Chiara Briani, Luca Massarotti, Antonio Branca, Marco Rossato, Tamara Berno, Andrea Visentin, Francesca Castellani, Chiara Dalla Torre, Marta Lucchetta, Tiziana Rosso, Alessandro Burlina, Giovanni Librizzi, Claudio Pagano, Manuele Marasca, Fabrizio Vianello, Renato Zambello, Livio Trentin, Alessandro Salvalaggio, Renzo Manara
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引用次数: 0
Abstract
Background and Aims
Brain pachymeningeal thickening (PT) is common in POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy, Skin changes) syndrome. Objective of our study was to assess PT changes in POEMS and correlation with hematologic and neurological response.
Methods
We performed a longitudinal brain MRI study on 18 POEMS patients. Inflammatory Neuropathy Cause and Treatment (INCAT) disability score assessed neurological impairment. Hematologic response was defined based on accepted criteria. Neurological and hematologic evaluations were performed the same week as brain MRI.
Results
Median disease duration at first MRI was 2 months (range 0–42). Median follow-up between first and last MRI was 44 months (range 3–167). At first MRI, 17/18 patients displayed PT. Twelve patients received bortezomib, 10 lenalidomide, 6 autologous stem-cell transplantation, 3 had ≥ 3 lines of therapy. The overall hematologic response was 72% with 44% achieving complete response. PT remained stable in 10 patients while decreased in 7 patients: all hematologically improved, 83% also neurologically improved. Among the 13 patients with hematologic improvement, 61% showed PT reduction. Among the 8 patients with neurological improvement, 63% displayed PT decrease.
Interpretation
PT is a common feature in POEMS syndrome and may support diagnosis. However, its evolution does not reliably reflect treatment response, limiting its use as a monitoring biomarker.
期刊介绍：
The Journal of the Peripheral Nervous System is the official journal of the Peripheral Nerve Society. Founded in 1996, it is the scientific journal of choice for clinicians, clinical scientists and basic neuroscientists interested in all aspects of biology and clinical research of peripheral nervous system disorders.
The Journal of the Peripheral Nervous System is a peer-reviewed journal that publishes high quality articles on cell and molecular biology, genomics, neuropathic pain, clinical research, trials, and unique case reports on inherited and acquired peripheral neuropathies.
Original articles are organized according to the topic in one of four specific areas: Mechanisms of Disease, Genetics, Clinical Research, and Clinical Trials.
The journal also publishes regular review papers on hot topics and Special Issues on basic, clinical, or assembled research in the field of peripheral nervous system disorders. Authors interested in contributing a review-type article or a Special Issue should contact the Editorial Office to discuss the scope of the proposed article with the Editor-in-Chief.
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