8/30/2023 0 Comments Anti mog antibody![]() These studies have demonstrated improvement in annualized relapse rate with the use of mycophenolate, azathioprine, rituximab, and IVIG, and a poor response to standard injectable DMTs used to treat MS, such as interferons and glatiramer acetate. In children with relapsing disease, studies suggest use of intravenous immunoglobulin (IVIG) and/or disease modifying therapies (DMTs). In the acute setting, most patients respond well to intravenous steroids followed by a gradual steroid taper over 3 to 4 weeks, though some centers recommend a prolonged steroid taper of up to 6 months. The optimal treatment strategy for relapsing disease is currently unknown. The most common residual deficits are visual impairment, erectile dysfunction, and bladder sphincter dysfunction. Complete recovery from the onset attack is common in children both clinically and radiographically, and in general the outcome is favorable. The disease course is most commonly monophasic but relapses can occur. Neuro-imaging of the spine characteristically shows longitudinally extensive lesions that can affect the cervical cord, but most frequently involves the lower cord, including the conus medullaris. Lesions of the corpus callosum, juxtacortical lesions, and well-defined ovoid lesions are uncommonly described. Neuro-imaging of the brain classically reveals poorly demarcated, widespread lesions, or confluent white matter changes, but can also be normal. ![]() CSF oligoclonal bands are not typically identified. CSF typically demonstrates a pleocytosis with variable protein elevation. Neurodiagnostically, patterns have emerged to distinguish this disorder from other demyelinating diseases. Most studies have found a bimodal distribution of clinical phenotypes, with younger patients predominantly presenting with ADEM and older children presenting with optic neuritis. The most common clinical phenotype of MOG-antibody associated disorders in children is ADEM, followed by optic neuritis and transverse myelitis. The precise function of MOG is poorly understood however, it likely plays a role in myelin maturation, myelin integrity, and cell surface interactions. MOG is a myelin glycoprotein that is exclusively expressed in the central nervous system and has been identified in about one-third of children diagnosed with an acquired demyelinating syndrome (ADS). MOG-antibody associated disease is an emerging demyelinating disorder that is characterized by a broad range of clinical phenotypes and neuroimaging findings with a disease course that is distinct from multiple sclerosis (MS). The combination of subacute encephalopathy with ataxia and hyperreflexia, neuro-imaging consistent with a demyelinating process, and serum MOG-antibody positivity led to the diagnosis of MOG-antibody associated encephalomyelitis. Serum antibodies to myelin oligodendrocyte glycoprotein (MOG) were reported positive after the patient was discharged. She was treated with high-dose intravenous steroids followed by a steroid taper leading to significant clinical improvement. ![]() The differential diagnosis included acute disseminated encephalomyelitis (ADEM), neuromyelitis optica (NMO), viral encephalomyelitis, and anti-NMDA receptor encephalitis. MRI of the brain and spine revealed diffuse patchy areas of abnormal signal in the cerebral peduncles, bilateral thalami, and cortical/subcortical areas bilaterally (Figure 3 A-C) and a longitudinally extensive spinal cord lesion from C2-T2, as well as involvement of the conus medullaris (Figure 3 D) - all suggestive of an inflammatory process.įigure 3 (above): MRI of the brain and spine T2 Axial FLAIR imaging demonstrating bilateral cortical T2 hyperintensities (A), left thalamic T2 hyperintensity (B), bilateral cerebral peduncle hyperintensities (C), and spinal imaging revealing a longitudinally extensive lesion predominantly affecting the grey matter from C2-T2 (D) A lumbar puncture showed an elevated white blood cell count of 225 (lymphocytic predominance), red blood cell count of 56, elevated protein of 55, and normal glucose. Her neurologic exam was notable for meningismus, hyperreflexia, bilateral sustained ankle clonus, dysmetria, and ataxia. Over the course of 3 weeks prior to hospitalization, her mother noticed a change in her gait and behavior in addition to episodes of headaches and emesis, which seemed to begin after a minor trauma.įigure 3 (A-D clockwise starting in upper left) During her hospitalization, she became increasingly agitated, with episodes of increased sleepiness, prompting neurologic consultation for encephalopathy. A 5-year-old female with history of behavioral issues, constipation, and mild developmental delays presents for medical evaluation of headaches, gait abnormality, generalized weakness, and weight loss.
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |