FLAMES with Elevated Myelin Basic Protein Followed by Myelitis

Hiroyuki Hokama1, Yuki Sakamoto1, Toshiyuki Hayashi1

  • 1Department of Neurology, Graduate School of Medicine, Nippon Medical School, Japan.

Insights

This study investigates anti-myelin oligodendrocyte glycoprotein (MOG)-associated encephalitis with seizures (FLAMES), revealing potential demyelination alongside inflammation. Elevated myelin basic protein in CSF suggests this dual pathology in FLAMES patients.

Area of Science:

  • Neuroimmunology
  • Neurology
  • Neuroinflammation

Background:

  • The underlying mechanisms of unilateral cortical lesions in anti-myelin oligodendrocyte glycoprotein (MOG)-associated encephalitis with seizures (FLAMES) remain poorly understood.
  • FLAMES is an inflammatory neurological condition characterized by seizures and specific brain imaging findings.

Observation:

  • A 26-year-old male presented with seizures and unilateral cortical FLAIR hyperintense lesions.
  • Cerebrospinal fluid (CSF) analysis revealed elevated myelin basic protein (MBP) and detected MOG antibodies.
  • The patient showed significant improvement following intravenous methylprednisolone treatment.

Findings:

  • The presence of MOG antibodies in serum and CSF confirmed the diagnosis of FLAMES.
  • Elevated MBP levels in the CSF suggest that demyelination is a component of the inflammatory process in this FLAMES case.
  • The findings indicate that both inflammation and demyelination may contribute to the pathophysiology of FLAMES.

Implications:

  • This case highlights the potential for demyelination in FLAMES, expanding our understanding of the disease spectrum.
  • The results suggest that therapeutic strategies targeting both inflammation and demyelination might be beneficial for FLAMES patients.
  • Further research is warranted to elucidate the precise role of demyelination in FLAMES and its impact on patient outcomes.

Related Concept Videos

Nervous Tissue: Myelin01:25

Nervous Tissue: Myelin

The myelin sheath is a multilayered lipid and protein covering that insulates the axon of a neuron, enhancing the speed of nerve impulse conduction. Axons without this sheath are referred to as unmyelinated. Two types of neuroglia, Schwann cells in the peripheral nervous system (PNS) and oligodendrocytes in the central nervous system (CNS) are responsible for producing myelin sheaths.
Schwann cells begin to form myelin sheaths around axons during fetal development. They wrap around a small...
3.3K
Myocarditis II: Clinical Features and Diagnostic Tests01:27

Myocarditis II: Clinical Features and Diagnostic Tests

Myocarditis is an inflammation of the heart muscle. The symptoms vary widely, encompassing asymptomatic presentations to severe, acute manifestations.Clinical PresentationAsymptomatic cases: In some instances, myocarditis may be asymptomatic, with the infection resolving without intervention. These cases often go undetected unless discovered incidentally through diagnostic imaging or tests conducted for other reasons.General Early Symptoms: Early symptoms of myocarditis are non-specific and can...
34
Myasthenia Gravis: Diagnostic Tests01:15

Myasthenia Gravis: Diagnostic Tests

Myasthenia gravis is an autoimmune condition affecting neuromuscular transmission, causing generalized weakness in skeletal muscles. Initial diagnoses rely on patients' signs, symptoms, and medical history. The challenge lies in distinguishing myasthenia from other muscular dystrophies. An important diagnostic feature is the significant improvement of symptoms after administering anticholinesterase inhibitors.
The edrophonium test is a diagnostic tool for myasthenia gravis. It involves...
1.4K
Myocarditis I: Introduction01:21

Myocarditis I: Introduction

Myocarditis is inflammation of the myocardium, which is the muscular layer of the heart.EtiologyMyocarditis has a diverse etiology, including a wide range of infectious and non-infectious causes:Infectious CausesViral: Common viruses include Coxsackie A and B, adenovirus, parvovirus B19, enteroviruses, and influenza A.Bacterial: Examples include infections caused by Streptococcus, Staphylococcus, and Mycoplasma species.Rickettsial: Infections like Rocky Mountain spotted fever can result in...
49