Добавил:
Sekretar
kiopkiopkiop18@yandex.ru
t.me/Prokururor I Вовсе не секретарь, но почту проверяю
Опубликованный материал нарушает ваши авторские права? Сообщите нам.
Вуз:
Предмет:
Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5526_Библиотеки_им_академика_М_И_Перельмана.pdf
448
M. Carvalho Soares and P. A. Sampaio Rocha-Filho
[1]. The headache is often described as pressing or tightening in quality and may
present features of tension-type headache or migraine. However, its association
with dystonic muscle overactivity is a dening characteristic [19].
The differential diagnosis includes several primary and secondary headache
types. Cervicogenic headache is a key condition to distinguish, as it is typically
provoked by neck movement or sustained awkward postures, commonly unilateral
and often relieved by anesthetic nerve blocks, features that are less characteristic of
HACCD.An evidence of a cervical disorder or lesion capable of causing headache,
based on clinical or imaging ndings, is necessary for its diagnosis [1]. Tension-type
headache is generally bilateral, dull, and non-pulsatile, and it does not exhibit a
temporal relationship with dystonic movements. Myofascial pain syndrome may
present with referred pain in the occipital or cervical region but is identied by the
presence of palpable trigger points and absence of involuntary movements [8]. Also,
it can coexist with HACCD and other headaches. Notably, HACCD may clinically
resemble migraine or tension-type headache in characteristics, but the temporal and
anatomical correlation with dystonia is the key feature that supports its diagnosis
according to ICHD-3 [1]. Accurate recognition requires careful clinical correlation
between headache features, its distribution, and timing of dystonic activity.
46.6 Treatment
Some studies have evaluated the effect on BoNT-A for treating CCD and associated
headaches and have shown an improvement in pain [14–19]. A prospective cohort
study compared patients with CCD with HACCD versus patients with CCD and
other headaches. Only those with HACCD showed a decrease in the impact of headache after BoNT-A (measured by HIT-6 score) [15]. It is not clear whether the
improvement in headache secondary to BoNT-A occurs due to the improvement in
dystonia or due to the effect of botulinum toxin on pain itself.
Therapeutic responses to botulinum toxin in the context of CD-associated head-
aches remain variable. Hulzenga etal. noted limited efcacy of botulinum toxin for
headache in CD, with only 3 of 19 patients receiving treatment, and only two reporting symptomatic relief [9]. Conversely, Barbanti etal. reported more favorable outcomes: among 45 patients with primary CCD, 12 (26.7%) experienced headache
relief following botulinum toxin injection [6]. These ndings highlight both the
potential therapeutic role and the challenges in recognizing subtle forms of dystonia
contributing to head pain.
Chemodenervation with BoNT-A remains the most effective and preferred treat-
ment for CD, requiring a highly individualized approach to optimize outcomes.
However, achieving the best outcomes depends on the precise and tailored application of injection techniques [7]. Nevertheless, no clinical trials evaluate the specic
treatment of HACCD.While BoNT-A injections are generally well-tolerated, mildto- moderate side effects such as dysphagia, local injection pain, and muscle weakness can occur, but these are typically manageable. The therapeutic effects of
46 Headache Attributed toCraniocervical Dystonia
449
BoNT-A may be mediated through multiple mechanisms. First, BoNT-A inhibits
muscle contractions and subsequent peripheral release of neurotransmitters involved
in pain regulation. Additionally, there is evidence suggesting that BoNT-A can be
retrogradely transported into the central nervous system, where it may modulate
pain-related pathways in the spinal cord, brainstem, and cerebral cortex [16]. These
mechanisms likely help prevent or reverse peripheral and central sensitization, providing a possible explanation for the efcacy of BoNT-A in treating not only
dystonia- related pain but also other painful conditions [8].
46.7 Conclusion
CCD is a complex and multifaceted neurological disorder that not only presents
with debilitating motor symptoms but also with signicant pain, often in the form of
HACCD.Understanding the interplay between motor dysfunction and pain in CCD
is crucial for developing effective, comprehensive treatment strategies. While
BoNT-A remains the cornerstone of treatment for both the motor and pain symptoms associated with CCD, its efcacy in managing headache-related pain highlights the importance of addressing both peripheral and central mechanisms of pain
in these patients. Although BoNT-A is highly effective for many patients, the individualized nature of its application requires careful consideration of injection techniques, muscle targeting, and patient response.
