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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5526_Библиотеки_им_академика_М_И_Перельмана.pdf
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- •Foreword
- •Contents
- •1.1 Introduction
- •1.2 Pathophysiology
- •1.3 Case Presentation
- •1.4 Case Discussion
- •1.5 Clinical Characteristics
- •1.6 Diagnostic Algorithm
- •1.8 Management
- •1.9 Conclusion
- •References
- •2.1 Introduction
- •2.2 Pathophysiology
- •2.3 Case Presentation
- •2.4 Case Discussion
- •2.5 Clinical Characteristics
- •1.7 Differential Diagnosis
- •2.6 Diagnostic Algorithm
- •2.7 Management
- •2.8 Conclusion
- •References
- •3.1 Introduction
- •3.2 Pathophysiology
- •3.3 Case Presentation
- •3.4 Case Discussion
- •3.5 Clinical Characteristics
- •3.6 Diagnostic Algorithm
- •3.7 Management
- •3.8 Conclusion
- •References
- •4.1 Introduction
- •4.2 Pathophysiology
- •4.3 Case Presentation
- •4.4 Case Discussion
- •4.6 Diagnostic Algorithm
- •4.7 Management
- •4.8 Conclusion
- •References
- •5.1 Introduction
- •5.2 Pathophysiology
- •5.3 Case Presentation
- •5.4 Case Discussion
- •5.5 Diagnostic Algorithm
- •5.6 Management
- •5.7 Conclusion
- •References
- •6.1 Introduction
- •6.2 Pathogenesis
- •6.3 Case Presentation
- •6.4 Case Discussion
- •6.5 Diagnostic Algorithm
- •6.6 Management
- •6.7 Conclusion
- •References
- •7.1 Introduction
- •7.2 Pathophysiology
- •7.3 Case Presentation
- •7.5 Differential Diagnosis
- •7.7 The Following Strategies Are Essential
- •7.7.1 Acute Symptom Relief
- •7.7.1.1 Pharmacological Treatment
- •7.7.2.1 Pharmacologic Prophylaxis
- •7.8 Conclusion
- •References
- •8.1 Introduction
- •8.3 Case Study
- •8.4 Case Discussion
- •8.5 Clinical Management
- •8.7 Diagnosis
- •8.8 Treatment
- •8.9 Conclusion
- •References
- •9.1 Introduction
- •9.2 Case Presentation
- •9.4 Diagnosis Algorithm
- •9.5 Secondary SUNCT
- •9.6 Management
- •9.8 Conclusion
- •References
- •10.1 Introduction
- •10.2 Pathophysiology
- •10.3 Case Presentation
- •10.4 Case Discussion
- •10.5 Clinical Characteristics
- •10.6 Diagnostic Algorithm
- •10.7 Management
- •10.8 Conclusion
- •References
- •11.1 Introduction
- •11.2 Pathophysiology
- •11.3 Case Presentation
- •11.4 Case Discussion
- •11.5 Clinical Characteristics
- •11.6 Diagnostic Algorithm
- •11.6.1 Step 1: Detailed Patient History
- •11.8 Management
- •11.9 Conclusions
- •12.2 Pathophysiology
- •12.3 Case Presentation
- •12.4 Case Discussion
- •12.6 Treatment
- •12.7 Conclusion
- •References
- •References
- •12.1 Introduction
- •13.1 Introduction
- •13.2 Pathophysiology
- •13.3 Case Presentation
- •13.4 Case Discussion
- •13.5 Clinical Characteristics
- •13.6 Diagnostic Algorithm
- •13.7 Management
- •13.8 Conclusion
- •References
- •14.1 Introduction
- •14.2 Pathophysiology
- •14.3 Case Presentation
- •14.3.1 Clinical Case 1
- •14.3.2 Clinical Case 2
- •14.4 Case Discussion
- •14.5 Clinical Characteristics
- •14.7 Treatment/Management
- •14.8 Conclusion
- •References
- •15.1 Introduction
- •15.2 Case Presentation
- •15.3 Case Discussion
- •15.4 Diagnostic Algorithm
- •15.5 Pathophysiology
- •15.6 Clinical Presentation
- •15.6.1 External-Compression Headache (ECH)
