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Saturday, 2 November 2013

Reversible cerebral vasoconstriction syndrome - Headache/Stroke

I. Stroke is a result of a blockage in the blood vessels (ischemic stroke) or bleeding (hemorrhagic stroke) of that associated ischemic stroke, transient ischemic attack, or non-traumatic intracranial hemorrhage, including intracerebral and subarachnoid hemorrhage, etc. according to "Headache attributed to stroke, TIA, intracerebral haemorrhage, or vascular malformation" by Carolei A, Sacco S.(1)

II. A headache or cephalalgia is defined as a condition of pain in the region around the head or neck. Normally, it is a symptom of a number of different effects of certain diseases or the head and neck themselves. High headache impact was proven to be associated with worse academic performance.

III. Reversible cerebral vasoconstriction syndrome (RCVS)
Reversible cerebral vasoconstriction syndrome, sometimes called Call-Fleming syndrome is defined as a condition of disease of the arteries of which the brain develops a blood vessels spasm that leads to multifocal arterial constriction and dilation, causing the sudden onset of a severe headache (2)
1. Does stroke causes headache?
A sudden severe headache or recurrent of thunderclap headache may be a beginning of a stroke as a result of a hemorrhagic stroke.
 
2. Does headache causes stroke?
Cerebral vasoconstriction due to severe headache may cause completely stop blood flow to a portion of the brain, causing stroke if the blood vessel can not relax fast for blood to flow again(2)

II. Symptoms
1. Sudden-onset thunderclap headache or recurrent severe headache
2. Dysarthria
Dysarthria is defined as a condition of motor speech disorder resulting from neurological injury of the motor component of the motor-speech system due to the can spontaneously constriction of and relax back and forth over a period of time of cerebral arteries.
3. Nausea, Vomiting, Photophobia, Confusion and Blurred vision(3)
4. Unsteady movement of the limbs
5. Overactive or overresponsive reflexes
As a result of upper motor neuron damage causes of focal neurological symptoms.
6. Etc.


V. Causes and risk factors
Some researchers suggested reversible cerebral vasoconstriction syndrome (RCVS) may be a results from a transient disturbance of the circle of arteries that supply blood to the brain of which leads to its constriction.
1. Antidepressants
Antidepressants used to treat depression, anxiety disorders, and some personality disorders may increase risk of diffuse cerebral vasoconstriction(5)

2. Nasal decongestants
In the investigation conducted by Lariboisière Hospital of clinical, neuroimaging and outcome data of 67 consecutive patients prospectively diagnosed over 3 years in our institution with an angiographically confirmed RCVS, 43 females and 24 males with a mean age of 42 years (19-70). RCVS was spontaneous in 37% of patients and secondary in the 63% others, to postpartum in 5 and to exposure to various vasoactive substances in 37, mainly cannabis, selective serotonin-recapture inhibitors and nasal decongestants(6)

3. Eletriptan  
A pediatric case of reversible cerebral vasoconstriction syndrome with cortical subarachnoid hemorrhage, suggested that Eletriptan might cause vasoconstriction of cerebral arteries. Although most patients with RCVS are adults and pediatric cases are rare, RCVS should be considered in a child complaining of severe headache(7)

4. Vasoconstrictive drug exposure and migraine
In the study of clinical, laboratory, and imaging features of patients with reversible cerebral vasoconstriction syndromes evaluated at 2 academic centers, compare subgroups, conducted by

6. Evoked by pregnancy(4)

7. Exposure to vasoactive substances such as angiotensin II, epinephrine, norepinephrine, vasopressin can lead to constrictor dilate of blood vessels)(4)

8. Etc.

VI. Diagnosis
A. Misdiagnosis
Reversible cerebral vasoconstriction syndrome (RCVS) constitutes an under-recognised but clinically important diagnosis, because it can be complicated by a cerebrovascular accident. The syndrome is often misdiagnosed as it resembles. Misdiagnosis of Reversible Cerebral Vasoconstriction Syndromes as primary cerebral vasculitis and aneurysmal subarachnoid hemorrhage is common because of overlapping clinical and angiographic features, researchers at Cleveland Clinic Lerner College of Medicine of Case Western Reserve University and Cleveland Clinic Foundation, said, as reversible cerebral vasoconstriction syndromes (RCVS) comprise a group of diverse conditions, all characterized by reversible multifocal narrowing of the cerebral arteries heralded by sudden (thunderclap), severe headaches with or without associated neurologic deficits(11). Other study reported demonstration of two female patients presented with severe headache with subsequent angiographic findings of ‘bead and string’ appearances of the cerebral arteries at Circle of Willis, which resolved spontaneously within 3 months. Diagnosis of reversible cerebral vasoconstriction syndrome was made. The clinical and imaging characteristics in different modalities are discussed(10)