References
1. Olesen. Headache Classication Committee of the International Headache Society (IHS) The
International Classication of Headache Disorders, 3rd edition. Cephalalgia. 2018;38(1):1–211.
2. Albanese A, Bhatia K, Bressman SB, DeLong MR, Fahn S, Fung VSC, Hallett M, Jankovic
J, Jinnah HA, Klein C, Lang AE, Mink JW, Teller JK.Phenomenology and classication of
dystonia: a consensus update. Mov Disord. 2013;28(7):863–73.
3. Balint B, Mencacci NE, Valente EM, Pisani A, Rothwell J, Jankovic J, Vidailhet M, Bhatia
KP.Dystonia. Nat Rev Dis Primers. 2018;4(1)
4. Bezerra ME, Rocha-Filho PA.Headache attributed to Craniocervical dystonia—a little known
headache. Headache J Head Face Pain. 2017;57(2):336–43.
5. Kutvonen O, Dastidar P, Nurmikko T.Pain in spasmodic torticollis. Pain. 1997;69(3):279–86.
6. Barbanti P, Fabbrini G, Pauletti C, Defazio G, Cruccu G, Berardelli A.Headache in cranial and
cervical dystonia. Neurology. 2005;64(7):1308–9.
7. Lizarraga KJ, Al-Shorafat D, Fox S.Update on current and emerging therapies for dystonia.
Neurodegener Dis Manag. 2019;9(3):135–47.
8. Rosales RL, Cuffe L, Regnault B, Trosch RM.Pain in cervical dystonia: mechanisms, assessment and treatment. Expert Rev Neurother. 2021;21(10):1125–34.
9. Hulzenga MA, Beumer D, Koehler PJ.Dystonic head tremor and the coexistence of headache.
Tremor Other Hyperkinet Mov. 2017;7:485.
10. LaHue SC, Albers K, Goldman S, Lo RY, Gu Z, Leimpeter A, Fross R, Comyns K, Marras
C, De Kleijn A, Smit R, Katz M, Ozelius LJ, Bressman S, Saunders-Pullman R, Comella C,
450
Klingman J, Nelson LM, Van Den Eeden SK, Tanner CM.Cervical dystonia incidence and
diagnostic delay in a multiethnic population. Mov Disord. 2020;35(3):450–6.
11. Defazio G, Belvisi D, Comella C, Hallett M, Jinnah HA, Cimino P, Latorre A, Mascia MM,
Rocchi L, Gigante AF, Ercoli T, Berardelli A.Validation of a guideline to reduce variability in
diagnosing cervical dystonia. J Neurol. 2023;270(5):2606–12.
12. Comella C, Bhatia K.An international survey of patients with cervical dystonia. J Neurol.
2015;262(4):837–48.
13. Boyce MJ, Canning CG, Mahant N, Morris J, Latimer J, Fung VSC.The Toronto Western
Spasmodic Torticollis rating scale: reliability in neurologists and physiotherapists.
Parkinsonism Relat Disord. 2012;18(5):635–7.
14. Zolin A, Broner SW, Yoo A, Guan I, Lakhani S, Trabilsy M, Klebanoff L, Vo M, Sarva
H.Dystonia phenomenology and treatment response in migraine. Headache J Head Face Pain.
2023;63(2):255–63.
15. Bezerra ME, Rocha-Filho PA.Headache attributed to craniocervical dystonia: a prospective
cohort study. Eur J Pain. 2020;24(8):1484–94.
16. Ondo WG, Gollomp S, Galvez-Jimenez N.A pilot study of botulinum toxin a for headache in
cervical dystonia. Headache J Head Face Pain. 2005;45(8):1073–7.
17. Dowson AJ, Kilminster SG, Salt R. Clinical prole of botulinum toxin a in patients with
chronic headaches and cervical dystonia: a prospective, open-label, longitudinal study conducted in a naturalistic clinical practice setting. Drugs R D. 2008;9(3):147–58.
