The Calm Before the Storm? An Unusual Case of Measles Retinopathy.

A 30-year-old male presented with sudden painless loss of vision in the right eye for the last two days. Slit-lamp examination of the right eye revealed a quiet anterior chamber and anterior vitreous. Fundus examination of the right eye revealed a large, focal retinitis lesion centered around the fovea, whereas examination of the left was unremarkable. He gave us a history of Measles at the age of 5 years but denied any history of systemic illness. Optical coherence tomography (OCT) revealed disruption of retinal architecture with ballooning of intact internal limiting membrane. His serology was negative for syphilis, human immunodeficiency virus and toxoplasmosis. Based on clinical suspicion, he was investigated by a neurologist. His electroencephalogram and Magnetic Resonance Imaging of brain were within normal limits, but high titres of anti-measles antibodies were found in serum and cerebrospinal fluid. The diagnosis of measles retinopathy should be considered in cases with focal necrotizing retinitis, even when classical findings of CNS involvement do not exist.

Super refractory status in a case of Febrile Infection-Related Epilepsy Syndrome due to hemophagocytic lymphocytic histiocytosis.

A 14-year-old boy presented with a prodromal respiratory infection followed by super refractory status epilepticus. A diagnosis of Febrile Infection-Related Epilepsy Syndrome (FIRES) was made. Initial MRI study and CSF analysis were normal. He required multiple anticonvulsants owing to the refractory nature of the seizures. The course of the illness was rather stormy, laced with various medical problems viz. hepatic dysfunction, sepsis, hemodynamic, and hematological abnormalities which posed several challenges in the management. Hemophagocytic lymphocytic histiocytosis (HLH) was identified as the etiology of the illness and was treated but without success. The case report highlights the several immunomodulatory strategies that were employed to treat the disease, despite which the outcome was unfavorable.

Comparison of clinical and electrophysiological characteristics between ictal and cardiac asystole encountered during video-EEG monitoring.

Differentiation between syncope secondary to epileptic seizures and cardiac disease in patients displaying transient loss of consciousness associated with convulsive movements is a diagnostic challenge both for neurologists and cardiologists. In such patients, prolonged video-EEG monitoring not only helps in identifying asystole as the cause of syncope, but also in categorizing asystole as primarily cardiac in origin (cardiac asystole) and secondary to epileptic seizures (ictal asystole). We carried out this study to ascertain the prevalence of asystole in an epilepsy monitoring unit, and to contrast the clinical and electrophysiological characteristics between ictal asystole and cardiac asystole. Through a retrospective search, we identified patients who were shown to have had asystole using a database of patients who underwent prolonged video-EEG monitoring during a 68-month period. We compared the data of 18 consecutive patients; five with ictal asystole and 13 with cardiac asystole, with 121 and 64 events recorded from them, respectively. Of the 10,096 patients who underwent prolonged video-EEG monitoring during the study period, we identified 18 (0.17%) patients with asystole. Cardiac asystole was 2.6 times more frequent than ictal asystole. Older age at onset, heralding symptoms of presyncope, occurrence during wakefulness, and brief duration of the events supported the diagnosis of cardiac asystole. Ictal asystole events were more protracted, and prolonged asystole more frequently occurred in patients with extratemporal seizures compared to temporal lobe seizures. Asystole occurred in only half of the recorded seizures. The accurate categorization of asystole as seizure-related or heart disease-related has huge implications for management strategy and outcome. The necessity of permanent pacemaker implantation is more frequent and urgent in patients with cardiac asystole because of the greater risk of sudden death. Hence, in patients with an ominous diagnosis of cardiac asystole, a thorough cardiac evaluation should surpass neurological evaluation.

Persistent extreme delta brush in anti-NMDA-receptor encephalitis: Does it portend a poor prognosis?

We describe an adolescent girl with non-paraneoplastic anti-NMDA-receptor encephalitis (ANMDARE), who despite persistence of the extreme delta brush (EDB) pattern for nearly 2 years in her serial EEGs, she exhibited a speedy and sustained response to immunotherapy. To the best of our knowledge, our patient had the longest persistence of the EDB pattern on EEG reported to date. Our patient illustrates that, although presence of EDB supports the diagnosis of ANMDARE, its presence and persistence may not be a reliable predictor of response to immunotherapy and overall clinical prognosis.