Headache and Facial Pain 3 Flashcards
Secondary headaches
1) Meningitis
2) Sinus headache
3) Ocular causes of headache (e.g. acute glaucoma)
4) Hypertension
5) Subarachnoid hemorrhage
6) Brain tumor
7) Cerebral venous sinus thrombosis
8) Idiopathic intracranial hypertension
9) Intracranial hypotension
10) Giant cell arteritis
11) Exertional headache
12) Sexually induced headache
13) Cardiac cephalalgia
14) Carotid or vertebral dissection & carotidynia
15) Cold stimulus headache
16) Headaches associated with sleep (hypnic headache, headache associated with sleep apnea, exploding head syndrome)
Cerebral venous sinus thrombosis: Mechanisms and clinical findings
Symptoms and signs of CVT can be grouped into three major syndromes:
●Isolated intracranial hypertension syndrome (headache with or without vomiting, papilledema, and visual problems)
●Focal syndrome (focal deficits, seizures, or both)
●Encephalopathy (multifocal signs, mental status changes, stupor, or coma)
Less common presentations include cavernous sinus syndrome, subarachnoid hemorrhage, and multiple cranial nerve palsies
Cerebral venous sinus thrombosis: Transient Risk factors
1) Infection
Central nervous system
Ear, sinus, mouth, face, and neck
Systemic infectious disease
2) Pregnancy and puerperium
3) Dehydration
4) Mechanical precipitants
Head injury
Lumbar puncture
Neurosurgical procedures
Jugular catheter occlusion
5) Drugs
Oral contraceptives
Hormone replacement therapy
Androgens
Asparaginase
Tamoxifen
Glucocorticoids
VITT - after vaccine for COVID 19
Cerebral venous sinus thrombosis: Permanent Risk factors
1) Inflammatory diseases
Systemic lupus erythematosus
Behçet disease
Granulomatosis with polyangiitis
Thromboangiitis obliterans
Inflammatory bowel disease
Sarcoidosis
2) Malignancy
Central nervous system
Solid tumour outside central nervous system
Hematologic
3) Hematologic condition
Prothrombotic states, genetic or acquired
Protein C deficiency
Protein S deficiency
Antithrombin deficiency
Factor V Leiden mutation
G20210A prothrombin gene mutation
Antiphospholipid syndrome
Myeloproliferative neoplasms
Nephrotic syndrome
Paroxysmal nocturnal hemoglobinuria
Hyperhomocysteinemia
Polycythemia, thrombocythemia
Severe anemia, including paroxysmal nocturnal hemoglobinuria
4) Central nervous system disorders
Dural fistulae
5) Other disorders
Congenital heart disease
Thyroid disease
Obesity!
Characteristics of headache in CVT
- Head pain may be localized or diffuse.
- It is typically characterized by severe head pain that worsens with Valsalva maneuvers and with recumbency.
- Headache onset with CVT is usually gradual, increasing over several days.
However, some patients with CVT have sudden explosive onset of severe head pain (ie, thunderclap headache) that mimics subarachnoid hemorrhage - Headache due to CVT may also resemble migraine with aura
Which condition must be excluded in persisting headache after LP?
CVT must be included as a possible cause of persisting headache following lumbar puncture, because lumbar puncture can rarely precipitate a CVT
CVT neuroimaging
- focal areas of edema
- venous infarction
- hemorrhagic venous infarction
- diffuse brain edema
- isolated subarachnoid hemorrhage (rarely)
In a minority of cases, CT may demonstrate direct signs of CVT, which include the dense triangle sign, the empty delta sign, and the cord sign
Brain MRI in combination with magnetic resonance venography is the most informative technique for demonstrating the presence of dural thrombus, cortical vein thrombosis, and extent of brain injury
Contrast-enhanced MRV, gradient-recalled echo, or susceptibility-weighted imaging sequences are the recommended techniques for the diagnosis of cortical venous thrombosis (AHA 2024)
In which patients should CVT be suspected?
The diagnosis of CVT should be suspected in patients who present with one or more of the following:
●New-onset headache
●Headache with features that differ from the usual pattern (eg, progression or change in attack frequency, severity, or clinical features) in patients with a previous primary headache
●Symptoms or signs of intracranial hypertension
●Encephalopathy
●Focal neurologic symptoms and signs, especially those not fitting a specific vascular distribution or those involving multiple vascular territories
●Seizures
In addition, the diagnosis of CVT should be suspected in patients who have atypical neuroimaging features on routine CT or MRI at presentation, such as cerebral infarction that crosses typical arterial boundaries, hemorrhagic infarction, or lobar intracerebral hemorrhage of otherwise unclear origin.
In any of these scenarios, suspicion for CVT should be particularly high for patients with known risk factors, including prothrombotic conditions, oral contraceptive use, pregnancy and the puerperium, malignancy, infection, and head injury, even if the initial neuroimaging study (most often a CT) is normal.
A) Direct and B) Indirect signs of CVT in CT scan
A) In about one-third of cases, CT demonstrates direct signs of CVT, including the following
●The cord sign is a curvilinear or linear hyperdensity from a thrombosed cortical vein typically found over the cerebral cortex.
●The dense triangle sign is a triangular or round hyperdensity reflecting a thrombosed sinus on a cross-section view.