18. Winner PK, Sadowsky CH, Martinez WC, Zuniga JA, Poulette A. Concurrent
OnabotulinumtoxinA treatment of cervical dystonia and concomitant migraine. Headache J
Head Face Pain. 2012;52(8):1219–25.
19. Galvez-Jimenez N, Lampuri C, Patiño-Picirrillo R, Hargreave MJ, Hanson MR. Dystonia
and headaches: clinical features and response to botulinum toxin therapy. Adv Neurol.
2004;94:321–8.
M. Carvalho Soares and P. A. Sampaio Rocha-Filho
Chapter 47
Headache Attributed toHeterophoria Or
Heterotropia
CaioViniciusde MeiraGravaSimioni
47.1 Introduction
Headache attributed to heterophoria or heterotropia represents a unique intersection
between neurology and ophthalmology. From the start, heterophoria is a latent deviation of the eyes that surfaces when binocular fusion is disrupted. Heterotropia, in
turn, is the overt misalignment observable during routine examination. In both pediatric and adult populations, ocular misalignment and related refractive issues play a
signicant role in headache pathogenesis.
Given that a considerable proportion of patients (especially children) with headaches are found to have ocular ndings that might be causally related, clinicians
must approach headache evaluation with an interdisciplinary mindset.
In this context, collaboration between neurologists and ophthalmologists is paramount. Timely identication and management of subtle ocular aberrations can be
key to effective headache treatment.
47.2 Pathophysiology
Headaches in patients with heterophoria or heterotropia are associated with distinct
neural and muscular processes related to binocular vision dysfunction [1]. Several
studies report that altered neural control of vergence plays a role. For example, differences in the fast and slow fusional disparity systems, with associated activity
changes in the secondary visual cortex, right cuneus, and oculomotor vermis, have
C. V. de MeiraGravaSimioni (*)
University of São Paulo, Faculdade de Medicina, Hospital das Clínicas, Departamento de
Neurologia, São Paulo, SP, Brazil
e-mail: caiosimioni@gmail.com
Switzerland AG 2026
D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_47
451© The Author(s), under exclusive license to Springer Nature
452
C. V. de MeiraGravaSimioni
been observed in patients with convergence insufciency [2]. In migraine cases,
activation of the trigeminovascular system and lateral inhibition in the visual cortex
are noted during episodes of binocular dysfunction. At the same time, interruptions
in sensory-motor feedback compromise the continuous calibration of vergence [3].
Muscular mechanisms are also implicated. Multiple reports describe how disruptions in the coordination between accommodation and convergence increase the
effort required by extraocular muscles. In one study, associations between exophoria, convergence insufciency, and inadequate positive fusional vergence at near
xation were identied in 150 headache patients [1]. Other observations reveal that
in cases of vertical heterophoria, competition between a faulty vestibular reex and
a corrective fusional reex leads to muscle overuse and fatigue [4]. These ndings
collectively suggest that both neural pathway alterations and compensatory muscular strain contribute to the pathogenesis of headaches in these patients.
Studies examined binocular anomalies and headaches in populations ranging
from children to adults. In a sample with a mean age of 25years, near-xation
assessments revealed 82% exophoria, 7.34% esophoria, and 10.7% orthophoria;
this study also noted a statistically signicant association between headache and
female gender (p<0.0001) [5]. In a young adult group (ages 20–30), exophoria
correlated with headache, with frontal and hemicranial pain reported by 44 and
34% of patients, respectively [6]. A pediatric study (mean age 10years) documented a 4.4% prevalence of strabismus, with no specic data on heterophoria [7].
Another study covering ages 5–36years found that 26.2% of headache patients
had convergence insufciency, with 78.7% of these reporting frontal headaches
[8]. In studies reporting gender, females constituted 60–79% of participants, with
one study nding a ratio of 1.85:1 compared to males [5]. Heterotropia data were
limited to the single pediatric study, and no study provided incidence rates [7].
While reports in adult and mixed-age populations consistently show high prevalences of heterophoria (notably exophoria) and associated headache symptoms,
pediatric-specic data on heterophoria remain sparse, limiting denitive age-based
comparisons [5].
