scholarly journals Hemorrhagic primary CNS angiitis and vasoconstrictive drug exposure

2016 ◽  
Vol 7 (1) ◽  
pp. 26-34
Author(s):  
Mehmet A. Topcuoglu ◽  
Ruchira M. Jha ◽  
Jacob George ◽  
Matthew P. Frosch ◽  
Aneesh B. Singhal
Keyword(s):  
Drug Exposure ◽  
Brain Mri ◽  
Brain Biopsy ◽  
High Rate ◽  
Thunderclap Headache ◽  
Drug Induced ◽  
Cerebral Vasoconstriction ◽  
Sympathomimetic Drug ◽  
Nasal Decongestants ◽  
Primary Angiitis

AbstractBackground:Primary angiitis of the CNS (PACNS) typically manifests with accumulating neurologic deficits from ischemic strokes. Intracerebral hemorrhage (ICH) is an uncommon complication. There is limited knowledge about the risk factors and features of hemorrhagic PACNS.Methods:We identified 49 patients (20 biopsy-proven) with PACNS diagnosed at our hospital from 1993 to 2015. We compared the features of hemorrhagic and nonhemorrhagic PACNS and analyzed the hemorrhagic PACNS cases in detail.Results:The mean age was 51 ± 15 years; 13 patients were men. Five patients had ICH (mean age 52 ± 14 years; 4 men) including 4 where ICH was the first manifestation of PACNS. All ICH patients reported recent exposure to sympathomimetic drugs (e.g., diet pills, nasal decongestants). Patients with ICH had higher rates of headache (100% vs 43%, p = 0.022), especially thunderclap headache (60% vs 0%, p = 0.001), and eosinophilic vascular infiltrates on brain biopsy (50% vs 9%, p = 0.084). In all ICH patients, brain MRI showed lobar hemorrhages with concurrent punctate diffusion-restricted lesions, suggesting an acute inflammatory process. Four received a short course of immunosuppressive therapy. All patients showed complete clinical resolution or significant improvement within weeks.Conclusions:In this study, hemorrhagic PACNS was exclusively associated with sympathomimetic drug exposure. The high rate of thunderclap headache, lobar hemorrhages, and the self-limited clinical course suggests a shared mechanism between hemorrhagic PACNS and the reversible cerebral vasoconstriction syndrome (RCVS), a PACNS mimic. This RCVS-PACNS overlap syndrome may result from sympathomimetic drug–induced prolonged distal vasoconstriction, culminating in inflammation.

Ischaemic Strokes with Reversible Vasoconstriction and without Thunderclap Headache: A Variant of the Reversible Cerebral Vasoconstriction Syndrome?

2014 ◽  
Vol 39 (1) ◽  
pp. 31-38 ◽  
Author(s):  
Valérie Wolff ◽  
Jean-Paul Armspach ◽  
Valérie Lauer ◽  
Olivier Rouyer ◽  
Anne Ducros ◽  
...  
Keyword(s):  
Young Patients ◽  
Acute Stage ◽  
Thunderclap Headache ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Cerebral Magnetic Resonance Imaging ◽  
Cerebral Vasoconstriction ◽  
Posterior Leukoencephalopathy ◽  
Nasal Decongestants ◽  
Diagnostic Work ◽  
Arterial Stenoses

Background: Reversible vasoconstriction (RV) may cause ischaemic stroke (IS) in the absence of any other defined stroke aetiology. The three objectives of our study were to evaluate the frequency of RV in a prospective series of young IS patients, to describe the detailed clinical-radiological features in the patients with RV and IS, and to compare these characteristics with those of reversible cerebral vasoconstriction syndrome (RCVS). Methods: We identified between October 2005 and December 2010, 159 consecutive young patients (<45 years) hospitalized for an acute IS confirmed by cerebral magnetic resonance imaging. An extensive diagnostic work-up was performed including toxicological urinary screening for cannabis, cocaine and amphetamines, and the usual biological, cardiac and vascular investigations for an IS in the young. We specifically studied patients with IS and RV, which was defined as multifocal intracranial arterial stenoses confirmed by intracranial arterial imaging that resolved within 3-6 months. Results: Out of 159 patients with IS, 21 (13%, 12 males, 9 females; mean age 32 years) had multifocal cerebral arterial stenoses that were fully reversible at 3-6 months, and no other cause for stroke. IS were located on posterior territory in 71% of cases, and vasoconstriction predominated on posterior cerebral and superior cerebellar arteries. Precipitating factors of IS and RV were the use of cannabis resin (n = 14), nasal decongestants (n = 2) and triptan (n = 1). Most cases (74%) had unusual severe headache, but none had thunderclap headache. None of 21 cases had reversible posterior leukoencephalopathy, cortical subarachnoid or intracerebral haemorrhage. Conclusion: RV was the sole identified cause of IS in 13% of our cohort. These young patients with IS and RV may have a variant of RCVS, related to an increased susceptibility to vasoactive agents in some individuals. RV in our patients differs from the classical characteristics of RCVS by the absence of thunderclap headache, reversible brain oedema and subarachnoid or intracranial haemorrhage. Intracranial arteries should be looked for, by appropriate vascular imaging, in young patients with IS at the acute stage and during the follow-up period.

