MS/MG & ALS Flashcards
Define MS
- inflammatory demyelinating disease of CNS: Brain, SC, and optic nerve
- White and Grey matter
What gender is MS MC in? Age range?
Females
20-40 y.o.
MS etiology
- Autoimmune: HLA alleles
- Vitamin D deficiency
- Identical twins association
Define optic neuritis
- Loss of vision in affected eye and pain with eye movement
- Phosphenes: Circular “stars”
Define acute transverse myelitis
Acute partial loss of:
- Motor
- Sensory
- Autonomic
- Reflex
Lhermittes Sign
“Barber shop” test
Neck flexion causes lightning-shock type pain radiation from spine down leg
What are the MC MS sx’s?
- Paresthesia, Trigeminal neuralgia
- Optic neuritis
- Acute Transverse myelitis
- Fatigue
- Autonomic SC sx: Bowel (constipation), bladder dysfunction
- Heat intolerance
- Ataxia
Uhthoff’s phenomen
Worsening symptoms with heat intolerance
Neuro PEx findings in MS
- Spasticity and Positive Babinski. (toes move up)
2. Ataxic gait
Eye PEx findings in MS
- Interneuclear Ophthlmoplegia
2. Nystagmus
What is the imaging of choice to help confirm and monitor the disease progression of MS?
MRI of brain and SC WITH Gadolinum
LP findings in CSF suggestive of MS
- Oligoclonal bodies
2. Immunoglobulin G (IgG)
MS diagnostic criteria
Dissemination in time and space
Management of acute MS exacerbations?
High dose corticosteroids (IV Methylprednisone)
First line treatment for RRMS to reduce frequency and severity of relapse?
B-Interferon or Glatiramer acetate
What helps with fatigue in MS?
Amantadine and Modafinil
Define Myasthenia Gravis
Rare autoimmune disorder=antibodies against Ach postsynaptic receptor @ neuromuscular junction= Decreased Ach receptors= Progressive SKELETAL MUSCLE weakness
Who is MG MC in? Age?
Young Females= 30’s
MG Clinical Manifestations
Fluctuating, fatiguable muscle weakness
- Ocular weakness: Diplopia and ptosis
- Generalized muscle weakness: Bulbar and respiratory
What is NORMAL in MG that. helps differentiate it from other diseases?
- Normal Sensory
2. Normal DTRs
MG PEx
- Myasthenic snarl: Bilateral facial muscle weakness
2. Recovery after rest
What is the most reliable test for diagnosing MG?
anti-acetylcholine receptor antibody test
Why would you want to order a CT scan in MG?
Rule out Thymoma or thymus abnormality
What is first line treatment in MG?
Anti-cholinesterase inhibitors
List Anti-cholinesterase inhibitors
- Pyridostigmine
2. Neostigmine
List immunosuppression therapy in MG and indication
- IV Immune Globulin (IVIg): in myasthenia crisis for rapid response
- Plasmapheresis
- Prednisone: Short and Long term benefit
What is the major complication of myasthenia gravis
Myasthenia crisis= respiratory failure
Define ALS
Mixed UMN and LMN neurodegenerative disease: Progressive motor degeneration
What is another name for ALS?
Lou Gehrig’s disease
UMN sx’s
- Spasticity in LE
- Stiffness
- Hyperreflexia
LMN sx’s
- Progressive bilateral fasciculations
- Muscle atrophy
- Muscle weakness
- Hyporeflexia
What dysfunction do we not see in ALS?
No dysfunction of:
- Cognitive/behavioral
- Sensory
- Autonomic
What movements are spared in ALS?
- Urinary sphincter
2. Voluntary eye movements
What are common first sx’s?
- Drop foot
- Walking
- Speech
- Dexterity
Bulbar sx’s in ALS
- Dysphagia
2. Dysarthria
Later stages in ALS
- Swallowing-Aspiration pneumonia
2. Intercostal-Poor lung function
ALS treatment
Riluzole- Reduce progression by 2-3 months
Riluzole MOA
Inhibits presynaptic release of Glutamate