47.3 Case Presentation
Sara, a 14-year-old female, presents to the neurologic clinic with complaints of
frequent headaches, particularly when exposed to smartphone or computer screens.
She reports that these headaches have been occurring for the past 6 months and have
gradually increased in frequency and intensity.
She describes her headaches as dull, aching pain localized in her forehead and
around her eyes. The headaches typically begin after about an hour of screen time
and worsen with continued use. She denies any throbbing sensation, nausea, vomiting, photophobia, or phonophobia. She reports occasional blurred vision, especially
when switching her gaze between the screen and distant objects. She also notes that
her eyes feel tired and strained after prolonged use of screens. Sara has no signicant past medical history and no known allergies. She does not have a personal or
47 Headache Attributed toHeterophoria Or Heterotropia
family history of migraine or other headache disorders. The patient occasionally
takes over-the-counter ibuprofen for headache relief, which provides only temporary relief. Her mother has a history of refractive errors but no signicant headache
disorders. She is a healthy-appearing adolescent female. Her vital signs are within
normal limits. Her neurological examination is normal, with no cranial nerve decits, motor or sensory abnormalities, or reex asymmetry. The patient’s visual acuity was 20/25in both eyes without correction. Subjective refraction revealed a mild
hyperopic refractive error bilaterally. Extraocular movements were full and smooth,
indicating normal ocular motility. The cover test demonstrated exophoria at near
xation, which became more pronounced with prolonged near work. Slit-lamp
examination of the anterior segment was normal. Fundoscopic evaluation showed
healthy optic nerves and retinal vessels, with no abnormalities noted.
Sara is prescribed a course of vision therapy to improve her fusional reserves and
reduce the strain on her extraocular muscles. The therapy includes exercises to
strengthen convergence and improve binocular coordination. She is advised to take
frequent breaks during screen use, adjust her screen distance and lighting, and
ensure proper posture.
Ultimately, Sara is prescribed glasses with a mild hyperopic correction and a
small amount of prism to help align her eyes at nearby distances.
After 6 weeks of vision therapy, ergonomic adjustments, and wearing her prescribed glasses, Sara reports a signicant reduction in the frequency and intensity of
her headaches. She also notes improved visual comfort and less eye strain during
screen use.
453
47.4 Case Discussion
Based on Sara’s history and examination ndings, she is diagnosed with a headache
attributed to heterophoria, specically exophoria, exacerbated by prolonged screen
use. Sara’s case illustrates the typical presentation of headaches associated with
ocular misalignment. Her symptoms of frontal headache, blurred vision, and eye
strain, which are exacerbated by screen time, are consistent with the strain on the
extraocular muscles when compensating for exophoria. The absence of migraine
features and a normal neurological examination further supports the diagnosis of
headache attributed to heterophoria.
47.5 Clinical Presentations
Three pediatric studies (n=2904) provided limited information on headache symptoms in patients with strabismus. No study reported headache frequency, intensity,
or duration by specic strabismus subtype [7, 9, 10]. One study noted that 57.8% of
a high-risk group experienced headaches, yet no quantitative measures of pain
intensity were provided [10]. Headache duration was described only qualitatively,
454
C. V. de MeiraGravaSimioni
with one report linking shorter episodes and morning pain to ocular ndings such as
optic nerve elevation [7].
Siqueira etal. proposed a scale that can predict the likelihood of headache in
pediatric patients with ophthalmic disorders, a tool called “HAMS Score”.
According to the HAMS score, strabismus is more likely to have headache (5.21),
followed by hyperopia (3.10), myopia (2.67), and, nally, astigmatism (1.86) [10].
47.6 Diagnostic Algorithm
Step 1: Clinical Suspicion
• Identify Key Symptoms:
– Headache related to visual effort
– Aggravated by sustained visual tasks (i.e., prolonged exposure to screens).
Step 2: Initial Diagnostic Workup
• Refer the patient to an ophthalmologic examination
• Rule out other secondary headache disorders, based on “red ags” symptoms
[11]; if necessary, order neuroimaging tests (i.e., magnetic resonance imaging
[MRI] or computed tomography [CT] scan).
