Reversible Vasoconstriction Syndrome Research Articles

Reply to the Letter "Reversible Vasoconstriction Syndrome Is a Complication of SARS-CoV-2 Infection/Vaccination Rather than That of Leigh Syndrome".

Reply to the Letter "Reversible Vasoconstriction Syndrome Is a Complication of SARS-CoV-2 Infection/Vaccination Rather than That of Leigh Syndrome".

Noninvasive investigations of human glymphatic dynamics in a diseased model.

To explore human glymphatic dynamics in a diseased model via a noninvasive technique. Patients with reversible vasoconstriction syndrome (RCVS) presenting with blood-brain barrier disruption, i.e., para-arterial gadolinium leakage on 3-T 3-dimensional isotropic contrast-enhanced T2-fluid-attenuated inversion recovery (CE-T2-FLAIR) magnetic resonance imaging (MRI), were prospectively enrolled. Consecutive 9-min-CE-T2-FLAIR for 5-6 times (early panel) after intravenous gadolinium-based contrast agent (GBCA) administration and one time-varying deferred scan of noncontrast T2-FLAIR (delayed panel) were performed. In Bundle 1, we measured the calibrated signal intensities (cSIs) of 10 different anatomical locations. In Bundle 2, brain-wide measurements of para-arterial glymphatic volumes, means, and medians of the signal intensities were conducted. We defined mean (mCoIs) or median (mnCoIs) concentration indices as products of volumes and signal intensities. Eleven subjects were analyzed. The cSIs demonstrated early increase (9min) in perineural spaces: (cranial nerve [CN] V, p = 0.008; CN VII + VII, p = 0.003), choroid plexus (p = 0.003), white matter (p = 0.004) and parasagittal dura (p = 0.004). The volumes, mCoIs, and mnCoIs demonstrated increasing rates of enhancement after 9 to 18min and decreasing rates after 45 to 54min. The GBCA was transported centrifugally and completely removed within 961-1086min after administration. The exogenous GBCA leaked into the para-arterial glymphatics could be completely cleared around 961 to 1086min after administration in a human model of BBB disruption. The tracer enhancement started variously in different intracranial regions but was eventually cleared centrifugally to brain convexity, probably towards glymphatic-meningeal lymphatics exits. Glymphatic clearance time intervals and the centrifugal directions assessed by a noninvasive approach may have implications for clinical glymphatic evaluation in the near future. • This study aimed to investigate the human glymphatic dynamics in a noninvasive diseased model. • The intracranial MR-detectable gadolinium-based contrast agents were removed centrifugally within 961 to 1086min. • The glymphatic dynamics was demonstrable by enhancing MRI in an in vivo diseased model noninvasively.

Ischemic stroke due to spontaneous reversible extra- cranial internal carotid artery vasospasm: A case report

Reversible idiopathic extracranial internal carotid artery (ICA) vasospasm is a rare cause of ischemic stroke especially seen among young patients and the pathophysiology is not fully understood. We report here a 23-year-old female who presented with left hemiparesis and hemihypoesthesia. She had several similar reversible attacks which last about 30 minutes in the past years. She had migraine- type headaches five times a month and history of bipolar disease. MRI showed multiple infarction areas on the right middle cerebral artery (MCA) territory with no large vessel abnormality. We could not find any cardioembolic or other etiology except for a PAI-1 heterozygote 4G/5G mutation. She was given warfarin and had several ischemic attacks in different vascular areas in the following years with various treatment strategies. MR angiography showed reversible stenosis of both internal carotid arteries (ICA) in every attack with different severities. We diagnosed the patient as reversible vasoconstriction syndrome of the ICA and started prednisolone, nimodipine, and ASA treatment. In conclusion, spontaneous reversible extracranial internal carotid artery vasospasm is a rare condition with uncertain pathology. There is also no effective treatment.

Thunderclap Headache in Children and Adolescents.

