Central and Peripheral Nervous System Complications of Vasculitis Syndromes From Pathology to Bedside: Part 1-Central Nervous System. 2022

Gelsomina Mansueto, and Giuseppe Lanza, and Francesco Fisicaro, and Danielle Alaouieh, and Emily Hong, and Sara Girolami, and Marco Montella, and Alessandro Feola, and Mario Di Napoli
Department of Advanced Medical and Surgical Sciences (DAMSS), University of Campania "Luigi Vanvitelli", Piazza L. Miraglia 2, 80138, Naples, Italy.

The aim of this review is to provide a comprehensive update on the clinical assessment, diagnosis, complications, and treatment of primary central nervous system vasculitis (PCNSV). The developments in neuroimaging, molecular testing, and cerebral biopsy have enhanced clinical assessment and decision making, providing novel insights to prevent misdiagnosis increasing diagnostic certainty. Advances in imaging techniques visualizing the wall of intracranial vessels have improved the possibility to distinguish inflammatory from non-inflammatory vascular lesions. Large recent studies have revealed a more varied histopathological pictures and disclosed an association with amyloid angiopathy. Unfortunately, therapy remains largely empiric. PCNSV is a heterogeneous group of disorders encompassing different clinical subsets that may differ in terms of prognosis and therapy. Recent evidence has described a more benign course, with good response to therapy. New diagnostic techniques will play soon a pivotal role in the appropriate diagnosis and prompt management of PCNSV.

UI MeSH Term Description Entries
D002490 Central Nervous System The main information-processing organs of the nervous system, consisting of the brain, spinal cord, and meninges. Cerebrospinal Axis,Axi, Cerebrospinal,Axis, Cerebrospinal,Central Nervous Systems,Cerebrospinal Axi,Nervous System, Central,Nervous Systems, Central,Systems, Central Nervous
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013577 Syndrome A characteristic symptom complex. Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes
D016657 Cerebral Amyloid Angiopathy A heterogeneous group of sporadic or familial disorders characterized by AMYLOID deposits in the walls of small and medium sized blood vessels of CEREBRAL CORTEX and MENINGES. Clinical features include multiple, small lobar CEREBRAL HEMORRHAGE; cerebral ischemia (BRAIN ISCHEMIA); and CEREBRAL INFARCTION. Cerebral amyloid angiopathy is unrelated to generalized AMYLOIDOSIS. Amyloidogenic peptides in this condition are nearly always the same ones found in ALZHEIMER DISEASE. (from Kumar: Robbins and Cotran: Pathologic Basis of Disease, 7th ed., 2005) Amyloid Angiopathy, Cerebral,Congophilic Angiopathy,Sporadic Cerebral Amyloid Angiopathy,Angiopathy, Cerebral Amyloid,Angiopathy, Congophilic,Cerebral Amyloid Angiopathies,Congophilic Angiopathies
D017933 Peripheral Nervous System The nervous system outside of the brain and spinal cord. The peripheral nervous system has autonomic and somatic divisions. The autonomic nervous system includes the enteric, parasympathetic, and sympathetic subdivisions. The somatic nervous system includes the cranial and spinal nerves and their ganglia and the peripheral sensory receptors. Nervous System, Peripheral,Nervous Systems, Peripheral,Peripheral Nervous Systems,System, Peripheral Nervous,Systems, Peripheral Nervous
D020293 Vasculitis, Central Nervous System Inflammation of blood vessels within the central nervous system. Primary vasculitis is usually caused by autoimmune or idiopathic factors, while secondary vasculitis is caused by existing disease process. Clinical manifestations are highly variable but include HEADACHE; SEIZURES; behavioral alterations; INTRACRANIAL HEMORRHAGES; TRANSIENT ISCHEMIC ATTACK; and BRAIN INFARCTION. (From Adams et al., Principles of Neurology, 6th ed, pp856-61) Angiitis, Central Nervous System,Central Nervous System Vasculitis,Cerebral Angiitis,Cerebral Vasculitis,Granulomatous Angiitis,Postzoster Arteritis,Primary CNS Vasculitis,Secondary CNS Vasculitis,CNS Vasculitis,Central Nervous System Angiitis,Granulomatous Arteritis,Primary Central Nervous System Vasculitis,Vasculitis, CNS, Secondary,Angiitis, Cerebral,Angiitis, Granulomatous,Arteritis, Granulomatous,Arteritis, Postzoster,CNS Vasculitis, Primary,CNS Vasculitis, Secondary,Vasculitis, CNS,Vasculitis, Cerebral,Vasculitis, Primary CNS,Vasculitis, Secondary CNS

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