Wednesday, 27 November 2013

Reversible cerebral vasoconstriction syndrome - The Misdiagnosis

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)
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. 

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