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
264
E. KochanKizilkilic and D. Uluduz
variations in headache frequency and intensity may correlate with cerebral blood
ow dynamics and compensatory collateral load [8]. In pediatric populations, headache is also a common symptom of MMD, though more overt neurological decits
frequently overshadow it. An extensive review of 204 pediatric patients showed that
headache was a primary complaint in nearly one-third of cases [11], emphasizing
the need for age-specic diagnostic consideration.
Given the often-subtle and misleading nature of headache presentations in
Moyamoya disease, clinicians must maintain a high index of suspicion, especially
in cases of late-onset, changing headache patterns or those refractory to typical
migraine therapies. Advanced neuroimaging remains the cornerstone of diagnosis,
and timely identication may prevent progression to more debilitating vascular
events [3, 7].
This chapter explores Moyamoya-related headaches through the lens of a detailed
case vignette, discussing clinical features, diagnostic challenges, and the current
understanding of pathophysiology and management. By shedding light on this rare
but crucial cause of secondary headaches, we aim to bridge the gap between clinical
suspicion and denitive diagnosis, thereby improving patient outcomes through
timely intervention.
28.2 Case Presentation
A 51-year-old right-handed female patient presented to the neurology outpatient
clinic with a complaint of gradually increasing frequency and intensity of headaches over the past 2–3months. She reported that she had been experiencing headaches intermittently for the past 30 years. Initially, the headaches had a pattern
consistent with episodic migraine without aura—unilateral, throbbing in character,
accompanied by nausea, vomiting, and photophobia. Attacks occurred approximately one to two times per month in earlier years, lasted for about 24h if untreated,
and were typically moderate in intensity, often responsive to over-the-counter
analgesics.
Interestingly, over the last 5years, the frequency of her attacks had signicantly
declined, almost to the point of remission. However, during the past few months, the
headache frequency abruptly increased, now occurring once or twice a week, with
some attacks reaching high intensity and lasting nearly a full day, sometimes requiring her to rest in a dark room due to severe photophobia. The headache pattern
remained predominantly unilateral and pulsatile, localized to the frontotemporal
region. No aura, focal neurological decits, or syncope episodes were described.
There were no triggers she could identify. Despite the increased burden, she had
never undergone a formal neurological evaluation or neuroimaging prior to this visit.
Her past medical history was unremarkable. She had never been diagnosed with
hypertension, diabetes, or hyperlipidemia and was not on any regular medication.
She denied alcohol or illicit drug use but reported smoking one pack of cigarettes
28 Headache Attributed toMoyamoya Angiopathy
265
per day for over 20years. Her family history was notable for similar headaches in
her mother, which had never been investigated or formally diagnosed.
Neurological examination at the time of admission was entirely normal. Routine
blood work revealed no abnormalities, including thyroid function, C-reactive protein (CRP), autoimmune panels, coagulation studies, and vasculitis screening.
Initial non-contrast cranial computed tomography (CT) was normal. However, a
contrast-enhanced cranial magnetic resonance imaging (MRI) was performed due
to the patient’s atypical headache evolution, particularly the recurrence after a prolonged quiescent phase and the increased burden. MRI revealed contrast enhancement of basal collateral vessels, along with increased uid-attenuated inversion
recovery (FLAIR) signal intensities at the level of the cerebral convexities, suggestive of slow-ow collateral channels (Figs.28.1 and 28.2). These ndings prompted
further vascular imaging.
Cranial MR angiography (MRA) demonstrated bilateral occlusion of the supraclinoid segments of the internal carotid arteries (ICA), along with prominent basal
collaterals, producing the characteristic “puff of smoke” appearance. Subsequent
digital subtraction angiography (DSA) (Figs.28.3 and 28.4) conrmed the diagnosis, revealing:
• Bilateral occlusion of ICA supraclinoid segments.
• Proliferation of basal collaterals adjacent to the occlusion.
• Convexity collaterals arising from the external carotid arteries.
• Posterior circulation compensation via vertebrobasilar collaterals.
