S3_L3: Demyelinating Diseases Flashcards
It is currently the most widely used CSF test for confirmation of MS
Oligoclonal bands
It is the most powerful diagnostic tool and most helpful in the diagnosis of MS
Magnetic Resonance Imaging
Enumerate the 4 risk factors for MS
- Caucasian / northern european
- Temperate latitude
- Family hx of MS
- Female preponderance
Determine whether the prognosticating factor is favorable or worse in the prognosis of MS
- Female
- Progressive form from onset
- Visual or somatosensory presentation
- Poor recovery from first attack
A. Favorable
B. Worse
- A
- B
- A
- B
Determine whether the prognosticating factor is favorable or worse in the prognosis of MS
- Late Onset
- Motor symptoms from onset
- Onset before age 40
- Male
A. Favorable
B. Worse
- B
- B
- A
- B
Determine the pathology of the ff demyelinating diseases
- Alternating bands of destruction and preservation of myelin in a series of concentric rings
- Multifocal white matter lesions in the CNS
- Multifocal meningeal infiltration
- Large, sharply outline, asymmetrical, foci of myelin destruction in the cerebral hemispheres
A. Multiple Sclerosis
B. Schilder’s sclerosis
C. Balo’s sclerosis
D. ADEM
- C
- A
- D
- B
What is the most common symptom of multiple sclerosis?
Pyramidal weakness
Which manifestation of multiple sclerosis involves the spinal cord?
Transverse myelitis / acute myelitis
What white matter fiber tract is affected in bilateral internuclear ophthalmoplegia?
Medial Longitudinal Fasciculus
The clinical features of bilateral internuclear ophthalmoplegia are paralysis of (1)___ and nystagmus of the (2)___ eye.
- adduction
- abducting
What does the Lhermitte sign indicate?
Lesion in the posterior column in the cervical spinal cord
What is felt when the Lhermitte sign is attempted?
Sensation of electricity/tingling down the back
How is the Lhermitte sign performed?
Do passive or active flexion of the neck
Determine whether it is a clinical feature of MS or not
- Uveitis
- Visual loss
- Early dementia
- Sensory loss
- Agnosia
A. Suggestive
B. Not suggestive
- B
- A
- B
- A
- B
Determine whether it is a clinical feature of MS or not
- Seizures
- Homonymous or bilateral hemianopsia
- Fatigue
- Apraxia
- Sphincter disturbance
A. Suggestive
B. Not suggestive
- B
- B
- A
- B
- A
Determine whether it is a clinical feature of MS or not
- Onset before the age of 10 and after 55
- Brainstem dysfunction
- Extrapyramidal symptoms
- Continued progression from onset without relapse
- Cerebellar ataxia & tremor
A. Suggestive
B. Not suggestive
- B
- A
- B
- B
- A
A type of Acute disseminated encephalomyelitis that is more common in children and the most fulminant
Acute necrotizing hemorrhagic encephalomyelitis
What are the two virus candidates most commonly implicated in the pathogenesis of MS?
- Epstein-Barr virus
- Human Herpesvirus 6
Concentric Sclerosis of Balo is prevalent among which nationality/race?
Filipinos
Devic Neuromyelitis Optica is also known as?
Opticospinal MS
Demyelination of the posterior and lateral funiculi of the spinal cord or subacute combined degeneration is caused by?
Vitamin B12 Deficiency
Determine which area is affected by the ff secondary demyelination diseases
- Ventral pons
- Posterior and lateral funiculi
- Corpus callosum
A. Marchiafava-Bignami disease
B. Central Pontine Myelinolysis
C. Subacute Combined Degeneration of the Spinal Cord
- B
- C
- A
Marchiafava-Bignami disease is commonly seen in what gender, their lifestyle, and location?
Male heavy alcoholic beverage drinkers from South America (Brazil)
What is the chronic inflammatory autoimmune disease of unknown etiology that is the most common and most recognized demyelinating disease?
Multiple Sclerosis
Multiple Sclerosis is more prevalent in (1)___ and symptoms typically occur at ages (2)___
- women
- 21-40 y/o
Multiple sclerosis has a low prevalence in what 3 locations?
- Asia
- Latin America
- South America
TRUE OR FALSE: The white population is at a greater risk for acquiring multiple sclerosis than the asian and african populations.
True
In the pathology of MS, multifocal plaques represent a variable combination of 1-4)___?
- Demyelination
- Inflammation
- Axonal loss
- Remyelination
In MS, there is a predilection for ___ matter structures such as the cerebrum, cerebellum, brainstem, spinal cord and optic nerve
white
The autoimmune mechanisms in multiple sclerosis are possibly triggered by what 2 factors?
- Environmental
- Genetic susceptibility
Rapid correction of hyponatremia is the cause of what secondary demyelination disease?
Central pontine myelinolysis
Determine which sx are present in the ff clinical manifestations
- Acute ataxia
- Midline back pain
- Bowel and bladder, and sensory problems
- Sudden fever, headache, confusion, somnolence
- Paraparesis or quadriparesis, depressed DTRs
A. Encephalitis
B. Acute transverse myelitis
C. Cerebellitis
- C
- B
- B
- A
- B
What 2 viral infections commonly cause acute necrotizing hemorrhagic encephalomyelitis?
- Measles
- Varicella (chickenpox)
What 2 vaccines can cause acute necrotizing hemorrhagic encephalomyelitis?
- Old anti rabies vaccine
- Smallpox vaccine
A patient presents with numerous large confluent edematous lesions in the cerebral hemisphere. The MD also saw hemorrhages in the gray and white matter. What encephalomyelitis does the patient present with?
