Spontaneously resolving seronegative autoimmune limbic encephalitis

Souhel Najjar1, Daniel Pearlman, David Zagzag

  • 1Departments of Neurology, Pathology (Division of Neuropathology), Neurosurgery & Psychiatry, NYU-Comprehensive Epilepsy Center, New York University School of Medicine, New York, NY, USA.

Abstract

Insights

Seronegative autoimmune limbic encephalitis (SNALE) can mimic glioma. This case highlights distinctive pathology and a rare spontaneous remission of SNALE lesions without immune therapy, offering new insights.

Area of Science:

  • Neurology
  • Immunology
  • Pathology

Background:

  • Seronegative autoimmune limbic encephalitis (SNALE) is rare, with limited reported cases and even fewer with pathological data.
  • Distinguishing SNALE from neoplastic lesions like glioma is clinically challenging.

Observation:

  • A 66-year-old man presented with severe memory loss, cognitive decline, and intractable seizures.
  • Initial MRI suggested viral encephalitis, but treatment was ineffective. Subsequent MRI revealed a contrast-enhancing lesion mimicking glioma.

Findings:

  • Pathological examination of the resected lesion showed inflammatory infiltrates characteristic of SNALE, with no infectious agents or autoantibodies detected.
  • The patient experienced a new lesion and worsening symptoms, yet achieved spontaneous resolution and significant recovery without immunotherapy.

Implications:

  • SNALE can present with imaging and clinical features indistinguishable from glioma, necessitating careful differential diagnosis.
  • Distinctive pathological findings aid in SNALE diagnosis.
  • This case suggests that SNALE lesions may undergo spontaneous resolution, challenging the necessity of immediate immune therapy in all instances.