment of Lyme disease are discussed in Chapter 126.
Bacterial Ear Infections
Facial paralysis can be caused by acute bacterial infections of the
middle ear, mastoid, or external auditory canal. In the pre-
antibiotic era, facial paralysis was associated with acute otitis
media in approximately 2% of cases; today, however, it occurs in
only 0.2% of cases. Malignant otitis externa can be associated with
facial paralysis. This disease entity is most commonly seen in
immunocompromised patients and usually is caused by Pseudo-
monas infection.
Differential Diagnosis
In addition to the disorders previously discussed, paralysis of CN
VII can be caused by facial trauma where temporal bone fracture
can cause nerve transection and neoplasia. A history of recurrent
ipsilateral paralysis or slow progression of symptoms is more
characteristic of a tumor. Associated CN abnormalities, such as a
gaze paresis, point to the possibility of a tumor or cerebral isch-
emic insult. Patients will occasionally describe “numbness” in the
same distribution as the weakness to the face, although they rarely
experience the dense sensory loss or weakness of mastication seen
in CN V lesions. Potential involvement of multiple CNs suggests
a stroke or a mass lesion.
Tumors of the facial nerve itself or tumors anywhere along its
course that invade or compress the nerve may lead to facial
paralysis. The course is typically progressive for at least 3 weeks
and typically of slower onset than that which is found in Bell’s
palsy, although up to 25% of tumors will present with a history
of sudden onset of paralysis. Although very rare overall, a neo-
plastic cause should be suspected in patients who suffer from
recurrent ipsilateral facial paralysis, significant pain, prolonged
symptoms, or any other concomitant CN abnormality.
Diagnostic Strategies
The diagnostic evaluation of acute facial nerve paresis is based on
whether the clinical picture is suggestive of a disease process other
than Bell’s palsy. If the clinical history is classic for Bell’s palsy, the
American Academy of Otolaryngology recommends no imaging
or laboratory studies.6 A history that poses potential exposure to
Lyme warrants serologic evaluation for the disease. Although
outpatient testing including electroneurography may ultimately
be performed, this usually is not part of the initial evaluation.
The presence of a “central” seventh nerve paralysis (upper face
sparing) should prompt imaging with computed tomography
(CT) or MRI, and consideration given to the possibility of an
acute stroke or other hemispheric lesion. History or physical
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findings suggestive of a possible tumor require imaging to rule
out a neoplasm. The study of choice will depend on the institution
and preferences of the consultant but typically involves MRI.
Management
Bell’s Palsy
Both medical and surgical treatments of Bell’s palsy are available.
The primary medical therapies for Bell’s palsy center on reducing
inflammatory changes to the nerve with corticosteroids and treat-
ing the presumed viral cause. If these therapies are unsuccessful,
surgical decompression may be considered.
Available evidence strongly favors the use of corticosteroids for
the treatment of Bell’s palsy, and earlier treatment is associated
with better outcomes.7,8 Steroid therapy is believed to inhibit
edema of the nerve, confined within the facial canal, which is
thought to cause or contribute to the nerve injury. Based on the
results of high quality, randomized trials, we recommend treat-
ment with prednisolone, 50 to 60 mg/day per day for 10 days, with
or without a short taper. Therapy should be started as soon as
possible, ideally within the first 24 hours, but it is still recom-
mended for patients without contraindications who seek treat-
ment within 1 week of symptom onset.
A number of studies have supported the contention that Bell’s
palsy may be caused by herpes virus infection. Herpes simplex
virus type 1 DNA has been demonstrated in the endoneurial tissue
of Bell’s palsy patients, although there is conflicting evidence on
the efficacy of antiviral treatment. Some studies have found treat-
ment with corticosteroids plus antiviral medications to be superior
to steroids alone, particularly in the setting of severe palsy or in
those treated within 24 hours of symptom onset.9 Other studies
have found conflicting results.10 Summary statements from the
American Academy of Otolaryngology recommend that antivirals
“be considered” in the treatment of Bell’s palsy, although they
should only be offered in combination with oral steroids and
should be considered more strongly for severe loss of function.10
We recommend valacyclovir, 1000 mg orally three times daily for
7 days, or famciclovir, 750 mg orally for 7 days (Table 95.2).
Valacyclovir and famciclovir have better oral absorption, are
better tolerated, and are dosed less frequently, resulting in higher
compliance than with acyclovir treatment. Although earlier treat-
ment is preferred, treatment should be considered for patients
presenting within 1 week of symptom onset.
Ramsay Hunt Syndrome
The treatment of Ramset Hunt syndrome is similar to that of
Bell’s palsy, although antiviral treatment is more strongly recom-
mended in this disease process in addition to steroid therapy.
Both prednisone and antiviral therapy should be continued for 7
to 10 days.
TABLE 95.2 
Treatment of Bell’s Palsy
MILD/MODERATE DISEASE SEVERE DISEASE
Steroids Prednisolone 50 to 60 mg Prednisolone 50 to 60 mg
daily for 10 days, with or daily for 10 days, with or
without taper without taper
Antivirals Not recommended Consider in addition to
steroids, valacyclovir
1000 mg tid or famciclovir
750 qD for 7 days

Lyme Disease
The treatment of Lyme disease is discussed in Chapter 126.
Bacterial Infections
Treatment bacterial infections causing a CN VII palsy involves
prolonged intravenous (IV) antipseudomonal antibiotic therapy
and may require surgical débridement.
Disposition
Most patients who have a seventh CN paralysis will have a clinical
diagnosis of Bell’s palsy and may be discharged with referral
for short-term follow-up. Patients with a possible hemispheric
process, such as stroke or tumor, require further evaluation and
often hospitalization. Patients thought to have Lyme disease
require immediate initiation of antibiotic therapy.
