A Case of Neuromyelitis Optica: Puerto Rican Woman with an Increased Time Lag to Diagnosis and a High Response to Eculizumab Therapy

A link between intractable hiccups, as the initial symptom, and a possible neuromyelitis optica spectrum disorder (NMOSD) diagnosis is confusing but vital and may not be made by health care providers (HCPs) if they are not aware of the 2015 NMOSD criteria. Early diagnosis and adequate treatment are...

Full description

Saved in:
Bibliographic Details
Main Authors: Ramón Vega, Benjamín González, Kiara Ortiz, Viviana Martínez, David Carmona, Ivonne Vicente, Javier Chapa, Ángel Chinea
Format: Article
Language:English
Published: Wiley 2022-01-01
Series:Case Reports in Neurological Medicine
Online Access:http://dx.doi.org/10.1155/2022/4311382
Tags: Add Tag
No Tags, Be the first to tag this record!
_version_ 1832564057971884032
author Ramón Vega
Benjamín González
Kiara Ortiz
Viviana Martínez
David Carmona
Ivonne Vicente
Javier Chapa
Ángel Chinea
author_facet Ramón Vega
Benjamín González
Kiara Ortiz
Viviana Martínez
David Carmona
Ivonne Vicente
Javier Chapa
Ángel Chinea
author_sort Ramón Vega
collection DOAJ
description A link between intractable hiccups, as the initial symptom, and a possible neuromyelitis optica spectrum disorder (NMOSD) diagnosis is confusing but vital and may not be made by health care providers (HCPs) if they are not aware of the 2015 NMOSD criteria. Early diagnosis and adequate treatment are essential to prevent disease progression. We report the case of a 46-year-old Puerto Rican female who presented intractable hiccups when she was 31 (in 2004). Almost 15 years passed since the initial symptom, and after two severe relapses, she received a formal NMOSD diagnosis in March 2019. Treatment started with rituximab 1000 mg IV in April 2019. However, a lack of response to treatment led to a switch to eculizumab therapy in August 2019. The patient had cervical and brain magnetic resonance imaging (MRI) conducted in June 2020, which depicted a remarkable decrease in swelling and hyperintensity within the cervical spinal cord with no enhancing lesions when compared with the first MRI from February 2019. In addition, the patient suffered no new relapses, an improvement regarding disability, and a reduction of the cervical spinal cord lesion size. Nonetheless, this substantial decrease does not occur on all NMOSD patients, but more awareness of the disease is needed, especially in Puerto Rico. This case illustrates the efficacy of eculizumab therapy and the importance of differentiating the clinical, histopathological, and neuroimaging characteristics that separate demyelinating autoimmune inflammatory disorders, such as NMOSD and multiple sclerosis (MS).
format Article
id doaj-art-63af17f452874eb4ac485b15ce30d418
institution Kabale University
issn 2090-6676
language English
publishDate 2022-01-01
publisher Wiley
record_format Article
series Case Reports in Neurological Medicine
spelling doaj-art-63af17f452874eb4ac485b15ce30d4182025-02-03T01:11:55ZengWileyCase Reports in Neurological Medicine2090-66762022-01-01202210.1155/2022/4311382A Case of Neuromyelitis Optica: Puerto Rican Woman with an Increased Time Lag to Diagnosis and a High Response to Eculizumab TherapyRamón Vega0Benjamín González1Kiara Ortiz2Viviana Martínez3David Carmona4Ivonne Vicente5Javier Chapa6Ángel Chinea7Puerto Rico Multiple Sclerosis FoundationSan Juan Bautista School of MedicineSan Juan Bautista School of MedicineSan Juan MS CenterSan Juan MS CenterSan Juan MS CenterNeurologist, Neurophysiologist, Epileptologist, and NeuroimagerSan Juan MS CenterA link between intractable hiccups, as the initial symptom, and a possible neuromyelitis optica spectrum disorder (NMOSD) diagnosis is confusing but vital and may not be made by health care providers (HCPs) if they are not aware of the 2015 NMOSD criteria. Early diagnosis and adequate treatment are essential to prevent disease progression. We report the case of a 46-year-old Puerto Rican female who presented intractable hiccups when she was 31 (in 2004). Almost 15 years passed since the initial symptom, and after two severe relapses, she received a formal NMOSD diagnosis in March 2019. Treatment started with rituximab 1000 mg IV in April 2019. However, a lack of response to treatment led to a switch to eculizumab therapy in August 2019. The patient had cervical and brain magnetic resonance imaging (MRI) conducted in June 2020, which depicted a remarkable decrease in swelling and hyperintensity within the cervical spinal cord with no enhancing lesions when compared with the first MRI from February 2019. In addition, the patient suffered no new relapses, an improvement regarding disability, and a reduction of the cervical spinal cord lesion size. Nonetheless, this substantial decrease does not occur on all NMOSD patients, but more awareness of the disease is needed, especially in Puerto Rico. This case illustrates the efficacy of eculizumab therapy and the importance of differentiating the clinical, histopathological, and neuroimaging characteristics that separate demyelinating autoimmune inflammatory disorders, such as NMOSD and multiple sclerosis (MS).http://dx.doi.org/10.1155/2022/4311382
spellingShingle Ramón Vega
Benjamín González
Kiara Ortiz
Viviana Martínez
David Carmona
Ivonne Vicente
Javier Chapa
Ángel Chinea
A Case of Neuromyelitis Optica: Puerto Rican Woman with an Increased Time Lag to Diagnosis and a High Response to Eculizumab Therapy
Case Reports in Neurological Medicine
title A Case of Neuromyelitis Optica: Puerto Rican Woman with an Increased Time Lag to Diagnosis and a High Response to Eculizumab Therapy
title_full A Case of Neuromyelitis Optica: Puerto Rican Woman with an Increased Time Lag to Diagnosis and a High Response to Eculizumab Therapy
title_fullStr A Case of Neuromyelitis Optica: Puerto Rican Woman with an Increased Time Lag to Diagnosis and a High Response to Eculizumab Therapy
title_full_unstemmed A Case of Neuromyelitis Optica: Puerto Rican Woman with an Increased Time Lag to Diagnosis and a High Response to Eculizumab Therapy
title_short A Case of Neuromyelitis Optica: Puerto Rican Woman with an Increased Time Lag to Diagnosis and a High Response to Eculizumab Therapy
title_sort case of neuromyelitis optica puerto rican woman with an increased time lag to diagnosis and a high response to eculizumab therapy
url http://dx.doi.org/10.1155/2022/4311382
work_keys_str_mv AT ramonvega acaseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy
AT benjamingonzalez acaseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy
AT kiaraortiz acaseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy
AT vivianamartinez acaseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy
AT davidcarmona acaseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy
AT ivonnevicente acaseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy
AT javierchapa acaseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy
AT angelchinea acaseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy
AT ramonvega caseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy
AT benjamingonzalez caseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy
AT kiaraortiz caseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy
AT vivianamartinez caseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy
AT davidcarmona caseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy
AT ivonnevicente caseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy
AT javierchapa caseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy
AT angelchinea caseofneuromyelitisopticapuertoricanwomanwithanincreasedtimelagtodiagnosisandahighresponsetoeculizumabtherapy