- •15.6.2 External-Traction Headache (ETH)
- •15.7 Management
- •15.7.1 Nonpharmacological Strategies
- •15.7.2 Pharmacological Strategies
- •15.7.3 Patient Education and Awareness
- •15.8 Conclusion
- •References
- •16.1 Introduction
- •16.2 Pathophysiology
- •16.3 Case Presentation
- •16.4 Case Discussion
- •16.6 Diagnostic Algorithm
- •16.7 Management
- •16.8 Conclusion
- •References
- •17.1 Introduction
- •17.2 Pathophysiology
- •17.3 Case Presentation
- •17.4 Case Discussion
- •17.5 Clinical Characteristics
- •17.6 Diagnosis
- •17.7 Differential Diagnosis
- •17.8 Treatment
- •17.9 Conclusion
- •References
- •18.1 Introduction
- •18.2 Pathophysiology
- •18.3 Case Presentation
- •18.4 Case Discussion
- •18.5 Clinical Presentation
- •18.6 Diagnosis
- •18.7 Differential Diagnosis
- •18.8 Treatment
- •18.9 Conclusion
- •References
- •19.1 Introduction
- •19.2 Pathophysiology
- •19.3 Case Presentation
- •19.4 Case Discussion
- •19.5 Diagnostic Approach
- •19.6 Management
- •19.7 Conclusion
- •References
- •20.1 Introduction
- •20.3 Case Report
- •20.4 Case Discussion
- •20.6 Clinical Presentation
- •20.7 Diagnostic Algorithm
- •20.8 Conclusion
- •References
- •21.1 Introduction
- •21.2 Case Presentation
- •21.3 Clinical Characteristics
- •21.4 Diagnosis
- •21.5 Treatment
- •References
- •22.1 Introduction
- •22.3 Case Presentation 1
- •22.4 Case Discussion
- •22.5 Case Presentation 2
- •22.6 Case Discussion 2
- •22.7 Clinical Characteristics
- •22.8 Diagnostic Workup
- •22.9 Treatment
- •22.10 Prognosis
- •References
- •23.1 Introduction
- •23.2 Pathophysiology
- •23.3 Case Presentation
- •23.4 Case Discussion
- •23.6 Diagnostic Algorithm
- •23.7 Management
- •23.8 Conclusion
- •References
- •24.1 Introduction
- •24.2 Case Presentation
- •24.3 Case Discussion
- •24.4 Pathophysiology
- •24.6 Clinical Characteristics
- •24.8 Treatment Approaches
- •24.10 Conclusion
- •References
- •25.1 Introduction
- •25.2 Case Presentation
- •25.3 Case Discussion
- •25.4 Conclusion
- •References
- •26.1 Introduction
- •26.2 Pathophysiology
- •26.3 Case Presentation
- •26.4 Case Discussion
- •26.5 Clinical Characteristics
- •26.6 Diagnostic Algorithm
- •26.7 Management
- •26.8 Conclusion
- •References
- •27.1 Introduction
- •27.2 Case Presentations
- •27.3 Clinical Characteristics
- •27.4 Discussion
- •27.5 Conclusion
- •References
- •28.1 Introduction
- •28.2 Case Presentation
- •28.3 Case Discussion
- •28.4 Clinical Characteristics
- •28.5 Diagnosis
- •28.6 Conclusion
- •28.7 Key Messages
- •References
- •29.1 Introduction
- •29.2 Pathophysiology
- •29.3 Case Presentation
- •29.4 Clinical Presentation
- •29.5 Diagnosis
- •29.6 Treatment
- •29.7 Conclusion
- •References
- •30.1 Introduction
- •30.2 Clinical Case
- •30.3 Clinical Presentation
- •30.4 Differential Diagnosis
- •30.5 Diagnosis
- •30.6 Treatment
- •30.7 Conclusion
- •References
- •31.1 Introduction
- •31.2 Pathophysiology
- •31.3 Case Presentation
- •31.4 Case Discussion
- •31.5 Clinical Presentation
- •31.7 Conclusion
- •References
- •32.1 Introduction
- •32.2 Pathophysiology
- •32.3 Case Presentation
- •32.4 Case Discussion
- •32.6 Diagnosis
- •32.7 Additional Diagnostic Evaluations
- •32.8 Apply ICHD-3 Diagnostic Criteria [9]
- •32.10 Management