Acute necrotizing hemorrhagic encephalomyelitis
Devic neuromyelitis optica is a necrotizing immune mediated demyelination of what 2 structures?
- Optic nerve [Optic neuritis]
- Spinal cord [transverse myelitis]
TRUE OR FALSE: Devic neuromyelitis optica is more common among young adults in Asia and Africa and affects men and women equally.
True
A case states that the 24 y/o female patient had nausea, vomiting, and hiccups; had blurry vision and pain with eye movements. Symptoms relapsed within 3 years. What condition does the patient present with?
Devic neuromyelitis optica
A case states that a 23 y/o male patient presents with devic neuromyelitis optica. What do we expect to find in the ancillary procedures done on the pt?
Note that we did a cranial and spinal MRI, and lumbar puncture.
Cranial MRI: -
Spinal MRI: +, shows signal abnormality extending of 3 vertebral segments
Lumbar puncture: Elevated WBC count, CSF pleocytosis
Devic neuromyelitis optica has a poor prognosis and may often lead to what?
Enumerate all 3 possible results
- Blindness (Unilateral or bilateral)
- Hemi- to paraplegia
- Death (d/t respiratory compromise)
Determine whether the clinical manifestation is found in Balo’s Sclerosis or Schilder’s Sclerosis
- Dementia
- Headache
- Pseudobulbar palsy
- Akinetic mutism
- Deafness
A. Concentric sclerosis of Balo
B. Diffuse cerebral sclerosis of Schilder
- B
- A
- B
- A
- B
Determine whether the clinical manifestation is found in Balo’s Sclerosis or Schilder’s Sclerosis
- Homonymous hemianopia
- Mental & behavioral changes
- Hemiplegia or quadriplegia
- Communication and consciousness disturbances
- Fever
A. Concentric sclerosis of Balo
B. Diffuse cerebral sclerosis of Schilder
- B
- A
- B
- A
- A
Determine whether the clinical manifestation is found in Balo’s Sclerosis or Schilder’s Sclerosis
- Primitive reflexes
- Protracted remitting course
- Bowel/bladder symptoms
- Paralysis
- Cortical blindness
A. Concentric sclerosis of Balo
B. Diffuse cerebral sclerosis of Schilder
- A
- B
- A
- A
- B
A patient presents with Schilder’s sclerosis. What do you expect and not expect to find in the CSF examination?
Expect to find: Myelin basic protein
Not expect: Oligoclonal bands
A short course of ___ followed by 2 weeks of oral prednisone usually increases the speed of recovery of vision by 2-3 weeks in MS
IV Methylprednisolone
This condition presents with signs of neurologic dysfunction in motor, sensory, or autonomic nerves and nerve tracts of the spinal cord
Transverse Myelitis / Acute Myelitis
Enumerate the 4 signs of optic neuritis
- Reduced visual acuity
- Reduced contrast sensitivity
- Reduced color vision
- Central scotoma
Fill in the blanks of the steps in the immunopathogenesis of MS
- (1)___ activated in the periphery
- Up-regulate surface molecules that enable them to more efficiently (2)___
- Adhere to the endothelial cells of the (3)___
- Active secretion of (4)___
- Facilitates immune cell invasion into the (5)___ where they may become reactivated and impact on the biology of CNS elements
- Immune cells
- Adhere
- Blood brain barrier
- Matrix proteases
- CNS
Arrange the steps of MS immunopathogenesis in order:
Activation, Reactivation, Attraction, Adhesion, Damage vs Repair, & Invasion
Activation
Adhesion
Attraction
Invasion
Reactivation
Damage vs Repair
TRUE OR FALSE: MS has no clear genetic predisposition, but susceptibility to MS is inherited.
True
Match the secondary demyelinating disease with its viral infection
- Subacute Sclerosing Panencephalitis
- Human T-cell lymphotropic or leukemia virus type I
- Progressive multifocal leukoencephalopathy
A. HAM or Tropical Spastic Paraparesis
B. JC Papilloma virus
C. Measles virus
- C
- A
- B
TRUE OR FALSE: There is no specific reliable diagnostic test for MS
True
What is the known treatment for acute demyelinating optic neuritis that can improve the ultimate visual prognosis?
None
In MS, what does a high number of attacks indicate?
Neurologic deficit becomes more severe leading to devastation
Match the CSF exam component with its corresponding finding
- Total protein
- IgG
- WBC
- Oligoclonal bands
- Protein electrophoresis
A. Oligoclonal bands
B. Normal
C. Seen in more than 90% of definite MS
D. Normal or modest lymphocytic pleocytosis
E. Increased, more than 12% of total protein
- B
- E
- D
- C
- A
It may be the only measure of activity of the disease as it occurs during acute onset or exacerbation
Pleocytosis
Addt’l: Polymorphonuclear may predominate in hyperacute cases
Fill in the blanks for criteria of definite MS
- Two separate (1)___ symptoms
- Two separate attacks and onset of symptoms is separated by at least (2)___.
- Symptoms must involve the (3)___
- Objective deficits are present on the neurologic examination
- Age is usually (4)___
- No other medical problem can be found
- central nervous
- 1 month
- white matter
- 20-40
TRUE OR FALSE: The key to the clinical criteria for the diagnosis of MS is 2 separate symptoms at 2 separate times or lesion disseminated in time
True
TRUE OR FALSE: In the Schumacher’s criteria, there must be evidence of the involvement of two or more separate parts of the CNS
True
In Schumacher’s criteria, involvement of the neuraxis must have occurred temporarily in one of the ff patterns:
- In one or more episodes of worsening supported by a period of one month or more, each episode lasting at least (1)___
- Stepwise progression of signs/symptoms over a period of at least (2)___
- 24 hours
- 6 months
