angiopathy cerebral reversible

This is a case of a 32-year-old primigravid who developed sudden severe headache on the 7th day postpartum associated with focal neurologic deficits and altered sensorium. She had a GCS score of 6, anisocoric pupils and an NIHSS score of 31. Cranial MRI with MRA showed multifocal hyperacute to acute infarcts on the left occipital lobe, left thalamus, and midbrain which was more prominent on the right. Due to clinical deterioration, a repeat Cranial MRI with MRA was done and showed progression of infarcts involving both thalami and right pons with interval appearance of contour irregularities in the proximal anterior cerebral, posterior cerebral, basilar and internal carotid arteries...

BACKGROUND AND OBJECTIVE: To report a triplication of the amyloid-β precursor protein ( APP ) locus along with relative messenger RNA (mRNA) expression in a family with autosomal dominant early-onset cerebral amyloid angiopathy (CAA) and Alzheimer disease (AD). METHODS: Four copies of the APP gene were identified by quantitative multiplex PCR of short fluorescent fragments, fluorescent in situ hybridization (FISH), and array comparative genomic hybridization. APP mRNA levels were assessed using reverse-transcription-digital droplet PCR in the proband's whole blood and compared with 10 controls and 9 APP duplication carriers...

Background Multifocal convexity subarachnoid hemorrhage (cSAH) has generally been described in the setting of traumatic brain injury, however, it has also been reported in the absence of trauma in conditions such as with reversible cerebral vasoconstriction syndrome. We describe the clinical and radiographic characteristics of multifocal cSAH in an academic center. Methods We analyzed our single-center retrospective database of nontraumatic convexity subarachnoid hemorrhage from January 2015-January 2018. Convexity subarachnoid hemorrhage was defined as blood in one or more cortical sulci in the absence of trauma; patients with blood in the cisterns or Sylvian fissure were excluded...

Acute neurological problems are significant disorders of pre- and postpartum period in women. We analyzed the magnetic resonance imaging (MRI) profile of patients presenting with peripartum encephalopathy over 2 years. Of 51 patients, MRI was abnormal in 40 patients and normal in 11 patients. Posterior reversible encephalopathy (13/40), cerebral venous thrombosis (6/40), and postpartum angiopathy (3/40) are the three most common causes of peripartum encephalopathy as identified in MRI. The other less common but important etiology include HELLP (Hemolysis, Elevated Liver enzymes and Low Platelet) syndrome (2), osmotic demyelination (2), antiphospholipid syndrome (2), tubercular meningitis/cerebritis (3), pituitary hyperplasia with hemorrhage (2), postictal edema (2), cerebellitis (1), transient splenial lesion (1), and changes of old trauma and stroke (one each)...

Intracerebral hemorrhage is a stroke subtype with high mortality and poor functional outcome in survivors. Its main causes are hypertension, cerebral amyloid angiopathy, and anticoagulant treatment. Hematomas have a high frequency of expansion in the first hours after symptom onset, a process associated with neurologic deterioration and poor outcome. Control of severe hypertension, reversal of anticoagulant effect, and management of increased intracranial pressure are the mainstays of management of intracerebral hemorrhage in the acute phase...

Inflammatory cerebral amyloid angiopathy is a largely reversible inflammatory vasculopathy that develops in an acute or subacute fashion in reaction to amyloid protein deposition in the central nervous system blood vessels. There are two recognized pathologically characterized variants: cerebral amyloid angiopathy-related inflammation (CAAri) and A beta-related angiitis (ABRA). Both variants produce a clinical picture that resembles primary angiitis of the CNS but is distinguished by a characteristic radiologic appearance...

IMPORTANCE: Primary angiitis of the central nervous system (PACNS) is a rare condition which is often misdiagnosed. In order to avoid mistakes in the management, a step by step approach is necessary. OBSERVATIONS: The most common presenting symptoms of PACNS are encephalopathy-related cognitive and affective abnormalities, headaches and multifocal symptoms associated with recurrent episodes of ischemia or hemorrhage. Seizures and spinal cord lesions may also occur...

Human cystatin C ( h CC), a member of the superfamily of papain-like cysteine protease inhibitors, is the most widespread cystatin in human body fluids. Physiologically active h CC is a monomer, which dimerization and oligomerization lead to the formation of the inactive, insoluble amyloid form of the protein, strictly associated with cerebral amyloid angiopathy, a severe state causing death among young patients. It is known, that biological membranes may accelerate the oligomerization processes of amyloidogenic proteins...

The amyloid-β (Aβ) peptide, a key pathogenic factor in Alzheimer's disease, attenuates the increase in cerebral blood flow (CBF) evoked by neural activity (functional hyperemia), a vital homeostatic response in which NMDA receptors (NMDARs) play a role through nitric oxide, and the CBF increase produced by endothelial factors. Tissue plasminogen activator (tPA), which is reduced in Alzheimer's disease and in mouse models of Aβ accumulation, is required for the full expression of the NMDAR-dependent component of functional hyperemia...

Primary angiitis of the central nervous system (CNS) is an inflammatory vasculopathy affecting the brain and spinal cord. It is a difficult diagnosis to make because of its insidious nonspecific course and its multiple mimics. This review identifies and discusses some noninfectious mimickers of primary CNS angiitis, including: reversible cerebral vasoconstriction syndrome, Sneddon's Syndrome, amyloid-beta-related angiopathy, Susac Syndrome, and neurosarcoidosis. Each condition will be reviewed in terms of epidemiology, pathology, clinical presentation, diagnostic approach, and treatment...