In patients with a complete facial nerve paralysis and inability
to close the eye, the ipsilateral eye should be patched. Patients
should be advised of the risk for corneal abrasion and corneal
dryness, which is associated with the inability to blink properly or
to close the eye completely. Patients should be counselled regard-
ing their expected timeline for recovery. Although mild paresis
typically recovers within 2 to 3 weeks, complete paralysis may take
up to 6 to 12 months for recovery. Patients with a complete facial
nerve paralysis should be provided with an urgent referral to a
head and neck surgeon.
VESTIBULAR SCHWANNOMA
(CRANIAL NERVE VIII)
Principles
Vestibular schwannoma, formally referred to as acoustic neuroma,
is a rare but important cause of sensorineural hearing loss. It
occurs in middle-aged individuals and is usually unilateral in
nature. Vestibular schwannoma is rarely bilateral, occurring in
approximately 5% of cases and generally associated with type 2
neurofibromatosis. Although histologically benign, vestibular
schwannoma can cause neurologic damage by direct compression
on CN VIII and the other structures in the cerebellopontine angle.
Vestibular schwannomas arise from the Schwann cells covering
the vestibular branch of the CN VIII as it passes through the
internal auditory canal. The tumor may compress the cochlear
(acoustic) branch of the CN VIII, causing hearing loss, tinnitus,
and dysequilibrium. Continued growth of the tumor may result
in compression of structures in the cerebellopontine angle, where
CN V and CN VII may be compressed and damaged. Larger
tumors may further encroach on the brainstem and, if large
enough, may compress the fourth ventricle, ultimately resulting
in signs of increased intracranial pressure (ICP).
Clinical Features
Asymmetrical sensorineural hearing loss is the hallmark of ves-
tibular schwannoma. Up to 15% of patients with this tumor,
however, will have normal results on audiometry. These patients
typically have symptoms, such as unilateral tinnitus, imbalance,
headache, fullness in the ear, otalgia, and facial nerve weakness.
Thus, patients with asymmetrical symptoms should be further
evaluated for vestibular schwannoma even with normal findings
on audiometry.
Vestibular schwannomas are extremely slow-growing tumors,
averaging an approximately 1-mm increase per year, although
many do not grow at all. The median time from symptom onset
to diagnosis is 12 months.
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CHAPTER 95  Brain and Cranial Nerve Disorders 1293
Differential Diagnosis
A majority of disease entities included in the differential diagnosis
for vestibular schwannoma cause symmetrical sensorineural
hearing loss. Asymmetrical sensorineural hearing loss has few
causes other than vestibular schwannoma. Ménière’s disease may
present with asymmetrical findings, but it can be differentiated
from vestibular schwannoma in that the tinnitus of Ménière’s
disease usually is intermittent, whereas the tinnitus of vestibular
schwannoma typically is continuous. In addition, patients with
Ménière’s disease typically describe true vertigo, whereas patients
with a vestibular schwannoma are more likely to describe imbal-
ance or dysequilibrium.
Vestibular schwannomas account for 80% of all cerebellopon-
tine angle tumors, meningiomas are the second most common.
Meningiomas more frequently cause symptoms of facial palsy or
trigeminal nerve abnormality. Of note, however, considerable
similarity between the clinical picture of a meningioma and that
of vestibular schwannoma in the cerebellopontine angle has been
described.
Diagnostic Strategies
When vestibular schwannoma is suspected, the patient is evalu-
ated by audiometry or gadolinium-enhanced MRI. This imaging
technique is extremely sensitive and has led to earlier diagnosis
and a decrease in mean size at detection of vestibular schwannoma.
CT lacks the necessary sensitivity in the posterior cranial fossa to
reliably rule out the presence of vestibular schwannoma. The
smaller the tumor at the time of diagnosis, the more options there
are for therapy and the better the prognosis.
Management
Vestibular schwannoma may be removed surgically or ablated
with stereotactic radiation therapy. In appropriately selected
patients, there is little difference in long-term quality-of-life seg-
regated by type of treatment.11 In general, tumors larger than 3 cm
are recommended for microsurgery because radiation treatments,
such as with the gamma knife, are less effective for local control
and growth arrest in larger masses.12 Smaller tumors are amenable
to use of stereotactic radiation therapy. Stereotactic radiation
therapy generally has good long-term outcomes of local growth
arrest, with nerve salvage approaching 90% or greater. In patients
who are minimally symptomatic with small tumors, serial moni-
toring with MRI is a viable option.
Disposition
Patients with suspected acoustic neuroma should be referred for
audiometry or MRI and evaluation by a specialist in either oto-
laryngology or neurosurgery.
DIABETIC CRANIAL MONONEUROPATHY
Principles
Cranial mononeuropathies occur uncommonly, and usually are a
complication of diabetes. They most often affect the extraocular
muscles. The oculomotor nerve (CN III) is most commonly
affected, followed by the trochlear (CN IV) and abducens (CN VI)
nerves. CN palsies occur in 1% of diabetics versus 0.1% among
nondiabetics. Whereas ophthalmoplegia appears to be closely
related to diabetes, facial palsy is less strongly correlated with this
disease.13
The pathologic basis of diabetic mononeuropathy appears to
be ischemia caused by occlusion of an intraneural nutrient artery
1294 PART III  Medicine and Surgery  |  SECTION Seven  Neurology
serving the nerve. This occlusion leads to injury located primarily
in the core fibers, whereas the peripheral nerve fibers are less
affected because they also are supplied by collateral vessels. In the
oculomotor nerve, the preservation of the circumferentially
located parasympathetic fibers explains the pupillary sparing that
usually is found in this syndrome.
