Pain Medicine Headache and Facial Pain A Volume in Pain

Medicine A Case Based Learning series

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 ACKNOWLEDGEMENTS

A very special thanks to my editors, Michael Houston, PhD, Jeannine Carrado,

and Karthikeyan Murthy, for all of their hard work and perseverance in the face
of disaster. Great editors such as Michael, Jeannine, and Karthikeyan make their
authors look great, for they not only understand how to bring the Three Cs of
great writing. . .Clarity 1 Consistency 1 Conciseness. . .to the author’s work, but
unlike me, they can actually punctuate and spell!
Steven D. Waldman, MD, JD
P.S. . . .Sorry for all the ellipses, guys!

ix
 CONTENTS

Preface vii
Acknowledgements ix

1 Renaldo Saldana A 58-Year-Old Male With Left-Sided Facial

Pain and Rash 2

2 Stephanie Ellison A 32-Year-Old Graphic Designer With

Severe Throbbing Left-Sided Headaches 18

3 Abby Austin A 30-Year-Old Administrative Assistant With

Frequent Headaches Involving the Head and Neck 38

4 Gene Fiback A 57-Year-Old Accountant With Severe

Episodic Unilateral Retro-orbital Headaches With Associated
Neurologic Symptoms 50

5 Jeff Baker A 24-Year-Old Medical Student With Severe

Episodic Headache Associated With Sexual Activity 62

6 Brooke Johnson A 30-Year-Old Teacher With Frequent

Headaches Involving the Head and Neck 72

7 Christy Stierwalt A 28-Year-Old Librarian With

Postdural Headache Following an Epidural Block
for Vaginal Delivery 82

8 Amy Lin A 46-Year-Old Female With Posttraumatic Occipital

Headaches 96

9 Cassandra Elliot A 29-Year-Old Overweight Female With

Constant Headache Pain That Worsens With Valsalva
Maneuver 110

10 Shanice Williams A 52-Year-Old Female With a Severe

Headache and a Progressive Alteration of Consciousness 126

11 Hattie Harrison A 77-Year-Old Female With Headache and

Jaw Pain 142

12 Lynn Sparks A 26-Year-Old Sales Associate With Sharp,

Stabbing Pain With Swallowing 160

xi
xii CONTENTS

13 Brenda Brown A 66-Year-Old Bookkeeper With Severe,

Shocklike Facial Pain 174

14 Tommy Flannagan A 47-Year-Old Male With Severe Episodic

Throat and Ear Pain 192

15 Heather Shepard A 52-Year-Old Editor With Aching Jaw Pain

and a Clicking Sensation 212

Index 231
C H A P T E R

1
Renaldo Saldana
A 58-Year-Old Male With
Left-Sided Facial Pain and Rash

L E ARNING O B J E CTI V E S
• Learn the common causes of facial pain.
• Learn the common types of painful rashes.
• Develop an understanding of varicella zoster infection.
• Learn the clinical presentation of shingles.
• Develop an understanding of the treatment options for shingles.
• Learn the appropriate testing options to help diagnose shingles.
• Learn to identify red flags in patients who present with acute facial pain.
• Develop an understanding of postherpetic neuralgia.

2
1—MALE WITH LEFT-SIDED FACIAL PAIN AND RASH 3

Renaldo Saldana
Renaldo Saldana is a 58 y/o waiter
with the chief complaint of, “My left
forehead is killing me.” Renaldo went
on to say that he wouldn’t have both-
ered coming in just for the pain, which
had been present for a couple of days,
but when he developed a rash on his
left forehead, his boss told him he
couldn’t wait tables and would have
to wash dishes until the rash went
away. I asked Renaldo if he had anything like this happen before. He shook his
head and responded, “You know me, Doc, I am happy and healthy, but I am
really worried about this rash. The damn forehead pain was bad enough, but
when I woke up and saw this rash, it really freaked me out!” He continued,
“Doc, the crazy thing is that the rash wasn’t there when I went to bed. I am posi-
tive about this because I went to look in the bathroom mirror to see if I could see
why my forehead was hurting, and there was nothing there. I get up this morn-
ing, and I see a couple of little blisters over my eye. Now the damn rash is spread-
ing and my boss won’t let me work. I’m pretty tough, but this really has me
worried because if I don’t work, I don’t eat. The other crazy thing is it hurts
when I try to comb my hair. What is that all about? Do you think I got bit by one
of those brown recluse spiders?”
I asked Renaldo about any antecedent trauma to the forehead and he just
shook his head. “Doc, this kind of snuck up on me. Like I said, at first, my fore-
head began aching and then I woke up with this crazy rash. But, like I also said,
I gotta work.” I asked Renaldo what made his pain worse and he said, “Anytime
I forget and touch my forehead, it really hurts.” He added, “You know, Doc, the
other crazy thing is that if the fan in my room blows on my forehead, I get these
sharp pains. What the hell is that about?”
I asked Renaldo to point with one finger to show me where it hurts the most.
He pointed to the rash over his left eye, taking care not to touch the area. “Doc,
I can’t really point to one place. It kind of hurts all around my left eye and my fore-
head, and another crazy thing is, sometimes I feel like my hair hurts.” I asked if
he had any fever or chills and he shook his head no. I then asked, “What about
steroids? Did you ever take any cortisone or drugs like that?” Renaldo again shook
his head no. He denied any cancer or human immunodeficiency virus (HIV).
Renaldo said, “Doc, you know me, I am happy and healthy,” but with a worried
look, he added, “This really has me freaked out. I really need your help!”
On physical examination, Renaldo was afebrile. His respirations were 18
and his pulse was 84 and regular. His blood pressure (BP) was slightly
4 1—MALE WITH LEFT-SIDED FACIAL PAIN AND RASH