1. Primary Angiitis of the Central Nervous System
Primary angiitis of the central nervous system is defined as an idiopathic disorder characterized by vasculitis within the dural confines. Headache and encephalopathy are the most frequent initial symptoms. Stroke or focal symptoms develop in less than 20% of patients at the onset of disease and are uncommon in the absence of headache or encephalopathy. Symptoms or signs of vasculitis outside of the central nervous system are rare; serologic markers of inflammation are typically normal(16). A Medline search was performed to identify all case reports since 1966 describing RCVS and PACNS that provide sufficient clinical detail to permit diagnostic classification according to published criteria. RCVS included case studies in which there was angiographic or transcranial Doppler ultrasound evidence of near-to-complete resolution of cerebral vasoconstriction in the absence of a well-recognized secondary cause. PACNS included reports of histologically confirmed PACNS either through biopsy or necropsy(17)

2. Post-partum cervicocephalic artery dissection (pp-CAD)
In a report of a 41-year-old right-handed African-American woman who developed the syndrome of pp-CAD (headaches, trace subarachnoid hemorrhage and diffuse cerebral arteriopathy on angiogram), researchers at the Northwestern University, hypothesized whether transient arterial wall abnormalities, postpartum hormonal changes or subtle connective tissue aberrations play a similar role in the pathogenesis of these two associated Post-partum cervicocephalic artery dissection (pp-CAD) and Reversible cerebral segmental vasoconstriction (RCSV)(16)

3. Subarachnoid Haemorrhage
Subarachnoid hemorrhage is defined as a bleeding in the subarachnoid space area between the brain and the thin tissues that cover the brain, causing certain similar symptoms to those of Reversible cerebral vasoconstriction syndrome. In the report of the case of a 51-year-old woman who presented to hospital following a thunderclap headache, initially thought to be secondary to a subarachnoid haemorrhage (SAH). A tiny anterior choroidal artery aneurysm was demonstrated on cerebral angiogram. At surgical clipping, no evidence of haemorrhage was observed. Post-operatively, the patient developed delayed right-sided hemiparesis, managed with aggressive hypertensive treatment, and later, with onset of septicaemia, central visual loss. Computed tomography (CT) brain scans demonstrated oedematous changes within the parieto-occipital regions bilaterally and later areas of infarction. The initial diagnosis of SAH was revised to reversible cerebral vasoconstriction syndrome (RCVS), which gave rise to Posterior reversible encephalopathy syndrome (PRES)(18). Another report on a pediatric case of RCVS with cortical subarachnoid hemorrhage (SAH). A 12-year-old boy developed acute, severe headache with paralysis of lower extremities causing gait disturbance after administration of eletriptan. Brain magnetic resonance angiography (MRA) revealed multifocal narrowing of the cerebral arteries, whereas magnetic resonance imaging (MRI) demonstrated sulcal hyperintensity on fluid-attenuated inversion recovery, consistent with cortical SAH. The patient's clinical symptoms resolved spontaneously after a few days and the MRI and MRA findings disappeared 3months later, suggesting a diagnosis of RCVS. Eletriptan might cause vasoconstriction of cerebral arteries. Although most patients with RCVS are adults and pediatric cases are rare, RCVS should be considered in a child complaining of severe headache.(19)

4. Orgasmic headache
Orgasmic headache (OH) is a condition of an "explosive" headache that occurs at orgasm. In a report of 34-year-old woman who presented with isolated and recurrent TCH at orgasm, which fulfilled the diagnosis of OH. However, she was post-partum and had recent exposure to ecstasy, making her symptoms highly suggestive of RCVS. Brain magnetic resonance angiography showed segmental vasoconstriction(15)

5. Etc.

B. Diagnosis
1. Catheter angiogram 
Catheter angiogram, is the use of a thin plastic tube, called a catheter, is inserted into a large artery and threaded through the circulatory system to the carotid artery, through a small incision in the skin, together with X-rays and a contrast dye in visualizing the blood vessels of the brain. Then a series of radiographs is taken as the contrast agent spreads through the brain's arterial system, then a second series as it reaches the venous system.

2. CT scan
CT scan can dive your doctor a three-dimensional view of your blood vessels of the brain to look for masses and other abnormalities that cause Reversible cerebral vasoconstriction syndrome

3.  Magnetic resonance imaging (MRI)
By using radio waves and magnetic fields to take pictures, MRI scan provides very high quality of a cross-sectional slice and lengthwise slices of the brain and thus providing the better and detail image of location of tumor and the surrounding structures. It is one of most likely early test ordered by a doctor to diagnose tumors, strokes, aneurysms, neurological diseases and other brain abnormalities and the blood vessels around the brain.

4. Computed tomography angiography (CTA)
CTA is a test to create detailed images of the blood vessels of the brain to look for neurological diseases and any abnormality with the use of the combination of the technology of a conventional CT scan with that of traditional angiography.