Clinical Features
Patients typically describe acute onset of unilateral retro-ocular and
supraorbital pain, diplopia, and ptosis. The physical manifestations
of a CN III palsy include the inability to move the eye superiorly
and medially, accompanied by ptosis. The pupillary light reflex
usually is present. Although it is a less common finding, CN IV and
CN VI may be affected. Patients with a CN IV palsy are unable to
move the eye inferolaterally, and those with a CN VI palsy are
unable to move the eye laterally. Because of the long intracranial
course of CN VI, a patient with an isolated sixth nerve palsy should
be evaluated for an intracranial lesion or increased ICP.
Differential Diagnosis
Diabetic mononeuropathy generally is a diagnosis of exclusion.
Considerations in the differential diagnosis include trauma, tumor,
vertebrobasilar ischemia, aneurysm, and brainstem hemorrhage.
Diagnostic Studies
Diagnostic imaging is not required in the setting of a “classic”
oculomotor mononeuropathy. If a diabetic patient presents with
an isolated CN III palsy with sparing of the pupillary light reflex
in the absence of other CN or neurologic abnormalities, the
diagnosis can be made presumptively. If the pupillary reflex is lost
in the affected eye, one must be concerned about aneurysm and
a computerized tomographic angiogram (CTA) is indicated. If
other CNs are involved or there are other acute neurologic deficits
present, stroke remains a consideration and CT or MRI should be
obtained. In a patient who presents with a cranial mononeuropa-
thy but without a history of diabetes, a hemoglobin A1c might be
helpful for the providers who assume care of the patient.
Management
Treatment consists of patching the affected eye and administra-
tion of analgesics and antiplatelet therapy. Although there is no
specific cure, patient education regarding glucose control is
important. The prognosis is good and the neuropathy generally
resolves within 3 to 6 months. Antioxidant preparations, includ-
ing α-lipoic acid, have been used therapeutically and have not
shown harm, but such agents have yet to be shown to have con-
vincing clinical effect.14
CEREBRAL VENOUS THROMBOSIS
Principles
Cerebral blood is drained by several major veins that lead into the
dural sinuses. The major dural sinuses are the superior sagittal
sinus, the inferior sagittal sinus, the straight sinus, the lateral
sinuses, and the sigmoid sinuses. The variability in symptoms
and signs in patients who present with CVT stems from differ-
ences in thrombus location and acuity of thrombus formation.
Women represent 60% to 75% of those diagnosed. The short-
term mortality of CVT can be quite high, depending upon time
to diagnosis and severity of neurological symptoms at the time of
presentation.15 With early diagnosis and treatment, mortality rates
are low.
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Infectious causes of CVT include systemic infections and local
infections, such as sinusitis, otitis media, and facial cellulitis.
Noninfectious causes include direct injury to the cerebral venous
system from trauma, surgery, dehydration, or any other condi-
tions causing hypercoagulable states, including the presence of a
malignant neoplasm or the use of oral contraceptive agents.
Clinical Features
Patients with CVT often present with symptoms of intracranial
hypertension, especially headache; patients with CVT are more
prone to hemorrhagic infarction and localizing neurologic defi-
cits. Lethargy, seizures, decreased level of consciousness, or mental
status changes may be noted. A patient’s symptom onset will vary
in accordance with the extent of collateral vessel growth in the
venous territory. Symptoms will appear only when the compensa-
tion for venous thrombosis is no longer sufficient or when hemor-
rhagic infarction occurs. Variability in collateralization between
patients also adds to the variability and time course of symptoms.
The average time from symptom onset to diagnosis of 7 days,
reflecting the difficulty in diagnosis of this rare disease entity.
Because of the broad spectrum of possible clinical features, the
diagnosis of CVT may be difficult but should be a consideration
in any patient with unexplained headache, especially in combina-
tion with focal neurologic deficit, papilledema, or seizures.
Patients with CVT will sometimes complain of diplopia or will
be observed to have a dysconjugate gaze secondary to involvement
of CN IV and CN VI. This can occur due to thrombosis of the
cavernous sinus or simply from elevated ICP. In the presence of
headache or a visual complaint, a funduscopic examination
should be performed to look for papilledema, which is noted in
up to 45% of patients with CVT.
Differential Diagnosis
The differential diagnosis of CVT is broad and includes the condi-
tions that cause patients to present with the new onset of neuro-
logic deficits, alteration in consciousness, or severe headache.
Diagnostic Strategies
Non-contrast CT scanning is commonly employed in the evalua-
tion of patients with severe or unusual headaches, it is neither
sensitive nor specific enough to reliably confirm or exclude the
diagnosis of CVT. Findings on CT that are consistent with CVT
include hyperdensity of a thrombosed sinus or deep vein (referred
to as the cord sign or attenuated vein sign, respectively), brain
edema, and hemorrhage secondary to venous congestion.
MRI can demonstrate local changes secondary to venous
congestion, such as brain edema and hemorrhage. In addition,
MRI can demonstrate the possibility of CVT by the lack of a “flow
void.” On conventional MRI, a flow void indicates the presence of
moving blood within the sinus, whereas the absence of a flow void
indicates a possible thrombus. Diagnostic accuracy, however, is
greatly improved through use of magnetic resonance venography
(MRV). This technique takes advantage of the MRI signal char-
acteristics of flowing blood to create images of venous structures.
Combination of these imaging techniques further enhances diag-
nostic accuracy. For imaging of a particular dural sinus, presence
of the sinus on conventional MRI and lack of flow on MRV are
diagnostic of a sinus thrombosis. This combined approach has
diagnostic sensitivity similar to that of conventional angiography.
MRV and CT venography have similar sensitivities for the
diagnosis of CVT when the CT study is performed on a multide-
tector row CT scanner; the sensitivity of CT venography for CVT
approaches 100% and is comparable to that of MRV both in
sensitivity and in inter-rater reliability. The sensitivity of CT

venography performed by scanners that do not use multidetector
row technology is unknown. Based on the best available evidence,
we recommend MRI/MRV as the diagnostic study of choice when
CVT is suspected, because this continues to be the gold standard
against which all other diagnostic studies are compared.
