First Case Report of Association of Anti-N-Methyl- D-Aspartate Receptor Encephalitis and Pneumatosis Intestinalis

Case Report

Austin Crit Care Case Rep. 2017; 1(1): 1001.

First Case Report of Association of Anti-N-Methyl- D-Aspartate Receptor Encephalitis and Pneumatosis Intestinalis

Abdul-Aziz R¹*, Bout-Tabaku S¹, O’Donovan JC², Sivaraman V¹ and Spencer CH¹

¹Department of Pediatric Rheumatology, Ohio State University, USA

²Department of Radiology, Ohio State University, USA

*Corresponding author: Rabheh Abdul-Aziz, Department of Pediatric Rheumatology, Ohio State University, 700 Children’s Drive, Columbus, Ohio, 43205, USA

Received: November 02, 2016; Accepted: January 27, 2017; Published: January 30, 2017


Anti-N-Methyl-D-Aspartate Receptor (anti-NMDA-R) encephalitis is an immune-mediated syndrome that was first described in 2007. We describe two patients with anti-NMDAR encephalitis who developed Pneumatosis Intestinalis (PI) during the course of their disease. Pneumatosis intestinalis in the setting of anti-NMDA-R encephalitis has not previously been reported in the literature. That led to speculation whether the association between anti-N-methyl-Daspartate receptor encephalitis and pneumatosis intestinalis is secondary to immunosuppression, medication effect, or a direct manifestation of auto antibodies will require further study. Our patients highlight the occurrence of this rare complication in critically ill patients with NMDA-R encephalitis who usually admit to pediatric intensive care unit. Early recognition and appropriate therapy are essential to recovery in patients who develop this complication.

Keywords: Anti-n-methyl-d-aspartate receptor; Pneumatosis intestinalis; Encephalitis


Anti-NMDA-R: Anti-N-Methyl-D-Aspartate Receptor; PI: Pneumatosis Intestinalis; CSF: Cerebrospinal Fluid; EEG: Electroencephalogram; NG: Nasogastric; NJ: Nasojejunal; TPN: Total Parenteral Nutrition


Dalmau et al first described anti-NMDA-R encephalitis in 2007 [1]. The classic presentation of anti-NMDA-R encephalitis includes psychiatric, neurologic, and autonomic symptoms [2]. Patient evaluation includes examination of Cerebrospinal Fluid (CSF), brain MRI, Electroencephalogram (EEG), and Anti-N-methyl-D-aspartate receptor antibodies in blood and CSF fluid [3].

In the other hand, Pneumatosis Intestinalis (PI) is a wellrecognized manifestation of Necrotizing Enterocolitis (NEC) in the newborn. However, limited information is available concerning PI in older children. West et al reported sixteen older infants and children with PI [4]. Associated conditions include short bowel syndrome, congenital heart disease, iron ingestion, nesidioblastosis, hemolytic anemia, rheumatoid arthritis, bronchopulmonary dysplasia, and malrotation [4]. In adults, PI has been reported after treatment with cetuximab, docetaxel, sunitinib, a-glucosidase inhibitor, and after neoadjuvant chemotherapy for esophageal cancer [5-9].

In this report, we describe two patients with anti-NMDA-R encephalitis whose clinical course was complicated by PI.

Case Presentation

Case A

A 13-year old African American female with a past medical history of attention deficit hyperactivity disorder was admitted to the psychiatric unit due to sub-acute altered mental status, auditory and visual hallucinations, and violent behavior. Her condition progressed to fluctuating levels of alertness and she developed catatonia, difficulty breathing and a fever. Her EEG was consistent with encephalopathy without seizure. Lumbar puncture showed a normal opening pressure and mild pleocytosis with 35 white blood cell, and positive EBV by PCR. A head CT and brain MRI were normal. During the next few days of hospitalization, she developed tachycardia, clonus, garbled speech, agitation, aggressive behavior and hyperreflexia. Due to altered mental status and concerns for aspiration, she was started on Nasogastric (NG) feeding that was subsequently changed to Nasojejunal (NJ) due to feeding intolerance. A presumptive diagnosis of autoimmune encephalitis was made, after infectious diseases were ruled out. She was empirically treated with IV methylprednisolone and Rituximab. After this, she developed seizures and was started on levetiracetam. Risperidone, lorazepam, trihexyphenidyl hydrochloride, clonidine, gabapentin, and chloral hydrate were added for a symptomatic management of her agitation and pain. On day 10, the result of anti-NMDA-R antibody from cerebral spinal fluid was reported as positive, with a titer of 1:40. Due to the progressive course of her illness, she was started on plasmapheresis for five cycles followed by intravenous immunoglobulin for 2 doses. She received also piperacillin/tazobactam and vancomycin for possible pneumonia and ganciclovir for EBV. On day 12, she developed abnormal movements with limb stiffening, head deviation, and orolingual dyskinesia. On day 13, she developed sudden onset eye blinking, oral-facial dyskinesia, and tonic synchronized flexion of neck, upper and lower extremities associated with a brief desaturation. An EEG demonstrated seizure activity and she developed status epilepticus. She was transferred to the pediatric intensive care unit, treated with fosphenytoin, and required mechanical ventilation for 3 days. She had tachycardia and tachypnea with intermittent hypertension presumed to be due to an autonomic dysfunction. A week later, she developed fever, seizure, and diarrhea. Stool Clostridium difficile, rotavirus, and adeno virus studies were negative. Her examination showed abdominal distension. She continued to have diarrhea and on day 28 developed bilious emesis. Her abdominal radiograph revealed pneumatosis in her cecum and ascending colon (Figure 1A). An abdominal CT scan demonstrated extensive pneumatosis (Figure 1B-1D). All enteral feeding was discontinued and she was started on piperacillin/ tazobactam and Total Parenteral Nutrition (TPN). The pneumatosis resolved after 3 days based on imaging by serial abdominal radiographs. NJ Feeds were restarted gradually and finally a gastric-tube was placed. By day 46 her neurologic status improved, as did her sleep and communication. However, she continued to be emotionally labile. She was started on monthly IVIg and mycophenolate mofetil for long-term management. She received inpatient rehabilitation services and was then discharged home. During her outpatient follow up, she continued to show improvement and remained seizure free.

Citation:Abdul-Aziz R, Bout-Tabaku S, O’Donovan JC, Sivaraman V and Spencer CH. First Case Report of Association of Anti-N-Methyl-D-Aspartate Receptor Encephalitis and Pneumatosis Intestinalis. Austin Crit Care Case Rep. 2017; 1(1): 1001.