elevated at 144/88. I made a note to recheck it again before he left because he

was pretty anxious. He had obvious vesicular lesions over the left eye. He
had no lesions in his ear and both his eyes looked normal. His cardiopulmo-
nary examination was unremarkable other than the mild hypertension. His
thyroid was normal. His abdominal examination revealed no abnormal
mass or organomegaly. There was no costovertebral angle (CVA) tender-
ness. There was no peripheral edema or adenopathy. His low back examina-
tion was unremarkable. I did a rectal exam, which revealed no mass and a
normal prostate. The remainder of Renaldo’s physical examination was
within normal limits.

Key Clinical Points—What’s Important and What’s Not

THE HISTORY
’ A history of left forehead pain, which occurred prior to the onset of
vesicular rash
’ No history of acute trauma
’ No history of previous significant facial pain
’ No fever or chills
’ Acute onset of vesicular pain in the distribution of the left ophthalmic
branch of the trigeminal nerve (V1) following the onset of forehead
pain
’ Allodynia when the affected area is blown on by a fan

THE PHYSICAL EXAMINATION

’ The patient is afebrile
’ Obvious vesicular rash in the distribution of the left ophthalmic branch of
the trigeminal nerve (V1) (see photo of Renaldo Saldana)
’ No auricular lesions bilaterally

OTHER FINDINGS OF NOTE

’ Slightly elevated BP
’ Normal head, eyes, ears, nose, throat (HEENT) examination
’ Normal cardiovascular examination
’ Normal pulmonary examination
’ Normal abdominal examination
’ No peripheral edema
’ Normal prostate examination
’ No adenopathy
1—MALE WITH LEFT-SIDED FACIAL PAIN AND RASH 5

What Tests Would You Like to Order?

The following tests were ordered:
’ Complete blood count
’ Chemistry profile

’ Enzyme-linked immunosorbent assay (ELISA) test for HIV

TEST RESULTS
All testing was within normal limits.

Clinical Correlation—Putting It All Together

What is the diagnosis?
’Acute herpes zoster of the first division of the trigeminal nerve on the
left

The Science Behind the Diagnosis

ANATOMY OF THE TRIGEMINAL NERVE
The trigeminal nerve is the fifth cranial nerve and is denoted by the Roman
numeral V. The trigeminal nerve has three divisions and provides sensory inner-
vation for the forehead and eye (V1, ophthalmic), cheek (V2, maxillary), and
lower face and jaw (V3, mandibular), as well as motor innervation for the mus-
cles of mastication (Fig. 1.1). The fibers of the trigeminal nerve arise in the trigem-
inal nerve nucleus, which is the largest of the cranial nerve nuclei. Extending
from the midbrain to the upper cervical spinal cord, the trigeminal nerve nucleus
is divided into three parts: (1) the mesencephalic trigeminal nucleus, which
receives proprioceptive and mechanoreceptor fibers from the mandible and
teeth; (2) the main trigeminal nucleus, which receives the majority of the touch
and position fibers; and (3) the spinal trigeminal nucleus, which receives pain
and temperature fibers.
The sensory fibers of the trigeminal nerve exit the brainstem at the level of the
midpons with a smaller motor root emerging from the midpons at the same
level. These roots pass in a forward and lateral direction in the posterior cranial
fossa across the border of the petrous bone. They then enter a recess called
Meckel’s cave, which is formed by an invagination of the surrounding dura
mater into the middle cranial fossa. The dural pouch that lies just behind the gan-
glion is called the trigeminal cistern and contains cerebrospinal fluid.
The gasserian ganglion is canoe shaped, with three sensory divisions: (1) the oph-
thalmic division (V1), which exits the cranium via the superior orbital fissure; (2) the
6 1—MALE WITH LEFT-SIDED FACIAL PAIN AND RASH

