Refractory status epilepticus from NMDA receptor encephalitis successfully treated with an adjunctive ketogenic diet

Objective: Case report of NMDA receptor encephalitis in a young man with early refractory status epilepticus and atypical radiological findings. Background: Anti-NMDA receptor encephalitis is an autoimmune disorder due to antibodies to the NR1-NR2B heterodimer of NMDA receptor. On imaging, it typically presents with T2 hyperintensities in mesio-temporal lobes, cerebral cortex and basal ganglia. We present a case with a dramatic clinical evolution and novel imaging findings. Design/Methods: Case report and review of imaging. Results: 29-year-old male presented with mood disturbance followed by partial-complex seizures, facial dyskinesia and choreo-athetotic movements. Initial MRI showed subtle T2-hyperintensities in mesio-temporal lobes. Diagnosis of NMDA-receptor encephalitis was confirmed after CSF antibody detection. Prior to diagnostic confirmation, he developed refractory status epilepticus, and concomitant signs of herniation. A repeated MRI showed increased T2-hyperintensities of thalami and mesencephalon, with cerebellar involvement and transtentorial/foraminal herniation. Restricted diffusion was documented in the cerebellar cortex/thalami/putamina and caudate. IV corticosteroids and hypertonic fluid reversed herniation, and halted the seizures. Conclusions: To our knoweldge, we report the first case report of uncal and tonsillar herniation in NMDA-r encephalitis secondary to atypical, predominant cerebellar involvement. This case highlights life-threatening manifestation that physicians might encounter, and a possible role for high dose IV corticosteroids as an adjunct treatment for brain edema and seizures.

Download Full-text

Comment on “Anti-NMDA receptor encephalitis presenting as new onset refractory status epilepticus in COVID-19”

Seizure ◽

10.1016/j.seizure.2020.08.007 ◽

2020 ◽

Vol 81 ◽

pp. 166

Author(s):

Alessandro Dinoto ◽

Marta Cheli ◽

Arianna Sartori ◽

Paolo Manganotti

Keyword(s):

Status Epilepticus ◽

Nmda Receptor ◽

Refractory Status Epilepticus ◽

Nmda Receptor Encephalitis ◽

Refractory Status ◽

New Onset

Download Full-text

Anti-NMDA receptor encephalitis presenting with the new-onset refractory status epilepticus

Northern Clinics of Istanbul ◽

10.14744/nci.2021.70431 ◽

2021 ◽

Author(s):

Murat Alemdar

Keyword(s):

Status Epilepticus ◽

Nmda Receptor ◽

Refractory Status Epilepticus ◽

Nmda Receptor Encephalitis ◽

Refractory Status ◽

New Onset

Download Full-text

P.002 Successful Treatment of Supra-Refractory Status Epilepticus Secondary to Anti-N-Methyl-D- Aspartate Receptor Encephalitis With Electroconvulsive Therapy

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2021.285 ◽

2021 ◽

Vol 48 (s3) ◽

pp. S20-S20

Author(s):

A AlSabah ◽

A Alshukaili ◽

JS Teitelbaum

Keyword(s):

Status Epilepticus ◽

Nmda Receptor ◽

Electroconvulsive Therapy ◽

Refractory Status Epilepticus ◽

Bilateral Oophorectomy ◽

Nmda Receptor Encephalitis ◽

Refractory Status ◽

Healthy Female ◽

Nr2 Subunits ◽

New Onset

Background: Anti-N-Methyl-D-Aspartate (NMDA) receptor encephalitis is an autoimmune disease associated with antibodies against heteromers NR1 and NR2 subunits of the cell surface of the NMDA receptors, causing many psychiatric and neurological symptoms. This includes new-onset refractory status epilepticus. Methods: A 33-year-old previously healthy female developed new-onset refractory status epilepticus caused by anti- NMDA receptor encephalitis without the presence of tumours. Results: The clinical course was complicated by prolonged status epilepticus, which was refractory to many antiepileptic drugs (levetiracetam, phenytoin, carbamazepine, topiramate, lacosamide, valproic acid), ketamine, propofol, midazolam, including inhalation agents (isoflurane). Also, she received first (intravenous immunoglobulin, intravenous methylprednisolone, and plasmapheresis), second-line immunotherapy (rituximab) and prophylaxis bilateral oophorectomy without clinical or electrographic improvement. However, the patient drug-resistant status epilepticus markedly improved both clinically and electrographically following seven sessions of electroconvulsive therapy. Conclusions: Electroconvulsive therapy should be considered as adjuvant therapy for the treatment of immunotherapy resistant encephalitis.

Download Full-text