Step 3: Apply Third Edition of the International Classication of Headache
Disorders (ICHD-3) Diagnostic Criteria [12]
A. Headache fullling criterion C
B. Heterophoria or heterotropia has been identied, with at least one of the fol-
lowing symptoms:
1. Blurred vision
2. Diplopia
3. Difculty switching from near to far focus and/or vice versa
C. Evidence of causation demonstrated by at least two of the following:
1. Headache has developed in temporal relation to the onset of heterophoria
and/or heterotropia, or led to its discovery
2. Headache has signicantly improved after correction of the heterophoria
and/or heterotropia
3. Headache is aggravated by sustained visual tasks
4. Headache is alleviated by closing one eye and/or discontinuation of the
visual task
D. Not better accounted for by another ICHD-3 diagnosis.
47 Headache Attributed toHeterophoria Or Heterotropia
455
47.7 Differential Diagnosis
As mentioned earlier, the headache attributed to heterophoria and heterotropia
should be distinguished from other secondary headaches, especially other ocular
conditions that may cause headaches, such as glaucoma or uveitis.
It is worth noting that primary headaches, such as migraine and tension-type
headache, have a high prevalence and can coexist with headaches attributed to heterophoria and heterotropia, which warrants a distinct approach and treatment.
Headache
characteristics
Visual symptoms Specic inquiries about blurred vision, double vision, eye strain, and
Ocular history Prior diagnoses of refractive errors, strabismus, or other ocular conditions.
Triggers Identication of factors that exacerbate headaches, such as prolonged
Impact on daily
life
Detailed description of headache frequency, duration, intensity, location,
and associated symptoms.
difculties with near or distance vision.
reading, computer use, or specic visual tasks.
Assessment of how headaches and visual symptoms affect the patient’s
ability to perform daily activities, including work, school, and leisure.
47.8 Treatment
This case highlights the importance of considering ocular causes in the evaluation
of pediatric headaches, particularly in the context of increased screen time. Sara’s
diagnosis of headache attributed to exophoria, a type of heterophoria, was made
based on her history, symptoms, and ophthalmological examination ndings. The
successful management of her headaches with vision therapy, ergonomic adjustments, and corrective lenses underscores the effectiveness of addressing the underlying ocular misalignment. This vignette also emphasizes the importance of a
multidisciplinary approach, involving collaboration between neurologists and ophthalmologists, to optimize patient outcomes.
In one study of 111 adults with vertical heterophoria, treatment with micro-prism
lenses reduced headache disability. This study reported a decrease in the mean
Headache Disability Inventory score from 37.55 to 22.13 (effect size d=0.54) and
similar reductions in dizziness and situational anxiety scores [13]. Two other studies—in adults with convergence insufciency—evaluated ofce-based vision therapy (versus placebo or home-based pencil pushups) and a home-based computer
orthoptic program. Although these studies demonstrated signicant improvements
in near point of convergence and positive fusional vergence, they did not report
outcomes for headache frequency or intensity [14, 15].
Thus, among adults with heterophoria or heterotropia, only micro-prism lenses
have documented benets for reducing headache-related disability based on the
available ndings [13].
456
C. V. de MeiraGravaSimioni
47.9 Conclusion
Headache attributed to heterophoria or heterotropia represents a complex clinical
entity that requires a comprehensive and multidisciplinary approach. By integrating
clinical history, physical examination ndings, ophthalmological examination
results, and diagnostic tools such as the HAMS Score, clinicians can effectively
identify and manage these cases, leading to improved patient outcomes.
References
1. Saif Hassan Al-Rasheed. Clinical characteristics of patients presenting with headache at binocular vision clinic: a hospital based study. Pak J Ophthalmol. 2020:36(3):247–252. https://
doi.org/10.36351/pjo.v36i3.1046.
2. Alvarez TL, Scheiman M, Morales C, Gohel S, Sangoi A, Santos EM, Yaramothu C, d’AntonioBertagnolli JV, Li X, Biswal BB. Underlying neurological mechanisms associated with
symptomatic convergence insufciency. Sci Rep. 2021;11(1):6545. https://doi.org/10.1038/
s41598-021-86171-9. PMID: 33753864; PMCID: PMC7985149.