This work aimed to review the epidemiology, clinical criteria, and primary and secondary diagnoses of pediatric thunderclap headache and to compare to adult thunderclap headache. Thunderclap headache among children aged 6-18years are rare; this headache presented in 0.08% of the patients admitted to a pediatric emergency department in a tertiary pediatric center. In that recent single-center study, thunderclap was a headache of grade 10 on the pain scale and conferred a benign course. Contrary to adults, in children, most thunderclap headaches are due to either a primary thunderclap headache or another type of primary headache. A number of case reports have attributed pediatric thunderclap to reversible vasoconstriction syndrome and bleeding due to intracranial aneurysm. However, 3-year data from a pediatric emergency department of one center did not find these reasons to be causes of secondary thunderclap headache. This may be due to the rarity of these diagnoses in children compared to adults. Four of the 19 patients with thunderclap headache reported in that single study had secondary thunderclap; the causes were infection in three and malignant hypertension in one. All the patients had a benign course. Although urgent imaging and lumbar puncture are required in the workup of pediatric thunderclap, severe causes are very rare. More research is needed to investigate pediatric thunderclap headache.

Vascular headache

Headache is a common accompanying symptom of cerebrovascular diseases, and may represent the only symptom at onset or predominates over the other neurological features. Indeed, the third edition of the International Classification of Headache Disorders (ICHD-3) dedicates an entire chapter to headache attributed to cranial or cervical vascular disorder. It includes headaches attributed to non traumatic intracranial hemorrhage, arterial dissection, acute ischemic stroke, cerebral venous sinus thrombosis, reversible cerebral vasoconstriction syndrome, arteritis and chronic intracranial vasculopathy such as CADASIL or MELAS. Unfortunately, headache associated with cerebrovascular diseases lacks clear-cut characteristics. The descriptions may range from a thunderclap onset to a phenotype similar to tension type headache. However, many of these headaches can be present with peculiar characteristics as in the case of thunderclap headache following subarachnoid hemorrhage or cerebral reversible vasoconstriction syndrome. In cervicocerebral artery dissection pain is most commonly ipsilateral to the dissected vessel, mainly perceived in the temporal area in carotid artery dissection and in the occipital area in vertebral artery dissection. Otherwise, pain can mimic primary headaches, mostly migraine, tension-type headache, or cluster headache, as described in the case of vascular artery dissection or symptomatic arteriovenous malformations. Headache may lack specific features as seen in cerebral venous thrombosis. Therefore, it is essential to be aware of the relationship between headache and cerebrovascular diseases, in order to can timely recognise causes and intervene as soon as possible to avoid permanent sequelae that result in disability.

Evaluating thunderclap headache.

Thunderclap headache (TCH) is an abrupt-onset of severe headache that needs to be thoroughly investigated because the most common secondary cause is subarachnoid hemorrhage (SAH). There has been no consensus guideline regarding the diagnostic workup. This review aims to provide an update on the evaluation of TCH. The most important update in the 2019 American College of Emergency Physicians guideline for evaluation of acute headache in the emergency department is that negative noncontrast brain computed tomography (CT) findings within 6 h from ictus essentially excludes SAH. Additionally, the updated guideline recommends that after a negative brain CT, CT angiogram is a reasonable alternative to lumbar puncture if clinical suspicion of an intracranial source of SAH is high. An important update of reversible vasoconstriction syndrome (RCVS), the second most common etiology of TCH, is the RCVS2 score development based on clinical and radiological features, providing high specificity and sensitivity for distinguishing RCVS from other intracranial arteriopathies. Although the evaluation of TCH is exhaustive, the potentially catastrophic consequence of a missed diagnosis of sentinel headache justifies the efforts. Awareness of the clinical features and application of diagnostic tools specific for different pathological conditions can facilitate the diagnostic workup.

What Can Mimic Multiple Sclerosis?