V1

V2

V3

V1, Ophthalmic nerve

V2, Maxillary nerve

V3, Mandibular nerve

Fig. 1.1 The sensory divisions of the trigeminal nerve. (From Waldman S. Atlas of Interventional Pain
Management. ed. 5. Philadelphia: Elsevier; 2021 [Fig. 12.1].)

maxillary division (V2), which exits the cranium via the foramen rotundum into the
pterygopalatine fossa, where it travels anteriorly to enter the infraorbital canal to
exit through the infraorbital foramen; and the mandibular division (V3), which exits
the cranium via the foramen ovale anterior convex aspect of the ganglion (Fig. 1.2).
A small motor root joins the mandibular division as it exits the cranial cavity via the
foramen ovale. Three major branches emerge from the trigeminal ganglion (see
Fig. 1.2). Each branch innervates a different dermatome. Each branch exits the cra-
nium through a different site. The first division (V1; ophthalmic nerve) exits the cra-
nium through the superior orbital fissure, entering the orbit to innervate the globe
and skin in the area above the eye and forehead.
The second division (V2, maxillary nerve) exits through a round hole, the fora-
men rotundum, into a space posterior to the orbit, the pterygopalatine fossa. It
then reenters a canal running inferior to the orbit, the infraorbital canal, and exits
through a small hole, the infraorbital foramen, to innervate the skin below the
eye and above the mouth. The third division (V3, mandibular nerve) exits the
cranium through an oval hole, the foramen ovale. Sensory fibers of the third
1—MALE WITH LEFT-SIDED FACIAL PAIN AND RASH 7

V2, Maxillary division

Frontal n.
V1, Ophthalmic division

Gasserian ganglion
Supratrochlear n.

Supraorbital n.

Infraorbital n.

Mental n.

V3, Mandibular division

Lingual n.
Inf. alveolar n.

Fig. 1.2 The anatomy of the gasserian ganglion and the branches of the trigeminal nerve. (From
Waldman S. Atlas of Interventional Pain Management. ed. 5. Philadelphia: Elsevier; 2021 [Fig. 10.2].)

division either travel directly to their target tissues or reenter the mental canal to
innervate the teeth, with the terminal branches of this division exiting anteriorly
via the mental foramen to provide sensory cutaneous innervation to the skin
overlying the mandible.

CLINICAL PRESENTATION
Herpes zoster is an infectious disease caused by the varicella zoster virus (VZV).
Primary infection with VZV in a nonimmune host manifests clinically as the
childhood disease chickenpox (varicella). Investigators have postulated that dur-
ing the course of this primary infection, the virus migrates to the dorsal root or
cranial ganglia, where it remains dormant and produces no clinically evident
disease. In some individuals, the virus reactivates and travels along the sensory
pathways of the first division of the trigeminal nerve, where it produces the char-
acteristic pain and skin lesions of herpes zoster, or shingles.
8 1—MALE WITH LEFT-SIDED FACIAL PAIN AND RASH

Why reactivation occurs in some individuals but not in others is not fully
understood, but investigators have theorized that a decrease in cell-mediated
immunity may play an important role in the evolution of this disease by allow-
ing the virus to multiply in the ganglia, spread to the corresponding sensory
nerves, and produce clinical disease. Patients who are suffering from malignant
disease (particularly lymphoma) or chronic disease and those receiving immu-
nosuppressive therapy (chemotherapy, steroids, radiation) are generally debili-
tated and thus are much more likely than the healthy population to develop
acute herpes zoster (Fig. 1.3). These patients all have in common a decreased
cell-mediated immune response, which may also explain why the incidence of
shingles increases dramatically in patients older than 60 years and is relatively
uncommon in those younger than 20 years.
The first division of the trigeminal nerve is the second most common site for
the development of acute herpes zoster after the thoracic dermatomes. Rarely,
the virus attacks the geniculate ganglion and results in hearing loss, vesicles in
the ear, and pain (Fig. 1.4). This constellation of symptoms is called Ramsay
Hunt syndrome and must be distinguished from acute herpes zoster involving
the first division of the trigeminal nerve.