D-dimer assays may have a role as a screening tool to exclude
CVT, particularly when MRI or CT venography is not available.
Although the reported sensitivity rates are fair at 83% to 100%,
larger prospective studies are needed. In general, although a
normal D-dimer level does not exclude the diagnosis of CVT, the
diagnosis is much less likely, particularly in a patient with symp-
toms of less than 2 weeks in duration.
Management
CVT is a relatively rare disease, and controlled studies evaluating
its treatment are lacking. Current therapeutic consensus strongly
recommends systemic anticoagulation with low–molecular-
weight heparin (LMWH) or unfractionated heparin to prevent
further clot formation and to promote recanalization, even in
patients with intracranial hemorrhage on initial imaging. A reg-
istry comparing outcomes of patients treated with unfractionated
heparin compared with LMWH found more benefit in the LMWH
group, although the effect was modest.16 Despite a paucity of
randomized controlled trials, expert opinion favors anticoagula-
tion in all groups unless another contraindication is present.
Catheter-based intervention with thrombolysis has shown
promise in the management of CVT. Two case series have shown
good outcomes in patients with altered mental status or coma at
the time of presentation. All were treated in a non-randomized
fashion with catheter-based thrombolysis with urokinase or
tissue plasminogen activator (tPA), with 75% of patients recover-
ing to a modified Rankin Scale of 0 or 1.17,18 This promising
therapy is typically considered only for patients with symptoms
of decreased level of consciousness, elevated ICP, or rapid neuro-
logic deterioration.
Disposition
All patients with suspected CVT should be admitted to a unit
capable of providing a high level of care with neurologic consulta-
tion. Patients should be anticoagulated if no contraindication
exists, and catheter-based thrombolysis should be considered in
patients with depressed mental status or focal findings on neuro-
logic examination.
MULTIPLE SCLEROSIS
Principles
MS is an inflammatory disease that affects the central nervous
system (CNS). The pathologic manifestation of this inflammatory
disease is a demyelination of discrete regions (plaques) within the
CNS with a relative sparing of axons. The clinical picture is highly
variable, but it is classically characterized by episodes of neuro-
logic dysfunction that evolve over days and resolve over weeks.
The peak age at onset is 25 to 30 years old; women are slightly
younger than men at onset. The incidence in women exceeds that
of men by a ratio of 1.8 : 1. MS is more common in temperate
climates. The worldwide prevalence is greatest in the United
Kingdom, Scandinavia, and North America. Epidemiologic studies
indicate that both genetic and environmental factors are associ-
ated with an increased incidence of MS. It is rare in Africans and
Asians, but African Americans have a higher incidence than their
relatives who remain in Africa. Thus, an environmental cause
superimposed on genetic susceptibility appears to be a likely etio-
logic scenario.
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CHAPTER 95  Brain and Cranial Nerve Disorders 1295
MS is considered to be an organ-specific autoimmune disease.
One theory proposes that genetic factors interact with an environ-
mental trigger or infection to establish pathologically autoreactive
T cells in the CNS. After a long and variable latency period (typi-
cally 10 to 20 years), a systemic trigger, such as a viral infection or
superantigen, activates these T cells. The activated T cells, on
reexposure to the autoantigen, initiate the inflammatory response.
This sets off a complex immunologic cascade that leads to the
demyelination characteristic of MS. This demyelination process
releases CNS antigens that are hypothesized to initiate further
episodes of autoimmune-induced inflammation.
Clinical Features
The clinical picture in MS is one of marked heterogeneity. The
classic clinical syndrome consists of recurring episodes of neuro-
logic symptoms that rapidly evolve over days and slowly resolve
over weeks. Variability occurs in age at onset, location of CNS
lesions, frequency and severity of relapses, and degree and time
course of progression.
The clinical features of MS can be divided into areas of specific
CNS impairment: cognition; CNs; motor pathways; sensory
pathways; cerebellar pathways; and bowel, bladder, and sexual
dysfunction. Patients with MS have frequent complaints of
poor memory, distractibility, and decreased capacity for sustained
mental effort. Formal neuropsychological testing suggests that
cognitive involvement is common and underreported, affecting
up to 65% of patients. A correlation has been found between
the MRI-based total lesion load and presence of cognitive
impairment.
CN dysfunction is common in MS. The most common associ-
ated CN abnormality is optic neuritis, which is a unilateral syn-
drome characterized by pain in the eye and a variable degree of
visual loss affecting primarily central vision. It is often the first
symptom of MS. Within 2 years of an attack of optic neuritis, the
risk of MS is approximately 20%, and within 15 years, it is
approximately 45% to 80%.
As a result of lesions in the vestibulo-ocular connections, the
oculomotor pathways also may be affected. The deficit may
be manifested as diplopia or nystagmus. The nystagmus may
be severe enough that the patient may complain of oscillopsia (a
subjective oscillation of objects in the visual field). CN impair-
ment also may include impairment of facial sensation, which is
relatively common. Unilateral facial paresis also may occur. In
addition, the occurrence of trigeminal neuralgia in a young person
may be an early sign of MS.
Motor pathways also are commonly involved; specifically,
corticospinal tract dysfunction. Paraparesis or paraplegia occurs
with greater frequency than upper extremity lesions owing to the
common occurrence of lesions in the motor tracts of the spinal
cord. In patients with significant motor weakness, spasms of the
legs and trunk may occur on attempts to stand from a seated
position. This dysfunction is manifested on physical examination
as spasticity that typically is worse in the legs than in the arms.
The deep tendon reflexes are markedly exaggerated, and sustained
clonus may be demonstrated. Although these symptoms frequently
are bilateral, they generally are asymmetrical.
Sensory manifestations are a frequent initial feature of MS and
will be present in nearly all patients at some point during the
course of the disease. Sensory symptoms are commonly described
as numbness, tingling, “pins and needles” paresthesias, coldness,
or a sensation of swelling of the limbs or trunk.