This article discusses mimics of multiple sclerosis (MS). Excluded in this discussion are neuromyelitis optica and vasculitis, discussed in other articles in this journal. Covered entities include posterior reversible encephalopathy syndrome, reversible vasoconstriction syndrome, acute disseminated encephalomyelitis, Sussac's Syndrome, and chronic idiopathic demyelinating polyneuropathy. There are also multiple infectious entities that mimic MS including; progressive multi-focal leukoencephalopathy (PML), Toxoplasmosis, Tuberculosis, Herpes Simplex Virus, Cytomegalovirus, Varicella zoster virus, Epstein Barr virus, Cryptococcus and Human immunodeficiency virus. In addition, there are leukoencephalopathies that can present in adulthood including Adrenoleukodystrophy, Metachromatic leukodystrophy, Cerebral autosomal dominant idiopathic leukoencephalopathy, Leigh's and Alexanders disease that could be mistaken for MS.

Reversible cerebral vasoconstriction syndrome

Reversible cerebral vasoconstriction syndrome (RCVS) is an increasingly recognized cause of acute headache. Postpartum reversible vasoconstriction syndrome is a recognized subset of RCVS. Differentiating RCVS from primary angiitis of the central nervous system can be dificult but has important therapeutic implications. This case highlights the clinical and imaging features of RCVS and stresses the importance of diagnosing this rare entity.

Acute Headache in the Emergency Setting.

Acute headache is a common symptom and is reported by approximately 2%-4% of patients who present to the emergency department. Many abnormalities manifest with headache as the first symptom, and it is crucial to obtain a patient's complete clinical history for correct diagnosis. Headache onset, duration, and severity; risk factors such as hypertension, immunosuppression, or malignancy; and the presence of focal neurologic deficits or systemic symptoms may aid the radiologist in deciding whether imaging is appropriate and which modality to choose. Imaging findings are more likely to be abnormal in patients with a "thunderclap" headache than in those with headaches of lesser severity. The causes of headache in the emergency setting are various. They may manifest at imaging as subarachnoid hemorrhage (ruptured aneurysm, reversible vasoconstriction syndrome, or pituitary apoplexy), parenchymal hemorrhage (hypertension, ruptured arteriovenous malformation, cerebral amyloid angiopathy, dural arteriovenous fistula, or sinus thrombosis), or parenchymal edema (posterior reversible encephalopathy syndrome, reversible cerebral vasoconstriction syndrome, sinus thrombosis, or encephalitis). Alterations in intracranial pressure that are related to idiopathic intracranial hypertension or spontaneous intracranial hypotension and prior lumbar puncture or epidural injection may manifest with specific imaging findings. With accumulating knowledge of disease pathophysiology, radiologists have started to play a more central role in making the correct diagnosis. This article reviews multiple causes of acute headache and their characteristic appearances at multimodality imaging and familiarizes the reader with current concepts in imaging.Online supplemental material is available for this article.©RSNA, 2019.

Current Hospital Demographics of Subarachnoid Hemorrhage Based on CT Angiography and 3D Rotational Angiography in a Neurosurgical Center.

Aneurysmal subarachnoid hemorrhage is an important cause of mortality and morbidity. Modern hospital demographics are scarce. We evaluated the diagnosis and treatment of patients with SAH in a neurosurgical referral center. Between March 2013 and April 2015, two hundred eighty-four patients with SAH diagnosed on CT or lumbar puncture were admitted. All patients underwent 64- to 128-detector row CT angiography. Additional imaging was with 3D rotational angiography of all vessels. In patients with aneurysms, characteristics and mode of treatment were recorded. In 197 of 220 patients with an aneurysmal bleeding pattern, we found a cause of the bleeding: One hundred ninety-five patients had a ruptured aneurysm (98%); 1 patient, a micro-AVM; and 1 patient, reversible vasoconstriction syndrome. Of 195 ruptured aneurysms, 6 were dissecting aneurysms and 3 were AVM-associated flow aneurysms. In 23 of 204 patients (11%) with an aneurysmal bleeding pattern and 3D rotational angiography performed, no cause was found. In 8 of 9 patients (89%) with lumbar puncture positive for SAH but CT negative for it, no cause was found. Of 180 patients with a ruptured aneurysm eligible for treatment, 147 (82%) were treated endovascularly and 30 aneurysms (17%) were clipped. Of 204 patients with an aneurysmal bleeding pattern and 3D rotational angiography, 72 (35%) had multiple aneurysms. These 72 patients had, altogether, 117 additional aneurysms, of which 24 (21%) were treated by either coiling or clipping. This study provides robust data on hospital demographics of SAH in a neurosurgical referral center, based on CTA and 3D rotational angiography of all vessels.

