Analysis of relapses in anti-NMDAR encephalitis

Gabilondo, Iñigo; Sáiz, Albert; Galán, Lisette Arias; González, Vanesa Arbesú; Jadraque, R; Sabater, Lidia; Sans, Anna; Sempere, Amparo; Vela, Amaia; Villalobos, F; M, Viñals; Villoslada, Pablo; Graus, Francesc

doi:10.1212/wnl.0b013e31822cfc6b

Cited by 226 publications

(179 citation statements)

References 10 publications

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“…Social behavior and executive function recovery is usually prolonged and may be incomplete, with a significant proportion of patients with considerable recovery remaining with frontal dysfunction [4][5][6]28]. Relapses occur in 12 to 25% of patients [5,15,29,30], more commonly in patients without associated tumor, so immunosuppression with azathioprine or mycophenolate mofetil during at least 1 year after withdrawal of the initial immunotherapies and periodic tumor screening is advised in these patients [4][5][6].…”

Section: Discussionmentioning

confidence: 99%

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

Marques

Teotónio

Cunha

et al. 2014

Journal of the Neurological Sciences

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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.

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Section: Discussionmentioning

confidence: 99%

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

Marques

Teotónio

Cunha

et al. 2014

Journal of the Neurological Sciences

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“…Patients showed clinical improvements with a combination of tumour removal and immunotherapies. Since this important finding, it has become clear that the disease affects both males and females and it is more commonly nonparaneoplastic [21][22][23] . Tumours are most frequently detected in women between 20 and 40 years-old, and they are almost always ovarian teratomata.…”

Section: The Nmda-receptormentioning

confidence: 99%

The expanding spectrum of clinically-distinctive, immunotherapy-responsive autoimmune encephalopathies

Irani

Vincent

2012

Arq. Neuro-Psiquiatr.

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The autoimmune encephalopathies are a group of conditions that are associated with autoantibodies against surface neuronal proteins, which are likely to mediate the disease. They are established as a frequent cause of encephalitis. Characteristic clinical features in individual patients often allow the specificity of the underlying antibody to be confidently predicted. Antibodies against the VGKC-complex, mainly LGI1(leucine-rich glioma-inactivated 1), CASPR2 (contactin-associated protein 2), and contactin-2, and NMDA (N-methyl, D-aspartate) -receptor are the most frequently established serological associations. In the minority of cases, an underlying tumour can be responsible. Early administration of immunotherapies, and tumour removal, where it is relevant, offer the greatest chance of improvement. Prolonged courses of immunotherapies may be required, and clinical improvements often correlate well with the antibody levels. In the present article, we have summarised recent developments in the clinical and laboratory findings within this rapidly expanding field.

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“…9 Data from case series seem to suggest a lower risk of relapse in patients treated with immunotherapy. 13 Exploration of this patient's persistent high fevers ultimately led to the serendipitous diagnosis of the increasingly recognized syndrome of anti-NMDAR encephalitis, although in retrospect nearly all of the features of her presentation fit well with this condition. Thus, it was only by a chance finding on her abdominal CT scan that this patient was ultimately diagnosed with a treatable, noninfectious encephalitis associated with an ovarian teratoma.…”

Section: Commentarymentioning

confidence: 89%

Chance favors the prepared mind

Prins

Gladwin

Brod

et al. 2012

Journal of Hospital Medicine

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The approach to clinical conundrums by an expert clinician is revealed through the presentation of an actual patient's case in an approach typical of a morning report. Similarly to patient care, sequential pieces of information are provided to the clinician, who is unfamiliar with the case. The focus is on the thought processes of both the clinical team caring for the patient and the discussant. This icon represents the patient's case. Each paragraph that follows represents the discussant's thoughts.

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Analysis of relapses in anti-NMDAR encephalitis

Abstract: Relapses in anti-NMDAR encephalitis are common (24%). They may occur many years after the initial episode. Relapses may present with partial aspects or with isolated symptoms of the full-blown syndrome. Immunotherapy at first episode reduces the risk of relapses.

Cited by 226 publications

References 10 publications

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

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

The expanding spectrum of clinically-distinctive, immunotherapy-responsive autoimmune encephalopathies

Chance favors the prepared mind

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