Professional Documents
Culture Documents
Atlas of
UNCOMMON
PAIN SYNDROMES
Steven D. Waldman, MD, JD
Vice Dean
Chairman and Professor
Department of Humanities and Bioethics
University of Missouri—Kansas City
Kansas City, Missouri
1600 John F. Kennedy Blvd.
Ste 1800
Philadelphia, PA 19103-2899
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WHAT OCCAM, KISS, ZEBRAS, AND MICKEY symptoms. But sometimes, in our almost obsessive desire to
make the diagnosis, simplicity is our enemy. In our haste to
GILLEY ALL HAVE IN COMMON make the patient fit the diagnosis, we get it wrong. Uncommon
It has been said that the three most dangerous things in medicine diseases are called uncommon diseases because they are uncom-
are: (1) a medical student with a sharp object; (2) a resident armed mon—they are not called unknown diseases (see below). Since
with a recently published study from NEJM, and; (3) an attending the beginning of time healers have recognized that the correct
physician with an anecdote. One must suspect that #2 was at play diagnosis is the key to getting the patient well, and as a corol-
when in the 1940’s, while on rounds at the University of Mary- lary, they also realized that the wrong diagnosis is not a “practice
land Hospital in Baltimore, Maryland, Theodore Woodward, MD, builder.” Which brings us to country music legend Mickey Gilley.
stated that “If you hear hoof beats out on Green Street, don’t look for In 1976 Mickey Gilley recorded the classic country ballad “The
zebras”! How this admonition to aspiring physicians morphed into Girl’s All Get Prettier At Closing Time. (FYI, 12 straight weeks
“when you hear hoof beats, look for horses, not zebras” is anybody’s charted at #1). This song is a plaintive lament about loneliness and
guess. (My son who was an ophthalmology resident in Baltimore late-night desperation. It explores how one’s perception of things
suggests that this sage piece of advice was most likely accompa- can change as circumstances change. What turns an unknown and
nied by a long-winded and confusing anecdote—see #3 above). undiagnosable disease into an uncommon disease is knowledge.
On the surface, most of us would agree with Dr. Woodward’s What changes our perception of what a constellation of symp-
logic that the most common things are the most common. Occam toms and physical findings mean when we are confronted with
agreed, when in the 14th century he put forth the philosophical a sick patient with an elusive diagnosis is knowledge. As we gain
tenant of parsimony that proposed that simpler explanations are, all more clinical experience, things that were once unknown become
things being equal, almost always better than more complex ones. known—even commonplace. The more we hone our clinical
He used a razor to “shave away” unnecessary or extraneous data acumen, the easier it is to put the pieces together—the jumble of
to get to the simplest solution. When you think about it, a razor disparate signs and symptoms come into focus—and then all of a
was all the rage as a medical instrument in the 14th century, so it is sudden, we have a diagnosis—a diagnosis we will never miss again!
not surprising that Occam chose it as his preferred medical device. The Atlas of Uncommon Pain Syndromes, Fourth Edition,
Occam’s razor certainly has a nice ring to it—better than Occam’s seeks to accomplish three things: The first is to familiarize the
MRI—which would no doubt be the name of his maxim if he had clinician with a group of uncommon pain syndromes that occur
lived in the 21st century, given that the MRI is certainly our most with enough frequency that they merit serious study—not rare
current popular medical device for “shaving away” extraneous data. or orphan diseases—just uncommon ones that are often mis-
Which brings us to KISS. Not the Gene Simmons’ rock band diagnosed. Second, this text is written with the goal of helping
KISS, but the admonition “Keep It Simple Stupid.” KISS was set the clinician reinforce his or her knowledge of common pain
forth by Lockhead aeronautical engineer Kelly Johnson when he syndromes to help in those situations when Occam is relatively
handed his design team a few simple tools and challenged them correct—when the pieces of the puzzle don’t quite fit the simple
to design military jets that could be easily fixed with the simple diagnosis. The third goal is more about the clinician and a little
tools that were available in combat situations. It is still not exactly less about the patient. It is about what attracted many of us to
clear to me who was “stupid,” but I certainly hope it is not the guys medicine to begin with. It is the irresistible charm of being pre-
who fix the jets I fly on. KISS makes sense when designing jet sented with a difficult clinical problem and getting it right. What
engines, but one has to ask what KISS has to do with the individual a great feeling that is! I hope you enjoy reading Atlas of Uncom-
patient—the sick one—the scared one—the one you worry about mon Pain Syndromes, Fourth Edition, as much as I did writing it.
in the middle of the night. Unfortunately, very little. Because for Steven D. Waldman, MD, JD
the individual patient with a difficult diagnosis, it turns out that a PS: The following ad recently ran in a local Kansas newspaper.
guy named Harry Hickam was probably more correct than Occam. Perhaps the hoof beats were zebras after all!
While on teaching rounds at Duke University, Harry
Hickam, MD, admonished his students and residents that TAMED YOUNG MALE AND FEMALE ZEBRAS FOR
“patients can have as many diseases as they damn well please”! SALE
(See also #3 above.) He correctly posited that when diagnosing ($2,000.00)
the individual patient, using Occam’s razor often provides the
correct diagnosis. But more often than we would care to admit, I have available young Male and Female zebra babies for sale to good
when dealing with a patient presenting with a perplexing con- and lovely homes who knows about the tamed zebras, feel free to send
stellation of signs and symptoms, it can just as easily provide the us emails for more details and pictures, please only serious inquiries.
wrong one. In fact, overreliance on Occam’s razor can be down-
right dangerous for both the patient and physician. Category: Pets »Horses
Often the simplest or, in the case of medical diagnosis, the Ad ID: 1564632
most common illness is exactly what is causing the patient’s Date: May 16, 2018
vii
SECTION 1 Headache and Facial Pain Syndromes
1
Ice Pick Headache
1
2 SECTION 1 Headache and Facial Pain Syndromes
TREATMENT
Ice pick headache uniformly responds to treatment with indo-
methacin. Failure to respond to indomethacin puts the diag-
nosis of ice pick headache in question. A starting dosage of 25
mg daily for 2 days and titrating to 25 mg three times per day
is a reasonable treatment approach. This dose may be carefully
increased to 150 mg per day. Indomethacin must be used care-
fully, if at all, in patients with peptic ulcer disease or impaired
renal function. Anecdotal reports of a positive response to
cyclooxygenase-2 (COX-2) inhibitors in the treatment of ice
pick headache have been noted in the headache literature.
Underlying sleep disturbance and depression are best treated
with a tricyclic antidepressant compound, such as nortriptyline,
which can be started at a single bedtime dose of 25 mg.
