Longitudinally extensive transverse myelitis

W Oliver Tobin1, Brian G Weinshenker, Claudia F Lucchinetti

  • 1Department of Neurology, College of Medicine, Mayo Clinic, Rochester, Minnesota, USA.

Abstract

Insights

Longitudinally extensive transverse myelitis (LETM) is a heterogeneous condition. New diagnostic tests and treatments for neuromyelitis optica (NMO) are available, but other causes of LETM must be excluded in NMO-IgG-seronegative patients.

Area of Science:

  • Neurology
  • Immunology

Background:

  • Longitudinally extensive transverse myelitis (LETM) is a severe neurological condition often associated with neuromyelitis optica (NMO).
  • Advances in NMO diagnosis and therapy have emerged, yet LETM can stem from various causes.
  • Investigating alternative etiologies for myelopathy in LETM patients is crucial.

Purpose of the Study:

  • To review recent advancements in the diagnosis and treatment of NMO.
  • To discuss the differential diagnosis of LETM, particularly in NMO-IgG-seronegative cases.

Main Methods:

  • Review of current literature on NMO and LETM.
  • Analysis of diagnostic assays for NMO-IgG, including fluorescence-activated cell sorting (FACS).
  • Evaluation of therapeutic strategies for NMO spectrum disorders.

Main Results:

  • FACS and cell binding assays offer the highest sensitivity for detecting NMO spectrum disorders.
  • Patients with suspected NMO and negative results from older assays should be retested using FACS.
  • Eculizumab is a potential novel therapy for active NMO, especially after failure of azathioprine and rituximab.

Conclusions:

  • LETM represents a diverse group of conditions.
  • While NMO has new treatment options, identifying alternative causes is essential for NMO-IgG-seronegative patients.

Related Concept Videos

Multiple Sclerosis l: Introduction01:19

Multiple Sclerosis l: Introduction

Multiple sclerosis is a chronic autoimmune disease of the central nervous system (CNS) that affects the brain, spinal cord, and optic nerves. It is an inflammatory demyelinating disorder and a leading cause of neurological disability in young adults.EpidemiologyMS commonly begins between 20 and 40 years of age and is twice as common in women. Its exact cause remains unclear, but genetic susceptibility contributes, with higher risk in first-degree relatives and identical twins. A greater...
20
Spinal Cord: Cross-sectional Anatomy01:16

Spinal Cord: Cross-sectional Anatomy

The cross-sectional anatomy of the spinal cord offers a detailed view of its complex structure and function within the central nervous system. At the core of the spinal cord lies the gray matter, characterized by its butterfly or "H"-shaped appearance in cross-section. This central region is enveloped by white matter, with the overall structure divided into symmetrical halves by the dorsal median sulcus and the ventral median fissure.
Gray Matter and its Components
Central to the gray matter is...
5.7K
Secondary Spinal Cord Injury llI: Pathophysiology01:25

Secondary Spinal Cord Injury llI: Pathophysiology

Early Ischemia and Ionic ImbalanceWithin minutes of spinal cord injury, a secondary cascade begins, progressing over hours to weeks. Vascular damage reduces blood flow, causing ischemia and mitochondrial dysfunction. ATP depletion leads to ion pump failure, membrane depolarization, sodium influx, potassium efflux, and water accumulation, resulting in cellular swelling. Increased intracellular calcium further disrupts mitochondria and accelerates cellular injury.Excitotoxicity and Neuronal...
52
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...
22
Myasthenia Gravis ll: Pathophysiology01:22

Myasthenia Gravis ll: Pathophysiology

The disease process of myasthenia gravis begins at the neuromuscular junction, where antibodies attack key proteins needed for muscle activation. This immune reaction weakens signal transmission, leading to the characteristic muscle fatigue and weakness that define the condition.Immune-Mediated DamageIn most individuals, antibodies target acetylcholine receptors (AChRs) on the postsynaptic membrane of muscle cells. By blocking acetylcholine binding, these antibodies prevent the nerve signal...
53