●The empty delta sign (also called the empty triangle or negative delta sign) is a triangular pattern of contrast enhancement surrounding a central region without contrast enhancement found in the posterior part of the superior sagittal sinus on head CT performed with contrast.
B) Indirect signs of CVT on head CT are more frequent.
These can include intense contrast enhancement of falx (δρέπανο) and tentorium (σκηνίδιο), dilated transcerebral veins, small ventricles, and parenchyma abnormalities.
In addition, associated brain lesions may be depicted in 60 to 80 percent of patients with CVT. These may be hemorrhagic or nonhemorrhagic:
●Hemorrhagic lesions include intracerebral hemorrhage, hemorrhagic infarcts, or rarely (<1 percent) subarachnoid hemorrhage usually limited to the convexity
●Nonhemorrhagic lesions include focal areas of hypodensity caused by vasogenic edema or venous infarction, usually not respecting the arterial boundaries, as well as diffuse brain edema.
With serial imaging, some lesions may disappear (“vanishing infarcts”), and new lesions may appear.
MRI signs of CVT
MRI using gradient echo T2* susceptibility-weighted sequences in combination with MR venography is the most sensitive imaging method for demonstrating the thrombus and the occluded dural sinus or vein
The characteristics of the MRI signal depend on the age of the thrombus:
●In the first five days, the thrombosed sinuses appear isointense on T1-weighted images and hypointense on T2-weighted images.
●Beyond five days, venous thrombus becomes more apparent because signal is increased on both T1- and T2-weighted images.
●After the first month, thrombosed sinuses exhibit a variable pattern of signal, which may appear isointense.
On T2-weighted gradient echo and T2 susceptibility-weighted MRI sequences, the acute thrombus can be directly visualized as an area of hypointensity in the engorged sinus or cortical vein.
In addition, a chronically thrombosed sinus may also demonstrate low signal on these sequences.
Parenchymal brain lesions secondary to venous occlusion, including brain swelling, vasogenic edema, or venous infarction, are hypointense or isointense on T1-weighted MRI and hyperintense on T2-weighted MRI. Venous congestion may show reversible reduced diffusivity on diffusion-weighted MRI sequences.
D-dimers in CVT
An elevated plasma D-dimer level supports the diagnosis of CVT, but a normal D-dimer does not exclude the diagnosis in patients with suggestive symptoms and predisposing factors.
CSF abnormalities in CVT
The CSF abnormalities in CVT are nonspecific and may include a lymphocytic pleocytosis, elevated red blood cell count, and elevated protein; these abnormalities are present in 30 to 50 percent of patients with CVT
lumbar puncture is valuable in such patients to measure and decrease CSF pressure when vision is threatened.
CVT: Acute Management
Acute anticoagulation
initial anticoagulation therapy with subcutaneous low molecular weight heparin or intravenous heparin
Management of acute complications
Complications that require intervention during the acute phase of CVT include elevated intracranial pressure, brain swelling, seizures, and infection.
*Measures to control acutely increased intracranial pressure and impending herniation, including decompressive surgery, may be required in patients with CVT.
*For patients with CVT who have both seizures at presentation and focal cerebral supratentorial lesions (eg, edema, infarction, or hemorrhage on admission computed tomography or magnetic resonance imaging), seizure prophylaxis with an antiseizure medication is recommended
For patients with a single early symptomatic seizure due to CVT in the absence of a supratentorial cerebral lesion, the benefit of seizure prophylaxis is uncertain due to low likelihood of recurrence
*Antibiotic treatment is mandatory whenever there is meningitis or other intracranial infection or an infection of a neighboring structure, such as otitis or mastoiditis.
++ Given the lack of controlled studies (and poorer outcomes in meta-analyses), endovascular therapies (mechanical thrombectomy or intrasinus thrombolysis) are reserved for patients with evidence of thrombus propagation, for individuals with neurological deterioration despite medical therapy, or for those with contraindications to anticoagulation
++ AHA 2024
CVT Management after the acute phase
After the acute phase of CVT, anticoagulation for most patients with either a direct oral anticoagulant or warfarin for 3 to 12 months is recommended.
Exceptions include patients with comorbid malignancy (for whom LMWH is preferred), antiphospholipid syndrome or chronic kidney disease (for whom warfarin is preferred), and those who are pregnant (for whom heparins are preferred)
It is reasonable to continue anticoagulation for three to 12 months for patients with a provoked CVT associated with a transient risk factor and indefinetely for unprovoked CVT
For pregnant patients with a history of CVT, temporary prophylactic anticoagulation with subcutaneous LMWH throughout pregnancy and continuing up to eight weeks postpartum is suggested
++ AHA 2024
Etiologies of thunderclap headache
Evaluation of thunderclap headache
Suggested diagnostic workup in patients with carotid artery dissection
AHA/ ASA 2024
Algorithm for antithrombotic treatment in patients with cervical artery dissection
duration of treatment 3-6 months
AHA/ ASA 2024
Clinical findings of cerebrovascular fibromuscular dysplasia
++ Compared with patients with cervical artery dissection without fibromuscular dysplasia, fibromuscular dysplasia–related cervical artery dissection was associated with >3-fold increase risk of recurrent cervical artery dissection!!
AHA/ ASA 2024
RCVS diagnosis
