Anti-NMDA receptor encephalitis presenting with total insomnia — A case report

Abstract

Fatal insomnia (FI) is the first diagnosis to be considered by most neurologists when approaching a patient presenting with total insomnia followed by personality and cognitive changes, disturbance of alertness, autonomic hyperactivation and movement abnormalities. We report the case of a 30 year-old male patient who presented with total insomnia followed by episodes of psychomotor restlessness resembling anxiety attacks. Twenty days later, he developed refractory convulsive status epilepticus with admission to Intensive Care Unit. He progressed to a state of reduced alertness and responsiveness, presenting periods of agitation with abnormal dyskinetic movements, periods of autonomic instability and central hypoventilation. Workup revealed antibodies against N-methyl-D-aspartate receptor (NMDAR). Immunotherapy treatment led to a very significant improvement with the patient presenting only slight frontal lobe dysfunction after one year of recovery. To the best of our knowledge this is the first report of a patient with anti-NMDAR encephalitis first presenting with total insomnia. Our aim is to alert that anti-NMDAR encephalitis must be considered in the differential diagnosis of FI, especially in sporadic cases. Distinguishing the two conditions is very important as, contrarily to the fatal disclosure of FI, anti-NMDAR encephalitis is potentially reversible with adequate treatment even after severe and prolonged disease.

Unilateral predominance of abnormal movements: A characteristic feature of the pediatric anti-NMDA receptor encephalitis?

“…The unilateral predominance of the abnormal movements observed in our patient has been previously reported in anti-NMDA receptor encephalitis, mainly in series of pediatric patients [5], [12], [13], [14], [23], [24], [25], [26], [27], [28], [29]. The unilateral predominance of the abnormal movements or seizures is a classical feature in other systemic antibody-mediated diseases including Rasmussen syndrome, systemic lupus erythematosus involving the central nervous system, Hashimoto encephalitis and Sydenham chorea, however the underlying pathophysiology of unilateral or bilateral asymmetric symptoms in autoimmune diseases remains poorly understood [12], [30], [31], [32], [33], [34].…”

Testing for N-methyl-d-aspartate Receptor Autoantibodies in Clinical Practice

“…Deciphering the factors that influence rates of NMDAR IgG autoantibody detection in serum has important implications for research and clinical care. Anti-NMDAR encephalitis is a potentially treatable cause of first-episode psychoses, 34 postpartum psychoses, 35,36 refractory mood disorders, 37 unexplained behavioral changes, [38][39][40] and neuroleptic sensitivity. 41,42 Accordingly, screening for serum NMDAR IgG autoantibodies has been performed in individuals with unexplained psychiatric presentations in whom obtaining CSF may present undue challenges.…”

New Onset Insomnia in a Pediatric Patient: A Case of Anti-NMDA Receptor Encephalitis

“…Multiple studies have reported sleep-wake cycle disturbances and insomnia as characteristic symptoms in anti-NMDAR encephalitis [7] and while they have been described previously as the presenting feature in adults [16, 17], insomnia in children with anti-NMDAR encephalitis is typically described later in the clinical course [1, 26–29] or has not been mentioned as a feature of the illness [30–32]. Other studies suggest that children are more likely to present with predominantly neurologic symptoms (seizures, abnormal movements, and focal neurologic deficits) than with psychiatric symptoms [26].…”