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J Neurol Sci. 2014 Jan 15;336(1-2):276-80. doi: 10.1016/j.jns.2013.10.034. Epub 2013 Oct 30.

Anti-NMDA receptor encephalitis presenting with total insomnia--a case report.

Author information

1
Department of Neurology, Coimbra University Hospital, Praceta Prof. Mota Pinto, 3000-075 Coimbra, Portugal. Electronic address: inesmbmarques@gmail.com.
2
Department of Neurology, Coimbra University Hospital, Praceta Prof. Mota Pinto, 3000-075 Coimbra, Portugal.

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.

KEYWORDS:

Autoimmune encephalitis; Immunotherapy; NMDAR encephalitis; Sporadic fatal insomnia; Total insomnia; Video-EEG monitoring

PMID:
24210076
DOI:
10.1016/j.jns.2013.10.034
[Indexed for MEDLINE]
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