A B
C
Fig. 2.3 Subdural Empyema in a Patient With Sinusitis (A) T2-weighted MRI shows high-signal-intensity
extraaxial fluid collection in the right frontal convexity and along the falx on the right side. (B and C) Gado-
linium-enhanced MRI shows extraaxial fluid collections in the right frontal convexity and along the falx with
intense peripheral enhancement. The signal intensity of the fluid collection is slightly higher than that of
cerebrospinal fluid. (From Haaga JR, Lanzieri CF, Gilkeson RC, eds. CT and MR Imaging of the Whole Body.
4th ed. Philadelphia: Mosby; 2003:209.)
6 SECTION 1 Headache and Facial Pain Syndromes
Fixed
semidilated
Hazy corneal reflex pupil
signifying edema
Cataractous
lens
Opaque thickened
edematous cornea
Cataractous lens
Shallow anterior
chamber
Fig. 2.4 Acute Angle Closure Resulting From an Intumescent Cataractous Lens The eye is red with a
hazy view of the anterior segment from corneal edema, with a fixed, irregular, semidilated pupil from iris
infarction. The slit image shows the corneal edema and a very shallow anterior chamber. Some uveitis may be
present because of ischemia, and this must be differentiated from the larger accumulations of lens material
and macrophages seen with phacolytic glaucoma. (From Spalton DJ, Hitchings RA, Hunter P. Atlas of Clinical
Ophthalmology. 3rd ed. London: Mosby; 2005:225.)
Supraorbital n.
Supraorbital notch
the area of the block immediately after injection. Application of SUGGESTED READINGS
cold packs for 20-minute periods after the block also decreases
the amount of postprocedure pain and bleeding. Hillerup S, Jensen RH, Ersbøll BK. Trigeminal nerve injury associated
with injection of local anesthetics: needle lesion or nneurotoxicity?
J Am Dental Assoc. 2011;142(5):531–539.
CLINICAL PEARLS Supraorbital nerve block is especially useful in the Levin M. Nerve blocks and nerve stimulation in headache disorders.
diagnosis and palliation of pain secondary to supraorbital neuralgia. The first Tech Reg Anesth Pain Manage. 2009;13:42–49.
step in the management of this unusual cause of headache is the correct fit- Levin M. Nerve blocks in the treatment of headache. Neurotherapeu-
ting of swimming goggles that do not compress the supraorbital nerves. Coex- tics. 2010;7:197–203.
istent frontal sinusitis should be ruled out in patients who do not respond Waldman SD. Swimmer’s headache. In: Waldman SD, ed. Atlas of
rapidly to a change in swim goggles and a series of the previously mentioned Pain Management Injection Techniques. Philadelphia: Saunders;
nerve blocks. Any patient with headaches severe enough to require neural 2007:7–10.
blockade as part of the treatment plan should undergo MRI of the head to rule Waldman SD. The trigeminal nerve. In: Waldman SD, ed. Pain Re-
out unsuspected intracranial pathological conditions. view. Philadelphia: Saunders; 2009:15–17.
3
Chronic Paroxysmal Hemicrania
TESTING
Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents. Fig. 3.1 In contrast to cluster headache, which occurs primarily in men,
MRI is highly accurate and helps to identify abnormalities that chronic paroxysmal hemicrania occurs primarily in women.
8
CHAPTER 3 Chronic Paroxysmal Hemicrania 9
DIFFERENTIAL DIAGNOSIS
Chronic paroxysmal hemicrania is a clinical diagnosis sup-
ported by a combination of clinical history, abnormal physi-
cal examination during attacks, radiography, and MRI. Pain
syndromes that may mimic chronic paroxysmal hemicrania
include cluster headache, trigeminal neuralgia involving the
A
first division of the trigeminal nerve, demyelinating disease,
and ice pick headache. Trigeminal neuralgia involving the first
division of the trigeminal nerve is uncommon and is charac-
terized by trigger areas and tic-like movements. Demyelinating
disease is generally associated with other neurological find-
ings, including optic neuritis and other motor and sensory
abnormalities. The pain of cluster headache lasts much longer
than the pain of chronic paroxysmal hemicrania, and cluster
headache has a male predominance, a chronobiological pat-
tern of attacks, and a lack of response to treatment with indo-
methacin (Fig. 3.3).
TREATMENT
Chronic paroxysmal hemicrania uniformly responds to
treatment with indomethacin. Failure to respond to indo-
methacin puts the diagnosis of chronic paroxysmal hemicra-
nia in question. A starting dose of 25 mg daily for 2 days
and titrating to 25 mg three times per day is a reasonable
treatment approach. This dose may be carefully increased up
to 150 mg per day. Indomethacin must be used carefully, if
at all, in patients with peptic ulcer disease or impaired renal
function. Anecdotal reports of a positive response to cyclo-
B oxygenase-2 (COX-2) inhibitors in the treatment of chronic
paroxysmal hemicrania have been noted in the headache
Fig. 3.2 Sagittal (A) and semiaxial (B) T2-weighted images of a massive
prolactinoma in a 41-year-old man with chronic daily headache. (From literature. Underlying sleep disturbance and depression are
Benitez-Rosario MA, McDarby G, Doyle R, et al. Chronic cluster-like best treated with a tricyclic antidepressant compound, such
headache secondary to prolactinoma: uncommon cephalalgia in asso- as nortriptyline, which can be started at a single bedtime
ciation with brain tumors. J Pain Symptom Manage. 2009;37:271–276.) dose of 25 mg.
Fig. 4.1 Hemicrania continua is a unilateral side-locked headache with associated signs of autonomic dys-
function. In contrast to cluster headache, which occurs primarily in men, hemicrania continua occurs primarily
in women.
chemistry testing should be performed if the diagnosis of hemi- 25 mg daily for 2 days and titrating to 25 mg three times per day
crania continua is in question. Intraocular pressure should be is a reasonable treatment approach. This dose may be carefully
measured if glaucoma is suspected. increased up to 150 mg per day. Indomethacin must be used
carefully, if at all, in patients with peptic ulcer disease or impaired
renal function. Anecdotal reports of a positive response to cyclo-
DIFFERENTIAL DIAGNOSIS oxygenase-2 (COX-2) inhibitors in the treatment of hemicrania
Hemicrania continua is a clinical diagnosis supported by a continua have been noted in the headache literature. Underlying
combination of clinical history, abnormal physical examina- sleep disturbance and depression are best treated with a tricyclic
tion during exacerbation of baseline headache, radiography, antidepressant compound, such as nortriptyline, which can be
and MRI. Pain syndromes that may be confused for hemicra- started at a single bedtime dose of 25 mg.