Fig. 1.3 Lateral view of a patient suffering from lymphoma post stem cell transplant with facial lesions
includes severe crusting and oozing in a clearly demarcated dermatomal distribution along the right
cranial nerve V distribution with associated right facial edema. (From Cheema H, Diedrich A, Kyne B,
et al. A case of tri-segmental cranial nerve V herpes zoster. IDCases. 2019;18:e00642. ISSN 2214-
2509. https://doi.org/10.1016/j.idcr.2019.e00642, http://www.sciencedirect.com/science/article/pii/
S2214250919302811 [Fig. 2].)
1—MALE WITH LEFT-SIDED FACIAL PAIN AND RASH 9

Fig. 1.4 Ramsay Hunt syndrome. (From Waldman S. Atlas of Common Pain Syndromes. ed. 4.
Philadelphia: Elsevier; 2019 [Fig. 1.1].)

SIGNS AND SYMPTOMS

As viral reactivation occurs, ganglionitis and peripheral neuritis cause pain that
may be accompanied by flulike symptoms. The pain generally progresses from a
dull, aching sensation to dysesthetic or neuritic pain in the distribution of the first
division of the trigeminal nerve. In most patients, the pain of acute herpes zoster
precedes the eruption of rash by 3 to 7 days, and this delay often leads to an erro-
neous diagnosis (see “Differential Diagnosis”). However, in most patients, the
clinical diagnosis of shingles is readily made when the characteristic rash appears.
As with chickenpox, the rash of herpes zoster appears in crops of macular lesions
that rapidly progress to papules and then to vesicles. Eventually, the vesicles
coalesce, and crusting occurs (Fig. 1.5). The affected area can be extremely painful,
and the pain tends to be exacerbated by any movement or contact (e.g., with
clothing or sheets). As the lesions heal, the crust falls away, leaving pink scars that
gradually become hypopigmented and atrophic (Fig. 1.6).
10 1—MALE WITH LEFT-SIDED FACIAL PAIN AND RASH

Fig. 1.5 Acute herpes zoster involving the ophthalmic division of the left trigeminal nerve. (From
Waldman SD. Pain management. Philadelphia: Elsevier; 2007.)

Fig. 1.6 Patient with healing herpes zoster in the second division of the trigeminal nerve. (A) Patient
presentation 2 weeks after onset of shingles. (B) Patient presentation 4 weeks after onset of pain.
(From Paquin R, Susin L, Welch G, et al. Herpes zoster involving the second division of the trigeminal
nerve: case report and literature review. J Endodont. 2017;43(9):1569 1573 [Fig. 3].)
1—MALE WITH LEFT-SIDED FACIAL PAIN AND RASH 11

<65 years of age

25%

>65 years of age

75%

>65 years <65 years

Fig. 1.7 Age of patients suffering from acute herpes zoster. (Data from Waldman S. Pain Management.
ed. 2. Philadelphia: Saunders; 2011.)

In most patients, the hyperesthesia and pain resolve as the skin lesions heal.
In some patients, however, pain persists beyond lesion healing. This common
and feared complication of acute herpes zoster is called postherpetic neuralgia,
and older persons are affected at a higher rate than the general population suffer-
ing from acute herpes zoster (Fig. 1.7). The symptoms of postherpetic neuralgia
can vary from a mild, self-limited condition to a debilitating, constantly burning
pain that is exacerbated by light touch, movement, anxiety, or temperature
change. This unremitting pain may be so severe that it completely devastates the
patient’s life; ultimately, it can lead to suicide. To avoid this disastrous sequela to
a usually benign, self-limited disease, the clinician must use all possible thera-
peutic efforts in patients with acute herpes zoster of the trigeminal nerve.
Ideally, prevention of acute herpes zoster by immunization with Zostrix should
be undertaken in all patients 60 years of age and older.

TESTING
Although in most instances the diagnosis is easily made on clinical grounds,
confirmatory testing is occasionally required. Such testing may be desirable in
patients with other skin lesions that confuse the clinical picture, such as in
patients with acquired immunodeficiency syndrome who are suffering from
Kaposi sarcoma. In such patients, polymerase chain reaction testing and immu-
nofluorescent antibody testing can rapidly identify herpes zoster virus and dis-
tinguish it from herpes simplex infections (Fig. 1.8). In uncomplicated cases, the
diagnosis of acute herpes zoster may be strengthened by obtaining a Tzanck
smear from the base of a fresh vesicle; this smear reveals multinucleated giant
cells and eosinophilic inclusions (Fig. 1.9). However, this inexpensive bedside
12 1—MALE WITH LEFT-SIDED FACIAL PAIN AND RASH

Fig. 1.8 Detection of antivaricella zoster virus immunoglobulin G by the fluorescent antibody to
membrane antigen assay. (A) Positive result and (B) negative control. (From Sauerbrei A, Färber I,
Brandstädt A, et al. Immunofluorescence test for sensitive detection of varicella-zoster virus-specific IgG: an
alternative to fluorescent antibody to membrane antigen test. J Virol Methods. 2004;19(1):15 30 [Fig. 1].)

test does not have the ability to distinguish between lesions caused by the VZV
and herpes simplex infections.

