Lesional demyelinations of the central nervous system

Lesional Demyelinations of the Central Nervous System

Lesional demyelinations of the central nervous system (CNS) refer to the pathological process where myelin, the protective sheath surrounding nerve fibers, is damaged or destroyed. This process is a hallmark of several neurological disorders, most notably multiple sclerosis (MS). Demyelination disrupts the normal conduction of electrical impulses along the nerve fibers, leading to a variety of neurological symptoms.

Pathophysiology

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Dawson's fingers, a characteristic MRI finding in multiple sclerosis.

Demyelination in the CNS is primarily mediated by an autoimmune response, where the body's immune system mistakenly attacks its own myelin. This results in the formation of lesions or plaques, which are areas of inflammation and subsequent myelin loss. The exact cause of this autoimmune response is not fully understood, but it is believed to involve a combination of genetic and environmental factors.

Types of Demyelinating Lesions

Demyelinating lesions can vary in size, location, and severity. In multiple sclerosis, lesions are commonly found in the white matter of the brain and spinal cord. These lesions can be classified based on their activity:

• Active lesions: Characterized by ongoing inflammation and myelin breakdown.
• Chronic active lesions: Show a rim of active inflammation surrounding a core of inactive demyelination.
• Inactive lesions: Lack active inflammation and are characterized by gliosis, a process where glial cells proliferate to form a scar.

Cellular Mechanisms

Types of glial cells involved in CNS demyelination.

The process of demyelination involves several types of cells:

• Oligodendrocytes: These are the myelin-producing cells in the CNS. Damage to oligodendrocytes leads to myelin loss.
• Microglia: These are the resident immune cells of the CNS that become activated during demyelination.
• Astrocytes: These cells contribute to the formation of the glial scar in chronic lesions.

Clinical Manifestations

The symptoms of demyelination depend on the location and extent of the lesions. Common symptoms include:

• Visual disturbances: Such as optic neuritis, which is inflammation of the optic nerve.
• Motor symptoms: Including weakness, spasticity, and coordination problems.
• Sensory symptoms: Such as numbness, tingling, and pain.
• Cognitive and emotional changes: Including memory problems and depression.

Diagnosis

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Histological image showing demyelination in multiple sclerosis.

Diagnosis of demyelinating diseases often involves a combination of clinical evaluation, imaging studies, and laboratory tests. Magnetic resonance imaging (MRI) is a key tool in identifying demyelinating lesions, as it can reveal characteristic patterns such as Dawson's fingers.

Histopathology

Histological image showing CD68-positive macrophages in a demyelinating lesion.

Histopathological examination of demyelinating lesions reveals:

• Loss of myelin: Evident as areas of pallor in myelin-stained sections.
• Inflammatory infiltrates: Composed of lymphocytes and macrophages.
• Gliosis: Proliferation of glial cells, particularly astrocytes.

Treatment

Treatment of demyelinating diseases focuses on managing symptoms, reducing inflammation, and modifying the disease course. Common treatments include:

• Corticosteroids: To reduce acute inflammation.
• Disease-modifying therapies: Such as interferon-beta and monoclonal antibodies to alter the immune response.
• Symptomatic treatments: To manage specific symptoms like spasticity and pain.

Related Pages

• Multiple sclerosis
• Oligodendrocyte
• Autoimmune disease
• Magnetic resonance imaging