nia continua include cluster headache, chronic paroxysmal
hemicrania, trigeminal neuralgia involving the first division of
the trigeminal nerve, demyelinating disease, ice pick headache,
COMPLICATIONS AND PITFALLS
and other indomethacin responsive headaches. Trigeminal Failure to diagnose hemicrania continua correctly may put the
neuralgia involving the first division of the trigeminal nerve patient at risk if intracranial pathological conditions or demy-
is uncommon and is characterized by trigger areas and tic- elinating disease, which may mimic the clinical presentation
like movements. Demyelinating disease is generally associated of hemicrania continua, is overlooked. MRI is indicated in all
with other neurological findings, including optic neuritis and patients thought to have hemicrania continua. Failure to diag-
other motor and sensory abnormalities. The pain of cluster nose glaucoma, which may cause intermittent ocular pain, may
headache is episodic, whereas the pain of hemicrania conti- result in permanent loss of sight.
nua is continuous with acute severe exacerbations (Fig. 4.3).
Cluster headache also has a male predominance, a chronobio- CLINICAL PEARLS Hemicrania continua is classified as a trigeminal
logical pattern of attacks, and a lack of response to treatment autonomic cephalgia. The trigeminal autonomic cephalgias are a group of
with indomethacin. distinct headache syndromes that share a number of common and often over-
lapping clinical characteristics (Table 4.2). The diagnosis of hemicrania con-
tinua is made by obtaining a thorough, targeted headache history. Between
TREATMENT attacks, patients with hemicrania continua should have a normal neurological
examination. If the neurological examination is abnormal between attacks,
Hemicrania continua uniformly responds to treatment with
the diagnosis of hemicrania continua should be discarded and a careful search
indomethacin. Failure to respond to indomethacin puts the
for the cause of the patient’s neurological findings should be undertaken.
diagnosis of hemicrania continua in question. A starting dose of
CHAPTER 4 Hemicrania Continua 13
A B
C D
Fig. 4.2 A 57-Year-Old Woman With Recurrent GBM Postcontrast T1-weighted image (A) and rCBV map
(B) demonstrate two adjacent necrotic, peripherally enhancing lesions with elevated rCBV (white arrow) con-
sistent with recurrent GBM. After subtotal reresection of the peripheral lesion and 2 weeks of treatment
with TMZ and bevacizumab, postcontrast T1-weighted image (C) and rCBV map (D) show reduced size and
decreased intensity of enhancement of the residual medial necrotic rim-enhancing lesion with no significant
rCBV elevation (white arrow). Findings are consistent with response to treatment. The patient had a rela-
tively long OS (>17 months) after bevacizumab initiation. (From Boxerman JL, Shiroishi MS, Ellingson BM,
et al. Dynamic susceptibility contrast MR imaging in glioma: review of current clinical practice. Magn Reson
Imaging Clin N Am. 2016;24(4):649–670.) rCBV, Cerebral blood volume; GBM, glioblastoma multiforme; OS,
overall survival rate; TMZ, temozolomide.
SUGGESTED READINGS
Becker M, Kohler R, Vargas MI, Viallon M, Delavelle J. Pathology of
the trigeminal nerve. Neuroimaging Clin N Am. 2008;18:283–307.
Craven J. Anatomy of the cranial nerves. Anaesth Intensive Care Med.
2010;11:529–534.
Lewis DW, Gozzo YF, Avner MT. The “other” primary headaches in
children and adolescents [review]. Pediatr Neurol. 2005;33:303–313.
Fig. 5.1 Patients suffering from Charlin syndrome present with the Waldman SD. The trigeminal nerve. In: Waldman SD, ed. Pain Re-
complaint of severe paroxysms of ocular or retroorbital pain that radi- view. Philadelphia: Saunders; 2009:15–17.
ates into the ipsilateral forehead, nose, and maxillary region. The pain is Waldman SD. Charlin’s syndrome. In: Waldman SD, ed. Atlas of
associated with voluminous ipsilateral rhinorrhea and congestion of the Pain Management Injection Techniques. Philadelphia: Saunders;
nasal mucosa and significant inflammation of the affected eye. 2007:20–24.
6
Sexual Headache
occipital, but some patients volunteer that the pain felt “like the
ICD-10 CODE R51
top of my head was going to blow off.” The pain is usually bilat-
eral, but isolated cases of unilateral explosive sexual headache
have been reported. The pain usually remains intense for 10 to
15 minutes and then gradually abates. Some patients note some
THE CLINICAL SYNDROME
residual headache pain for 2 days.
Sexual headache, which is also known as primary headache
associated with sexual activity, is a term used to describe a Dull Type of Sexual Headache
group of headaches associated with sexual activity. Clinicians The dull type of sexual headache begins during the early por-
have identified the following three general types of headache tion of sexual activity. This headache type has an aching char-
associated with sexual activity: acter and begins in the occipital region. The headache becomes
• Explosive type holocranial as sexual activity progresses toward orgasm. It may
• Dull type peak at orgasm, but in contrast to the explosive type of sexual
• Postural type headache, the dull type disappears rapidly after orgasm. Ceasing
Each of these sexual headache types was previously called sexual activity usually aborts the dull type of sexual headache.
benign coital headache, but this term has been replaced by sex- Some headache specialists think the dull type of sexual head-
ual headache because each may occur with sexual activity other ache is simply a milder version of the explosive type of sexual
than coitus (Fig. 6.1). In general, sexual headache includes a headache.
benign group of disorders, but a rare patient may have acute
subarachnoid hemorrhage during sexual activity, which may be Postural Type of Sexual Headache
erroneously diagnosed as the benign explosive type of sexual The postural type of sexual headache is similar to the explosive
headache. There is no gender predilection for sexual headache, type of sexual headache in that it occurs just before or during
and the occurrence of all types of sexual headache may be epi- orgasm. Its rapid onset to peak and severe intensity also are sim-
sodic rather than chronic. Rarely, more than one type of sexual ilar to that of the explosive type. It differs from the explosive
headache occurs in the same patient. Sexual headaches have type of headache in that the headache symptoms recur when the
been associated with the use of cannabis, pseudoephedrine, oral
contraceptives, and amiodarone.
17
18 SECTION 1 Headache and Facial Pain Syndromes
TESTING
Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents.