- •32.11 Conclusion
- •References
- •33.1 Introduction
- •33.2 Pathophysiology
- •33.3 Case Presentation
- •33.4 Clinical Characteristics
- •33.5 Diagnostic Algorithm
- •33.6 Treatment
- •33.7 Conclusion
- •References
- •34.1 Introduction
- •34.2 Pathophysiology
- •34.3 Case Presentation
- •34.4 Case Discussion
- •34.6 Diagnostic Algorithm
- •34.7 Treatment
- •34.8 Conclusion
- •References
- •35.1 Introduction
- •35.3 Case Presentation
- •35.4 Case Discussion
- •35.7 Treatment
- •35.7.1 Oxygen Therapy (100% Oxygen)
- •35.8 Conclusion
- •References
- •36.1 Introduction
- •36.2 Pathophysiology
- •36.3 Case Presentation
- •36.5 Diagnostic Algorithm
- •36.6 Treatment
- •36.7 Conclusion
- •References
- •37.1 Introduction
- •37.2 Pathophysiology
- •37.3 Case Presentation
- •37.4 Headache Characteristics
- •37.5 Case Discussion
- •37.6 Treatment
- •37.7 Conclusion
- •References
- •38.1 Introduction
- •38.2 Pathophysiology
- •38.3 Case Presentation
- •38.4 Clinical Presentation
- •38.5 Diagnostic Algorithm
- •38.6 Treatment
- •38.7 Conclusion
- •References
- •39.1 Introduction
- •39.3 Case Presentation
- •39.4 Case Discussion
- •39.6 ICHD-3 Diagnostic Criteria [28]
- •39.6.1 Diagnostic Criteria
- •39.7 Diagnostic Algorithm
- •39.9 Conclusion
- •References
- •40.1 Introduction
- •40.3 Case Presentation
- •40.4 Case Discussion
- •40.5.1 Diagnostic Algorithm
- •40.6 Treatment
- •40.7 Conclusion
- •References
- •41.1 Introduction
- •41.3 Case Presentation
- •41.4 Clinical Presentation
- •41.5 Differential Diagnosis
- •41.6 Conclusion
- •41.7 Key Messages
- •References
- •42.1 Introduction
- •42.2 Pathophysiology
- •42.3 Case Presentation
- •42.5 Case Discussion
- •42.6 Clinical Presentation
- •42.7 Diagnostic Algorithm [9]
- •42.8 Preeclampsia
- •42.9 Eclampsia
- •42.10 Fetal Assessment
- •42.11 Treatment
- •42.12 Antihypertensive Management [8]
- •42.14 Conclusion
- •References
- •43.1 Introduction
- •43.2 Pathophysiology
- •43.3 Case Presentation
- •43.4 Case Discussion
- •43.5 Clinical Manifestations
- •43.6 Diagnosis
- •43.7 Treatment
- •43.8 Conclusion
- •References
- •44.1 Introduction
- •44.2 Pathophysiology
- •44.3 Case Presentation
- •44.4 Case Discussion
- •44.6 Diagnostic Approach
- •44.7 Management
- •44.8 Conclusion
- •References
- •45.1 Introduction
- •45.2 Pathophysiology
- •45.3 Case Presentation
- •45.6 Treatment
- •45.7 Conclusion
- •References
- •46.1 Introduction
- •46.2 Pathophysiology
- •46.3 Case Presentation
- •46.4 Clinical Characteristics
- •46.5 Differential Diagnosis
- •46.6 Treatment
- •46.7 Conclusion
- •References
- •47.1 Introduction
- •47.2 Pathophysiology
- •47.3 Case Presentation
- •47.4 Case Discussion
- •47.5 Clinical Presentations
- •47.6 Diagnostic Algorithm
- •47.7 Differential Diagnosis
- •47.8 Treatment
- •47.9 Conclusion
- •References
- •48.1 Introduction
- •48.2 Pathophysiology
- •48.3 Case Presentation
- •48.4 Case Discussion
- •48.5 Clinical Characteristics
- •48.7 Treatment
- •48.8 Conclusion
- •References
- •49.1 Introduction
- •49.2 Pathophysiology
- •49.3 Case Presentation
- •49.4 Clinical Presentation
- •49.5 Diagnosis
- •49.6 Treatment
- •49.7 Conclusion
- •References
- •50.1 Introduction
- •50.2 Pathophysiology
