Rate of neuropathic progression in hereditary transthyretin amyloidosis with polyneuropathy and other peripheral neuropathies: a systematic review and meta-analysis
Abstract Background We aimed to compare neuropathic progression rate between hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN) and other peripheral neuropathies, including diabetic peripheral neuropathy (DPN) and Charcot-Marie-Tooth disease (CMT). Methods Literature searches identi...
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| Format: | Article |
| Language: | English |
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BMC
2021-02-01
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| Series: | BMC Neurology |
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| Online Access: | https://doi.org/10.1186/s12883-021-02094-y |
| _version_ | 1818569838857027584 |
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| author | Xiaochen Lin Aaron Yarlas Montserrat Vera-Llonch Nishtha Baranwal Josh Biber Duncan Brown Braden Vogt Chafic Karam |
| author_facet | Xiaochen Lin Aaron Yarlas Montserrat Vera-Llonch Nishtha Baranwal Josh Biber Duncan Brown Braden Vogt Chafic Karam |
| author_sort | Xiaochen Lin |
| collection | DOAJ |
| description | Abstract Background We aimed to compare neuropathic progression rate between hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN) and other peripheral neuropathies, including diabetic peripheral neuropathy (DPN) and Charcot-Marie-Tooth disease (CMT). Methods Literature searches identified studies reporting neuropathic progression, measured by Neuropathy Impairment Score (NIS) or NIS-Lower Limbs (NIS-LL). Our study also included unpublished data from a clinical registry of patients who were diagnosed with different peripheral neuropathies and seen at the Oregon Health & Science University (OHSU) during 2016–2020. Meta-analysis and meta-regression models examined and compared annual progression rates, calculated from extracted data, between studies of ATTRv-PN and other peripheral neuropathies. Results Data were synthesized from 15 studies in which NIS and/or NIS-LL total scores were assessed at least twice, with ≥12 weeks between assessments, among untreated patients with ATTRv-PN or other peripheral neuropathies. Meta-analysis models yielded that the annual progression rate in NIS total scores was significantly different from zero for studies in ATTRv-PN and CMT (11.77 and 1.41; both P < 0.001), but not DPN (− 1.96; P = 0.147). Meta-regression models showed significantly faster annual progression in studies in ATTRv-PN, which statistically exceeded that in other peripheral neuropathies by 12.45 points/year for NIS, and 6.96 for NIS-LL (both P < 0.001). Conclusions Peripheral nervous function deteriorates more rapidly in patients with ATTRv-PN than for other peripheral neuropathies. These findings may improve understanding of the natural history of neuropathy in ATTRv-PN, facilitate early diagnosis, and guide the development of assessment tools and therapies specifically targeting neuropathic progression in this debilitating disease. |
| first_indexed | 2024-12-14T06:52:57Z |
| format | Article |
| id | doaj.art-614b7d3469c148ddbf872a0e61198f2f |
| institution | Directory Open Access Journal |
| issn | 1471-2377 |
| language | English |
| last_indexed | 2024-12-14T06:52:57Z |
| publishDate | 2021-02-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Neurology |
| spelling | doaj.art-614b7d3469c148ddbf872a0e61198f2f2022-12-21T23:12:50ZengBMCBMC Neurology1471-23772021-02-0121111310.1186/s12883-021-02094-yRate of neuropathic progression in hereditary transthyretin amyloidosis with polyneuropathy and other peripheral neuropathies: a systematic review and meta-analysisXiaochen Lin0Aaron Yarlas1Montserrat Vera-Llonch2Nishtha Baranwal3Josh Biber4Duncan Brown5Braden Vogt6Chafic Karam7QualityMetric Incorporated, LLCQualityMetric Incorporated, LLCAkcea TherapeuticsQualityMetric Incorporated, LLCQualityMetric Incorporated, LLCAkcea TherapeuticsDepartment of Neurology, Oregon Health & Science UniversityDepartment of Neurology, Oregon Health & Science UniversityAbstract Background We aimed to compare neuropathic progression rate between hereditary transthyretin amyloidosis with polyneuropathy (ATTRv-PN) and other peripheral neuropathies, including diabetic peripheral neuropathy (DPN) and Charcot-Marie-Tooth disease (CMT). Methods Literature searches identified studies reporting neuropathic progression, measured by Neuropathy Impairment Score (NIS) or NIS-Lower Limbs (NIS-LL). Our study also included unpublished data from a clinical registry of patients who were diagnosed with different peripheral neuropathies and seen at the Oregon Health & Science University (OHSU) during 2016–2020. Meta-analysis and meta-regression models examined and compared annual progression rates, calculated from extracted data, between studies of ATTRv-PN and other peripheral neuropathies. Results Data were synthesized from 15 studies in which NIS and/or NIS-LL total scores were assessed at least twice, with ≥12 weeks between assessments, among untreated patients with ATTRv-PN or other peripheral neuropathies. Meta-analysis models yielded that the annual progression rate in NIS total scores was significantly different from zero for studies in ATTRv-PN and CMT (11.77 and 1.41; both P < 0.001), but not DPN (− 1.96; P = 0.147). Meta-regression models showed significantly faster annual progression in studies in ATTRv-PN, which statistically exceeded that in other peripheral neuropathies by 12.45 points/year for NIS, and 6.96 for NIS-LL (both P < 0.001). Conclusions Peripheral nervous function deteriorates more rapidly in patients with ATTRv-PN than for other peripheral neuropathies. These findings may improve understanding of the natural history of neuropathy in ATTRv-PN, facilitate early diagnosis, and guide the development of assessment tools and therapies specifically targeting neuropathic progression in this debilitating disease.https://doi.org/10.1186/s12883-021-02094-yHereditary transthyretin amyloidosis with polyneuropathyNeuropathy impairment scoreMeta-analysisPeripheral neuropathyNeuropathic progression |
| spellingShingle | Xiaochen Lin Aaron Yarlas Montserrat Vera-Llonch Nishtha Baranwal Josh Biber Duncan Brown Braden Vogt Chafic Karam Rate of neuropathic progression in hereditary transthyretin amyloidosis with polyneuropathy and other peripheral neuropathies: a systematic review and meta-analysis BMC Neurology Hereditary transthyretin amyloidosis with polyneuropathy Neuropathy impairment score Meta-analysis Peripheral neuropathy Neuropathic progression |
| title | Rate of neuropathic progression in hereditary transthyretin amyloidosis with polyneuropathy and other peripheral neuropathies: a systematic review and meta-analysis |
| title_full | Rate of neuropathic progression in hereditary transthyretin amyloidosis with polyneuropathy and other peripheral neuropathies: a systematic review and meta-analysis |
| title_fullStr | Rate of neuropathic progression in hereditary transthyretin amyloidosis with polyneuropathy and other peripheral neuropathies: a systematic review and meta-analysis |
| title_full_unstemmed | Rate of neuropathic progression in hereditary transthyretin amyloidosis with polyneuropathy and other peripheral neuropathies: a systematic review and meta-analysis |
| title_short | Rate of neuropathic progression in hereditary transthyretin amyloidosis with polyneuropathy and other peripheral neuropathies: a systematic review and meta-analysis |
| title_sort | rate of neuropathic progression in hereditary transthyretin amyloidosis with polyneuropathy and other peripheral neuropathies a systematic review and meta analysis |
| topic | Hereditary transthyretin amyloidosis with polyneuropathy Neuropathy impairment score Meta-analysis Peripheral neuropathy Neuropathic progression |
| url | https://doi.org/10.1186/s12883-021-02094-y |
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