MRI is highly accurate and helps identify abnormalities that
may put the patient at risk for neurological disasters secondary
to intracranial and brainstem pathological conditions, including
tumors, demyelinating disease, and hemorrhage. More impor-
tantly, MRI helps identify bleeding associated with leaking intra-
cranial aneurysms. Magnetic resonance angiography (MRA)
and cerebral arteriography may be useful in helping identify
aneurysms or other arterial abnormalities responsible for the
patient’s neurological symptoms (Figs 6.2 and 6.3). In patients B
who cannot undergo MRI, such as patients with pacemakers,
Fig. 6.3 Cerebral angiogram revealed segmental (boundaries denoted
computed tomography (CT) is a reasonable second choice. Even
by large arrows) irregularities (small arrows) of the basilar artery in
if blood is not present on MRI or CT, if intracranial hemorrhage both anterior-posterior (A) and lateral (B) views. (From Delasobera BE,
is suspected, lumbar puncture should be performed. Osborn SR, Davis JE. Thunderclap headache with orgasm: a case of
Screening laboratory tests consisting of complete blood cell basilar artery dissection associated with sexual intercourse. J Emerg
count, erythrocyte sedimentation rate, and automated blood Med. 2012;43[1]:e43–e47, fig. 1.)
chemistry testing should be performed if the diagnosis of sexual
headache is in question. Intraocular pressure should be mea- cluster headache, migraine, and chronic paroxysmal hemi-
sured if glaucoma is suspected. crania. Trigeminal neuralgia involving the first division of the
trigeminal nerve is uncommon and is characterized by trigger
areas and tic-like movements. Demyelinating disease is gener-
DIFFERENTIAL DIAGNOSIS ally associated with other neurological findings, including optic
Sexual headache is a clinical diagnosis supported by a combi- neuritis and other motor and sensory abnormalities. The pain of
nation of clinical history, normal physical examination, radi- chronic paroxysmal hemicrania and cluster headache is associ-
ography, MRI, and MRA. Pain syndromes that may mimic ated with redness and watering of the ipsilateral eye, nasal con-
sexual headache include trigeminal neuralgia involving the gestion, and rhinorrhea during the headache. These findings are
first division of the trigeminal nerve, demyelinating disease, absent in all types of sexual headache. Migraine headache may
CHAPTER 6 Sexual Headache 19
or may not be associated with nonpainful neurological findings headache, is overlooked. MRI, MRA, and occasionally cerebral
known as aura, but the patient almost always reports some sys- angiography are indicated in all patients thought to have sex-
temic symptoms, such as nausea or photophobia, not typically ual headache. Failure to diagnose glaucoma, which also may
associated with sexual headache. cause intermittent ocular pain, may result in permanent loss
of sight.
TREATMENT CLINICAL PEARLS The diagnosis of sexual headache is made by obtain-
It is generally thought that avoiding the inciting activity for a ing a thorough, targeted headache history. As mentioned earlier, patients may
few weeks decreases the propensity to trigger sexual headaches. not be forthcoming about the events surrounding the onset of their headache,
and the clinician should be sensitive to this fact. Patients suffering from sexual
If this avoidance technique fails or is impractical because of
headache should have a normal neurological examination. If the neurological
patient preference, a trial of propranolol is a reasonable next
examination is abnormal, the diagnosis of sexual headache should be dis-
step. A low dose of 20 to 40 mg as a daily dose and titrating carded and a careful search for the cause of the patient’s neurological findings
in 20-mg increments to 200 mg as a divided daily dose until should be undertaken.
prophylaxis occurs treats most patients suffering from sexual
headache. Propranolol should be used with caution in patients
with asthma or cardiac failure and patients with brittle diabetes.
If propranolol is ineffective, indomethacin may be tried. A SUGGESTED READINGS
starting dose of 25 mg daily for 2 days and titrating to 25 mg Delasobera BE, Osborn SR, Davis JE. Thunderclap headache with
three times per day is a reasonable treatment approach. This dose orgasm: a case of basilar artery dissection associated with sexual
may be carefully increased to 150 mg per day. Indomethacin intercourse. J Emerg Med. 2012;43(1):e43–e47.
must be used carefully, if at all, in patients with peptic ulcer dis- Evans RW. Diagnostic testing for migraine and other primary head-
ease or impaired renal function. Anecdotal reports of a positive aches. Neurol Clin. 2009;27:393–415.
response to cyclooxygenase-2 (COX-2) inhibitors and magne- Hu CM, Lin YJ, Fan YK, et al. Isolated thunderclap headache during
sium in the treatment of sexual headache have been noted in the sex: orgasmic headache or reversible cerebral vasoconstriction
headache literature. Underlying sleep disturbance and depres- syndrome? J Clin Neurosci. 2010;17:1349–1351.
Jolobe OMP. The differential diagnosis includes reversible cerebral
sion are best treated with a tricyclic antidepressant compound,
vasoconstrictor syndrome. Am J Emerg Med. 2010;28:637.
such as nortriptyline, which can be started at a single bedtime
Kim HJ, Seo SY. Recurrent emotion-triggered headache following
dose of 25 mg. primary headache associated with sexual activity. J Neurol Sci.
2008;273:142–143.
COMPLICATIONS AND PITFALLS Tuğba T, Serap Ü, Esra O, et al. Features of stabbing, cough, exer-
tional and sexual headaches in a Turkish population of headache
Failure to diagnose sexual headache correctly may put the patients. J Clin Neurosci. 2008;15:774–777.
patient at risk if intracranial pathology or demyelinating
disease, which may mimic the clinical presentation of sexual
7
Cough Headache
20
CHAPTER 7 Cough Headache 21
Herniation of
cerebellar tonsil
Spinal cord
Fig. 7.1 Symptomatic cough headache is often associated with structural abnormalities, such as Arnold-
Chiari malformation, and usually occurs in the third decade of life.
DIFFERENTIAL DIAGNOSIS
Cough headache is a clinical diagnosis supported by a combina-
tion of clinical history, physical examination, radiography, MRI,
and MRA. Pain syndromes that may mimic cough headache
include benign exertional headache, ice pick headache, sexual
headache, trigeminal neuralgia involving the first division of
the trigeminal nerve, demyelinating disease, cluster headache,
and chronic paroxysmal hemicrania. Trigeminal neuralgia
involving the first division of the trigeminal nerve is uncom-
mon and is characterized by trigger areas and tic-like move-
Fig. 7.2 Low-lying cerebellar tonsils (straight arrows) of a Chiari mal- ments. Demyelinating disease is generally associated with other
formation are shown deforming the medulla (curved arrow) in a sagit- neurological findings, including optic neuritis and other motor
tal T1-weighted spin echo image. 4, Fourth ventricle. (From Stark DD,
and sensory abnormalities. The pain of chronic paroxysmal
Bradley WG Jr, eds. Magnetic Resonance Imaging. 3rd ed. St Louis:
Mosby; 1999:1841.) hemicrania and cluster headache is associated with redness and
watering of the ipsilateral eye, nasal congestion, and rhinorrhea
during the headache. These findings are absent in all types of
22 SECTION 1 Headache and Facial Pain Syndromes
cough headache. Migraine headache may or may not be associ- SUGGESTED READINGS
ated with painless neurological findings known as aura, but the
patient almost always reports some systemic symptoms, such Berciano J, Poca M-A, García A, Sahuquillo J. Paroxysmal cervi-
as nausea or photophobia, not typically associated with cough cobrachial cough-induced pain in a patient with syringomy-
elia extending into spinal cord posterior gray horns. J Neurol.
headache.
