Author(s): Huda S.; Whittam D.; Bhojak M.; Das K.; Jacob A.; Kneen R.

Source: Developmental Medicine and Child Neurology; Jan 2019; vol. 61 ; p. 73-74

Publication Date: Jan 2019

Publication Type(s): Conference Abstract

Available  at Developmental Medicine and Child Neurology –  from Wiley Online Library Full Collection

Abstract:Introduction: The most common presentation of MOG-IgG in children is acute disseminated encephalomyelitis (ADEM). MOG-IgG has been reported in adults with cortical encephalitis but similar cases in children have not been described. Case report: A 6-year old girl was admitted with headaches, speech disturbance, altered consciousness, and a right-sided focal seizure following a coryzal illness. She was treated with anti-epileptics, broad-spectrum antibiotics and aciclovir. Her MR brain demonstrated bilateral multifocal non-enhancing cortical T2 hyperintensities with diffusion restriction. MR spectroscopy supported an inflammatory aetiology. Her EEG recorded infrequent right central focal spikes with diffuse slowing (1-2 Hz), particularly over the right hemisphere. Her CSF demonstrated a monocytic pleocytosis (14 white blood cells) with matched oligoclonal bands. CSF bacteriology/virology were negative as was screening for mitochondrial encephalopathy with lactic acidosis and stroke like episodes. Her serum was positive for MOG-IgG but she had already started to improve clinically. Four months later there were no signs of physical or cognitive sequela. Her follow-up MR demonstrated gliosis in the right occipital and left occipitalparietal regions and repeat MOG-IgG was positive. Discussion(s): The sparing of white matter and absence of contrast enhancement were atypical for ADEM although the clinical course was typical with a good recovery reported to date. Although an MR angiogram was not performed, MR spectroscopy findings were more consistent with inflammation than ischaemia and vasculitis is also less common than a demyelinating disorder in children. The persistence of MOGIgG supports a non-multiple sclerosis disease course and may have prognostic implications. Long term immunosuppression has been discussed but a wait and see policy has been adopted. Conclusion(s): This case suggests that MOG-IgG maybe associated with a multifocal cortical encephalitic syndrome in children. The long term follow up will be carefully monitored and documented.

Database: EMBASE


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