@article{SommerCarrollKoikeetal.2021,
  author    = {Sommer, Claudia and Carroll, Antonia S. and Koike, Haruki and Katsuno, Masahisa and Ort, Nora and Sobue, Gen and Vucic, Steve and Spies, Judith M. and Doppler, Kathrin and Kiernan, Matthew C.},
  title     = {Nerve biopsy in acquired neuropathies},
  series = {Journal of the Peripheral Nervous System},
  volume    = {26},
  journal   = {Journal of the Peripheral Nervous System},
  number    = {S2},
  doi       = {10.1111/jns.12464},
  url       = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-259555},
  pages     = {S21-S41},
  year      = {2021},
  abstract  = {A diagnosis of neuropathy can typically be determined through clinical assessment and focused investigation. With technological advances, including significant progress in genomics, the role of nerve biopsy has receded over recent years. However, making a specific and, in some cases, tissue-based diagnosis is essential across a wide array of potentially treatable acquired peripheral neuropathies. When laboratory investigations do not suggest a definitive diagnosis, nerve biopsy remains the final step to ascertain the etiology of the disease. The present review highlights the utility of nerve biopsy in confirming a diagnosis, while further illustrating the importance of a tissue-based diagnosis in relation to treatment strategies, particularly when linked to long-term immunosuppressive therapies,},
  language  = {en}
}
@article{MerkiesvanSchaikLegeretal.2019,
  author    = {Merkies, Ingemar S.J. and van Schaik, Ivo N. and L{\´e}ger, Jean-Marc and Bril, Vera and van Geloven, Nan and Hartung, Hans-Peter and Lewis, Richard A. and Sobue, Gen and Lawo, John-Philip and Durn, Billie L. and Cornblath, David R. and De Bleecker, Jan L. and Sommer, Claudia and Robberecht, Wim and Saarela, Mika and Kamienowski, Jerzy and Stelmasiak, Zbigniew and Tackenberg, Bj{\"o}rn and Mielke, Orell},
  title     = {Efficacy and safety of IVIG in CIDP: Combined data of the PRIMA and PATH studies},
  series = {Journal of the Peripheral Nervous System},
  volume    = {24},
  journal   = {Journal of the Peripheral Nervous System},
  organization    = {PRIMA Trial Investigators and the PATH Study Group},
  doi       = {10.1111/jns.12302},
  url       = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-224013},
  pages     = {48-55},
  year      = {2019},
  abstract  = {Intravenous immunoglobulin (IVIG) is a potential therapy for chronic inflammatory demyelinating polyneuropathy (CIDP). To investigate the efficacy and safety of the IVIG IgPro10 (Privigen) for treatment of CIDP, results from Privigen Impact on Mobility and Autonomy (PRIMA), a prospective, open-label, single-arm study of IVIG in immunoglobulin (Ig)-na{\"i}ve or IVIG pre-treated subjects (NCT01184846, n = 28) and Polyneuropathy And Treatment with Hizentra (PATH), a double-blind, randomized study including an open-label, single-arm IVIG phase in IVIG pre-treated subjects (NCT01545076, IVIG restabilization phase n = 207) were analyzed separately and together (n = 235). Efficacy assessments included change in adjusted inflammatory neuropathy cause and treatment (INCAT) score, grip strength and Medical Research Council (MRC) sum score. Adverse drug reactions (ADRs) and ADRs/infusion were recorded. Adjusted INCAT response rate was 60.7\% in all PRIMA subjects at Week 25 (76.9\% in IVIG pre-treated subjects) and 72.9\% in PATH. In the pooled cohort (n = 235), INCAT response rate was 71.5\%; median time to INCAT improvement was 4.3 weeks. No clear demographic differences were noticed between early (responding before Week 7, n = 148) and late responders (n = 21). In the pooled cohort, median change from baseline to last observation was -1.0 (interquartile range -2.0; 0.0) point for INCAT score; +8.0 (0.0; 20.0) kPa for maximum grip strength; +3.0 (1.0; 7.0) points for MRC sum score. In the pooled cohort, 271 ADRs were reported in 105 subjects (44.7\%), a rate of 0.144 ADRs per infusion. This analysis confirms the efficacy and safety of IgPro10, a recently FDA-approved IVIG for CIDP, in a population of mainly pre-treated subjects with CIDP [Correction added on 14 March 2019 after first online publication: the INCAT response rate has been corrected.].},
  language  = {en}
}