Unique combination of hyperintense vessel sign on initial FLAIR and delayed vasoconstriction on MRA in reversible cerebral vasoconstriction syndrome: A case report

Cephalalgia ◽  
2014 ◽  
Vol 34 (13) ◽  
pp. 1093-1096 ◽  
Author(s):  
Tomoaki Kameda ◽  
Michito Namekawa ◽  
Haruo Shimazaki ◽  
Daisuke Minakata ◽  
Tohru Matsuura ◽  
...  
Keyword(s):  
Brain Mri ◽  
Sudden Onset ◽  
Arterial Stenosis ◽  
Thunderclap Headache ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Unique Combination ◽  
Cerebral Vasoconstriction ◽  
Fluid Attenuated Inversion Recovery ◽  
Angiographic Findings

Background Reversible cerebral vasoconstriction syndrome is characterized by thunderclap headache and reversible cerebral vasoconstriction on angiographic findings. It can be difficult to diagnose when initial angiography is normal. Case results A 30-year-old woman was admitted because of sudden-onset thunderclap headache and seizure on postpartum day 7. Brain MRI on fluid-attenuated inversion recovery (FLAIR) showed hyperintense vessel sign (HVS), which usually means slow flow due to severe proximal arterial stenosis. However, magnetic resonance angiography (MRA) indicated that proximal arteries was normal. After nicardipine treatment, her symptoms improved dramatically. Follow-up FLAIR on day 7 showed complete resolution of HVS, while a series of MRAs revealed reversible multifocal segmental vasoconstriction. Conclusions HVS on initial FLAIR is useful for an early diagnosis of reversible cerebral vasoconstriction syndrome. As the delayed vasoconstriction on MRA can be observed, reversible cerebral vasoconstriction syndrome may progress from distal small to proximal larger arteries.

Primary angiitis of the CNS and reversible cerebral vasoconstriction syndrome

Neurology ◽  
2018 ◽  
Vol 91 (16) ◽  
pp. e1468-e1478 ◽  
Author(s):  
Hubert de Boysson ◽  
Jean-Jacques Parienti ◽  
Jérôme Mawet ◽  
Caroline Arquizan ◽  
Grégoire Boulouis ◽  
...  
Keyword(s):  
Vasogenic Edema ◽  
White Matter Lesions ◽  
Careful Analysis ◽  
Thunderclap Headache ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Motor Deficit ◽  
Artery Dissection ◽  
Cervical Artery Dissection ◽  
Cerebral Vasoconstriction ◽  
Primary Angiitis

ObjectivesTo further improve the distinction between primary angiitis of the CNS (PACNS) and reversible cerebral vasoconstriction syndrome (RCVS).MethodsWe compared 2 large French cohorts of patients with PACNS (n = 110, retrospectively and prospectively enrolled) and RCVS (n = 173, prospectively enrolled).ResultsPatients with RCVS were predominantly female (p < 0.0001), with migraines (p < 0.0001), and were more often exposed to vasoactive substances (p < 0.0001) or postpartum (p = 0.002) than patients with PACNS. Headache, especially thunderclap headache, was more frequent in RCVS (both p < 0.0001). Thunderclap headache was absent in only 6% of patients with RCVS and was mainly recurrent (87%) and provoked (77%) mostly by sexual intercourse, exertion, or emotion. All other neurologic symptoms (motor deficit, seizure, cognitive disorder, or vigilance impairment, all p < 0.0001) were more frequent in PACNS. At admission, brain CT or MRI was abnormal in all patients with PACNS and in 31% of patients with RCVS (p < 0.0001). Acute ischemic stroke was more frequent in PACNS than in RCVS (p < 0.0001). Although intracerebral hemorrhage was more frequent in PACNS (p = 0.006), subarachnoid hemorrhage and vasogenic edema predominated in RCVS (p = 0.04 and p = 0.01, respectively). Multiple small deep infarcts, extensive deep white matter lesions, tumor-like lesions, or multiple gadolinium-enhanced lesions were observed only in PACNS, whereas cervical artery dissection was found only in RCVS.ConclusionsOur study confirms that careful analysis of clinical context, headache features, and patterns of brain lesions can distinguish PACNS and RCVS within the first few days of admission in most cases. However, diagnosis remains challenging in a few cases.