Impairment of the cerebellar pathway may result in gait imbal-
ance, difficulty with coordinated actions, and dysarthria. Physical
examination reveals the typical features of cerebellar dysfunction,
including dysmetria, dysdiadochokinesis (an impairment of rapid
alternating movements), breakdown in the ability to perform
1296 PART III  Medicine and Surgery  |  SECTION Seven  Neurology
complex movements, intention tremor in the limbs and head,
truncal ataxia, and dysarthria.
Impairment of bowel, bladder, and sexual function also is
common. The extent of sphincter and sexual dysfunction usually
parallels the motor impairment in the lower extremities. Urinary
frequency may progress to urinary incontinence as the disease
advances. An atonic bladder may develop, which empties by
simple overflow and often is associated with the loss of perception
of bladder fullness and with anal and genital hypoesthesia. Con-
stipation becomes common in time, and almost all patients with
paraplegia require special measures to maintain effective bowel
habits. Sexual dysfunction, although frequently overlooked, is
common in MS. Approximately 50% of patients become com-
pletely sexually inactive as a result of this disease.
Differential Diagnosis
Other diseases that affect the CNS white matter may be clinically
and radiographically similar to MS. These include CNS tumors
(especially lymphomas and gliomas), spinal cord compression,
vasculitides, Behçet’s disease, neuro-sarcoidosis, postinfectious
and postvaccinal encephalomyelitis, human immunodeficiency
virus (HIV) encephalopathy, Lyme disease, and vitamin B12
deficiency.
Diagnostic Strategies
MS is a relapsing-remitting disorder with symptoms that fluctuate
over time. Therefore, the clinical diagnosis rests on occurrence of
at least two clinical episodes with different neurologic symptoms
at different times.
Although no laboratory tests are diagnostic for MS, one clinical
feature remains unique to this disease: Uhthoff ’s phenomenon,
temporary worsening of current or preexisting signs or symptoms
of MS secondary to small increases in the patient’s body tempera-
ture. Accordingly, exercise, a hot bath, exposure to a warm envi-
ronment, or fever can bring about Uhthoff ’s phenomenon. This
phenomenon reflects subclinical demyelination or preexisting
injury to nerves without obvious significant clinical involvement
before heat exposure or temperature elevation.
Lumbar puncture is recommended for evaluation of patients
with suspected MS, but mass lesions and elevated ICP should be
ruled out before lumbar puncture is attempted. CSF analysis is
abnormal in 90% of the cases. Fifty percent of patients will have
pleocytosis, with more than five lymphocytes per high-power
field. Approximately 70% of patients will have an elevated gamma
globulin level, with immunoglobulin G (IgG) ranging from 10%
to 30% of the CSF total protein. Electrophoresis of the CSF
demonstrates oligoclonal bands of IgG in 85% to 95% of patients
who carry a diagnosis of MS; however, oligoclonal bands of IgG
also are seen with neurosyphilis, fungal meningitis, and other CNS
infections.
The initial imaging test to aid in the diagnosis of MS is
gadolinium-enhanced MRI of the brain and spinal cord. MRI is
a sensitive test for the detection of lesions consistent with MS and
also is useful to assess disease severity. Lesions usually are multiple
and commonly are found in the periventricular white matter. In
patients with an initial neurologic event consistent with CNS
demyelination and an MRI cranial study showing multiple white
matter lesions, the 5-year risk for development of MS is 60%.
Patients with similar clinical syndromes and a normal MRI
appearance have less than a 5% risk.
Management
Management of patients with MS has essentially three aspects:
(1) therapies aimed at halting the progression of the disease,
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(2) treatment of acute exacerbations, and (3) therapies designed
to modify complications.
Treatment of Disease Progression
Although there are many therapies under development for treat-
ment of early or established disease, standard therapies aimed
at halting disease progression are based primarily on the use
of either interferon-β or glatiramer acetate. The interferons are
a group of natural compounds with antiviral and immuno-
modulatory actions used in therapy for MS. Side effects of the
agents interferon-β1a and interferon-β1b include influenza-like
symptoms, depression, anxiety, and confusion. Interferon-β1a
lowers relapse rate, prolongs time to first relapse, and lowers
the accumulation of brain lesions on MRI. Interferon-β also
has been shown to retard progression to clinically definite
MS and to decrease the total number of brain lesions seen on
subsequent MRI studies in patients who have their first demy-
elinating episode with MRI abnormalities at initial presentation.
This finding highlights the importance of early evaluation and
treatment.
Glatiramer acetate is a mixture of synthetic polypeptides
designed to mimic myelin basic protein, which has successfully
been used in the treatment of MS. The mechanism of action by
which glatiramer acetate exerts its effect is unknown, but it is
thought to modify the immune processes responsible for the
pathogenesis of MS. Patients receiving glatiramer acetate experi-
ence significantly fewer relapses and are more likely to demonstrate
neurologic improvement. It has also been shown to slow the
progression to clinically definite MS after a first clinical demyelin-
ating episode.
Current recommendations for management of relapsing-
remitting MS are to initiate treatment with interferon-β or glat-
iramer acetate.19 Such regimens have been demonstrated to
decrease the volume of plaques seen on MRI and to diminish
relapses. Newer disease-modifying agents include fingolimod,
laquinimod, daclizumab, natalizumab, and teriflunomide; their
role is yet to be fully defined.
Treatment of Acute Exacerbations
Acute exacerbations of MS will generally resolve without therapy;
however, steroids diminish the duration. More than 85% of
patients with relapsing-remitting MS show improvement with IV
methylprednisolone. IV steroids have been shown in controlled
trials to speed the recovery from the visual loss of optic neuritis
compared with placebo. In addition, when patients with acute
optic neuritis are treated with high-dose IV steroids, the 2-year
rate of development of MS is reduced, although this effect dimin-
ishes over time. Oral prednisone is not helpful and is associated
with a potential increase in disease flares.