MITOCHONDRIAL DISEASES (Posters): P.183MELAS and reversible cerebral vasoconstriction

A 22-year-old woman was admitted due to fever and severe headache for 10 days and altered mental status and generalized tonic-clonic seizures for 8 days. She had a short stature and hairy body surface, and her mother passed away about ten years ago due to ``encephalitis''. Laboratory data showed elevated lactate in both peripheral blood and CSF. Brain MRI using diffusion weighted imaging revealed hyperintense lesions located in the whole right temporal and occipital lobes and pulvinar of thalamus. MR angiography revealed segmental stenoses at the right P1 and M2-3 junction. Muscle biopsy showed typical RRF and SSV. A genetic study revealed a mitochondrial DNA A3243G point mutation. The patient's clinical symptoms and MRI/MR angiography (MRA) findings improved after the administration of L-arginine, L-carnitine, lipoic acid, vitamin B and dexamethasone. Therefore we speculate the possible pathophysiology just like RCVS (reversible cerebaral vasoconstriction syndrome) involving in the stroke-like episodes of MELAS.

Diagnosing causes of headache within the postpartum period

Background: Headache is one of the most common symptoms following delivery and the underlying cause may be benign or life threatening. Identification of the cause of headache in the postpartum period can be challenging and relies on a comprehensive history and thorough examination, with particular focus on the presence or absence of neurological signs, which may suggest a more serious diagnosis (Nelson-Piercy 2010). However, through clinical experience and research, we have noted that post-partum headache is significantly under-recognised and treated (Goldszmidt et al. 2005).Aims: To ensure a standardised approach to the management of patients presenting with postpartum headache, we sought to introduce a diagnostic tool for postpartum headache.Methods: This was a literature review combined with a postpartum headache case example that occurred within our institution in 2015. Risk factors and potential clinical failures from the case were noted and compared to the literature.Results: Our case is a 28-year-old woman who developed a postpartum headache following spontaneous vaginal delivery. Of note, the patient had an epidural inserted in labour for analgesia. Initially, systemic and neurological examinations were normal and the patient was discharged home with simple analgesics and a diagnosis of tension-type headache. She represented with worsening headache, pyrexia and signs of meningism. Lumbar puncture confirmed a diagnosis of bacterial meningitis caused by Haemophilus influenzae. She was treated with IV ceftriaxone and discharged home without any long-term sequelae.In an attempt to improve recognition of post-partum headache, especially rare causes such as meningitis, NHS Tayside has chosen to adopt the PARTUM mneumonic (Lim et al. 2014).Pressure (blood pressure for pre-eclampsia/eclampsia)Anaesthetic (post-dural puncture headache)Reversible (vasoconstriction syndrome)Thrombosis (cerebral venous sinus thrombosis, ischaemic stroke)Use your brain (there are so many other causes of headache)Migraine (Lim et al. 2014).PARTUM was first devised in 2014 as part of a research paper published by the Journal Practical Neurology (Lim et al. 2014). The aim of the mnemonic is to allow the treating physician a basic list of postpartum headache causes to use as a working diagnosis (Lim et al. 2014).Conclusions: Research has shown that many postpartum headaches go undiagnosed and as a result are poorly treated, often returning following discharge from secondary care (Nelson-Piercy 2010). The hope is that the introduction of this adjuvant will allow clinicians to identify the cause of postpartum headaches earlier and will allow life threatening diagnoses to be quickly excluded.