2007;54:678–681.
Chen YY, Lirng JF, Fuh JL, et al. Primary cough headache is associat-
TREATMENT ed with posterior fossa crowdedness: a morphometric MRI study.
Cephalalgia. 2004;24:694–699.
Indomethacin is the treatment of choice for benign cough head- Rothrock JF. Headaches caused by vascular disorders. Neurol Clin.
ache. A starting dose of 25 mg daily for 2 days and titrating to 2014;32(2):305–319.
25 mg three times per day is a reasonable treatment approach. Cutrer M, DeLange FJ. Cough, exercise, and sex headaches. Neurol
This dose may be carefully increased up to 150 mg per day. Clin. 2014;32(2):433–450.
Indomethacin must be used carefully, if at all, in patients with Langridge B, Phillips E, Choi D. Chiari malformation type 1: a sys-
peptic ulcer disease or impaired renal function. Headache spe- tematic review of natural history and conservative management.
cialists have noted anecdotal reports of a positive response to World Neurosurg. 104:213–219
Pascual J, Rubén Martín A, Oterino A. Headaches precipitated by
cyclooxygenase-2 (COX-2) inhibitors in the treatment of benign
cough, prolonged exercise or sexual activity: a prospective etiolog-
cough headache. Underlying sleep disturbance and depression ical and clinical study. J Headache Pain. 2008;9:259–266.
are best treated with a tricyclic antidepressant compound, such Pascual J. Primary cough headache. Curr Pain Headache Rep.
as nortriptyline, which can be started at a single bedtime dose 2005;9:272–276.
of 25 mg. Pascual J. Cough and exertional headache, primary. In: Encyclopedia
The only uniformly effective treatment for symptomatic of the Neurological Sciences. 2nd ed. Oxford: Academic Press;
cough headache is surgical decompression of the foramen mag- 2014:881–884.
num. This surgery is usually done via suboccipital craniectomy. Waldman SD. Arnold Chiari malformation type I. In: Waldman
Surgical decompression prevents the low-lying cerebellar ton- SD, Campbell RS, eds. Imaging of Pain. Philadelphia: Saunders;
sils from obstructing the flow of spinal fluid from the cranium 2011:27–28.
to the spinal subarachnoid space during a Valsalva maneuver. Waldman SD. Arnold Chiari malformation type II. In: Waldman
SD, Campbell RS, eds. Imaging of Pain. Philadelphia: Saunders;
2011:29–30.
COMPLICATIONS AND PITFALLS
Failure to diagnose cough headache correctly may put the
patient at risk if intracranial pathology or demyelinating dis-
ease, which may mimic the clinical presentation of cough head-
ache, is overlooked. MRI and MRA are indicated in all patients
thought to have cough headache. Failure to diagnose glaucoma,
which also may cause intermittent ocular pain, may result in
permanent loss of sight.
Overlap Overlap
SIGNS AND SYMPTOMS between duration between duration
Fig. 8.1 Overlap Between Attack Duration in Trigeminal Autonomic
Patients with SUNCT headache present with the complaint Cephalalgias SUNCT, Sudden unilateral neuralgiform conjunctival injec-
of severe paroxysms of ocular or periorbital pain that radi- tion tearing. (From Leone M, Bussone G. Pathophysiology of trigeminal
ate into the ipsilateral temple, forehead, nose, cheek, throat, autonomic cephalalgias. Lancet Neurol. 2009;8:755–774.)
23
24 SECTION 1 Headache and Facial Pain Syndromes
SUNCT, Sudden unilateral neuralgiform conjunctival injection tearing. Fig. 8.3 Autonomic signs and symptoms, ipsilateral to the pain,
observed in a patient with sudden unilateral neuralgiform conjunctival
injection tearing. CI, Conjunctival injection; EO, eyelid edema; FF, fore-
head and facial flushing; La, lacrimation; Mi, miosis; NC, nasal conges-
tion; Pt, ptosis; Rh, rhinorrhea. (From Kitahara I, Fukuda A, Imamura Y,
et al. Pathogenesis, surgical treatment, and cure for SUNCT syndrome.
World Neurosurg. 2015;84[4]:1080–1083, fig. 1.)
D
Fig. 8.4 Magnetic resonance imaging (MRI) with right-sided short-lasting unilateral neuralgiform headache
attacks with conjunctival injection and tearing syndromes, demonstrating an aberrant loop of the causative
vessels abutting the right trigeminal nerve. Right (R) and left (L) sides of the patient are indicated on the MRI
images. MRI (A) and operative (B) images for case 1. Axial (A, upper panel) and sagittal (lower panel) images
are shown. The side of the first branch of the right trigeminal nerve (RTN) was compressed by the vertebral
artery (VA). MRI (C) and operative (D) images for case 2. Axial (C, top panel) and 2 sagittal images are shown.
Compression of the side of the first branch of the right trigeminal nerve by the superior cerebellar artery (SCA)
was suspected (C, middle panel). The side of the third branch of the right trigeminal nerve was compressed
by the anterior inferior cerebellar artery (AICA) (C, bottom panel). FTN, The first branch of the trigeminal nerve;
LTN, left trigeminal nerve; PV, petrosal vein; RC, right cerebellum; TTN, the third branch of the trigeminal
nerve; VII–VIII N, VII and VIII nerves. (From Kitahara I, Fukuda A, Imamura Y, et al. Pathogenesis, surgical
treatment, and cure for SUNCT syndrome. World Neurosurg. 2015;84[4]:1080–1083, fig. 2.)