The current standard therapy for an acute exacerbation in
MS is IV methylprednisolone, 250 to 500 mg every 12 hours
for 3 to 7 days. Whether this should be followed by an oral
prednisolone taper remains controversial. Potential adverse
effects of methylprednisolone therapy include fluid retention,
gastrointestinal hemorrhage, anxiety, psychosis, infection, and
osteoporosis. Diagnostic diligence should be exercised in the
evaluation of an acute exacerbation of MS, because many ex-
acerbations are brought on by other medical issues, including
infection. This is especially true of patients with severe preexisting
disease.14
Treatment of Complications
Several therapies directed toward the complications of MS may
be helpful. The associated spasticity generally is treated with

baclofen starting at 5 mg tid and eventually increasing up to
20 mg tid. This is a highly effective therapy aimed at reducing the
painful flexor and extensor spasms. A major side effect is drowsi-
ness, which generally diminishes with continued use. Higher-dose
therapy can cause confusion, especially in the setting of baseline
cognitive impairment. For patients with intractable spasticity,
baclofen is available for intrathecal administration by either bolus
therapy or continuous implanted pump therapy. Additional thera-
peutic agents for control of spasticity include tizanidine, diazepam,
and dantrolene.
The tremor and ataxia associated with MS occasionally are
treated with propranolol, diazepam, or clonazepam. The results
of these therapies, however, generally are unsatisfactory. Pain
often is associated with MS and affects the shoulders, pelvic girdle,
and face. The facial pain may be indistinguishable from that
of trigeminal neuralgia. Treatment options include carbamaze-
pine, baclofen, and tricyclic antidepressants.
KEY CONCEPTS
Trigeminal Neuralgia
• Patients with unilateral, intermittent, lancinating facial pain without
abnormalities on physical examination are likely to have trigeminal
neuralgia.
• Carbamazepine starting at 100 mg bid is the first-line agent for
medical treatment, with a typical therapeutic dose of 600 to
800 mg/day in divided doses.
• Patients who do not tolerate treatment or whose pain is refractory to
medical management may be candidates for microvascular
decompression or ablation.
Facial Nerve Paralysis
• Patients who have facial muscle paresis with intact forehead
movement should be considered to have a central (upper motor
neuron) lesion until the diagnostic investigation proves otherwise.
• Slowly progressive or recurrent facial paralysis is suggestive of a
neoplasm. Recurrent unilateral paralysis may occur with Bell’s palsy
but frequently (30%) is seen in patients with tumor.
• Simultaneous bilateral facial paralysis is suggestive of Lyme disease,
especially in endemic regions.
• Patients with Bell’s palsy should be treated early in the course with
corticosteroids, prednisolone at 50 mg/day for 10 days. Antiviral
medication, valacyclovir, 1000 mg orally three times daily for 7 days,
or famciclovir, 750 mg orally for 7 days should be considered in
patients with severe loss of function.
Vestibular Schwannoma
• The onset of unilateral auditory symptoms, especially sensorineural
hearing loss, requires evaluation and referral to an ear, nose, and
throat specialist.
• Neurologic symptoms of lower CN dysfunction, ataxia, or raised ICP
may be caused by a benign tumor at the cerebellopontine angle.
Diabetic Cranial Mononeuropathy
• Diabetic neuropathy is a diagnosis of exclusion because no definitive
diagnostic testing is available, although a CN III palsy with sparing of
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CHAPTER 95  Brain and Cranial Nerve Disorders 1297
Disposition
Patients with a history of MS who seek treatment for significant
symptoms must first be evaluated to rule out other, non–
MS-related pathologic processes. Other systemic illnesses, espe-
cially infections, which can worsen the symptoms of MS, should
be excluded. If the problem is thought to be a significant exacerba-
tion of MS, most patients will require hospital admission for IV
steroid therapy. Depending on the patient’s neurologic status, an
alternative to hospitalization may be the initiation of IV steroids
in the ED followed by a next-day appointment with the patient’s
primary care physician or neurologist for ongoing IV steroid
administration.
Patients with the new onset of symptoms suggestive of MS
should be admitted or referred to a neurologist, because early
treatment has been shown to significantly decrease progression of
the disease.
the pupillary response in a patient with a history of diabetes is
classic for the presentation.
• Both ischemic and hemorrhagic brainstem lesions must be ruled out
in the case of an acute ophthalmoplegia.
• Extraocular mononeuropathy is sufficiently common in patients with
diabetes mellitus that its occurrence in isolation warrants evaluation
of the patient for previously undiagnosed diabetes.
Cerebral Venous Thrombosis
• The differential diagnosis for CVT includes other conditions
that present with new-onset neurologic deficits, alteration in
consciousness, or severe headache. CVT is more likely to be present
in these patients when the etiology is unclear, the patient is thought
to have a hypercoagulable state, and the head CT is normal in
appearance or shows subtle signs of CVT.
• Non–contrast-enhanced CT scanning is not adequate to rule out
CVT. MRI with MRV is recommended, although multidetector row CT
venography is an acceptable alternative.
• Treatment of most patients with CVT includes systemic
anticoagulation, even in the setting of hemorrhagic cerebral infarcts,
unless another contraindication exists.
Multiple Sclerosis
• MS should be suspected in patients who present with episodes of
neurologic dysfunction that evolve over days and resolve over weeks.
• Apparent exacerbations of known MS can be brought on by other
medical problems, most commonly infections.
• Therapy for patients with MS will require consultation with the
patient’s primary care provider or neurologist to provide consistent
disease management.
• IV methylprednisolone, 250 to 500 mg every 12 hours for 3 to 7
days effectively promotes earlier resolution of recurrences.
• IV methylprednisolone has been shown to speed the recovery from
vision loss from optic neuritis associated with MS.