OBJECTIVE: To assess cerebrovascular reactivity in response to a visual task in participants with cerebral amyloid angiopathy (CAA), Alzheimer disease (AD), and mild cognitive impairment (MCI) using fMRI. METHODS: This prospective cohort study included 40 patients with CAA, 22 with AD, 27 with MCI, and 25 healthy controls. Each participant underwent a visual fMRI task using a contrast-reversing checkerboard stimulus. Visual evoked potentials (VEPs) were used to compare visual cortex neuronal activity in 83 participants...

Heavy metals are considered a continuous threat to humanity, as they cannot be eradicated. Prolonged exposure to heavy metals/metalloids in humans has been associated with several health risks, including neurodegeneration, vascular dysfunction, metabolic disorders, cancer, etc. Small blood vessels are highly vulnerable to heavy metals as they are directly exposed to the blood circulatory system, which has comparatively higher concentration of heavy metals than other organs. Cerebral small vessel disease (CSVD) is an umbrella term used to describe various pathological processes that affect the cerebral small blood vessels and is accepted as a primary contributor in associated disorders, such as dementia, cognitive disabilities, mood disorder, and ischemic, as well as a hemorrhagic stroke...

Cerebral amyloid angiopathy-related inflammation is a syndrome of reversible encephalopathy with cerebral amyloid angiopathy, however the pathology is not well understood. We clear a part of the pathology through the first case of an 80-year-old man with cerebral amyloid angiopathy-related inflammation induced by relapsing polychondritis (RP) analysis. An 80-year-old man was diagnosed with RP by auricular cartilage biopsy. Almost no abnormality including intracranial microbleeding was detected by cranial magnetic resonance image (MRI) at diagnosis...

Circulating adiponectin (APN) levels decrease with age and obesity. On the other hand, a reduction in APN levels is associated with neurodegeneration and neuroinflammation. We previously showed that aged adiponectin knockout (APN-/- ) mice developed Alzheimer's like pathologies, cerebral insulin resistance, and cognitive impairments. More recently, we also demonstrated that APN deficiency increased Aβ-induced microglia activation and neuroinflammatory responses in 5xFAD mice. There is compelling evidence that deregulated insulin activities or cerebral insulin resistance contributes to neuroinflammation and Alzheimer's disease (AD) pathogenesis...

Spreading depolarization is observed as a large negative shift of the direct current potential, swelling of neuronal somas, and dendritic beading in the brain's gray matter and represents a state of a potentially reversible mass injury. Its hallmark is the abrupt, massive ion translocation between intraneuronal and extracellular compartment that causes water uptake (= cytotoxic edema) and massive glutamate release. Dependent on the tissue's energy status, spreading depolarization can co-occur with different depression or silencing patterns of spontaneous activity...

Ring finger protein (RNF) 213 is known as a susceptibility gene for moyamoya disease (MMD), which is characterized by bilateral carotid folk stenosis. Cerebral angiopathy after viral infection has been known to present angiographical appearance resembling MMD, however its pathogenesis and genetic background are not well known. We report a case of reversible cerebral angiopathy after viral infection in a pediatric patient with genetic variant of RNF213 mutation. The patient had developed a severe headache after hand, foot, and mouth disease...

Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare and treatable variant of CAA likely due to an autoimmune response directed toward beta-amyloid deposits. Cognitive and behavioral manifestations are the most common symptoms, followed by focal neurological signs, headache and seizures, associated with characteristics neuroradiological features on brain magnetic resonance imaging (MRI). We describe the clinical course, radiological features and therapeutic approach of two patients with probable CAA-ri with the aim of emphasizing the importance of an early diagnosis of this potentially reversible disease in different neurological settings, such as memory clinics and stroke units...

BACKGROUND: Nontraumatic convexity subarachnoid hemorrhage (cSAH) is a nonaneurysmal variant that is associated with diverse etiologies. METHODS: With IRB approval, we retrospectively reviewed consecutive nontraumatic cSAH from July 1, 2006 to July 1, 2016. Data were abstracted on demographics, medical history, neuroimaging, etiology, and clinical presentation. RESULTS: We identified 94 cases of cSAH. The cases were classified according to the following etiologies: reversible cerebral vasoconstriction syndrome (RCVS) 17 (18%), cerebral amyloid angiopathy (CAA) 15 (16%), posterior reversible encephalopathy syndrome 16 (17%), cerebral venous thrombosis 10 (11%), large artery occlusion 7 (7%), endocarditis 6 (6%), and cryptogenic 25 (27%)...

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...

Various imaging techniques play a role in the diagnosis of CNS vasculopathies, which comprise a heterogeneous group of disorders, including various noninflammatory and inflammatory etiologies. Noninflammatory vasculopathies include entities such as CADASIL, Susac, moyamoya, fibromuscular dysplasia, vasculopathy of connective tissue disorders, and reversible vasoconstriction syndrome. Inflammatory vasculopathies include vasculitides of different vessel sizes, primary angiitis of the CNS, vasculitis of systemic disease, and vasculitis secondary to specific causes...