Pediatric Reversible Cerebral Vasoconstriction Syndrome: Two Unique Cases with a Review of all Reported Children

2021 ◽  
Author(s):  
Neelu Desai ◽  
Rahul Badheka ◽  
Nitin Shah ◽  
Vrajesh Udani
Keyword(s):  
Magnetic Resonance ◽  
Cerebral Arteries ◽  
Brain Mri ◽  
Brain Magnetic Resonance Imaging ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Neurological Deficits ◽  
Cerebral Vasoconstriction ◽  
Magnetic Resonance Imaging Mri ◽  
Reversible Encephalopathy ◽  
Mr Angiogram

AbstractReversible cerebral vasoconstriction syndrome (RCVS) has been well described in adults, but pediatric cases are yet under recognized. We describe two children with RCVS and review similar already published pediatric cases. The first patient was a 10-year-old girl who presented with severe headaches and seizures 3 days after blood transfusion. Brain magnetic resonance imaging (MRI) showed changes compatible with posterior reversible encephalopathy syndrome and subarachnoid hemorrhage. Magnetic resonance angiogram showed diffuse vasoconstriction of multiple cerebral arteries. The second patient was a 9-year-old boy who presented with severe thunderclap headaches. Brain MRI showed isolated intraventricular hemorrhage. Computed tomography/MR angiogram and digital subtraction angiogram were normal. A week later, he developed focal neurological deficits. Repeated MR angiogram showed diffuse vasospasm of multiple intracranial arteries. Both children recovered completely. A clinico-radiological review of previously reported childhood RCVS is provided.

scholarly journals Hematologic emergencies

2012 ◽  
pp. 238-248
Author(s):  
Daniele Vallisa ◽  
Carlo F. Moroni
Keyword(s):  
New England ◽  
Superior Vena ◽  
Tumor Lysis Syndrome ◽  
Hematologic Malignancies ◽  
Cord Compression ◽  
High Rate ◽  
Cochrane Library ◽  
Drug Induced ◽  
Hematologic Diseases ◽  
Emergency Situations

In recent years, the surprising progress made in other areas of hematology (advances in the understanding of leukemogenesis, improved transplant techniques) has been conspicuously absent in the management of hematologic emergencies. And yet, every step toward greater knowledge, every new treatment option will be of little value unless we are able to manage the acute complications of hematologic diseases. These complications are better defined as hematologic emergencies, and they are characterized by a high rate of mortality. This review is based on a search of the literature that was initially confined to articles published in the journal Hematology from 2000 to 2009. The search was then extended to the Cochrane Library and to Pub Med in February 2010 with the following Keywords emergencies; urgencies; hematology. The same key words were employed in a search of the archives of Blood and the New England Journal of Medicine from 2000 to 2010. The results confirm that hematologic emergencies can be caused by hematologic malignancies as well as by non-neoplastic hematologic diseases. Within the former category; this review examines the causes; manifestations; treatment and prevention of disseminated intravascular coagulation; superior vena caval syndrome; spinal cord compression; tumor lysis syndrome; hyperleukocytosis; and hypercalcemia. We also review emergency situations associated with non-neoplatic haematological diseases; such as thrombotic thrombocytopenic purpura; drug-induced hemolytic anemia; and acute sickle-cell crisis.

scholarly journals DIAGNOSTICS OF REVERSIBLE CEREBRAL VASOCONSTRICTION SYNDROME: CLINICAL MANIFESTATION AND IMAGING PATTERNS

2018 ◽  
pp. 50-55
Author(s):  
E. G. Klocheva ◽  
V. V. Goldobin
Keyword(s):  
Magnetic Resonance ◽  
Magnetic Resonance Angiography ◽  
Clinical Manifestation ◽  
Thunderclap Headache ◽  
Reversible Cerebral Vasoconstriction Syndrome ◽  
Treatment Modification ◽  
Cerebral Vasoconstriction

Data of 130 patients with thunderclap headache are presented. The data include clinical manifestation analysis and neuroimaging results (magnetic resonance angiography). Magnetic resonance angiography was performed 15 days after acute clinical manifestation and permitted to verify cerebral vasoconstriction, that led to treatment modification with clinical and neuroimaging signs of vasoconstriction reverse.

scholarly journals Neurologic manifestation in children parenterally infected by AIDS

2001 ◽  
Vol 82 (5) ◽  
pp. 356-358
Author(s):  
T. Yu. Burdaeva ◽  
N. V. Bobrysheva
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Clinical Manifestations ◽  
Brain Biopsy ◽  
High Rate ◽  
Neurologic Manifestation ◽  
Progressive Encephalopathy ◽  
Neuropsychologic Testing ◽  
Magnet Resonance ◽  
Standard Tests