26 SECTION 1 Headache and Facial Pain Syndromes
28
CHAPTER 9 Primary Thunderclap Headache 29
A B C
D E F
Fig. 9.2 Magnetic Resonance Imaging Showing Arteriovenous Malformation Patient with aneurysm-re-
lated false aneurysm (FA) in right parietal region. Preangiographic T1-weighted magnetic resonance axial
image (A) and T2-weighted magnetic resonance coronal image (B) show round lesion (arrow) with flow void
and mixed signal in the center and mixed signal on the periphery. Fluid-attenuated inversion recovery image
(C) reveals small area of surrounding edema (arrow). (D) Flow in the center of FA (arrow) on two-dimensional
time-of-flight magnetic resonance angiography. (E) Preembolization digital subtraction angiography image. (F)
Residual inflow to FA (arrow) on postembolization digital subtraction angiography (DSA) image (arrow indicates
arteriovenous malformation). (From Brzozowski K, Frankowska E, Piasecki P, et al. The use of routine imaging
data in diagnosis of cerebral pseudoaneurysm prior to angiography. Eur J Radiol. 2011;80:e401–e409.)
SUGGESTED READINGS
Arenaza-Basterrechea N, Iglesias Díez. F, López Sarnago P. Thunder-
clap headache secondary to pneumocephalus. Neurología (English
Ed). 2017;32(2):132–133.
Chih-Ming H, Ya-Ju L, Yang-Kai F, Shih-Pin C, Tzu-Hsien L. Iso-
lated thunderclap headache during sex: orgasmic headache or
reversible cerebral vasoconstriction syndrome? J Clin Neurosci.
2010;17:1349–1351.
10
Hypnic Headache
32
CHAPTER 10 Hypnic Headache 33
TESTING
Magnetic resonance imaging (MRI) of the brain provides the
best information regarding the cranial vault and its contents.
MRI is highly accurate and helps identify abnormalities that Fig. 11.1 Patients Suffering From Nummular Headache Complain
may put the patient at risk for neurological disasters secondary of Unifocal Area of Pain and Scalp Sensitivity.
35
36 SECTION 1 Headache and Facial Pain Syndromes
A B
Fig. 11.2 Calvarial Metastases (A) Abnormal enhancement (arrows) is present within the diploë on this
gadolinium-enhanced T1-weighted image. Expansion of the left parietal bone occurs, affecting the inner table
more than the outer table. (B) Heterogeneous hyperintensity (arrows) persists within the calvaria on this
T2-weighted image. The right parietal lesion is no longer imaged on this more superior section. (From Edel-
man RR, Hesselink JR, Zlatkin MB, Crues JV III, eds. Clinical Magnetic Resonance Imaging. 3rd ed. Philadel-
phia: Saunders; 2005.)
TREATMENT
Nummular headache uniformly responds to treatment with SUGGESTED READINGS
indomethacin. Failure to respond to indomethacin puts the Cohen GL. Nummular headache: what denomination? Headache.
diagnosis of nummular headache in question. A starting dosage 2005;10:1417–1418.
of 25 mg daily for 2 days and titrating to 25 mg three times a day Evans RW, Pareja JA. Nummular headache. Headache. 2005;45:164–
is a reasonable treatment approach. This dose may be carefully 165.
increased to 150 mg/day. Indomethacin must be used carefully, Mathew NT. Indomethacin responsive headache syndromes. Head-
if at all, in patients with peptic ulcer disease or impaired renal ache. 1981;21:147–150.
Pareja JA, Caminero AB, Serra J, et al. Nummular headache: a coin-
function. Anecdotal reports of a positive response to cycloox-
shaped cephalgia. Neurology. 2002;58:1678–1679.
ygenase-2 (COX-2) inhibitors in the treatment of nummular
Pareja JA, Pareja J, Barriga FJ, et al. Nummular headache: a prospec-
headache have been noted in the headache literature, as well as tive series of 14 new cases. Headache. 2004;44:611–614.
a successful treatment with gabapentin. Underlying sleep dis- Pareja JA, Pareja J, Yangüela J. Nummular headache, trochleitis, su-
turbance and depression are best treated with a tricyclic antide- praorbital neuralgia, and other epicranial headaches and neural-
pressant compound, such as nortriptyline, which can be started gias: the epicranias. J Headache Pain. 2003;4:125–131.
at a single bedtime dose of 25 mg. Iwanowski P, Kozubski W, Losy J. Nummular headache in a patient
with ipsilateral occipital neuralgia—a case report. Neurol Neuro-
chir Pol. 2014;48(2):141–143.
COMPLICATIONS AND PITFALLS Chen WH, Chen YT, Lin CS, Li TH, Lee LH, Chen CJ. A high preva-
Failure to diagnose nummular headache correctly may put lence of autoimmune indices and disorders in primary nummular
headache. J Neurol Sci. 2012;320(1–2):127–130.
the patient at risk if an intracranial pathological condition or
calvarial disease, which may mimic the clinical presentation
of nummular headache, is overlooked. MRI is indicated in all
patients thought to have nummular headache.
12
Headache Associated With
Temporal Arteritis
Temporal artery
External
carotid artery
Ophthalmic
artery
Fig. 12.1 (A) Temporal arteritis is a disease of the sixth decade that occurs almost exclusively in whites, with
a predilection of 3:1 for women. (B) The sine qua non of temporal arteritis is jaw claudication.
DIFFERENTIAL DIAGNOSIS
Headache associated with temporal arteritis is a clinical diag-
nosis supported by a combination of clinical history, abnormal
Halo sign
findings on physical examination of the temporal artery, normal
radiography, MRI findings, an elevated erythrocyte sedimen-
Cephalad to the orbit tation rate, and a positive temporal artery biopsy result. Pain
Superficial temporal artery transverse view syndromes that may mimic temporal arteritis include tension
type headache, brain tumor, other forms of arteritis, trigeminal
neuralgia involving the first division of the trigeminal nerve,
Fig. 12.2 Positive Halo Sign on Transverse Ultrasound Image in a demyelinating disease, migraine headache, cluster headache,
Patient With Temporal Arteritis The patient’s sedimentation rate was
98.
and chronic paroxysmal hemicrania. Trigeminal neuralgia
involving the first division of the trigeminal nerve is uncom-
mon and is characterized by trigger areas and tic-like move-
If the diagnosis of temporal arteritis is in doubt, magnetic res- ments. Demyelinating disease is generally associated with other
onance imaging (MRI) of the brain provides the best information neurological findings, including optic neuritis and other motor
regarding the cranial vault and its contents. MRI is highly accu- and sensory abnormalities. The pain of chronic paroxysmal
rate and helps identify abnormalities that may put the patient hemicrania and cluster headache is associated with redness and
at risk for neurological disasters secondary to intracranial and watering of the ipsilateral eye, nasal congestion, and rhinorrhea
brainstem pathological conditions, including tumors and demy- during the headache. These findings are absent in all types of
elinating disease. More importantly, MRI helps identify bleed- sexual headache. Migraine headache may or may not be associ-
ing associated with leaking intracranial aneurysms. Magnetic ated with painless neurological findings known as aura, but the
resonance angiography (MRA) may be useful to help identify patient almost always reports some systemic symptoms, such as
aneurysms responsible for neurological symptoms. In patients nausea or photophobia, not typically associated with the head-
who cannot undergo MRI, such as patients with pacemakers, ache of temporal arteritis.