REFERENCES
1. Zuniga C, et al: Acute treatment of trigeminal neuralgia with onabotulinum toxin A.
Clin Neuropharmacols 36:146, 2013.
2. Wu C, et al: Botulinum toxin type A for the treatment of trigeminal neuralgia: results
from a randomized, double-blind, placebo-controlled trial. Cephalalgia 32:443, 2012.
3. Kouzounias K: Comparison of percutaneous balloon compression and glycerol rhi-
zotomy for the treatment of trigeminal neuralgia. J Neurosurg 113:486, 2010.
4. Baschnagel AM, et al: Trigeminal neuralgia pain relief after gamma knife sterotactic
radiosurgery. Clin Neurol Neurosurg 117:107, 2014.
5. Katz A, et al: Bell’s palsy during pregnancy: is it associated with adverse perinatal
outcome? Laryngoscope 121:1395, 2011.
6. Baugh RF, et al: Clinical practice guideline: Bell’s palsy. Otolaryngol Head Neck Surg
149:S1, 2013.
7. Salinas RA, Alvarez G, Daly F, et al: Corticosteroids for Bell’s palsy (idiopathic facial
paralysis). Cochrane Database Syst Rev (3):CD001942, 2010.
8. Berg T, et al: The effect of prednisolone on sequelae in Bell’s palsy. Arch Otolaryngol
Head Neck Surg 138:445, 2012.
9. Lee HY, et al: Steroid-antiviral treatment improves the recovery rate in patients with
severe Bell’s palsy. Am J Med 126:336, 2013.
10. Axelsson S, et al: Bell’s palsy—the effect of prednisolone and/or valacyclovir versus
placebo in relation to baseline severity in a randomized controlled trial. Clin Otolar-
yngol 37:283, 2012.
CHAPTER 95: QUESTIONS & ANSWERS
95.1. A 53-year-old woman presents with complaints of
increasing left facial pain. She describes a pattern of brief,
excruciatingly painful lancinating sensations along the left
jaw associated with chewing and brushing her teeth. She
notes intermittent clusters of pain that last seconds to a
minute and have not occurred at night. Physical
examination is normal except for triggered left jaw and
buccal pain with palpation of the left mandibular area.
What is the most likely finding in this patient?
A. Analgesia from a left inferior alveolar nerve block
B. Analgesia from subcutaneous sumatriptan
C. Immediate pain relief with high-flow oxygen
D. Magnetic resonance imaging (MRI) evidence of
multiple sclerosis (MS)
E. Vascular compression of the trigeminal nucleus
Answer: E. In 80% to 90% of cases of trigeminal neuralgia, a
vascular compression of the trigeminal nucleus is found in series
of surgical cases. Microvascular decompression of the trigeminal
nerve is curative in a high percentage of patients who fail to
respond to medical management. Approximately 5% of patients
with trigeminal neuralgia have MS. Cluster headache and
migraine treatments are not effective. Peripheral nerve block is
also ineffective because the pathologic process is more central at
the nucleus.
95.2. A 26-year-old man presents with complaints of left facial
drooping. The symptoms began painlessly 3 days ago
without a prodrome. Examination reveals a left facial
droop with an inability to wrinkle the forehead without
other associated physical examination abnormalities.
Which of the following will provide the largest potential
benefit to the patient’s recovery?
A. High-volume lumbar puncture
B. Initiation of oral antiviral treatments
C. Initiation of oral corticosteroids
D. Intravenous (IV) thrombolysis
E. Urgent non–contrast-enhanced computed tomography
(CT) scan of the head
Answer: C. The patient has Bell’s palsy, which is a painless left
facial nerve palsy. The primary symptom is a left peripheral nerve
paralysis often with unilateral dysgeusia, hyperacusis (stapedius
muscle paralysis), and external canal and pharyngeal numbness.
Facial sensation is intact. Corticosteroids are helpful, and they
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For personal use only. No other uses without permission. Copyright ©2017. Elsevier Inc. All rights reserved.
CHAPTER 95  Brain and Cranial Nerve Disorders 1297.e1
11. Brooker JE, et al: Quality of life among acoustic neuroma patients managed by
microsurgery, radiation, or observation. Otol Neurotol 31:977, 2010.
12. Boari N, et al: Gamma knife radiosurgery for vestibular schwannoma: clinical results
at long-term follow-up in a series of 379 patients. J Neurosurg 121:123, 2014.
13. Greco D, Gambina F, Pisciotta M, et al: Clinical characteristics and associated
comorbidities in diabetic patients with cranial nerve palsies. J Endocrinol Invest
35:146–149, 2012.
14. Oynhausen S, et al: Emergency medical care of multiple sclerosis patients: primary
data from the Mount Sinai resource utilization in multiple sclerosis project. J Clin
Neurol 10:21, 2014.
15. Dentali F, et al: Long-term outcomes of patients with cerebral vein thrombosis: a
multicenter study. J Thromb Haemost 10:1297, 2012.
16. Coutinho JM, et al: Unfractionated or low-molecular weight heparin for the treat-
ment of cerebral venous thrombosis. Stroke 41:2575–2580, 2010.
17. Li G, et al: Safety and validity of mechanical thrombectomy and thrombolysis on
severe cerebral venous sinus thrombosis. Neurosurgery 72:730, 2013.
18. Mohammadian R, et al: Treatment of progressive cerebral sinuses thrombosis with
local thrombolysis. Interv Neuroradiol 18:89, 2012.
19. Sorenson PS: New management algorithms in multiple sclerosis. Curr Opin Neurol
27:246, 2014.
should be initiated as far out as a week after onset, although they
should be started within 24 hours if possible. Initiation of oral
antiviral treatment has shown benefit in some trials, although
conflicting evidence exists. In the absence of a contraindication
and in a patient who can afford the medicine, antivirals should be
considered. Given the classic picture and lack of other associated
neurologic findings, CT scan of the head is not required and is
not likely to provide benefit. Testing for Lyme disease is indicated
in Lyme endemic areas or in patients with a history of a tick bite.
