SynthesisNeurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology2025
Spinal cord involvement in primary central nervous system vasculitis. A systematic review of clinical, neuroradiological and pathological findings.
Synthesis in Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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Who cites it
1 citing paper in PubMed.
- Granulomatous Diseases of the CNS: Aetiology, Neuroradiological Features and Clinical Findings.Clinical neuroradiology · 2026Review
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Abstract
introductionPrimary Angiitis of Central Nervous System (PACNS) is a rare disease featured by transmural inflammation in vessels pertaining to brain, leptomeninges and spinal cord. It is a rare disease and the involvement of the spinal cord represents a rarer and not yet completely investigated subtype.
methodsWe performed a systematic search of the available literature on Pubmed and Embase, adding backward and forward citations, in order to retrieve the reported cases of PACNS i9nvolvimeng the spinal cord without time limitations. The main aim is to retrieve information about clinical and demographic features, pathological and neuroradiological findings on brain and spinal cord, and, finally, treatment and outcome.
resultsThe search provided 33 papers (mainly individual case reports) and 38 patients, with a large age frame (from 12 to 70 years of age), mainly adults. Among these ones 36/38 received a pathological diagnosis and granulomatous pattern was the main reported one. The description of spinal cord involvement in MRI is variable form extensive tumefactive lesions to spinal roots prominent involvement. The mortality is high (29% at the end of the individual follow-up). DISCUSSION: As in non-spinal involvement, the main limitation of the retrieved cases is the inhomogeneity of the diagnostic and therapeutic pathway with underusing and underreporting of neuroradiolgoical techniques relevant for the diagnosis according with the available diagnostic criteria. Spinal cord involvement confirms its rarity, but it has been associated to a high disability and mortality and the diagnosis of PACNS has therapeutic consequences.
conclusionsSpinal cord involvement is present in a minority of PACNS cases with a variety of neuroradiological and pathological findings. The standardization of the diagnostic pathway could help to improve the quality of information in prospective studies.
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