What is the Difference Between Leukodystrophy and Leukoencephalopathy

Leukodystrophy and leukoencephalopathy are two white matter diseases. The brain’s white matter constitutes a network of nerve fibers that allows the exchange of information between different areas of the gray matter. The white matter of the cerebrum plays a critical role in helping the body process information, while gray matter focuses on processing sensation, perception, voluntary movement, learning, speech, and cognition. White matter disease is an umbrella term for damage to the white matter of the brain. Both leukodystrophy and leukoencephalopathy can be caused by inherited or other genetic changes.

The key difference between leukodystrophy and leukoencephalopathy is the nature of white matter. leukodystrophy is a condition characterized by the abnormal growth of white matter in the brain while leukoencephalopathy is a condition characterized by changes or damages in the white matter in the brain.

CONTENTS

1. Overview and Key Difference
2. What is Leukodystrophy 
3. What is Leukoencephalopathy
4. Similarities – Leukodystrophy and Leukoencephalopathy
5. Leukodystrophy vs Leukoencephalopathy in Tabular Form
6. Summary – Leukodystrophy vs Leukoencephalopathy
7. FAQ: Leukodystrophy and Leukoencephalopathy

What is Leukodystrophy?

Leukodystrophy is caused by the imperfect growth of cerebral white matter. Leukodystrophy specifically damages the brain’s myelin sheath. The myelin sheath acts as an insulator around nerve fibers in the brain and spinal cord. This condition slows down or blocks messages between the brain and the rest of the body. There are a number of diseases that come under the term leukodystrophy, such as Aicardi-Goutières syndrome, adrenoleukodystrophy (ALD), Alexander disease, Canavan disease, cerebral adrenoleukodystrophy, Krabbe disease, etc.

This condition can be caused by inherited or sporadic genetic mutations. Leukodystrophy can result in particular signs and symptoms such as developmental regression, trouble with motor skills, seizures, cognitive decline, abnormal body and muscle tone, abnormal movements, trouble with speech, difficulty with eating, decline in vision or hearing, and decline in mental and physical development.

Figure 01: Leukodystrophy

Leukodystrophy can be diagnosed through MRI, blood work, genetic tests, urine tests, and neuropsychological screenings. Furthermore, treatment options for leukodystrophy medications for spasticity, such as baclofen, bone marrow and stem cell transplantation, enzyme replacement, and gene therapy.

What is Leukoencephalopathy?

Leukoencephalopathy is a condition due to changes or damage to the white matter of the cerebrum. There are a number of conditions under the term leukoencephalopathy, which include progressive multifocal leukoencephalopathy, toxic leukoencephalopathy, leukoencephalopathy with vanishing white matter, reversible posterior leukoencephalopathy syndrome, and hypertensive leukoencephalopathy. Leukoencephalopathy can be caused by inherited and sporadic gene mutations, viral infections, inhaled toxins, etc. The common signs and symptoms of leukoencephalopathy are loss of coordination, clumsiness, loss of language ability (aphasia), memory loss, vision problems, weakness in legs and arms that gets worse, personality changes, disturbance in coordination, cognitive impairment, sensory and motor deficits.

Figure 02: Leukoencephalopathy

Leukoencephalopathy can be diagnosed through physical examination, MRI, spinal tap, and genetic tests. Furthermore, treatment options for leukoencephalopathy include giving antiretroviral therapy, antioxidant therapy, reducing trauma, blood pressure management (nitroprusside), and seizure management (antiepileptic).

Similarities Between Leukodystrophy and Leukoencephalopathy

1. Leukodystrophy and leukoencephalopathy are two brain conditions that specifically affect the cerebral white matter.
2. Both these conditions can be caused by inherited or other genetic changes.
3. Both these conditions affect myelin sheath development.
4. They may cause similar symptoms, such as movement problems, vision problems, speech problems, etc.
5. Both these can be diagnosed through physical examination, imaging testing, and genetic testing.
6. They can be treated through specific medications and therapies.

Difference Between Leukodystrophy and Leukoencephalopathy

Definition

1. Leukodystrophy is a condition due to imperfect growth of the cerebral white matter of the brain.
2. Leukoencephalopathy is a condition due to changes, images, or malfunction of the cerebral white matter of the brain.