95.3. A 43-year-old woman presents with her fourth episode of
left facial paralysis in 1 year. She denies prodrome or
associated symptoms. Examination is consistent with an
isolated left peripheral facial nerve paralysis. What should
be the next step in her management?
A. Antinuclear antibody level and erythrocyte
sedimentation rate
B. Carotid angiography
C. Initiation of antivirals and corticosteroids
D. Magnetic resonance imaging (MRI) scan
E. Neurology referral
Answer: D. A neoplastic cause should be suspected in patients
who suffer from recurrent facial paralysis, significant pain,
prolonged symptoms, or any associated cranial nerve (CN)
dysfunction.
95.4. Which of the following statements regarding acoustic
neuroma is true?
A. An audiogram has a sensitivity of greater than 95%.
B. Gadolinium-enhanced magnetic resonance imaging
(MRI) is the diagnostic test of choice.
C. Symptom onset is generally during 1 to 3 months.
D. Symptoms of increased intracranial pressure (ICP) are
common.
E. The symptoms of Ménière’s disease are essentially
identical.
Answer: B. Computed tomography (CT) lacks the posterior fossa
sensitivity to detect small tumors. Although asymmetrical hearing
loss is the hallmark of this disease, audiograms may be normal in
up to 15% of cases. Symptom onset is generally during years
rather than months. Although increased ICP can happen, it is
uncommon. The tinnitus of Ménière’s disease is typically inter-
mittent rather than continuous.
1297.e2 PART III  Medicine and Surgery  |  SECTION Seven  Neurology
95.5. Which of the following symptoms is not associated with
diabetic cranial mononeuropathy?
A. Diplopia
B. Inability to move the eye inferolaterally
C. Nonreactive pupil
D. Orbital pain
E. Ptosis
Answer: C. Diabetic cranial mononeuropathy may affect the
third, fourth, or sixth cranial nerve (CN). Pain, diplopia, and
ptosis are common. Pupillary reactivity is usually preserved
because these fibers are on the third nerve periphery and less
affected by the occlusion of the “penetrating” neural nutrient
artery affecting the core motor fibers. Inferolateral movement
paralysis may be seen with a fourth nerve palsy and lateral paraly-
sis with a sixth nerve palsy.
95.6. A 64-year-old diabetic woman presents with the acute
onset of painless diplopia. She has a 25-year history of
type 2 diabetes. Her only other past history is
hypertension. Physical examination reveals normal vital
signs and a normal neurologic examination with the
exception of an inability to look laterally with the left eye.
What is the most appropriate next step?
A. Cerebral angiography
B. Contrast-enhanced computed tomography (CT) scan
C. Magnetic resonance imaging (MRI) scan
D. Ophthalmology consultation
E. Patching the affected eye and initiation of antiplatelet
therapy
Answer: C. Diabetic cranial mononeuropathy may affect the
third, fourth, or sixth cranial nerve (CN). It is a diagnosis of
exclusion, and brainstem ischemic or hemorrhagic lesions should
also be considered. The long intracranial course of the sixth nerve
makes MRI scanning particularly indicated to rule out a mass
lesion. Once it is diagnosed, patching, analgesics, and antiplatelet
therapy should be considered for management of this diabetic
complication.
95.7. A 38-year-old woman presents 8 weeks postpartum with a
1-week history of severe headache and progressively
altered mental status, which culminated in a seizure
several minutes before presentation. On examination, she
is normotensive, appears postictal, but has no focal
neurologic findings. Ophthalmoscopic examination reveals
papilledema, and non–contrast-enhanced head computed
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tomography (CT) reveals a dense sagittal sinus and a
small venous hemorrhage in the occipital region. The next
most appropriate management step is:
A. 325 mg aspirin per rectum
B. Dexamethasone 10 mg IV push
C. Hypertonic saline 500-mL bolus
D. Mannitol 1 g/kg
E. Systemic anticoagulation with unfractioned heparin or
low–molecular-weight heparin (LMWH)
Answer: E. The patient presents with a dural sinus thrombosis.
Although large, randomized trial data do not exist, case series and
expert consensus strongly suggest improved outcomes with sys-
temic anticoagulation, even in the setting of venous hemorrhage
on head CT. Osmotic agents and steroids have no proven benefit
in the management of sinus thrombosis and may cause harm.
Antiplatelet agents may be considered if absolute contraindica-
tions to anticoagulation exist but probably have lower therapeutic
efficacy.
95.8. A 20-year-old female college student presents with her
third episode of bilateral foot numbness after a game of
volleyball. Each episode has occurred approximately 1 or 2
hours after a full game of indoor volleyball, which she had
no trouble completing. Each of the episodes of foot
numbness resolved during 2 or 3 days with no residual
symptoms. Her only other complaint is of falling grades
in school due to subjective poor memory and
distractibility. What would be the most likely finding in
this patient?
A. Elevated cerebrospinal fluid (CSF) protein levels
B. Elevated erythrocyte sedimentation rate
C. Hypocalcemia
D. Increased intracranial pressure (ICP) on lumbar
puncture
E. Thrombocytopenia
Answer: A. Presenting symptoms for multiple sclerosis (MS) may
be myriad. Uhthoff ’s phenomenon is the finding in MS in which
small increases in body temperature exacerbate neurologic symp-
toms temporarily. Almost any neurologic complaint or finding
may be a feature of MS, with up to 60% having cognitive impair-
ment. CSF analysis is abnormal in 90% of cases with a pleocytosis
and elevated protein with oligoclonal bands. ICP is normal.
Lumbar puncture is undertaken after magnetic resonance imaging
(MRI), which is the initial imaging test of choice.