Longitudinal extensive transverse myelitis--it's not all neuromyelitis optica

Corinna Trebst1, Peter Raab, Elke Verena Voss

  • 1Department of Neurology, Hannover Medical School, Carl-Neuberg-Strasse 1, 30625 Hannover, Germany.

Nature Reviews. Neurology
|November 3, 2011
PubMed

Insights

Longitudinal extensive transverse myelitis (LETM) involves spinal cord lesions spanning three or more vertebrae. This review details its diverse causes, symptoms, and optimal treatments for accurate diagnosis and patient care.

Area of Science:

  • Neurology
  • Radiology
  • Immunology

Background:

  • Longitudinal extensive transverse myelitis (LETM) is defined by spinal cord lesions on MRI extending over three or more vertebral segments.
  • LETM presents with significant neurological deficits, including motor, sensory, and autonomic dysfunction.

Observation:

  • LETM is a hallmark of neuromyelitis optica but also occurs in other central nervous system (CNS) inflammatory diseases like multiple sclerosis, sarcoidosis, and Sjögren syndrome.
  • Infectious diseases, neoplastic disorders, and traumatic spinal cord injury can also manifest as longitudinal spinal lesions.

Findings:

  • This review outlines the clinical signs and symptoms indicative of various etiologies of LETM.
  • Educational case studies illustrate the differential diagnoses for LETM.
  • Optimal therapeutic strategies for each identified diagnosis are discussed.

Implications:

  • Accurate diagnosis of LETM etiology is crucial for effective treatment and patient management.
  • Understanding the diverse causes of LETM aids neurologists and clinicians in patient evaluation.
  • This review provides a comprehensive guide for diagnosing and managing LETM.

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...
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...
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...
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...
Myasthenia Gravis: Overview and Treatment01:20

Myasthenia Gravis: Overview and Treatment

Myasthenia gravis is a neuromuscular transmission disorder characterized by weakness and increased fatigability of skeletal muscles. It is an autoimmune disease affecting approximately one in 2000 people, where antibodies against the α1 subunit of nicotinic acetylcholine receptors are produced.
These antibodies interfere with the function of the nicotinic receptors in three ways: by binding to the receptor and disrupting acetylcholine binding; by causing cross-linking of receptors which leads...