Vasculitis profile, ANA, ANA profile, SS-A, SS-B and autoimmune encephalitis profile were negative. Further MRI imaging of spine and orbit revealed no abnormalities. 1) in stark contrast to the MRI brain that was done 4 years back which showed few indistinct scattered foci of altered signal changes in the superficial white matter of parietal lobe (Fig. Magnetic Resonance Imaging (MRI) Brain revealed extensive and scattered altered signal in bilateral superficial and deep white matters including the corpus callosum, iso-intense in T1-weighted image (T1WI), hyper-intense in T2-weighted image (T2WI), T2-fluid-attenuated-inverse-recovery (FLAIR), without any restricted diffusion or enhancement with contrast (Fig. Cerebrospinal fluid (CSF) analysis showed 3 cells, protein- 38 mg/dL (normal: 10–50 mg/dL) glucose- 66 mg/dL (corresponding serum glucose: 90 mg/dL), negative neuroviral-panel (Zoster, Ebstein–Barr, Herpes simplex, cytomegalovirus, adenovirus, enterovirus, coxsackie B virus, and herpes virus 6, Japanese B virus). Routine blood investigations were unrevealing. Neurological examination was unremarkable, apart from gaze evoked nystagmus. There was history of one episode right focal motor seizure with secondary generalization 4 years back, after which the patient was prescribed anti-epileptic drug (AED), however, was self-discontinued after 2 months. Authors herein intend to present a case of 16-year-old adolescent boy with sole manifestation of episodes of focal seizures 4 years apart, finally diagnosed to be a case of MOGAD, which not only boosts the evidence of establishing the possibility of MOG antibody-associated autoimmune epilepsy but also reinforces the importance of unexplained seizure as a clinical phenotype in MOGAD.Ī 16-year-old-male,with no prior co-morbidities, presented with a single episode of sudden facial deviation to left with neck deviation followed by tonic–clonic movement of all limbs with impaired awareness lasting for 5–6 min, with up-rolling of eyeball and frothing from mouth with a post-ictal confusion for 10 min, without any focal neurological deficits (FND). Pathogenesis is still unclear, though Myelin oligodendrocyte (MOG) antibody-associated autoimmune epilepsy may be one of the plausible mechanism underlying. Recently, there are evidences to correlate the presence of such antibodies with seizures, occurring in association with CNS demyelination, or even as isolated phenomena. Studies have revealed that MOGAD presented with seizures or an encephalitis-like illness more commonly than patients with AQP4-positive Neuromyelitis optica spectrum disorder (NMOSD), which is another acquired primary CNS demyelinating disorder having clinical phenotype similarity with MOGAD. MOGAD is a distinct primary CNS demyelinating disorder with seizure and encephalopathy being common manifestations. With the advancement of our understanding about primary demyelinating disorder of central nervous system (CNS) which also includes Myelin oligodendrocyte associated disease (MOGAD), seizure has becomes an integral part of diagnosis. Seizure as presenting manifestation or as an early diagnostic clue in primary demyelinating disorder like MS is rare. This case of an adolescent boy with sole manifestation of episodes of focal seizures 4 years apart, finally diagnosed to be a case of MOGAD, not only boosts the evidence of establishing the possibility of MOG antibody-associated autoimmune epilepsy but also reinforces the importance of unexplained seizure as a clinical phenotype in MOGAD. The patient received steroids and Rituximab therapy without any further recurrence of seizure or any neuro-deficits and gradual improvement in lesion burden in brain imaging. Brain imaging revealed bilateral superficial and deep white matter lesions including the corpus callosum. Neurological examination showed bilateral gaze evoked nystagmus. ![]() There was a history of right focal onset motor seizure with secondary generalized tonic–clonic seizure and impaired awareness 4 years ago. Case presentationĪ 16-year-old-male presented with single episode of left focal onset motor seizure with secondary generalization and impaired awareness, without any other focal neurological deficits. ![]() Isolated seizure as a manifestation of myelin oligodendrocyte antibody-associated disease (MOGAD) has rarely been reported previously.
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