Demyelinating Diseases Flashcards
What occurs when the immune system attacks the myelin sheath in Multiple Sclerosis?
Demyelination
This disrupts the transmission of electrical impulses along the nerves.
What is one of the most common symptoms of Multiple Sclerosis?
Fatigue
Fatigue is frequently reported by individuals with MS.
Name a type of vision problem associated with Multiple Sclerosis.
Blurred vision
Other vision problems include double vision and eye pain.
Often due to optic neuritis.
What symptom of MS involves sensations in the limbs?
Paraesthesia
This symptom can significantly affect daily activities.
What type of MS is characterised by episodes of symptoms followed by periods of remission?
Relapsing-Remitting MS (RRMS)
This is the most common form of MS.
What type of MS progresses steadily without periods of remission?
Primary Progressive MS (PPMS)
This type can be challenging to manage due to its continuous progression.
What is believed to be a contributing factor to the development of Multiple Sclerosis?
Genetic and environmental factors
A family history of MS can increase the risk.
Which diagnostic test is used to detect lesions in the central nervous system for MS?
MRI Scans
MRI is crucial for visualizing the extent of MS damage.
What is the purpose of Disease-Modifying Therapies (DMTs) in MS treatment?
To reduce the frequency and severity of relapses
DMTs are essential in managing the course of the disease.
Fill in the blank: A common symptom management approach for MS includes _______.
[Symptomatic Treatments]
These treatments address specific symptoms like muscle spasticity and pain.
What does rehabilitation for MS typically include?
Physical, occupational, and speech therapy
These therapies aim to improve function and quality of life.
True or False: There is currently a cure for Multiple Sclerosis.
False
While there is no cure, treatments can help manage symptoms.
How does the prognosis for MS vary among individuals?
Some may experience mild symptoms while others have severe disability
Early diagnosis and treatment can improve overall outcomes.
What is Progressive Multifocal Leukoencephalopathy (PML)?
A rare and often devastating demyelinating disease of the central nervous system (CNS)
PML is caused by the reactivation of the JC virus (JCV) in individuals with weakened immune systems.
What virus causes Progressive Multifocal Leukoencephalopathy?
JC virus (JCV)
JCV is kept in check by the immune system and remains latent in the kidneys and possibly other tissues.
In which individuals does PML primarily occur?
Individuals with severely weakened immune systems
This includes patients with HIV/AIDS, certain cancers, or those receiving immunosuppressive treatments.
What are common symptoms of PML?
- Clumsiness or lack of coordination
- Difficulty speaking
- Weakness
- Dementia
- Vision problems
- Headaches and epilepsy
Symptoms vary depending on which parts of the brain are affected.
What is the main characteristic of PML’s pathophysiology?
Subacute and progressive destruction of myelin
Myelin is the protective sheath around nerve fibers in the CNS.
Which cells are primarily infected by the JC virus in PML?
Oligodendrocytes
Oligodendrocytes produce and maintain myelin in the CNS.
What imaging technique is critical for diagnosing PML?
Magnetic Resonance Imaging (MRI)
MRI detects characteristic lesions of PML in the brain.
What analysis is key for diagnosing PML?
Cerebrospinal Fluid (CSF) Analysis
Testing for JC viral DNA using polymerase chain reaction (PCR) is a key diagnostic tool.
What is the primary approach to managing PML?
Restoring the immune response
There is currently no specific antiviral treatment for PML.
What therapy can improve immune function in patients with HIV/AIDS?
Antiretroviral Therapy (ART)
Effective ART can help control JCV reactivation.
What is the prognosis for PML?
Generally poor, with significant disability or death within a few months to a year
Early detection and intervention can improve outcomes.
What ongoing research is being conducted related to PML?
Better diagnostic methods, treatments, and ways to boost the immune response
Understanding molecular mechanisms of JCV reactivation is critical for developing new therapies.
True or False: PML symptoms can include cognitive impairment and personality changes.
True
Dementia symptoms can progress rapidly in PML patients.
Fill in the blank: PML is characterized by the _______ of myelin.
destruction
The destruction of myelin leads to neurological deficits.
What is Devic’s disease also known as?
Neuromyelitis optica (NMO) or neuromyelitis optica spectrum disorder (NMOSD)
Devic’s disease is a rare autoimmune disorder affecting the optic nerves and spinal cord.
What are the primary areas affected by Devic’s disease?
Optic nerves and spinal cord
The disease causes inflammation and demyelination in these areas.
What is a major symptom of Devic’s disease related to vision?
Optic neuritis
This includes pain and vision loss in one or both eyes.
What symptom describes weakness, numbness, or paralysis in the arms and legs?
Transverse myelitis
This is a common symptom associated with Devic’s disease.
List some symptoms of Devic’s disease.
- Optic neuritis
- Transverse myelitis
- Bladder and bowel dysfunction
- Pain
- Cognitive changes
Symptoms can vary among patients.
What causes Devic’s disease?
An autoimmune response attacking the myelin sheath
This leads to inflammation and demyelination, disrupting nerve signal transmission.