computed tomography (CT) is a reasonable second choice. If
intracranial hemorrhage is suspected, lumbar puncture should
be performed, even if blood is not present on MRI or CT. Case
TREATMENT
reports of the utility of positron emission tomography (PET)/ The mainstay of treatment for temporal arteritis and its associ-
computerized tomography scans in the diagnosis of giant cell ated headaches and other systemic symptoms is the immediate
arteritis of the temporal, occipital, and vertebral arteries may use of corticosteroids. If visual symptoms are present, an ini-
offer additional diagnostic options (Fig. 12.3). Intraocular pres- tial dose of 80 mg of prednisone is indicated. This dose should
sure should be measured if glaucoma is suspected. be continued until the symptoms of temporal arteritis have
CHAPTER 12 Headache Associated With Temporal Arteritis 39
completely abated. At this point, the dose may be decreased by SUGGESTED READINGS
5 mg/week if the symptoms remain quiescent and the eryth-
rocyte sedimentation rate does not increase. Cytoprotection of Bajkó Z, Bălaşa R, Szatmári S, et al. The role of ultrasound in the
the stomach mucosa should be considered because ulceration diagnosis of temporal arteritis. Neurol Neurochir Pol. 2015;49(2):
139–143.
and gastrointestinal bleeding are possible. If the patient cannot
Dickason A, McArdle P. Jaw claudication in the presentation of
tolerate corticosteroids, or the maintenance dose of steroids
temporal arteritis: a review of the epidemiology and presenting
remains so high as to produce adverse effects, azathioprine is a symptoms of 207 patients at Derriford Hospital, Plymouth. Br J
reasonable next choice. Oral Maxillofac Surg. 2015;53(10):e87.
Hazelman BL. Polymyalgia rheumatica. In: Waldman SD, ed. Pain
Management. Philadelphia: Saunders; 2009:449–454.
COMPLICATIONS AND PITFALLS Paget SA, Spiera RF. Polymyalgia rheumatica and temporal arteritis.
Failure to recognize, diagnose, and treat temporal arteritis In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadel-
promptly may result in the permanent loss of vision. Failure to phia: Saunders; 2007:1123–1127.
diagnose the headache associated with temporal arteritis cor- Rehak Z, Vasina J, Ptacek J, et al. PET/CT in giant cell arteritis: high
rectly may put the patient at risk if an intracranial pathological 18F-FDG uptake in the temporal, occipital and vertebral arteries.
Rev Esp Med Nucl Imagen Mol. 2016;35(6):398–401.
condition or demyelinating disease, which may mimic the clin-
Sloane J, Rice N, Kergozou E, et al. Temporal artery biopsy for giant
ical presentation of temporal arteritis, is overlooked. MRI of the
cell arteritis: an audit of 471 cases – what have we learnt? Br J Oral
brain is indicated in all patients thought to have headaches asso- Maxillofac Surg. 2015;53(10):e44.
ciated with temporal arteritis. Failure to diagnose glaucoma, Waldman SD. Connective tissue diseases. In: Waldman SD, ed. Pain
which also may cause intermittent ocular pain, may result in Review. 2nd ed. Philadelphia: Saunders Elsevier; 200916:43167–
permanent loss of sight. 44468.
Waldman SD. Temporal arteritis. In: Waldman SD, ed. Pain Review.
CLINICAL PEARLS The diagnosis of headache associated with tempo- 2nd ed. Philadelphia: Saunders Elsevier; 200916:22236–22328.
ral arteritis is made by obtaining a thorough, targeted headache history. As
mentioned, jaw claudication is pathognomonic for temporal arteritis, and its
presence should be sought in all elderly patients presenting with headache.
Failure to recognize, diagnose, and treat temporal arteritis promptly may result
in the permanent loss of vision.
13
Post–Dural Puncture Headache
Language: Italian
NUOVI RACCONTI
DI
Enrico Castelnuovo
MILANO
Fratelli Treves, Editori
1896
—
Secondo Migliaio.
PROPRIETÀ LETTERARIA.
Milano. — Tip. Treves.
INDICE.
Mercoledì, 2 giugno.
Il colèra è da lunedì in qualche descrescenza, ma seguita a colpire
più d’una trentina di persone al giorno. La città è squallida e triste.
Dietro le vetrine delle botteghe non si leggono che avvisi mortuari di
persone uccise dal fiero morbo, dal crudo morbo, dall’inesorabile
morbo, eleganti perifrasi per indicare il colèra senza nominarlo. Le
muraglie sono coperte di manifesti sesquipedali che vantano al
pubblico le glorie di questo o quel preservativo infallibile.
Si vanno aprendo collette e istituendo comitati: della Croce verde,
della Società del Bucintoro; si annunziano distribuzioni gratuite di
commestibili, questue per le case, ecc., ecc.; tutta roba che fa salir la
mosca al naso al colonnello Struzzi. L’ho sentito stamattina
esprimere le sue opinioni in proposito alla Gegia. Che Croce rossa, o
verde, o bianca?... Buffonate di gente che vuol mettersi in evidenza
e magari buscarsi un cavalierato.... Ci credete voi al colèra?.... Non
vi domando il vostro parere; può importarmene molto del vostro
parere!... Ma vi dico io che non c’è colèra, non c’è che un branco di
vigliacchi che scappano e un manipolo di vanitosi che si
arrampicherebbero sugli specchi per richiamare l’attenzione sopra di
sè.... Come quei dottorini della policlinica che girano per la città in
cerca di colerosi, e quando non ce ne sono se ne inventano....
Saltimbanchi, saltimbanchi!... Oh nel 1849 sì che ci fu il colèra a
Venezia, e avevamo più di quattrocento casi in un giorno.... Ma già
voi non eravate neanche nata nel 49... Peggio per voi che vi
toccherà stare di più in questo mondaccio.... Cosa c’è? Dove
andate?
— Ma.... — balbettò la ragazza — hanno suonato alla porta di
strada.
— Che aspettino.... Fin che parlo io, voi dovete rimanere.... Dove
avete imparato la creanza?
In quel momento suonarono di nuovo, e siccome sapevo che la
signora Celeste era uscita e ritenevo quindi che fosse lei, andai io
stessa ad aprire.
