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Rare case of multifocal extradural and intramedullary neurosarcoidosis without pulmonary involvement: a case report and literature review

INTRODUCTION: Sarcoidosis is a multisystem disease characterized histologically by noncaseating granulomas. Localization of sarcoidosis to the CNS is termed neurosarcoidosis, a complex and rare neuroinflammatory form of sarcoidosis. When the spinal cord is involved, lesions are often intradural. Her...

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Autores principales: Shields, Ryan, Sagan, Olivia, Roebke, Logan, Vander Maten, Josh, Shah, Shailen, Chang, George, Ibrahim, Dalia, Naz, Sumayya
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Nature Publishing Group UK 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8477626/
https://www.ncbi.nlm.nih.gov/pubmed/34584071
http://dx.doi.org/10.1038/s41394-021-00450-1
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author Shields, Ryan
Sagan, Olivia
Roebke, Logan
Vander Maten, Josh
Shah, Shailen
Chang, George
Ibrahim, Dalia
Naz, Sumayya
author_facet Shields, Ryan
Sagan, Olivia
Roebke, Logan
Vander Maten, Josh
Shah, Shailen
Chang, George
Ibrahim, Dalia
Naz, Sumayya
author_sort Shields, Ryan
collection PubMed
description INTRODUCTION: Sarcoidosis is a multisystem disease characterized histologically by noncaseating granulomas. Localization of sarcoidosis to the CNS is termed neurosarcoidosis, a complex and rare neuroinflammatory form of sarcoidosis. When the spinal cord is involved, lesions are often intradural. Here, we present a rare case of progressive myelopathy secondary to multifocal spinal extradural neurosarcoidosis with spinal cord compression and without pulmonary involvement. CASE PRESENTATION: A 29-year-old African American female presented to the emergency department with numbness and paresthesia of 2-month duration in her left lower extremity and 2-week duration in her right lower extremity. The patient reported difficulty ambulating, paresthesia below the umbilicus, and back pain radiating to bilateral lower extremities. She endorsed 9-month history of cough, subjective fevers, night sweats, and unintentional 15 kg weight loss. Examination revealed 4/5 strength in the left lower extremity. MRI of the brain and spinal cord revealed enhancing extradural lesions, with spinal cord compression at T8 measuring 1.3 × 1.9 cm. Lumbar puncture demonstrated oligoclonal bands and increased CSF neutrophils, lymphocytes, monocytes, and protein. T8 laminectomy with resection of the epidural lesion was performed. Histology showed granulomas, consistent with neurosarcoidosis. At follow-up, repeat spinal MRI revealed disease progression with intramedullary involvement. Long-term immunosuppressive treatment was eventually initiated with satisfactory response. DISCUSSION: This is a rare case of myelopathy secondary to spinal extradural neurosarcoidosis. Spinal neurosarcoidosis is predominantly an intradural process. Our review of the literature identified only seven cases of extradural neurosarcoidosis presenting with compressive myelopathy. Additional insight into management and rehabilitation following pathological diagnosis is of clinical significance.
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spelling pubmed-84776262021-09-28 Rare case of multifocal extradural and intramedullary neurosarcoidosis without pulmonary involvement: a case report and literature review Shields, Ryan Sagan, Olivia Roebke, Logan Vander Maten, Josh Shah, Shailen Chang, George Ibrahim, Dalia Naz, Sumayya Spinal Cord Ser Cases Case Report INTRODUCTION: Sarcoidosis is a multisystem disease characterized histologically by noncaseating granulomas. Localization of sarcoidosis to the CNS is termed neurosarcoidosis, a complex and rare neuroinflammatory form of sarcoidosis. When the spinal cord is involved, lesions are often intradural. Here, we present a rare case of progressive myelopathy secondary to multifocal spinal extradural neurosarcoidosis with spinal cord compression and without pulmonary involvement. CASE PRESENTATION: A 29-year-old African American female presented to the emergency department with numbness and paresthesia of 2-month duration in her left lower extremity and 2-week duration in her right lower extremity. The patient reported difficulty ambulating, paresthesia below the umbilicus, and back pain radiating to bilateral lower extremities. She endorsed 9-month history of cough, subjective fevers, night sweats, and unintentional 15 kg weight loss. Examination revealed 4/5 strength in the left lower extremity. MRI of the brain and spinal cord revealed enhancing extradural lesions, with spinal cord compression at T8 measuring 1.3 × 1.9 cm. Lumbar puncture demonstrated oligoclonal bands and increased CSF neutrophils, lymphocytes, monocytes, and protein. T8 laminectomy with resection of the epidural lesion was performed. Histology showed granulomas, consistent with neurosarcoidosis. At follow-up, repeat spinal MRI revealed disease progression with intramedullary involvement. Long-term immunosuppressive treatment was eventually initiated with satisfactory response. DISCUSSION: This is a rare case of myelopathy secondary to spinal extradural neurosarcoidosis. Spinal neurosarcoidosis is predominantly an intradural process. Our review of the literature identified only seven cases of extradural neurosarcoidosis presenting with compressive myelopathy. Additional insight into management and rehabilitation following pathological diagnosis is of clinical significance. Nature Publishing Group UK 2021-09-28 /pmc/articles/PMC8477626/ /pubmed/34584071 http://dx.doi.org/10.1038/s41394-021-00450-1 Text en © The Author(s), under exclusive licence to International Spinal Cord Society 2021
spellingShingle Case Report
Shields, Ryan
Sagan, Olivia
Roebke, Logan
Vander Maten, Josh
Shah, Shailen
Chang, George
Ibrahim, Dalia
Naz, Sumayya
Rare case of multifocal extradural and intramedullary neurosarcoidosis without pulmonary involvement: a case report and literature review
title Rare case of multifocal extradural and intramedullary neurosarcoidosis without pulmonary involvement: a case report and literature review
title_full Rare case of multifocal extradural and intramedullary neurosarcoidosis without pulmonary involvement: a case report and literature review
title_fullStr Rare case of multifocal extradural and intramedullary neurosarcoidosis without pulmonary involvement: a case report and literature review
title_full_unstemmed Rare case of multifocal extradural and intramedullary neurosarcoidosis without pulmonary involvement: a case report and literature review
title_short Rare case of multifocal extradural and intramedullary neurosarcoidosis without pulmonary involvement: a case report and literature review
title_sort rare case of multifocal extradural and intramedullary neurosarcoidosis without pulmonary involvement: a case report and literature review
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8477626/
https://www.ncbi.nlm.nih.gov/pubmed/34584071
http://dx.doi.org/10.1038/s41394-021-00450-1
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