The rate and spectrum of central nervous system lesion in children parenterally infected by AIDS the rate of clinical manifestations of lesions, life duration of such children are established. The differential diagnosis of nervous system lesion is made difficult due to the high rate of combining various symptoms in one patient, hence there are the complications of selecting the treatment tactics. Tomography, nuclear magnet resonance, brain biopsy should be used, neuropsychologic testing with standard tests should be performed to reveal the early signs of progressive encephalopathy.

scholarly journals Neurobrucellosis mimicking primary vasculitis of the central nervous system: we should perform a metagenomic analysis of the cerebrospinal fluid prior to brain biopsy

2021 ◽  
Author(s):  
Marina Barrionuevo Mathias ◽  
Fernando Gatti ◽  
Gustavo Bruniera ◽  
Vitor Paes ◽  
Gisele Sampaio Silva ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Psychiatric Symptoms ◽  
Brain Mri ◽  
Genetic Material ◽  
Clinical Manifestations ◽  
Brain Biopsy ◽  
Metagenomic Analysis ◽  
Normal Brain ◽  
The Central Nervous System

Context Primary angiitis of the central nervous system (PACNS) is characterized by the inflammation of small and medium CNS arteries; the clinical manifestations include headache, cognitive impairment and focal neurological deficits. The gold standard test for diagnosis is brain biopsy. Neurobrucellosis is an infection associated with cattle farming, which leads to neurological and psychiatric symptoms. We report a case of neurobrucellosis mimicking PACNS. Case report Male, 32 years old, with fever, headache, dizziness and cognitive impairments for 30 days. History of stroke 2 years before, with mild sequelae right hemiparesis; investigation showed suspected intracranial dissection. On physical examination, he had apathy, preserved strength, reduced reflexes with plantar flexor responses. General laboratory tests, autoantibodies and serology were normal. Brain MRI showed deep left nucleocapsular gliosis and cerebral angiography revealed stenosis of the ICA and MCA. CSF showed 42 cells/ mm³, glucose 46 mg/dL, protein 82 mg/dL. Blood PCR was negative for Brucella. Immunophenotyping of the CSF and PET-CT excluded neoplasia. Brain biopsy was inconclusive for vasculitis. Metagenomic analysis of the CSF detected 78% of Brucella genetic material. Serum agglutination test was 1:40 for brucella. Conclusions PACNS is diagnosed by exclusion. The patient filled criteria for possible PACNS, image compatible with vascular stenosis, but inconclusive brain biopsy. Brucellosis is an endemic disease in underdeveloped countries that can present as CNS vasculitis. Metagenomic analysis allows the detection of different pathogens using a single method. The case illustrates the use of metagenomics in rare diseases characterized by vasculitis, with change in clinical outcomes and conduct.

scholarly journals Mechanistic Investigations Support Liver Safety of Ubrogepant

2020 ◽  
Vol 177 (1) ◽  
pp. 84-93 ◽  
Author(s):  
Brenda Smith ◽  
Josh Rowe ◽  
Paul B Watkins ◽  
Messoud Ashina ◽  
Jeffrey L Woodhead ◽  
...  
Keyword(s):  
Drug Exposure ◽  
Liver Toxicity ◽  
Cgrp Receptor ◽  
Receptor Antagonists ◽  
Drug Induced ◽  
Clinical Dose ◽  
Liver Safety ◽  
Trial Experience ◽  
Dosing Regimens ◽  
Cgrp Receptor Antagonists

Abstract Small-molecule calcitonin gene-related peptide (CGRP) receptor antagonists have demonstrated therapeutic efficacy for the treatment of migraine. However, previously investigated CGRP receptor antagonists, telcagepant and MK-3207, were discontinued during clinical development because of concerns about drug-induced liver injury. A subsequent effort to identify novel CGRP receptor antagonists less likely to cause hepatotoxicity led to the development of ubrogepant. The selection of ubrogepant, following a series of mechanistic studies conducted with MK-3207 and telcagepant, was focused on key structural modifications suggesting that ubrogepant was less prone to forming reactive metabolites than previous compounds. The potential for each drug to cause liver toxicity was subsequently assessed using a quantitative systems toxicology approach (DILIsym) that incorporates quantitative assessments of mitochondrial dysfunction, disruption of bile acid homeostasis, and oxidative stress, along with estimates of dose-dependent drug exposure to and within liver cells. DILIsym successfully modeled liver toxicity for telcagepant and MK-3207 at the dosing regimens used in clinical trials. In contrast, DILIsym predicted no hepatotoxicity during treatment with ubrogepant, even at daily doses up to 1000 mg (10-fold higher than the approved clinical dose of 100 mg). These predictions are consistent with clinical trial experience showing that ubrogepant has lower potential to cause hepatotoxicity than has been observed with telcagepant and MK-3207.

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