Fig. 28.1 Contast
enhancement brain MRI
266
Fig. 28.2 Coronal image
of contast enhancement
Brain MRI
E. KochanKizilkilic and D. Uluduz
Fig. 28.3 Network of
blood vessels in cerebral
angiography
28 Headache Attributed toMoyamoya Angiopathy
Fig. 28.4 Cerebral
angiographic image of the
patients
267
No evidence of atherosclerosis, dissection, or secondary causes such as craniopharyngioma or sickle cell disease was found. The imaging and clinical ndings
met the diagnostic criteria for Moyamoya disease.
Given the absence of transient ischemic attacks, strokes, or hemorrhagic events
and the currently stable neurological status, no immediate surgical intervention was
planned. A conservative management strategy focusing on headache control was
adopted. The patient was counseled regarding the nature of Moyamoya disease and
the need for regular follow-up and vascular monitoring. Symptomatic treatment
with triptan alternatives and migraine prophylaxis was initiated cautiously, considering potential vasoconstrictive effects.
28.3 Case Discussion
This case highlights the often under-recognized presentation of Moyamoya disease
with migraine-like headaches as the primary or even sole manifestation, particularly
in adults. The long history of stable, non-disabling headaches followed by a recent,
unexplained escalation in attack frequency and severity should alert the clinician to
reconsider the initial diagnosis and evaluate for underlying structural or vascular
abnormalities.
The absence of focal decits or classic ischemic episodes may delay diagnosis,
especially in patients with a long-standing migraine history. However, certain red
ags—such as a late-onset increase in headache burden, resistance to usual medications, and headaches associated with signs of impaired cerebrovascular reserve—
warrant advanced neuroimaging. In this case, progressive bilateral ICA occlusion
268
and basal collateral formation were consistent with Moyamoya disease and dramatically changed the clinical trajectory.
While surgical revascularization is typically considered for ischemic or hemorrhagic complications, headache itself is increasingly recognized as a symptom worthy of monitoring in Moyamoya patients due to its possible association with cerebral
hypoperfusion or compensatory collateral circulation overload.
E. KochanKizilkilic and D. Uluduz
28.4 Clinical Characteristics
Headache is a frequently under-recognized symptom in Moyamoya disease (MMD),
often overshadowed by its more dramatic ischemic or hemorrhagic presentations.
Yet, growing evidence underscores that headache may be the disease’s rst—and in
some cases the only—manifestation, especially in adults [4–6]. As such, it carries
signicant diagnostic value and should prompt careful consideration in the appropriate clinical context.
The pathophysiology of Moyamoya-associated headaches is complex and multifactorial. One prominent theory involves chronic cerebral hypoperfusion, which can
result in ischemia-induced activation of nociceptors in pain-sensitive intracranial
structures [6]. Another mechanism includes the mechanical distension or tortuosity
of collateral vessels, which may irritate the dura or surrounding tissues, triggering
pain via the trigeminovascular pathway [1]. Additionally, altered cerebrovascular
autoregulation and uctuating perfusion pressures may sensitize central painprocessing networks and contribute to migrainous features [8].
In the case presented, the patient had a long history of migraine-like headaches,
but a recent shift in frequency and severity suggested a secondary etiology. Such
evolution in headache phenotype should be a red ag, especially in patients without
prior neuroimaging or a history of progressive disease [5, 7]. Importantly, this shift
represents a diagnostic opportunity: headache may act as a clinical “early warning
sign” preceding irreversible ischemic events, mainly when supported by subtle
imaging ndings [3].
28.5 Diagnosis
The differential diagnosis for a unilateral, throbbing headache with associated nausea, vomiting, and photophobia is broad. The primary consideration is migraine
without aura, given the symptomatic overlap. However, several distinguishing features can guide clinicians toward alternative diagnoses:
• Reversible Cerebral Vasoconstriction Syndrome (RCVS): Presents with recur-
rent thunderclap headaches, often bilateral and severe, and may include focal
28 Headache Attributed toMoyamoya Angiopathy
269
decits or seizures. Unlike Moyamoya, RCVS typically has a reversible angio-
graphic pattern over weeks [6].