5. Cerebral magnetic resonance angiography (MRA)
Magnetic resonance angiography (MRA) is an accurate non-invasive tool for imaging the cerebral vessels. It provides morphologic information about the cerebral vessels relying on blood flow as the physical basis for generating contrast between stationary tissues and moving spins. 'Selective' MRA gives functional information about the cerebrovascular system such as flow direction, origin of flow, and presence or absence of collaterals. Arteries and veins can be imaged selectively due to their usually opposite flow directions. Although at a relatively early stage of development, MRA has already become a widely used tool for the study of the cerebrovascular system(12)
In the study of One hundred five (79%) of all 133 aneurysms detected with MRA by a neuroradiologist, 100 (75%) detected by an experienced neurosurgeon, 84 (63%) detected by a general radiologist, and 80 (60%) detected by a resident neuroradiologist, conducted by Nagatomi Neurosurgical Hospital, found that although MRA is useful in the diagnosis of cerebral aneurysms, sufficient experience and careful attention are necessary for accurate diagnosis of aneurysms located at the internal carotid and anterior cerebral arteries(13)

6. Etc.

VIII. Treatments
1. Calcium channel inhibitors (nimodipine, nifedipine or verapamil)
a. In the observation of a 63-year-old female with developed visual field impairment and a right-sided hemiparesis. Brain MRI revealed bilateral posterior and left parietal ischaemic strokes and  Cerebral catheter angiography showed segmental arterial vasoconstriction, after 11 days onset of headache, a vasodilative therapy with calcium channel inhibitors was started and serial transcranial Doppler ultrasonography demonstrated resolution of cerebral arterial vasoconstriction(14)
b. Side effects are not limit to
b.1. Constipation
b.2. Nausea,
b.3 Headache
b.4. Rash,
b.5. Edema
b.6. Low blood pressure
b.7. Drowsiness, and dizziness
b.8. Etc.

2. Corticosteroids
a. Corticosteroids (commonly called steroids) are synthetic medicine closely resemble cortisol, a hormone produced adrenal glands produce naturally. In the report of a severe case of a 53-year-old woman with RCVS having an unruptured cerebral aneurysm and presenting as cortical subarachnoid hemorrhage, reversible posterior leukoencephalopathy syndrome, and cerebral infarction, by Juntendo University Shizuoka Hospital showed that the patient was successfully treated with corticosteroids and a calcium channel blocker and the aneurysm was clipped. Her various complications are due to the responsible vasoconstriction that started distally and progressed towards proximal arteries. This case demonstrates the spectrum of presentations of RCVS, a clinically complicated condition(20)

b. Side effects are not limit to
b.1. Bones thinning,
b.2. Avascular necrosis of bones
b.3. Swelling of the face
b.4. The medicine can inhibit natural hormones
b.5. It may cause liver damage
b.6. It may lower HDL cholesterol, and raise LDL cholesterol
b.7. Etc.

3. Intravenous magnesium sulfate
a. Magnesium sulfateis is a chemical compound with the formula MgSO4. In the report of four postpartum women aged 15 to 33 years developed acute neurologic deficits 1 to 8 days after uncomplicated deliveries. One had a history of migraine headaches and 2 had histories of spontaneous abortion. Two of the patients had uneventful pregnancies and 2 had preeclampsia, 1 of whom had acute hepatic failure, conducted by Jennifer E and the team showed that aggressive treatment was attempted with most patients including intravenous magnesium sulfate, corticosteroids, calcium channel blockers, balloon angioplasty, vasopressors, and osmotic agents. Two patients underwent serial angiography, with results showing severe, recurrent proximal vasoconstriction involving all major intracranial vessels.

b. Side Effects are not limit to
b.1. It may cause severe allergic reactions, including rash; hives; itching; difficulty breathing; tightness in the chest swelling of the mouth, face, lips, or tongue.
b.2. Dizziness
 b.3. Flushing;
b.4. Irregular heartbeat
b.5. Sweating
b.6. Etc.

4. Etc.
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Sources
(1) http://www.ncbi.nlm.nih.gov/pubmed/20816453
(2) http://www.ncbi.nlm.nih.gov/pubmed/19220301 
(3) http://www.ncbi.nlm.nih.gov/pubmed/20936928
(4) http://www.ncbi.nlm.nih.gov/pubmed/21179608
(5) http://www.ncbi.nlm.nih.gov/pubmed/16832100
(6) http://www.ncbi.nlm.nih.gov/pubmed/18025032
(7) http://www.sciencedirect.com/science/article/pii/S038776041200006X
(8) http://www.medscape.org/viewarticle/748234_3
(9) http://www.ncbi.nlm.nih.gov/pubmed/22000400
(10) http://www.hkcr.org/publ/Journal/vol13no3/full/149-53%20Imaging.pdf
(11) http://www.ncbi.nlm.nih.gov/pubmed?term=Narrative%20Review%3A%20Reversible%20Cerebral%20Vasoconstriction%20Syndromes
(12) http://www.biomedsearch.com/nih/Cerebral-magnetic-resonance-angiography/1355864.html
(13) http://www.ncbi.nlm.nih.gov/pubmed/12105357 
(14) http://www.ncbi.nlm.nih.gov/pubmed/12662190
(15) http://www.ncbi.nlm.nih.gov/pubmed/20655230
(16) http://archneur.ama-assn.org/cgi/reprint/66/6/704.pdf
(17) http://www.ncbi.nlm.nih.gov/pubmed/20534374
(18) http://www.ncbi.nlm.nih.gov/pubmed/22237929
(19) http://www.ncbi.nlm.nih.gov/pubmed/22285527