Juvenile stroke - what is important?

Stroke in young adults is adiagnostic and therapeutic challenge for all persons involved. Approximately 15% of ischemic strokes occur in young adults. Lack of awareness of the symptoms in emergency departments often results in delayed diagnosis and access to specific therapeutic options, such as revascularization. The causes are often heterogeneous and necessitate specific investigations. The etiology of juvenile stroke includes drug abuse, vasculitis and arteriopathies, such as reversible vasoconstriction syndrome and posterior reversible encephalopathy syndrome, although the prevalence of classical vascular risk factors is high. The most frequent causes of ischemic stroke in young adults are cardioembolism and microangiopathy; furthermore, dissection of vessels of the neck are more frequent compared to older patients. According to the results of currently available studies reperfusion strategies, such as intravenous fibrinolysis and mechanical thrombectomy are efficacious and safe in young patients.

Agrupación de casos de síndrome de Guillain-Barré atípico: ¿es necesario redefinir los criterios diagnósticos y los protocolos microbiológicos?

Guillain-Barre syndrome is classically defined as a symmetrical ascending acute polyradiculoneuropathy, although there are atypical variants that make diagnosis difficult. The medical data of six patients in our hospital area are collected during the first quarter of 2013. Lumbar punctures, imaging, neurophysiological studies, ganglioside antibodies and serologies have been proposed in all cases. We focus on the atypical features as late hyporeflexia, increased frequency of asymmetry and distal paresis and initial fever. From a neurophysiological point of view, all patients presented sensorimotor axonal forms. The most consistent datas in early studies is the F wave's alteration. A Miller Fisher variant associated with faciocervicobraquial paresis and cerebral reversible vasoconstriction syndrome has been detected. A bilateral brachial paresis and lumbar polyradiculopathy in the context of influenza A infection is other interesting case. The saltatory variant with cranial nerve involvement and lower limbs paresis has been demonstrated in one patient. Bands in cerebrospinal fluid are positive in three cases and anti-ganglioside antibodies in one patient. The syndrome of inappropriate secretion of antidiuretic hormone may explain some of the hyponatremias registered. The first line of treatment are inmunoglobulins in all patients. Plasmapheresis exchanges has been used as an additional therapy in four cases. These clusters of six axonal cases with atypical clinical features justifies the need for knowledge of these variants in order to achieve an early treatment. Late hyporeflexia and brachialfaciocervico, saltatory and lumbar forms should be considered in the spectrum of Guillain-Barre syndrome. The etiological study should rule out a lots of pathogens as influenza A.

Neurological Disorders and Pregnancy

Neurological disorders caused by pregnancy and puerperium include the posterior reversible encephalopathy syndrome, the amniotic fluid embolism syndrome (AFES), the postpartum angiopathy due to reversible vasoconstriction syndrome, and the Sheehan syndrome. Hypertension and proteinuria are the hallmarks of preeclampsia, seizures define eclampsia. Hemolysis, elevated liver enzymes and low platelets constitute the HELLP syndrome. Vision disturbances including cortical blindness occur in the posterior reversible encephalopathy syndrome (PRES). The Sheehan syndrome presents with panhypopituitarism post partum due to apoplexia of the pituitary gland in severe peripartal blood loss leading to longstandinghypotension. Some neurological disorders occur during pregnancy and puerperium with an increased frequency. These include stroke, sinus thrombosis, the restless legs syndrome and peripheral nerve syndromes, especially the carpal tunnel syndrome. Chronic neurologic diseases need an interdisciplinary approach during pregnancy. Some anticonvulsants double the risk of birth defects. The highest risk exists for valproic acid, the lowest for lamotrigine and levetiracetam. For MS interval treatment, glatiramer acetate and interferones seem to be safe during pregnancy. All other drugs should be avoided.