• Cerebral Arterial Dissection: Especially in younger patients, carotid or vertebral
dissections may cause hemicranial headaches with Horner’s syndrome or focal
neurological signs. MRI/MRA and vessel wall imaging help differentiate this
from Moyamoya.
• Giant Cell Arthritis (GCA) is an essential consideration in patients>over 50. It
presents with a new-onset headache, scalp tenderness, jaw claudication, and
elevated inammatory markers. Temporal artery biopsy and imaging are
diagnostic.
• Cerebral Venous Sinus Thrombosis (CVST): Typically presents with diffuse or
frontal headache, possibly with papilledema, seizures, or altered mental status.
MR venography is the gold standard for diagnosis.
• Idiopathic Intracranial Hypertension (IIH): It presents with diffuse headache,
visual symptoms, and papilledema, more common in obese women of childbear-
ing age. Routine imaging with elevated opening pressure on lumbar puncture is
diagnostic.
• Brain Tumors or Mass Lesions: A headache due to a mass effect is often worse
in the morning or with Valsalva and may be associated with vomiting or neuro-
logical decits.
• Primary Angiitis of the CNS (PACNS): This is rare but important. It causes sub-
acute headaches, cognitive decline, and multifocal infarcts. Vessel imaging and,
in some cases, a brain biopsy are needed to diagnose it.
When Moyamoya disease is the underlying pathology, standard migraine treatments are often inadequate or even contraindicated, particularly vasoconstrictive
agents like triptans or ergot derivatives, which may exacerbate cerebral hypoperfusion [4, 5]. Thus, accurate diagnosis is not only crucial for disease monitoring and
stroke prevention but also essential to tailor appropriate symptomatic treatments
and avoid iatrogenic harm.
In contrast to classic migraine, Moyamoya-related headaches may lack aura and
often evolve in frequency or severity over time. Moreover, they are sometimes exacerbated by physical exertion or hyperventilation, likely due to impaired vascular
reactivity and decreased reserve capacity in affected territories [6]. These distinguishing features, when recognized, can guide clinicians toward timely neurovascular imaging.
Radiological imaging is pivotal in identifying Moyamoya disease and excluding
other entities in this complex differential. Non-contrast CT is often normal in early
stages, but MRI with FLAIR sequences may reveal slow-ow signal abnormalities
and subtle ischemic changes. Magnetic resonance angiography (MRA) typically
reveals stenosis or occlusion of the terminal internal carotid arteries (ICAs) and the
proliferation of ne basal collateral vessels, which is often more visible with
contrast- enhanced techniques [10]. Digital subtraction angiography (DSA) remains
the gold standard for diagnosis, characteristically demonstrating bilateral or unilateral ICA occlusion at the supraclinoid segment; basal collateral vessels with a “puff
270
of smoke” appearance, resulting from compensatory lenticulostriate and thalamoperforator networks; collaterals arising from the external carotid system and the
vertebrobasilar circulation as disease progresses [1]. In some cases, perfusion MRI
or single photon emission computed tomography (SPECT) can reveal regions of
delayed cerebral blood ow and impaired vascular reactivity, offering insights into
headache mechanisms and guiding surgical planning.
E. KochanKizilkilic and D. Uluduz
28.6 Conclusion
In conclusion, headache should be regarded as a potential sentinel symptom of
Moyamoya disease, especially in patients with atypical features, progression of previously stable headache patterns, or resistance to standard treatments. Early neurovascular imaging and heightened clinical awareness can facilitate earlier diagnosis,
allowing for timely intervention and stroke prevention [6, 8]. In this context, the
neurology clinic becomes not only a place of symptom management but also of lifechanging early detection.
28.7 Key Messages
• Headache can be the rst and sometimes only symptom of Moyamoya disease,
particularly in adult patients. Clinicians should maintain a high index of suspi-
cion when evaluating patients with new-onset, changing, or refractory
headaches.
• Migraine-like features (unilateral, throbbing pain, photophobia, nausea) are stan-
dard in Moyamoya-related headaches, which may lead to misdiagnosis and
delayed imaging.
