Neurofibromatosis type I is a genetic condition with an autosomal dominant transmission characterized by neurocutaneous involvement and a predisposition to tumor development. Central nervous system manifestations include benign areas of dysmyelination and possibly hazardous glial tumors whose clinical management may result challenging. Here, we report on three patients diagnosed with Neurofibromatosis type I whose brain MRI follow-up showed the presence of gadolinium-enhancing lesions which spontaneously regressed. In none of the three cases, the lesions showed any clinical correlate and eventually presented a striking reduction in size while gadolinium enhancement disappeared despite no specific therapy administration during the follow-up. Although their nature remains undetermined, these lesions presented a benign evolution. However, they might be misdiagnosed as potentially life-threatening tumors. Hitherto, a similar behavior has been described only in scattered cases and we believe these findings may be of particular interest for the clinical management of patients affected by neurofibromatosis type I

Regression of gadolinium-enhanced lesions in patients affected by neurofibromatosis type 1

LUCCHETTA, MARTA;Manara, Renzo;PERILONGO, GIORGIO;CLEMENTI, MAURIZIO;TREVISSON, EVA
2016

Abstract

Neurofibromatosis type I is a genetic condition with an autosomal dominant transmission characterized by neurocutaneous involvement and a predisposition to tumor development. Central nervous system manifestations include benign areas of dysmyelination and possibly hazardous glial tumors whose clinical management may result challenging. Here, we report on three patients diagnosed with Neurofibromatosis type I whose brain MRI follow-up showed the presence of gadolinium-enhancing lesions which spontaneously regressed. In none of the three cases, the lesions showed any clinical correlate and eventually presented a striking reduction in size while gadolinium enhancement disappeared despite no specific therapy administration during the follow-up. Although their nature remains undetermined, these lesions presented a benign evolution. However, they might be misdiagnosed as potentially life-threatening tumors. Hitherto, a similar behavior has been described only in scattered cases and we believe these findings may be of particular interest for the clinical management of patients affected by neurofibromatosis type I
2016
Electronic
Inglese
121
3
214
217
4
Springer-Verlag Italia s.r.l.
Internazionale
anonymous
The Neurology category covers resources concerned with the central and peripheral nervous system including the brain, spinal cord, nerves, and fluids. Coverage includes general and clinical neurology including neurosurgery, neuropsychiatry, neuropsychology, neurophysiology, neuroradiology, neuropediatrics, neuropathology, and neurobiology. Resources on cerebrovascular diseases, movement and spinal disorders, pain, dementia, headache, aphasiology, brain injury, paraplegia, stroke, and acupuncture are also included.
The Research/Laboratory Medicine & Medical Technology category includes resources on pathology, forensic sciences, medical genetics, biotechnology, and biomedical engineering. Coverage also includes clinical monitoring and computing devices; medical instrumentation; and artificial organs, medical implants, and other emerging medical treatments.
25-set-2015
Gadolinium; Gliomas; Lesions; Magnetic resonance imaging; Neurofibromatosis type 1; Radiology, Nuclear Medicine and Imaging
http://link.springer.com/journal/11547
ITALIA
no
reserved
Lucchetta, Marta; Manara, Renzo; Perilongo, Giorgio; Clementi, Maurizio; Trevisson, Eva
01 CONTRIBUTO IN RIVISTA::01.01 - Articolo in rivista
info:eu-repo/semantics/article
5
262
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11577/3187214
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