- •50.3 Case Presentation
- •50.4 Case Discussion
- •50.5 Clinical Characteristics
- •50.6 Diagnosis
- •50.7 Treatment
- •50.8 Conclusion
- •References
- •51.1 Introduction
- •51.2 Case Presentation
- •51.3 Clinical Characteristics
- •51.4 Diagnosis
- •51.5 Treatment
- •51.6 Conclusion
- •References
- •52.1 Introduction
- •52.2 Pathophysiology
- •52.3 Case Presentation
- •52.4 Case Discussion
- •52.5 Clinical Characteristics
- •52.6 Diagnosis
- •52.6.1 Cervicogenic Headache
- •52.6.2 Migraine
- •52.6.3 Neck Pain
- •52.6.4 Demyelinating Lesions
- •52.6.5 Cervical Myelitis
- •52.6.6 Occipital Allodynia
- •52.6.7 Cervical Muscle Spasms
- •52.7 Treatment
- •52.7.2 Acupuncture
- •52.7.3 Transcutaneous Electrical Nerve Stimulations (TENS)
- •52.8 Minimally Invasive Treatment
- •52.8.1 Nerve Blocks
- •52.8.2 Botulinum Toxin A
- •52.8.3 Radio Frequency
- •52.8.4 Occipital Nerve Stimulation
- •52.9 Surgical Treatments
- •52.10 Conclusions
- •References
- •53.1 Introduction
- •53.2 Pathophysiology
- •53.3 Characteristics of Pain
- •53.4 Case Presentation
- •53.5 Case Discussion
- •53.6 Clinical Characteristics
- •53.8 Treatment
- •53.9 Conclusion
- •References
- •54.1 Introduction
- •54.2 Pathophysiology
- •54.3 Case Presentation
- •54.4 Case Discussion
- •54.5 Clinical Characteristics
- •54.6 Diagnostic Algorithm
- •54.7 Management
- •54.8 Conclusion
- •References
- •55.1 Introduction
- •55.2 Pathophysiology
- •55.3 Case Presentation

10
E. J. Piovesan and M. A. T. Utiumi
Table 1.1
a
weakness
b
for weeks
Diagnostic criteria for the diagnosis of hemiplegic migraine [27]
1.2.3 Hemiplegic migraine
A.Attacks fullling criteria for 1.2 Migraine with aura and criterion B below
B.Aura consisting of both of the following:
C.Fully reversible motor weakness
D.Fully reversible visual, sensory, and/or speech/language symptoms
1.2.3.1 Familial hemiplegic migraine
A.Attacks fullling criteria for 1.2.3 Hemiplegic migraine
B. At least one rst- or second-degree relative has had attacks fullling criteria for 1.2.3
Hemiplegic migraine
1.2.3.2 Sporadic hemiplegic migraine
A.Attacks fullling criteria for 1.2.3 Hemiplegic migraine
B.No rst- or second-degree relative fulls criteria for 1.2.3 Hemiplegic migraine
The term plegic means paralysis in most languages, but most attacks are characterized by motor
Motor symptoms generally last less than 72h but, in some patients, motor weakness may persist
a,b
Step 5: Apply the International Classication of Headache Disorders, third edition
(ICHD-3) Diagnostic Criteria (Table 1.1)
Step 6: Conrm diagnosis
• Conrm hemiplegic migraine:
– CACNA1A (FHM1), ATP1A2 (FHM2), SCN1A (FHM3), or PRRT2
(other types) gene mutations or sporadic types.
Step 7: Classication re-evaluation
• Reassess headache classication for other secondary types of headache.
– See below (Sect. “Differential Diagnosis”).
1.7 Differential Diagnosis
Differential diagnosis is essential in patients with no affected relatives (sporadic
forms) or when a reliable family history is lacking. The type and magnitude of HM
symptoms make ruling out other signicant disorders necessary, including diseases
such as cerebrovascular events, infectious conditions, inammatory diseases, epilepsy with hemiparesis, and tumors [28].