Era invece il professore Verdani che aveva dimenticato la chiave di
casa e veniva a prenderla. Figuriamoci com’egli rimase quando vide
me sul pianerottolo, come arrossì, e quante scuse mi fece. Gli
dispiaceva proprio d’avermi disturbata.
— Un disturbo piccolo — risposi; — La Gegia è tenuta in chiacchiere
dal signor colonnello.
— Ah! — fece il professore.
E voleva aggiungere qualche cosa, e qualche cosa volevo
aggiungere anch’io. Ma eravamo imbarazzati tutti e due e ci
limitammo a un saluto più espansivo del solito.
A guardarlo bene il professore non è mica un brutto giovine....
Probabilmente la lettera di Odoardo è in viaggio. Ma da Tiflis a
Venezia le lettere ci mettono un paio di settimane, sicchè ho da
aspettare almeno dieci o dodici giorni. Sono curiosa di vedere quanti
danari mio fratello mi manda, e aspetto la sua rimessa prima di fare
alcune spese necessarie pel mio viaggio e di comperare qualche
regaluccio per le mie amiche. S’egli non mi spedisce che quanto
occorre strettamente pel tragitto a Costantinopoli, mi converrà
vendere o impegnare i pochi oggetti preziosi che conservo come
ricordi di famiglia.... Sarebbe un principiar molto male.
Sabato, 5 giugno.
Questa mattina la signora Celeste s’era fitta in capo di condurmi alla
chiesa della Salute, ove c’è una funzione solenne per invocar dalla
Madonna la cessazione del morbo che ci affligge. Io rispetto le
credenze di tutti, ma non so simulare una fede che non ho. Rifiutai
quindi d’accompagnare la mia padrona di casa nel suo
pellegrinaggio, e per quietarla le promisi di non partir da Venezia
senza essermi recata una domenica con lei a San Marco, all’ora
della messa grande.... Ci andrò volentieri; la basilica è tanto bella! E
poi non sono mica una giacobina, non ho mica l’orrore dei templi,
non mi atteggio io, povera donna, ignorante, a libera pensatrice, a
spirito forte.... Ho una ripugnanza invincibile a fingere, ecco tutto.
Del resto, la signora Celeste non è punto intollerante e fanatica.
Siamo uscite insieme anche stamane di buonissimo accordo; ella
andò alla sua chiesa, io andai da altra parte. Nel ritorno presi il
vaporino a San Moisè e mi trovai seduta poco distante dal dottor
Negrotti, il nostro medico antico, quello che mi ha vista nascere.
Volevo salutarlo, ma egli era in compagnia, e miope com’è non mi
ravvisò.
Passammo dinanzi alla Salute. La superba chiesa era aperta,
sfavillante di ceri; moltissime gondole erano ferme dinanzi alla riva,
quelle tra l’altre del Municipio, con le bandiere a prora e i barcaiuoli
in tenuta di gala.
— Dottore — disse qualcheduno — ci crede lei alla Madonna della
Salute quale specifico contro il colèra?
— Caro mio — rispose il medico — credo appena al laudano, e poco
anche a quello.
Seguitarono così per un pezzo, tirando giù a campane doppie contro
i pregiudizi popolari, contro le processioni di fanciulle scalze, contro
la Giunta municipale che interveniva in pompa magna a una
cerimonia religiosa.
— Meno male la Giunta! — sospirò con comica gravità il dottor
Negrotti, — il peggio si è che ha voluto intervenirvi mia moglie,
pigliando per sè la gondola e sforzandomi a girar per la città in
vaporetto.
Il dottor Negrotti è molto invecchiato d’aspetto, ma è sempre lo
stesso uomo, scettico, sarcastico; e non dubito che si sarà
conservato buonissimo di fondo, caritatevole e leale a tutta prova.
Avevo rinunziato a salutarlo per oggi, quando alla stazione della Cà
d’Oro vidi con piacere ch’egli s’accommiatava dagli amici e
scendeva con me.
Me gli accostai tendendogli la mano. — Dottore, non mi riconosce?
— Oh! — fec’egli con un sorriso cordiale. — L’Elena?... Era in tram?
— Sì certo.... e a pochi passi da lei.... Ma non osavo disturbarlo.
— Perchè, perchè?... Oh come sono lieto di quest’incontro!... Dopo
tanto tempo! E come va, cara Elena?
Una volta il dottor Negrotti mi dava del tu; adesso si capisce che gli
faccio soggezione.
Camminavamo a fianco; egli era diretto dalla stessa parte ov’ero
diretta io. Gli raccontai le mie ultime vicende, la solitudine in cui ero
rimasta, la decisione che avevo presa di raggiunger mio fratello a
Tiflis.
— Oh diavolo, diavolo! — esclamò il dottore. — Che cosa mi
narra?... Ma lei deve appena conoscerlo quest’Odoardo. Era poco
più d’una bambina quando partì.
— Fu nel 66. Avevo cinqu’anni.
— Sicuro. Tra voi altri due ci devono essere almeno quindici anni di
differenza.
— Sedici ce ne sono.
— Già.... Odoardo è ormai un uomo maturo.... Come passa il
tempo!... Allora era un bel giovinetto.... molto vivace.... forse troppo
vivace....
Io non dissi nulla.... Pensavo alle lacrime che quel ragazzo aveva
fatto spargere a’ miei genitori.
— Non cattivo però — soggiunse Negrotti. — Era di quelli che hanno
bisogno di libertà, che non sanno adattarsi a star nelle file.... Ma una
volta che si sono aperta una strada, metton giudizio.... Deve aver
girato molto....
— Oh moltissimo!... Non s’è fissato a Tiflis che nell’83.
— E non ha mai fatto una corsa sin qui?
— Mai.
Il dottore rimase un momento soprappensiero; poi mi domandò: — È
rimasto scapolo?
— Sì.
— Capisco — riprese il vecchio medico. — Lei non ha altri appoggi,
non ha altri parenti....
— Nessuno, nessuno.... Ma — esclamai — sia sincero.... Crede che
io stia per commettere un grande sproposito?
— No, cara Elena, no.... È probabile che al suo posto avrei fatto lo
stesso anch’io..... A ogni modo, lei è una ragazza coraggiosa; se non
si trovasse bene saprebbe tornare nel suo paese.
— Oh! — diss’io.... tentennando la testa — non tornerò più.
E mi salivano le lacrime agli occhi.
Il dottore rallentò il passo, e mi mostrò un portone all’angolo della
calle. — Debbo fermarmi qui.... Ma lei non parte mica subito?...
Gli risposi che ritenevo di non partire prima della fine del mese.