The redefining of neuromyelitis optica spectrum disorder (NMOSD) in 2006 has revealed cases with unique and previously unrecognised presentations. We describe a case of an NMOSD relapse in which seizures were the main clinical feature.
A twenty three year old female first presented in 2006, then thirteen with hyponatraemia and bilateral, severe optic neuritis, in the context of hypothalamic and optic chiasm lesions on MRI brain. Central pontine myelinosis was initially diagnosed but this was revised to an acute disseminated encephalomyelitis (ADEM) in 2013. She remained well for nine years albeit with residual neurological disabilities, including mild spastic paraparesis, global hyperreflexia and bilateral extensor plantar responses. She had almost complete loss of vision in both eyes with peripheral sparing evidenced by bilateral optic atrophy. In September 2015 she presented with generalised seizures. MRI brain showed contrast enhancement in the right cingulate gyrus (DWI negative). Cerebrospinal fluid revealed a lymphocytic pleocytosis (white cells 28; 88% lymphocytes), mildly raised protein (0.75) and normal glucose. Other than aquaporin 4 antibody (AQ4Ab) seropositivity, full investigative work up was normal. She was commenced on Levetiracetam, a short course of intravenous methylprednisolone (500 mg) and subsequent high dose, reducing regime of oral prednisolone. She had no further seizures and follow up MRI brain and CSF results were normal.
This case adds to the widening spectrum of NMOSD presentations and to our knowledge, seizures have not been described before. Recognition of atypical NMOSD presentations leads to timely diagnosis and effective treatment. Past diagnoses of recurrent ADEM should be revisited and we would suggest AQ4Ab testing is performed.
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