[Fast and spectacular clinical response to plasmapheresis in a paediatric case of anti-NMDA encephalitis]

N Nunez-Enamorado1, A Camacho-Salas, S Belda-Hofheinz

  • 1Hospital Universitario 12 de Octubre, Madrid, Espana. noenu_enamorado@yahoo.es

Revista De Neurologia
|March 28, 2012
PubMed

Insights

Autoimmune encephalitis against N-methyl-D-aspartate (NMDA) receptors is increasingly diagnosed in children. Early recognition and immunotherapy, particularly plasmapheresis, are crucial for treating this potentially reversible neurological disorder.

Area of Science:

  • Pediatric Neurology
  • Neuroimmunology

Background:

  • Autoimmune encephalitis targeting N-methyl-D-aspartate (NMDA) receptors is increasingly recognized in pediatric populations.
  • Suspected in children presenting with psychiatric symptoms, encephalopathy, movement disorders, or seizures.

Observation:

  • A 2.5-year-old boy presented with subacute encephalopathy, seizures, behavioral changes, neurological regression, dyskinesias, and insomnia.
  • Initial treatments with corticosteroids and IVIG were ineffective; however, plasmapheresis led to rapid and significant recovery.
  • Cerebrospinal fluid was normal, MRI showed focal periventricular lesions and leptomeningeal enhancement, and EEG indicated diffuse delta activity with epileptiform discharges.

Findings:

  • The patient tested positive for antibodies against the NMDA receptor.
  • Plasmapheresis resulted in a swift and dramatic clinical improvement.
  • Long-term follow-up showed mild residual behavioral and language alterations without relapse or need for maintenance therapy.

Implications:

  • Anti-NMDA receptor encephalitis is a treatable condition, emphasizing the importance of early diagnosis.
  • Immunotherapy, including plasmapheresis, is effective for non-paraneoplastic cases, offering rapid recovery.
  • This case highlights the potential for significant neurological recovery with prompt and appropriate treatment in pediatric autoimmune encephalitis.
Abstract

Related Concept Videos

Encephalitis ll: Pathophysiology01:26

Encephalitis ll: Pathophysiology

Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...
Encephalitis l: Introduction01:19

Encephalitis l: Introduction

Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
Diphtheria01:28

Diphtheria

Diphtheria is an acute, toxin-mediated infectious disease that primarily affects the upper respiratory tract. It is caused by Corynebacterium diphtheriae, a Gram-positive, pleomorphic rod that lacks spore-forming capability and exhibits a characteristic club-shaped morphology under microscopic examination. While C. diphtheriae can asymptomatically colonize mucosal surfaces, clinical disease manifests only when the bacterial strain is lysogenized by a specific β-corynephage. This phage...