ABSTRACT
OBJECTIVE: Diagnosis of reversible cerebral vasoconstriction syndrome (RCVS) is currently based on luminographic findings of vasoconstriction. In addition to vasoconstriction, the blood-brain barrier (BBB) breakdown has been postulated as a central mechanism of RCVS. Our aim was to document BBB breakdown in patients with RCVS and its role for the pathophysiology-based diagnosis of RCVS. METHODS: We prospectively recruited 72 consecutive patients with thunderclap headache who did not have aneurysmal subarachnoid hemorrhage from April 2015 to July 2016 at the Samsung Medical Center. Based on the International Classification of Headache Disorders-3 beta criteria and neuroimaging, patients were classified as having RCVS (n = 41; "definite" in 29 imaging-proven patients and "probable" in 12 imaging-negative patients), other secondary causes (n = 7), and thunderclap headache of undetermined cause (n = 24). BBB breakdown was evaluated using contrast-enhanced fluid-attenuated inversion recovery magnetic resonance imaging. RESULTS: BBB breakdown was documented in 20 (69.0%) patients with definite RCVS, 3 (25.0%) patients with probable RCVS, and none with other secondary causes. BBB breakdown was present in RCVS patients with (n = 4) and without (n = 19) concomitant posterior reversible encephalopathy syndrome. In patients with RCVS, the extent of BBB breakdown was independently associated with neurological complications (multivariate odds ratio = 1.48 per 1 territorial increase, 95% confidence interval = 1.04-2.12, adjusted p = 0.032). Three (12.5%) patients with thunderclap headache of undetermined cause were newly classified as having RCVS by the presence of BBB breakdown. INTERPRETATION: This is the first study to show BBB breakdown in patients with RCVS. This finding might broaden our understanding of the pathophysiology and clinical spectrum of RCVS. Ann Neurol 2017;81:454-466.
Subject(s)
Blood-Brain Barrier/diagnostic imaging , Cerebral Arterial Diseases/diagnostic imaging , Headache Disorders, Primary/diagnostic imaging , Posterior Leukoencephalopathy Syndrome/diagnostic imaging , Vasoconstriction/physiology , Adult , Cerebral Arterial Diseases/cerebrospinal fluid , Cerebral Arterial Diseases/epidemiology , Cerebral Arterial Diseases/physiopathology , Comorbidity , Female , Headache Disorders, Primary/cerebrospinal fluid , Headache Disorders, Primary/epidemiology , Headache Disorders, Primary/physiopathology , Humans , Male , Middle Aged , Posterior Leukoencephalopathy Syndrome/cerebrospinal fluid , Posterior Leukoencephalopathy Syndrome/epidemiology , Posterior Leukoencephalopathy Syndrome/physiopathologyABSTRACT
Arteriopathy is an uncommon complication of primary varicella zoster virus (VZV) infection in the immunocompetent adult. We report a case of a 39-year-old woman known to be VZV negative prior to the event. She presented to the emergency department having experienced an episode of expressive aphasia and right upper limb paraesthesia lasting 15 min. The symptoms followed a 3-day period of general malaise, arthralgia and a generalised maculopapular itchy rash involving face and limbs. No immunocompromise was detected but an infectious contact was identified in the home. Imaging findings were consistent with a focal cerebritis/vasculopathy and VZV infection was confirmed with cerebrospinal fluid PCR analysis. Resolution of radiological signs occurred following prompt treatment with appropriate antivirals.
Subject(s)
Cerebral Arterial Diseases/virology , Chickenpox/complications , Adult , Aphasia/etiology , Cerebral Arterial Diseases/cerebrospinal fluid , Cerebral Arterial Diseases/diagnosis , Chickenpox/cerebrospinal fluid , Chickenpox/diagnosis , Female , Humans , Magnetic Resonance Imaging , Paresthesia/etiologyABSTRACT
A 42-year-old homosexual man without evidence of immune deficiency developed cerebral granulomatous angiitis in association with the isolation of human T-lymphotropic virus type III (HTLV-III) from brain tissue and cerebrospinal fluid. This syndrome may be an additional neurological sequela of HTLV-III infection.
Subject(s)
Brain/microbiology , Cerebral Arterial Diseases/microbiology , Granuloma/microbiology , HIV/isolation & purification , Vasculitis/microbiology , Cerebral Arterial Diseases/cerebrospinal fluid , Cerebral Arterial Diseases/pathology , Cerebral Arteries/pathology , Granuloma/pathology , Homosexuality , Humans , Male , Vasculitis/cerebrospinal fluid , Vasculitis/pathologyABSTRACT
The extent of edema related to infarction assessed by computed tomography was compared with the CSF-lactate concentration in patients with middle cerebral artery (MCA) infarction on the first, third and seventh day following the stroke. A linear correlation between the extent of infarction edema and CSF-lactate level was most distinct on the third day. CSF-lactate concentration on the third day can be considered as a measure of the extent of the accompanying edema which in our study reached its maximum at this time in comparison to the first and seventh day.
Subject(s)
Cerebral Arterial Diseases/diagnostic imaging , Cerebral Infarction/diagnostic imaging , Lactates/cerebrospinal fluid , Tomography, X-Ray Computed , Acute Disease , Adult , Aged , Brain Edema/etiology , Cerebral Arterial Diseases/cerebrospinal fluid , Cerebral Arterial Diseases/complications , Cerebral Infarction/cerebrospinal fluid , Cerebral Infarction/complications , Humans , Lactic Acid , Middle Aged , Time FactorsSubject(s)
Cerebral Arterial Diseases/etiology , Virus Diseases/complications , Adult , Antibodies, Viral/analysis , Cerebral Angiography , Cerebral Arterial Diseases/cerebrospinal fluid , Cerebral Arterial Diseases/diagnostic imaging , Child , Complement Fixation Tests , Female , Humans , Influenza, Human/cerebrospinal fluid , Influenza, Human/complications , Influenza, Human/diagnosis , Male , Pregnancy , Virus Diseases/cerebrospinal fluid , Virus Diseases/diagnosisABSTRACT
To clarify the causal relationship between spontaneous recanalization of the occluded cerebral artery and development of hemorrhagic infarction, 15 patients with internal carotid or middle cerebral arterial axis occlusion were submitted to consecutive lumbar punctures and follow-up cerebral angiography. Consequently, six of seven recanalized patients had sanguineous cerebrospinal fluid (CSF) on the second or third day after ictus, while only one of eight non-recanalized patients had bloody CSF. It was strongly suggested that recanalization might have an initimate relationship with the development of hemorrhagic infarction.