Severe rhabdomyolysis associated with catha edulis(khat) use

Severe rhabdomyolysis is a rare complication of exposure to the recreational drug khat. Adverse reactions to khat thathave been reported in the literature include myocardial infarction and reversible vasoconstriction syndrome. Theactive ingredient, cathinone, is a naturally occurring beta-ketone amphetamine that may be responsible for this severecatecholaminergic response. Here we report a case of severe rhabdomyolysis following exposure to khat.

Migraine and stroke

We reviewed the literature concerning the relationship between migraine and ischemic stroke, and compared our data with reported findings. The incidence of migrainous stroke, strictly defined according to the International Classification of Headache 3 beta version, is quite low. A European MRI study indicated that the relative risk of ischemic stroke is increased two-fold in migraine patients with aura, and white matter lesions are located predominantly in the posterior circulation region. The risk of ischemic stroke in patients with migraine aura under 45 years old was reduced to the control level in the group that avoided oral contraceptives and quit smoking. Migraine is a relatively low risk factor for ischemic stroke, in contrast to classic risk factors for stroke, such as hypertension, diabetes mellitus, and atrial fibrillation. Our data concerning the location of white matter lesions showed predominance in the anterior circulation region, in marked contrast to the European study. The reason for this difference remains unknown. It is possible that the white matter lesions in some migraine patients could be caused by reversible vasoconstriction syndrome.

Another cause of reversible splenial lesion: Hepatic encephalopathy

Background and purpose: Reversible splenial lesion has been reported to be associated with encephalitis due to different pathogens, mostly viruses, methotrexate toxicity, systemic lupus erythematosus, reversible vasoconstriction syndrome and migraine. But it has not been reported yet under imaging findings of hepatic encephalopathy.

Posterior reversible vasoconstriction syndrome in congenital disorders of glycosylation due to a defect in Conserved Oligomeric Golgi complex (COG4)

Background: Congenital disorders of glycosylation (CDGs/INS;) are a rare group of metabolic diseases that have been described in only a few individuals, where phenotypes are continually being updated. Some of these disorders present hemorrhagic or prothrombotic features, but there is not a clear correlation with each individual subtype.

Reversible cerebral vasoconstriction syndrome identification of prognostic factors

Object: Reversible cerebral vasoconstriction syndrome (RCVS) is described as a clinical and radiological entity characterized by thunderclap headaches, a reversible segmental or multifocal vasoconstriction of cerebral arteries with or without focal neurological deficits or seizures. The purpose of this study is to determine risk factors of poor outcome in patients presented a RCVS. MethodsA retrospective multi-center review of invasive and non-invasive neurovascular imaging between January 2006 and January 2011 has identified 10 patients with criterion of reversible segmental vasoconstriction syndrome. Demographics data, vascular risks and evolution of each of these patients were analyzed. ResultsSeven of the ten patients were females with a mean age of 46 years. In four patients, we did not found any causative factors. Two cases presented RCVS in post-partum period between their first and their third week after delivery. The other three cases were drug-induced RCVS, mainly vaso-active drugs. Cannabis was found as the causative factor in two patient, Sumatriptan identified in one patient while cyclosporine was the causative agent in also one patient. The mean duration of clinical follow-up was 10.2 months (range: 0–28 months). Two patients had neurological sequelae: one patient kept a dysphasia and the other had a homonymous lateral hemianopia. We could not find any significant difference of the evolution between secondary RCVS and idiopathic RCVS. The only two factors, which could be correlated to the clinical outcome were the neurological status at admission and the presence of intraparenchymal abnormalities (ischemic stroke, hematoma) in brain imaging. ConclusionsFulminant vasoconstriction resulting in progressive symptoms or death has been reported in exceptional frequency. Physicians had to remember that such evolution could happen and predict them by identifying all factors of poor prognosis (neurological status at admission, the presence of intraparenchymal abnormalities).