DIFFERENTIAL DIAGNOSIS
A careful initial evaluation, including a thorough history and physical examina-
tion, is indicated in all patients suffering from acute herpes zoster of the
1—MALE WITH LEFT-SIDED FACIAL PAIN AND RASH 13

Fig. 1.9 Tzanck smear showing intranuclear inclusion bodies within giant multinucleated cell. May-
Grünwald Giemsa stain; original magnification: 3 1000. (From Durdu M, Baba M, Seçkin D. The value
of Tzanck smear test in diagnosis of erosive, vesicular, bullous, and pustular skin lesions. J Am Acad
Dermatol. 2008;59(6):958 964 [Fig. 1]. ISSN 0190-9622. https://doi.org/10.1016/j.jaad.2008.07.059,
http://www.sciencedirect.com/science/article/pii/S0190962208010669)

trigeminal nerve. The goal is to rule out occult malignant or systemic disease that
may be responsible for the patient’s immunocompromised state. A prompt diag-
nosis allows early recognition of changes in clinical status that may presage the
development of complications, including myelitis or dissemination of the dis-
ease. Other causes of pain in the distribution of the first division of the trigeminal
nerve include trigeminal neuralgia, sinus disease, glaucoma, retro-orbital tumor,
inflammatory disease (e.g., Tolosa-Hunt syndrome), and intracranial disease,
including tumor (Box 1.1).

TREATMENT
The therapeutic challenge in patients presenting with acute herpes zoster of the
trigeminal nerve is twofold: (1) the immediate relief of acute pain and symptoms,
and (2) the prevention of complications, including postherpetic neuralgia. Most
pain specialists agree that the earlier treatment is initiated, the less likely it is that
postherpetic neuralgia will develop. Further, because older individuals are at the
highest risk for developing postherpetic neuralgia, early and aggressive treat-
ment of this group of patients is mandatory.

Nerve block
Sympathetic neural blockade with local anesthetic and steroid through stellate
ganglion block is the treatment of choice to relieve the symptoms of acute herpes
14 1—MALE WITH LEFT-SIDED FACIAL PAIN AND RASH

BOX 1.1 ’ Causes of Facial Pain

’ Trigeminal neuralgia
’ Atypical facial pain
’ Temporomandibular joint dysfunction
’ Temporal arteritis
’ Cluster headache
’ Autonomic trigeminal cephalgias
’ Dental abnormalities
’ Acute herpes zoster
’ Trauma
’ Neoplasm
’ Infection
’ Diseases of the eye
’ Sinus disease
’ Inflammatory disorders (e.g., Tolosa-Hunt syndrome)
’ Eagle syndrome
’ Multiple sclerosis
’ Referred pain
’ Salivary gland disease
’ Vasculitis
’ Aneurysms
’ Glossopharyngeal neuralia
’ Vidian neuralgia
’ Psychogenic disorders

zoster of the trigeminal nerve, as well as to prevent postherpetic neuralgia. As

vesicular crusting occurs, the steroid may also reduce neural scarring.
Sympathetic nerve block is thought to achieve these goals by blocking the pro-
found sympathetic stimulation caused by viral inflammation of the nerve and
gasserian ganglion. If untreated, this sympathetic hyperactivity can cause ische-
mia secondary to decreased blood flow of the intraneural capillary bed. If this
ischemia is allowed to persist, endoneural edema forms, thus increasing endo-
neural pressure and causing a further reduction in endoneural blood flow, with
irreversible nerve damage.
These sympathetic blocks should be continued aggressively until the
patient is pain free and should be reimplemented if the pain returns. Failure
to use sympathetic neural blockade immediately and aggressively, especially
in older patients, may sentence the patient to a lifetime of suffering from post-
herpetic neuralgia. Occasionally, some patients do not experience pain relief
from stellate ganglion block but do respond to blockade of the trigeminal
nerve.

Opioid analgesics
Opioid analgesics can be useful to relieve the aching pain that is common during
the acute stages of herpes zoster, while sympathetic nerve blocks are being