• Red ag signs, such as an abrupt increase in attack frequency, resistance to typi-
cal treatments, or changes in headache pattern after years of stability, should
prompt evaluation for secondary causes, including Moyamoya disease.
• Differential diagnosis includes primary headache disorders and secondary
causes such as RCVS, cerebral vasculitis, arterial dissection, CVST, and tumors.
Neurovascular imaging is essential to differentiate Moyamoya from
these mimics.
• Conventional migraine therapies, especially vasoconstrictors, may be inap-
propriate or harmful in Moyamoya disease due to compromised cerebral
perfusion.
• MRI, MRA, and DSA remain the gold standards for diagnosis, with the “puff of
smoke” angiographic appearance being a hallmark nding.
28 Headache Attributed toMoyamoya Angiopathy
271
References
1. Kuroda S, Houkin K.Moyamoya disease: current concepts and future perspectives. Lancet
Neurol. 2008;7(11):1056–66.
2. Research Committee on the Pathology and Treatment of Spontaneous Occlusion of the Circle
of Willis; Health Labour Sciences Research Grant for Research on Measures for Infractable
Diseases. Guidelines for diagnosis and treatment of moyamoya disease (spontaneous occlusion
of the circle of Willis). Neurol Med Chir (Tokyo). 2012;52(5):245–66. https://doi.org/10.2176/
nmc.52.245.
3. Smith ER, Scott RM.Moyamoya: epidemiology, presentation, and diagnosis. Neurosurg Clin
N Am. 2010;21(3):543–51. https://doi.org/10.1016/j.nec.2010.03.007.
4. Prabhu N, etal. Unraveling the mystery: headache as a presenting symptom of moyamoya
disease. J Neurol Sci. 2024;455:121659. https://doi.org/10.1016/j.jns.2023.121659.
5. Zach V, Bezov D, Lipton RB, Ashina S.Headache associated with moyamoya disease: a
case story and literature review. J Headache Pain. 2010;11(1):79–82. https://doi.org/10.1007/
s10194- 009- 0181- 8.
6. Chiang CC, Shahid AH, Harriott AM, Tietjen GE, Savastano LE, Klaas JP, etal. Evaluation and
treatment of headache associated with moyamoya disease– a narrative review. Cephalalgia.
2022;42(6):542–52. https://doi.org/10.1177/03331024211056250.
7. Malone M, Ritchie D.The mystery of a unilateral headache ultimately diagnosed as Moyamoya
disease. Cureus. 2022;14(7):e26816. https://doi.org/10.7759/cureus.26816.
8. Gao B, Kang K, Zhang J, Zhang D, Zhao X.Clinical characteristics and long-term outcome
of headaches associated with Moyamoya disease in the Chinese population– a cohort study.
Front Neurol. 2020;11:605636. https://doi.org/10.3389/fneur.2020.605636.
9. Kraemer M, Lee SI, Ayzenberg I, Schwitalla JC, Diehl RR, Berlit P, etal. Headache in
Caucasian patients with Moyamoya angiopathy – a systematic cohort study. Cephalalgia.
2017;37(5):496–500. https://doi.org/10.1177/0333102416643516.
10. Muljadi R, Koesbandono, Pratama TA, Octavius GS.Moyamoya disease and moyamoya syndrome: a case series from multicentre private hospitals in Indonesia. Neurocirugia (Astur :
Engl Ed). Forthcoming 2024. https://doi.org/10.1016/j.neucie.2024.11.001. (DOI assumed for
completion—please update when available).
11. Seol HJ, Wang KC, Kim SK, Hwang YS, Kim KJ, Cho BK.Headache in pediatric moyamoya
disease: review of 204 consecutive cases. J Neurosurg Pediatr. 2005;103(5 Suppl):439–42.
https://doi.org/10.3171/ped.2005.103.5.0439.
https://doi.org/10.1016/S1474- 4422(08)70240- 0.
Chapter 29
Migraine-Like Aura Attributed toCerebral
Amyloid Angiopathy (CAA)
MariaEduardaNobre
29.1 Introduction
Cerebral amyloid angiopathy (CAA), also known as cerebral congophilic angiopathy due to the presence of abnormal amyloid aggregations demonstrated by microscopic examination of brain tissue after staining with Congo red, is a condition
characterized by the deposition of amyloid-beta peptides in the walls of cerebral
blood vessels. This deposition can lead to various neurological symptoms and complications, including an increased risk of hemorrhagic stroke, particularly in normotensive and elderly patients [1].