In cases of transient ischemic attacks (TIA) and strokes, the condition is sudden,
while in HM, the neurological symptoms evolve gradually [1]. Motor aura may start
in the hand, spread to the arm, and nally reach the face. The manifestation is sudden in all regions in neurovascular conditions. Headaches precede weakness in
cases of vascular syndromes, while they occur closer in time in cases of HM [29].

1 Hemiplegic Migraine
11
Headache is rare in cases of TIA but common in patients with hemorrhagic stroke
and young patients with HM [29].
Other vascular disorders discussed elsewhere include hereditary cerebral angiopathy [30] and cerebral autosomal dominant arteriopathy with subcortical infarcts
(CADASIL).
Seizures followed by Todd’s syndrome (motor weakness secondary to a seizure)
can be confused with motor auras [1, 30]. Approximately 7% of those with FHM
may also have epilepsy, which often makes the differential diagnosis challenging
[26]. HM motor symptoms can last from 30min to days, while seizures usually last
only a few minutes. The typical motor patterns of seizures include limb spasms,
head-turning, and loss of consciousness at their onset [2]. Todd’s syndrome can be
as long-lasting as HM, and this differential diagnosis is often complex.
In infectious cases, the patient typically presents with fever and some degree of
altered mental status. Differential diagnosis with hypo- or hyperactive delirium of
infectious origin may be necessary. The diagnostic workup includes neuroimaging
to rule out structural lesions and the analysis of the cerebrospinal uid [31] when
the condition is unclear.
Stroke-like migraine attacks after radiation therapy (SMART) syndrome is a rare
condition that may occur in patients with a history of brain radiotherapy 20–30years
before the onset of symptoms. Imaging tests may show thick cortical gyral enhancement [32].
Alternating hemiplegia of childhood is a rare genetic disorder of childhood
caused by mutations in the ATP1A3 gene that produces periodic episodes of
hemi- or quadriplegia, associated with dystonia, epilepsy, and cognitive alterations [33].
HaNDL is a rare sporadic condition characterized by hemiparesis and aphasia,
associated with changes in cerebral blood ow and an increase in the lymphocyte
count in the cerebrospinal uid [34]. This condition can be related to FHM1 [23].
However, HaNDL is monophasic and lasts up to 3months, while FHM recurs for
years and is associated with frequent visual symptoms [
17].
Mitochondrial disorders [35] can present with recurrent migraine-like headaches, neurological decits, neuroimaging, and genetic ndings. Sturge-Weber disorder may manifest with symptoms like HM, although typical dermatological
changes help differentiate the disease [36]. Hypercapnia, hyponatremia, hypocalcemia, hepatic and renal failure, carotid dissection, antiphospholipid syndrome, systemic lupus erythematosus, and ornithine transcarbamylase deciency can all
present with symptoms that suggest HM [13].
1.8 Management
Non-pharmacological treatment begins by avoiding exposure to potentially seizuregenerating factors, including pharmacological substances. The triggering factors
can be emotional and intense physical stress, viral infections, and head trauma [37].

12
E. J. Piovesan and M. A. T. Utiumi
In isolated reports, the use of vasoconstrictive drugs (ergotamine and dihydroergotamine) in the acute phase of the disease can exacerbate the aura [30]. During the
interictal periods, the use of topiramate [38] and glucagon-like peptide-1 (GLP-1)
agonist (liraglutide) [39] can also worsen the HM evolution. An isolated case of
regadenoson use, a selective A2A adenosine receptor agonist, triggering HM was
reported [40].
In the acute phase with altered consciousness (brain edema), we favor a more
aggressive intervention. Magnesium sulfate 2g intravenous (IV) every 8h (3 doses)
and dexamethasone were administered, followed by 0.1mg/kg IV every 6h for
5days, resulting in complete improvement [41]. In cases of cerebral edema, we may
use dexamethasone 0.5mg/kg/day IV in 3 pulses/day for 3days, followed by gradual oral tapering and hypertonic solution at 3% 1.5 mL/kg/h, maintaining Na+
between 145 and 155mEq/L [42].
