A few weeks ago, we published a blog on SRNA-supported research that was presented at the 70th American Academy of Neurology Annual Meeting. Below is other research on rare neuroimmune disorders that was presented at the meeting.
A team from Johns Hopkins University, Johns Hopkins TM Center, and the Kennedy Krieger Institute presented research that aimed to investigate the differential diagnosis of pediatric myelopathy at the Johns Hopkins TM Center. They looked at the records of 55 children (under age 21) who had been seen at the Center between 2015 and 2017. They reviewed the temporal profile of symptoms, clinical presentation, and cerebrospinal fluid (CSF) analysis results of these children. They then compared the clinical features of non-inflammatory and inflammatory myelopathies. 71% of the cases they reviewed were inflammatory. Most of the patients presented with infectious (para-infectious and post-infectious) myelopathies (n=23, 42%), followed by vascular (n=15, 27%), autoimmune demyelinating (n=13, 24%) (including idiopathic transverse myelitis (n=3), neuromyelitis optica (NMO) spectrum (n=3), multiple sclerosis (n=7)) and of uncertain etiology (n=4, 7%). Those with non-inflammatory myelopathies were significantly more likely than those with inflammatory myelopathies to have a hyperacute onset (that is, less than six hours from onset to their peak neurological symptoms), sphincter dysfunction, lower extremity weakness, and symmetric involvement (meaning both sides of the body). Those with inflammatory myelopathies were significantly more likely than those with non-inflammatory myelopathies to have a preceding infection, cranial nerve involvement, and an increased white blood cell count in their cerebrospinal fluid.
Additional research was presented on Chiari malformations and TM. Chiari malformation is a condition in which brain tissue extends into the spinal canal. The researchers looked at patients (under age 21) who were referred to the Johns Hopkins TM Center between 2010 and 2017, and who had a diagnosis of TM and Chiari malformation. Of the 86 patients seen at the TM Center, seven patients (8%) with TM and Chiari malformation were identified. Six of seven (88%) were male. They all presented with hyperacute onset flaccid quadraparesis associated with longitudinally extensive cervical myelopathy. The prevalence of Chiari malformation in this study was eight times that of the prevalence of Chiari malformation in the general pediatric population reported in prior studies. The researchers believe these cases are not inflammatory but are vascular instead.
Maureen Mealy and Dr. Michael Levy from Johns Hopkins University School of Medicine, and Dr. Philippines Cabahug from the Kennedy Krieger Institute presented research on sexual quality of life in women with NMOSD. They conducted an analysis of women with NMOSD from the Johns Hopkins NMO Clinic Facebook Group, and online patient community. These women completed the Sexual Quality of Life-Female Questionnaire (SQOL-F), an 18-item assessment of the impact of sexual dysfunction on quality of life in women. They compared their data to healthy controls and to data from women with traumatic spinal cord injury (SCI). They found that sexual quality of life was significantly worse in women with NMOSD than women with SCI or the healthy controls.
We hope to see additional research presented at upcoming conferences and look forward to sharing the findings with you. For more information on this and other research, please visit https://wearesrna.org/shaping-the-future/research/.
Munoz LS, Gordon-Lipkin E, Barreras P, Gómez S, Piedra W, Castañeda-Valderrama MJ, Yeshokumar A, Mealy M, Murphy O, Levy M, Becker D, Newsome S, Pardo CA. The spectrum of myelopathies in children: Beyond idiopathic transverse myelitis. Poster presented at: 2018 American Academy of Neurology Annual Meeting; April 2018; Los Angeles, CA.
Gordon-Lipkin E, Munoz L, Barreras P, Castañeda M, Gailloud P, Pardo CA. Chiari malformations associated with transverse myelitis: Are these myelopathies truly inflammatory? Poster presented at: 2018 American Academy of Neurology Annual Meeting; April 2018; Los Angeles, CA.
Mealy MA, Cabahug P, Levy M. Impaired sexual quality of life in women with neuromyelitis optica spectrum disorder. Poster presented at: 2018 American Academy of Neurology Annual Meeting; April 2018; Los Angeles, CA.