CAA has been recognized since the early twentieth century, with early observations suggesting that CAA was a likely cause of nontraumatic primary cerebral
hemorrhage, producing stroke in a signicant proportion of affected individuals.
While CAA is more commonly associated with cognitive decline and intracerebral
hemorrhages, some patients may experience migraine-like aura as a symptom [1].
29.2 Pathophysiology
Migraine aura refers to a range of neurological symptoms that typically occur before
a migraine headache, including visual disturbances, sensory changes, or motor
weakness. The exact mechanism by which CAA may produce migraine-like aura is
not entirely understood, but it is believed to be related to the effects of amyloid
deposition on vascular function and cerebral blood ow [1, 2].
Amyloids are brillary protein assemblies composed of monomers that form
beta-sheet structures and aggregate into long bers. In September 2020, the
M. E. Nobre (*)
Fluminense Federal University, Rio de Janeiro, Brazil
Switzerland AG 2026
D. Uludüz et al. (eds.), Rare Causes of Headache Disorders, Headache,
https://doi.org/10.1007/978-3-032-10242-3_29
273© The Author(s), under exclusive license to Springer Nature
274
M. E. Nobre
International Amyloidosis Society identied 36 amyloid proteins that can cause disease in humans, six of which are associated with brain disease, either exclusively or
as part of systemic involvement. CAA primarily affects small arteries and arterioles,
especially in the cortex and leptomeninges. The deposition of beta-amyloid causes
age-related sporadic CAA, while early-onset CAA occurs before age 50 and may
include rare monogenic forms [2].
Amyloid deposition damages vessel walls, leading to thickening, hyalinization,
and smooth muscle cell loss. This ultimately causes parenchymal brain injury due
to both bleeding and ischemia. Sporadic beta-amyloid CAA is the most common
form of cerebrovascular amyloidosis, primarily associated with brain hemorrhage
and cognitive impairment, either occurring independently or alongside Alzheimer’s
disease. The distribution of sporadic amyloid-β CAA is predominantly posterior,
with the occipital lobe most affected [2, 3].
CAA is a signicant cause of intracerebral hemorrhage (ICH) in the elderly and
can also lead to transient focal neurological episodes (TFNE). TFNE presents as
recurrent, stereotyped attacks linked to cortical supercial siderosis (cSS). The denitions of TFNE and migraine aura—described as positive, reversible neurological
symptoms following a sequential pattern according to the third edition of
International Classication of Headache Disorders (ICHD-3)—overlap. The relationship between migraine and cerebrovascular disease has long been recognized,
but the underlying pathophysiological basis remains unclear [4].
29.3 Case Presentation
J.S. is a 72-year-old male engineer with a history of hypertension, hyperlipidemia,
and mild cognitive impairment who presented to the emergency department with
complaints of visual disturbances and transient episodes of right-sided weakness
occurring over the past few weeks. He described episodes lasting approximately
20–30min, beginning with ickering lights in his left visual eld that gradually
progressed to blurry vision. These visual symptoms were sometimes followed by
numbness and tingling in his right hand, which resolved once the visual disturbances subsided. He also reported mild frontotemporal pulsatile headache and photophobia, without accompanying nausea, which responded well to paracetamol.
These episodes had occurred four times over the past month, with increasing
frequency.
Neurological examination revealed that he was alert and fully oriented, with no
focal neurological decits identied. Brain magnetic resonance imaging (MRI)
showed cortical and subcortical microbleeds predominantly in the occipital and
parietal lobes, along with evidence of supercial siderosis. No signs of acute infarction or hemorrhage were detected. Routine blood work, including coagulation prole and metabolic panel, returned within normal limits.
Given the imaging ndings, the patient’s age, and cognitive history, a diagnosis
of cerebral amyloid angiopathy (CAA) was considered. The nature of his transient