Myelopathy in sickle cell disease: a case-oriented review

Introduction Although neurological complications are well recognized in patients with sickle cell disease, myelopathy has been rarely described, with few reported cases of compressive and ischemic myelopathy. We present the first case report of longitudinally extensive myelitis (LETM) in SCD and rev...

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Bibliographic Details
Published in:Spinal cord series and cases 2021-09, Vol.7 (1), p.85-85, Article 85
Main Authors: Brum, Igor Vilela, Silva, Guilherme Diogo, Sodre, Diego Sant’Ana, Nogueira, Felipe Melo, Pereira, Samira Luisa dos Apostolos, Castro, Luiz Henrique Martins
Format: Article
Language:English
Subjects:
692/699/375/1411
692/699/375/1824
Abscesses
Anatomy
Antibodies
Biomedical and Life Sciences
Biomedicine
Blood
Case Report
Chicken pox
Epidural
Hospitals
Human Physiology
Ischemia
Neurochemistry
Neuropsychology
Neurosciences
Patients
Pressure ulcers
Proteins
Salmonella
Sickle cell disease
Spinal cord
Urinary retention
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Summary:Introduction Although neurological complications are well recognized in patients with sickle cell disease, myelopathy has been rarely described, with few reported cases of compressive and ischemic myelopathy. We present the first case report of longitudinally extensive myelitis (LETM) in SCD and review the differential diagnosis of myelopathy in these patients. Case presentation We report the case of a 29-year-old African-Brazilian man with SCD, who experienced a subacute flaccid paraparesis, with T2 sensory level and urinary retention. Cerebrospinal fluid analysis showed a lymphocytic pleocytosis and increased protein levels. MRI disclosed a longitudinally extensive spinal cord lesion, with a high T2/STIR signal extending from C2 to T12. We searched Medline/PubMed, Embase, Scopus, and Google Scholar databases for myelopathy in SCD patients. Discussion Spinal cord compression by vertebral fractures, extramedullary hematopoietic tissue, and Salmonella epidural abscess have been reported in SCD. We found only three case reports of spinal cord infarction, which is unexpectedly infrequent compared to the prevalence of cerebral infarction in SCD. We found only one case report of varicella-zoster myelitis and no previous report of LETM in SCD patients. Specific and time-sensitive causes of myelopathy should be considered in SCD patients. In addition to compression and ischemia, LETM is a possible mechanism of spinal cord involvement in SCD patients.
ISSN:2058-6124
2058-6124
DOI:10.1038/s41394-021-00449-8