In acute cases without altered consciousness, triptans can produce good or excellent symptom control in up to 62% of cases [43]. Nasal administration of ketamine
reduced the duration of aura in those with FHM by 42% [44]. Verapamil 5mg IV
over 5min, followed by an oral maintenance dose of 120mg/day, reduced headache
symptoms but had little impact on hemiplegia [45].
Some oral drugs can be used for prophylactic management. Verapamil 120mg,
administered two to three times a day [24, 46, 47], reduced the intensity and impact
of HM.Acetazolamide, 250–500mg, reduces the burden of attacks and nystagmus
and is primarily used in cases related to the CACNA1A gene [22, 38]. Flunarizine
10mg daily [48], lamotrigine [42], and propranolol 10mg three to four times a day
[46] have been tried. Memantine 10mg twice daily and dextromethorphan have
been primarily used in individuals with the ATP1A2 variant [49]. Telcagepant [50]
showed some therapeutic effects.
Botulinum neurotoxin type A utilizing the PREEMPT protocol, has shown
improvement in patients with HM [51]. The use of CGRP (calcitonin gene-related
peptide) monoclonal antibodies such as galcanezumab in a family with PRRT2
mutation caused a >50% reduction of auras and pain frequency, analgesics consumption, and functional improvement. In another study, administering galcanezumab for 3months in six patients reduced the severity of the attacks to moderate
in half of them, motor weakness in four, and the MIDAS (Migraine Disability
Assessment Test) score improved in ve. There was no change in the number of
days with headache and the characteristics of aura [
52].
Gepants, small molecules with CGRP receptor antagonism activity, should
be avoided during HM attacks due to the risk of worsening cerebral vasospasm
[53]. Promising treatments, such as foramen ovale closure, have been described
in ve different cases, potentially providing control over HM [54]. No studies
have evaluated the presence of patent foramen ovale in groups of patients with
HM to date.

1 Hemiplegic Migraine
13
1.9 Conclusion
Hemiplegic migraine is a rare disorder whose main clinical feature is the presence
of motor symptoms frequently associated with other types of aura. The differential
diagnosis, including stroke, epilepsy, and other conditions, is an important step.
Because it is a disorder associated with ion channel disturbances, cortical spreading
depression is the primary pathophysiological mechanism. Symptoms can be as
severe as mental confusion, coma, and seizures. Acute treatment includes calcium
channel blockers, but prophylaxis is limited. Natural history consists of a typical
dramatic phase during the rst few years of life, with a favorable course afterward.
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Chapter 2
Migrainous Status
MauroEduardoJurno andLigiaBarrosde Oliveira
2.1 Introduction
Migrainous status is one of the most debilitating complications of migraine, characterized by a migraine attack lasting more than 72h, with persistent and uncontrollable symptoms resulting in signicant functional incapacitation. Although
recognized by the International Classication of Headache Disorders (ICHD-3;
code 1.4.1. Migrainous Status) [1], its pathophysiology and epidemiology remain
poorly understood, with signicant gaps in its investigation and effective treatment [2, 3].
This chapter aims to discuss status migrainosus, using a clinical case observed by
the authors as the foundation for the discussion.
2.2 Pathophysiology
The exact pathophysiology of migrainous status is not fully understood, but it shares
several characteristics with common migraine, such as activation of the trigeminovascular system and central and peripheral sensitization [4].
Evidence suggests that migrainous status may have a biological basis distinct
from common migraine, representing a more refractory and prolonged state that
does not respond well to conventional therapies. Genetic studies indicate that specic genetic variants may be associated with a higher susceptibility to more severe
and prolonged migraine attacks [4].
M. E. Jurno (*) · L. B. de Oliveira
Barbacena School of Medicine– FUNJOB and Minas Gerais State Hospital Foundation–
FHEMIG, Barbacena, 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_2
17© The Author(s), under exclusive license to Springer Nature

18
Furthermore, some research indicates that migrainous status may cause reversible structural changes in the brain, such as vasogenic edema and hypoperfusion,
suggesting deeper involvement than typical migraine. However, such ndings are
still rare and require validation through larger, controlled studies [4].