What diagnostic tool is used to detect lesions in the optic nerves and spinal cord?
MRI Scans
MRI is crucial for diagnosing Devic’s disease.
What is analyzed during a lumbar puncture in the diagnosis of Devic’s disease?
Cerebrospinal fluid for specific antibodies
This helps confirm the diagnosis.
Which antibodies are commonly tested for in blood tests for Devic’s disease?
- Anti-AQP4
- Anti-MOG
Detecting these antibodies aids in diagnosis.
What is the primary focus of treatment for Devic’s disease?
Managing symptoms and preventing relapses
There is currently no cure for the disease.
What treatment is used to reduce inflammation during acute attacks?
High-Dose Steroids
This is a common treatment approach.
What is plasma exchange used for in the treatment of Devic’s disease?
For patients who do not respond to steroids
It may provide relief for some patients.
Name some immunosuppressive medications used to prevent relapses.
- Rituximab
- Azathioprine
- Mycophenolate mofetil
These medications help manage the disease.
How does the prognosis of Devic’s disease vary among patients?
Some experience mild symptoms and recover, while others have severe disability
Early diagnosis and treatment are crucial for improving outcomes.
Fill in the blank: Devic’s disease results in _______ and _______ dysfunction.
vision loss and spinal cord
These are key features of the disease.
- What are some commonly used Disease-Modifying Therapies (DMTs) for Multiple Sclerosis (MS)?
Commonly used DMTs for MS include Interferon beta, Glatiramer acetate, Fingolimod, Dimethyl fumarate, Natalizumab, Ocrelizumab, and Alemtuzumab.
How does Interferon beta help in managing Multiple Sclerosis?
Interferon beta helps reduce inflammation and modulate the immune system, thereby reducing the frequency and severity of MS relapses.
What is the mechanism of action of Glatiramer acetate (Copaxone/Brabio) in the treatment of MS?
Glatiramer acetate works by mimicking myelin protein, which diverts the immune response away from myelin, thus protecting nerve fibers from autoimmune attack.
Why is Fingolimod used in the treatment of MS, and how does it work?
Fingolimod prevents lymphocytes from exiting lymph nodes, reducing their migration to the central nervous system (CNS). This reduces the immune system’s attack on the myelin sheath, thereby helping manage MS symptoms and progression.
What are the anti-inflammatory and neuroprotective effects of Dimethyl fumarate?
Dimethyl fumarate exerts its effects by activating the Nrf2 pathway, which has anti-inflammatory properties and helps protect nerve cells from damage.
How does Natalizumab block immune cells from entering the CNS in MS patients?
Natalizumab works by binding to the α4-integrin molecule on immune cells, blocking their interaction with vascular cell adhesion molecule-1 (VCAM-1). This prevents immune cells from crossing the blood-brain barrier into the CNS.
In what way does Ocrelizumab target B cells to help in treating MS?
Ocrelizumab is a monoclonal antibody that targets the CD20 protein on B cells. By binding to CD20, Ocrelizumab depletes B cells, which play a role in the pathogenesis of MS, thereby reducing inflammation and the immune system’s attack on myelin.
How does Alemtuzumab affect CD52 on immune cells in MS therapy?
Alemtuzumab targets CD52, a protein found on the surface of immune cells. By binding to CD52, Alemtuzumab depletes these immune cells, leading to immune reconstitution and reducing MS disease activity.
What are the main symptomatic treatments for MS and how do they help manage specific symptoms?
Symptomatic treatments for MS include muscle relaxants (e.g., baclofen, tizanidine) to relieve muscle spasms, pain relievers (e.g., gabapentin, pregabalin) for neuropathic pain, bladder control medications (e.g., oxybutynin, tolterodine) for bladder dysfunction, and corticosteroids to reduce inflammation during acute relapses.
What role do muscle relaxants like baclofen and tizanidine play in MS treatment?
Muscle relaxants like baclofen and tizanidine help relieve muscle spasms and stiffness, improving mobility and comfort for MS patients.
How do pain relievers like gabapentin and pregabalin assist in managing MS symptoms?
Gabapentin and pregabalin are used to treat neuropathic pain, which is common in MS. They help alleviate pain by modulating nerve signals.
What medications are used for bladder control in MS, and how do they work?
Medications like oxybutynin and tolterodine help manage bladder dysfunction by relaxing the bladder muscles, reducing urgency and frequency of urination.
How do corticosteroids help reduce inflammation during acute relapses of MS?
Corticosteroids reduce inflammation and immune activity during acute MS relapses, helping to shorten the duration and severity of the relapse.
What is the mechanism of action of Ocrelizumab in the treatment of MS?
Ocrelizumab targets CD20 on B cells. By depleting B cells, it helps reduce inflammation and the immune system’s attack on myelin, thereby reducing relapses and slowing disease progression.
What type of brain lesions does MS generally cause?
Periventricular, juxtacortical, pericallosal.
Which cells are affected in MS?
Oligodendroglia.