Group of Diseases Under the Term

1. Aicardi-Goutières syndrome, adrenoleukodystrophy (ALD), Alexander disease, Canavan disease, cerebral adrenoleukodystrophy, cerebrotendinous xanthomatosis, Krabbe disease, metachromatic leukodystrophy (MLD), Niemann-Pick disease, Pelizaeus-Merzbacher disease (PMD) and vanishing white matter are diseases that come under leukodystrophy.
2. Progressive multifocal leukoencephalopathy, leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation, toxic leukoencephalopathy, leukoencephalopathy with vanishing white matter, reversible posterior leukoencephalopathy syndrome, leukoencephalopathy with neuroaxonal spheroids, hypertensive leukoencephalopathy and megalencephalic leukoencephalopathy with subcortical cysts are diseases that come under leukoencephalopathy.

Cause

1. Causes of leukodystrophy are inherited and sporadic gene mutations.
2. Causes of leukoencephalopathy are inherited and sporadic gene mutations, viral infections, inhaled or ingestion of toxins, such as carbon monoxide, methanol, ethylene, toluene, ethanol methylenedioxymethamphetamine (MDMA or “ecstasy”), or paradichlorobenzene.

Symptoms

1. Symptoms of leukodystrophy are problems with balance, breathing, cognition, eating and swallowing, hearing, movement and coordination, speech, and vision.
2. Symptoms of leukoencephalopathy are coordination problems, memory loss, vision problems, Inattention, changes in personality, dysarthria, ataxia, dementia, coma, and even death.

Diagnosis

1. Physical examination, blood test, MRI, CT scan, saliva test, urine test, and genetic test are some of the tests taken to diagnose leukodystrophy.
2. Physical examination, MRI, spinal tap, and genetic tests are some of the tests taken to diagnose leukoencephalopathy.

Treatment

1. General treatment of leukodystrophy are medications for seizures, nutritional therapy, hormone therapy, physical and occupational therapy, speech therapy, and bone marrow transplant.
2. General treatment of leukoencephalopathy are antiviral medications, antiretroviral therapy, antioxidant therapy, reducing trauma, blood pressure management (nitroprusside), and seizure management (antiepileptic).

The following table summarizes the difference between leukodystrophy and leukoencephalopathy.

Summary – Leukodystrophy vs Leukoencephalopathy

Leukodystrophy and leukoencephalopathy are two brain conditions that specifically affect the cerebral white matter. Leukodystrophy is characterized by the abnormal growth or degeneration of white matter in the brain while leukoencephalopathy is characterized by changes or damages in white matter in the brain. This is the summary of the difference between leukodystrophy and leukoencephalopathy.

FAQ: Leukodystrophy and Leukoencephalopathy

1. What are the signs and symptoms of leukodystrophy?

• The symptoms leukodystrophy may include loss of the ability to detect sensations, such as touch, pain, heat and sound, loss of intellectual, thinking and memory skills, loss of motor skills, such as walking, moving, speaking and swallowing, stiffness, rigid muscles, poor muscle function, and paralysis.

2. What is the life expectancy of leukodystrophy?

• In late infantile form, death typically happens within five to six years of diagnosis, whereas in juvenile form, it typically results in death 10 to 20 years following diagnosis, and in adult form, death typically happens within six to 14 years after diagnosis.

3. Can leukodystrophy be cured?

• There is no proper cure for leukodystrophy. The symptoms can be managed by medication for seizures, muscle tightness, and movement problems, nutritional therapy or feeding tubes for eating and swallowing problems, hormone therapy, physical and occupational therapy, speech therapy, and bone marrow transplant (BMT).

4. What are the symptoms of leukoencephalopathy?

• The main symptoms of leukoencephalopathy may include loss of coordination, clumsiness, loss of language ability (aphasia), memory loss, vision problems, weakness of the legs and arms that gets worse, personality changes, disturbance in coordination, cognitive impairment, and sensory and motor deficits.

5. Is leukoencephalopathy treatable?

• There are no permanent treatments for leukoencephalopathy that have proved effective. However, symptoms can be managed by antiviral medications, antioxidant therapy, reducing trauma, blood pressure management, and seizure management.

Reference:

1. “Leukodystrophy: What Is It, Types, Symptoms, Treatment & Outlook.” Cleveland Clinic.
2. “Leukoencephalopathy.” ScienceDirect.

Image Courtesy:

1. “Globoid cell leukodystrophy PAS” By Jensflorian – Own work (CC BY-SA 3.0) via Commons Wikimedia

2. “Progressive multifocal leukoencephalopathy 002” By radswiki.net (CC BY 3.0) via Commons Wikimedia

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