M. E. Jurno and L. B. de Oliveira
2.3 Case Presentation
The patient, a female, 35years old, reports having experienced a severe unilateral
headache on the right side 4days before admission. The headache was characterized as pulsatile, accompanied by intense nausea, photophobia, and phonophobia.
She denies having osmophobia. The episode began similarly to her usual migraine
attacks. Still, this time the pain persisted for over 72h, continuously, despite using
her routine medications, which include sumatriptan and non-steroidal antiinammatory drugs (NSAIDs). She also took dipyrone and rested in a dark place,
but found no relief from her painful symptoms and general malaise.
The patient notes that before the current episode, she had been experiencing
episodic headaches two to three times a month, always triggered by stress and sleep
deprivation. Typically, her episodes lasted between 12 and 24h, but they had never
exceeded 48h. In the last 24h, she reported increasing difculty sleeping due to
persistent pain and nausea, having vomited twice.
The patient has been diagnosed with migraine without aura since the age of 25.
She reports that her most common triggers are stress, irregular sleep, and the menstrual period. She has no other signicant clinical comorbidities and denies chronic
medication use except for migraine control. Patient’s mother has a history of
migraine, controlled with preventive medications.
On physical examination, the patient was afebrile, with a blood pressure of
120/80mmHg and a heart rate of 76bpm. She appeared lucid and oriented but was
visibly distressed, holding her head and adopting a posture suggestive of signicant
discomfort. Neurological examination revealed no focal decits, and fundoscopy
showed no abnormalities. There were no signs of neck stiffness or other meningeal
irritation.
Based on the report of severe headache persisting for over 72h with inadequate
response to usual treatment, the probable diagnosis is Migrainous Status. This condition is exacerbated by associated vomiting and other debilitating symptoms, characterizing a medical emergency.
2.4 Case Discussion
This is a typical case of status migrainosus in a patient with a history of migraine,
triggered by stress and sleep deprivation. The progression to a prolonged crisis
unresponsive to standard medications justies the need for a more aggressive

2 Migrainous Status
19
approach, including intravenous (IV) therapies and hospital observation. Proper
management can prevent complications such as the progression to chronic
migraine and reduce the severe functional impact associated with the condition
[3–7]. Given the refractoriness of the headache to standard treatments and its prolonged duration, a more intensive management strategy in a hospital setting was
warranted. The proposed plan included intravenous hydration with a saline solution to address potential electrolyte imbalances and dehydration resulting from
vomiting. Dexamethasone (8mg IV) was administered to reduce neuroinammation and lower the risk of recurrence. Metoclopramide (10mg IV) was used to
manage associated nausea and vomiting. Intravenous magnesium sulfate was
added for its known prophylactic and crisis-modulating effects in refractory
migraine. Additionally, an anticonvulsant such as intravenous valproic acid was
considered due to its reported efcacy in treatment-resistant attacks. The patient
was admitted for observation over the rst 24h to monitor clinical response and
allow for timely adjustment of therapy as needed. With appropriate treatment, it is
expected that the patient will show improvement within 24–48h, with a gradual
resolution of symptoms. However, a subsequent evaluation should be considered
to review long-term preventive medication and discuss the management of comorbid conditions.
2.5 Clinical Characteristics
Epidemiological studies indicate that migrainous status is relatively uncommon,
although it is often underreported. A survey conducted in Olmsted County,
Minnesota, identied an age- and sex-adjusted incidence rate of 26.6 per 100,000
individuals per year, with a peak between the ages of 40 and 49, and a predominance
among women.
Other studies suggest that between 3% and 20% of individuals with migraine
may experience migrainous status at some point [5].
Risk factors for developing this condition include excessive use of pain medications during attacks, sleep disturbances, stress, and lack of effective treatment for
migraine attacks. One of the primary triggers includes irregular sleep patterns, both
excess and deprivation, which are strongly associated with the risk of migrainous
status recurrence. Patients with episodic migraine are also more likely to progress to
chronic migraine if they experience recurrent migrainous status.
Additionally, migraine attacks resistant to acute pharmacological therapy
increase the likelihood of complications, such as progression to chronic migraine
and the development of medication dependency [6].
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