3. Sara L, Ruzda K, Shabeebz N, Meghana KH. Binocular vision function and migraine: a review.
Int J Multidiscipl Res (IJFMR). 2022;4(6).
4. Rosner MS, Feinberg DL, Doble JE, Rosner AJ. Treatment of vertical heterophoria ameliorates
persistent post-concussive symptoms: A retrospective analysis utilizing a multi-faceted assessment battery. Brain Inj. 2016;30(3):311–7. https://doi.org/10.3109/02699052.2015.1113564.
Epub 2016 Feb 1. PMID: 26829465.
5. Alrasheed SH, Osman TM, Aljohani S, Alshammeri S. Clinical features of sudanese
patients presenting with binocular vision anomalies: a hospital-based study. J Med Life.
2023;16(8):1251–57. https://doi.org/10.25122/jml-2023-0132. PMID: 38024832; PMCID:
PMC10652681.
6. Kumar DG, Kumar DS. Evaluation of the relationship between binocular anomaly and headache: prospective cross-sectional study. Eur J Mol Clin Med (EJMCM). 2021;07(10).
7. Lin LY, Pan W, Ying GS, Binenbaum G. Ocular ndings in children with headache. Ophthalmic
epidemiology. 2022;30(4):392–99. https://doi.org/10.1080/09286586.2022.2125019.
8. Priya SV, Priya TV, Amudha P, Lalithambigai C. The relationship between headache and convergence insufciency. Panacea J Med Sci. 2021;11(1):147–150.
9. Mo Y, Zhang W, Tang X, Zhang R, Wang Y, Zheng L. Evaluation of postoperative discomfort after strabismus surgery under general anesthesia in children: a prospective observational study. J Pain Res. 2024;17:2717–2726. https://doi.org/10.2147/JPR.S468977. PMID:
39188912; PMCID: PMC11346475.
10. Siqueira P de TVV, Andrade-Valença LPA de, Andrade JR, Valença MM. Pediatric patients at
a high risk of headache of ocular origin: the HAMS Score (Hyperopia, Astigmatism, Myopia,
and Strabismus). Headache Med [Internet]. 2021 Oct. 19 [cited 2025 Apr. 29];12(2):134-40.
Available from: https://headachemedicine.com.br/index.php/hm/article/view/443.
11. Do TP, Remmers A, Schytz HW, Schankin C, Nelson SE, Obermann M, Hansen JM, Sinclair
AJ, Gantenbein AR, Schoonman GG. Red and orange ags for secondary headaches in
clinical practice: SNNOOP10 list. Neurology. 2019;92(3):134–144. https://doi.org/10.1212/
WNL.0000000000006697. Epub 2018 Dec 26. PMID: 30587518; PMCID: PMC6340385.
12. Headache Classication Committee of the International Headache Society (IHS) the international classication of headache disorders, 3rd edition. Cephalalgia 2018;38(1):1–211 https://
doi.org/10.1177/0333102417738202.
47 Headache Attributed toHeterophoria Or Heterotropia
13. Rosner MS, Feinberg DL, Rosner AJ. Vertical Heterophoria Treatment Ameliorates Headache,
Dizziness and Anxiety. Optometry & Visual Performance 2020;8(1):24–34.
14. Scheiman M, Mitchell GL, Cotter S, Kulp MT, Cooper J, Rouse M, Borsting E, London R,
Wensveen J. A randomized clinical trial of vision therapy/orthoptics versus pencil pushups for
the treatment of convergence insufciency in young adults. Optom Vis Sci. 2005;82(7):583–95.
https://doi.org/10.1097/01.opx.0000171331.36871.2f. PMID: 16044063.
15. Serna A, Rogers DL, McGregor ML, Golden RP, Bremer DL, Rogers GL. Treatment of symptomatic convergence insufciency with a home-based computer orthoptic exercise program. J
AAPOS. 2011;15(2):140–3. https://doi.org/10.1016/j.jaapos.2010.11.023. Epub 2011 Mar 31.
PMID: 21458340.
457