Neuromyelitis optica spectrum disorders

Huda, Saif; Whittam, Dan; Bhojak, Maneesh; Chamberlain, Jayne L.; Noonan, Carmel; Jacob, Anu; Kneen, Rachel

doi:10.7861/clinmedicine.19-2-169

Cited by 221 publications

(212 citation statements)

References 61 publications

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“…Notably, NMO and neuromyelitis optica spectrum disorders (NMOSD) were included in the differential diagnosis, because of the uncommon combination of optic nerve involvement with LETM. However, the core clinical characteristics for NMOSD including the production of antibodies against AQP − 4, an acute type of transverse myelitis, severe optic neuritis revealed on MRI, area postrema syndrome (APS), brainstem and diencephalic syndromes were absent [21,22]. Because of the time in which the transverse myelitis developed (weeks), the subacute subtype of transverse myelitis was diagnosed.…”

Section: Discussionmentioning

confidence: 99%

Subacute transverse myelitis with optic symptoms in neuroborreliosis: a case report

Opiełka¹,

Opielka²,

Sobocki³

et al. 2020

BMC Neurol

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Background: Subacute transverse myelitis is one of the late manifestations of neuroborreliosis with only a few cases described to the present day. Case presentation: We present magnetic resonance imaging, cerebrospinal fluid, and electroneurography findings of a young female patient suffering from neuroborreliosis-associated transverse myelitis with a wide constellation of symptoms including papilloedema. Magnetic resonance imaging of the cervical spine has shown an enlargement of the spinal cord in the mid-cervical region. Cerebrospinal fluid findings included lymphocytic pleocytosis, increased levels of anti-Borrelia antibodies, and increased intrathecal anti-Borrelia antibody index. Following the 28-day course of intravenous ceftriaxone, the patient attained complete recovery. Conclusions: Subacute transverse myelitis in the course of neuroborreliosis should be considered in the differential diagnosis of patients with abnormal magnetic resonance scans of the spinal cord, lymphocytic pleocytosis, and intrathecal antibody production, especially in the tick-endemic areas, even if the tick bite was not reported. Infrequent accompanying symptoms such as papilloedema are diagnostically challenging and cannot be treated as clinching evidence.

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Section: Discussionmentioning

confidence: 99%

Subacute transverse myelitis with optic symptoms in neuroborreliosis: a case report

Opiełka¹,

Opielka²,

Sobocki³

et al. 2020

BMC Neurol

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“…Acute brainstem syndrome can present with multiple cranial nerve palsies. 3 MRI of the brain remains the mainstay of neuroimaging for NMOSD. MRI abnormalities are often located at areas with high AQP4 expression.…”

Section: Discussionmentioning

confidence: 99%

Images of the month: Intractable vomiting and neuromyelitis optica spectrum disorder

Cheo¹,

Low

Tan

et al. 2020

Clinical Medicine

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“…In the case of neuromyelitis optica spectrum disorders, another type of autoimmune demyelinating disease, the optic nerve and spinal cord are preferentially damaged. 45 . In EAE, a “classical” spinal cord phenotype (ascending flaccid paralysis) can be distinguished from “atypical” EAE with inflammation localized to the cerebrum, cerebellum and brain stem.…”

Section: Discussionmentioning

confidence: 99%

Pericytes regulate vascular immune homeostasis in the CNS

Torok

Schreiner

Tsai

et al. 2019

Preprint

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21Brain endothelium possesses several organ-specific features collectively known as the blood-22 brain barrier (BBB). In addition, trafficking of immune cells in the healthy central nervous 23 system (CNS) is tightly regulated by CNS vasculature. In CNS autoimmune diseases such as 24 multiple sclerosis (MS), these homeostatic mechanisms are overcome by autoreactive 25 2 lymphocyte entry into the CNS causing inflammatory demyelinating immunopathology. 26Previous studies have shown that pericytes regulate the development of organ-specific 27 characteristics of brain vasculature such as the BBB and astrocytic end-feet. Whether pericytes 28 are involved in the control of leukocyte trafficking remains elusive. Using adult, pericyte-29 deficient mice (Pdgfb ret/ret ), we show here that brain vasculature devoid of pericytes shows 30 increased expression of VCAM-1 and ICAM-1, which is accompanied by increased leukocyte 31 infiltration of dendritic cells, monocytes and T cells into the brain, but not spinal cord 32 parenchyma. Regional differences enabling leukocyte trafficking into the brain as opposed to 33 the spinal cord inversely correlate with the pericyte coverage of blood vessels. Upon induction 34 of experimental autoimmune encephalitomyelitis (EAE), pericyte-deficient mice succumb to 35 severe neurological impairment. Treatment with first line MS therapy -fingolimod significantly 36 reverses EAE, indicating that the observed phenotype is due to the massive influx of immune 37 cells into the brain. Furthermore, pericyte-deficiency in mice that express myelin 38 oligodendrocyte glycoprotein peptide (MOG35-55) specific T cell receptor (Pdgfb ret/ret ; 2D2 Tg ) 39 leads to the development of spontaneous neurological symptoms paralleled by massive influx 40 of leukocytes into the brain, suggesting altered brain vascular immune quiescence as a prime 41 cause of exaggerated neuroinflammation. Thus, we show that pericytes indirectly restrict 42 immune cell transmigration into the CNS under homeostatic conditions and during 43 autoimmune-driven neuroinflammation by inducing immune quiescence of brain endothelial 44 cells. 45 46 leukocytes into brain parenchyma, thus contributing to immune privilege of the CNS. BBB 51 function is induced by neural tissue and established by all cell types constituting the 52 neurovascular unit (NVU). Pericytes and mural cells residing on the abluminal side of 53 capillaries and post-capillary venules, regulate several features of the BBB 1, 2 . Studies on Pdgfb 54and Pdgfrb mouse mutants, which exhibit variable pericyte loss, have demonstrated that 55 pericytes negatively regulate endothelial transcytosis which, if not suppressed, leads to 56 increased BBB permeability to plasma proteins 1, 2 . In addition, pericyte-deficient vessels show 57 abnormal astrocyte end-feet polarization 1 . Thus, pericytes regulate several characteristics of 58 brain vasculature during development and in the adult organism 1, 2 . Whether the non-59 permissive properties of brain vasculature to leukocyte traffic...

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Neuromyelitis optica spectrum disorders

Cited by 221 publications

References 61 publications

Subacute transverse myelitis with optic symptoms in neuroborreliosis: a case report

Subacute transverse myelitis with optic symptoms in neuroborreliosis: a case report

Images of the month: Intractable vomiting and neuromyelitis optica spectrum disorder

Pericytes regulate vascular immune homeostasis in the CNS

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