Relapse risk factors in anti‐N‐methyl‐D‐aspartate receptor encephalitis

Nosadini, Margherita; Granata, Tiziana; Matricardi, Sara; Freri, Elena; Ragona, Francesca; Papetti, Laura; Suppiej, Agnese; Valeriani, Massimiliano; Sartori, Stefano

doi:10.1111/dmcn.14267

Cited by 35 publications

(31 citation statements)

References 45 publications

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“…5,6 On the other hand, relapses of AE have been described even after five to ten years 12 and relapses in Anti- NMDAR encephalitis have been reported in 9 to 23 per cent of patients. 13 Indeed, early aggressive therapy is referred to reduce relapse rates, 14 which would be consistent with our personal experience. However, the choice of second-line treatment is not always easy, especially due to possible side-effects and missing licenses.…”

Section: Discussionsupporting

confidence: 79%

A critical reflection on our first patient presenting with Anti-Nmethyl- D-aspartate receptor encephalitis

et al. 2019

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One of the best characterized autoimmune encephalitis is the Anti-Nmethyl-D-aspartate receptor (NMDAR) encephalitis, which may occur in the presence of cancer. First- and second-line immunotherapy and oncological investigations are suggested. We present here a case of an 18-year-old female who was our first patient suffering from Anti-NMDAR encephalitis more than 9 years ago. She was satisfactorily treated with intravenous immunoglobulins and high dose steroid therapy. After more than one year the patient had a relapse. First-line immunotherapy was repeated; however, a complete recovery was achieved only after plasmapheresis. Afterwards, she continued maintenance immunotherapy with steroids for two years and with Azathioprine for about five years associated to regular oncological assessment. In the last years our therapeutical approach of Anti-NMDARencephalitis has significantly changed. Nevertheless, established treatment guidelines are still missing and the role of long-term maintenance immunotherapy is largely unexplored. In addition, oncological revaluation might be indicated in selected patients.

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Section: Discussionsupporting

confidence: 79%

A critical reflection on our first patient presenting with Anti-Nmethyl- D-aspartate receptor encephalitis

et al. 2019

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“…In fact, determining an ictal discharge is one of the most challenging issue for anti‐NMDAR encephalitis. Most paroxysmal attacks of psychomotor agitation need to be recorded by EEG to avoid mistakenly diagnosing as seizures, and most of paroxysmal rhythmic activities in anti‐NMDAR encephalitis are non‐epileptic 16 . As a result, recognition of an ictal pattern is important for clinical practice.…”

Section: Discussionmentioning

confidence: 99%

Delta brush variant: A novel ictal EEG pattern in anti‐NMDAR encephalitis

Huang

Liao

et al. 2020

Epilepsia Open

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Seizure is one of the main symptoms of anti‐NMDAR encephalitis, but data of ictal electroencephalogram (EEG) patterns remain limited. In this study, we aimed to introduce a unique ictal pattern. This delta brush variant (DBV) was characterized as generalized delta rhythm with fast spike activity riding on it. We retrospectively evaluated the ictal pattern from six patients with anti‐NMDAR encephalitis, and patients were grouped based on the presence of DBV. DBV was found in two patients who were in the florid phase of the disease: (a) A 17‐year‐old girl experienced rhythmical jerking of bilateral limbs. Corresponding EEG patterns showed generalized DBV. Seizure terminated after intravenous injection of midazolam, but oral‐facial dyskinesia reappeared; and (b) a 24‐year‐old man suffered stiffening of the right limbs and oral‐facial dyskinesia. The EEG pattern showed frontal DBV with left prominence. Seizure was controlled, but oral‐facial dyskinesia remained after intravenous injection of midazolam. Compared with patients without DBV, patients in this group were more likely to have prolonged excessive delta brush (100% vs 25%) and hyperpyrexia (39.7℃ vs 38.2℃). Duration in ICU (36 days vs 18 days) and hospital (52 days vs 36 days) was relatively longer in DBV group, and no significant difference was found in terms of the mRS score (1 vs 0.5) and seizure relapse rate (0% vs 25%) during 3‐month follow‐up. DBV is a peculiar pattern in anti‐NMDAR encephalitis. An EEG‐based monitoring should be considered to avoid misleading this ictal EEG pattern to the electromyographic artifact.

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“…CSF examination is useful to differentiate ADEM from other in ammatory or autoimmune diseases, and to determine the presence of autoantibodies (e.g. anti-myelin oligodendrocyte glycoprotein, MOG) which can be indicative for recurrence (3,(34)(35)(36)(37)(38)(39).…”

Section: Discussionmentioning

confidence: 99%

Acute Disseminated Encephal Omyelitis (ADEM) After Pneumococcal Meningitis In A Child

Foiadelli¹,

Trabatti²,

Bassanese³

et al. 2020

Preprint

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Background: Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder of the central nervous system (CNS), whose pathogenesis is still debated. It occurs more frequently in children, and the first neurological symptoms usually appear between 2 and 4 weeks after a trigger event, such as an infection or vaccination. Several viral agents have been related to the development of ADEM, while bacteria and parasites are less frequently involved. Severe Streptococcus pneumoniae infection has been rarely described as a trigger for CNS demyelination in particularly predisposed subjects.Case presentation: A 10 year old girl was evaluated for headache, fever and vomit. CSF analysis revealed pleocytosis and presence of S. pneumoniae antigen, and proper antibiotic therapy for bacterial meningitis was started, with rapid improvement. A breach in the right frontal bone, due to a car accident occurring the previous year, was considered the gateway for pneumococcal infection. Three days after admission, the girl developed drowsiness, altered speech and left hemiparesis. Brain MRI showed multiple T2-hyperintense bilateral lesions in the supratentorial white matter, and ADEM was diagnosed. Considering the underlying bacterial meningitis, intravenous immunoglobulins were preferred to steroid therapy, and the patient progressively recovered. However, due to recurrence of encephalopathic symptoms after 11 days, high-dose intravenous steroid therapy was performed. The neurological outcome was favourable, with complete regression of the white matter lesions after 4 months and absence of relapses over a follow-up period of 2 years.Conclusions: Occurrence of ADEM following pneumococcal meningitis is rare, and very few cases have been described in children. It should be suspected in case of persistence, recurrence or onset of new symptoms despite adequate antimicrobial treatment, relying on brain MRI for a thorough differential diagnosis. High level of surveillance is mandatory in patients with predisposing factors to invasive pneumococcal disease. In some cases, acute demyelination can occur few days after the onset of meningitis; pathogenetic mechanisms are not yet fully clarified and the choice of the correct therapeutic approach can be challenging.

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Relapse risk factors in anti‐N‐methyl‐D‐aspartate receptor encephalitis

Cited by 35 publications

References 45 publications

A critical reflection on our first patient presenting with Anti-Nmethyl- D-aspartate receptor encephalitis

A critical reflection on our first patient presenting with Anti-Nmethyl- D-aspartate receptor encephalitis

Delta brush variant: A novel ictal EEG pattern in anti‐NMDAR encephalitis

Acute Disseminated Encephal Omyelitis (ADEM) After Pneumococcal Meningitis In A Child

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