Clin Med part 2 Flashcards
Are Schwann cells found in the CNS or PNS?
PNS
What type of cells line the areas of the nervous system that have cerebrospinal fluid and help circulate the fluid?
Ependymal cells
What type of cells facilitate nutrient extraction from blood supply?
Astrocytes
What cells circulate through the nervous system and destroy bacteria or dead cells.
Microglial cells
Typically, what symptom do most MS patients present with first?
Optic neuritis – blurred or diminished vision
What criteria must be met to diagnose MS?
McDonald criteria
What must we see on MRI to diagnose MS?
Must show two or more different areas in central areas of white matter affected at different times
What other test besides MRI should we obtain when suspecting MS?
Lumbar puncture
What will the results of a lumbar puncture look like with a MS patient?
- mild lymphocytosis
- presence of oligoclonal bands
- if you do find albumin, this indicates disruption of the BBB
Most common type of MS?
relapsing remitting
What is Uhthoff’s phenomenon?
Heat intolerance seen in MS patients
How are acute attacks of MS treated?
Treated with glucocorticoids, most commonly IV methylprednisolone
What drug or MS is a mixture of amino acids antigenically similar to myelin protein and works by competing for T-cells?
Glatiramer [Copaxone]
What was the first oral med approved for MS?
Gilenya
What patient population cannot we not prescribe Gilenya to?
Diabetics
What drug results in down-regulation of receptors and T-cell sequestration in lymphoid tissue?
Gilenya
According to Alden-St.Pierre, what has historically been our first line drug for MS?
Glatiramer with out without interferon-B
“Cure” for MS?
Stem-cell transplant
what is an uncommon disease, but is the most common disease of neuromuscular transmission?
Myasthenia Gravis
Up to 75% of patients have __________ abnormalities.
thymic
Is there a genetic predisposition to myasthenia gravis?
yes
What is the main function of the thymus?
To provide an area for T lymphocyte maturation
Ptosis, diplopia, bulbar muscle weakness, expressionless face, neck muscles (head drop) are all symptoms of what disease?
Myasthenia gravis
On physical exam of MG, you will find fatigability of muscles. What muscle in particular?
Muscles of the eye and mouth
Is neuromuscular transmission improved at cooler or warmer temperatures?
Cooler – thats why we do the ice pack test with myasthenia gravis.
80-90% of MG patients will have circulating antibodies against?
NAchR
If your patient is seronegative for MG, but you still believe they have the disease, what other test can you do?
Electrophysiologic testing – single fiber EMG – more complex specialized procedure.
What is the cornerstone of treatment with MG?
Acetylcholinesterase inhibitors
Pyridostigmine, Neostigmine
What treatment is useful for acute decline from MG or myasthenic crisis?
Plasma exchange
What is MG crisis?
Respiratory weakness that can lead to respiratory failure
What are inherited disorders causing progressive muscle weakness and atrophy due to a genetic defect?
Muscular dystrophies
This muscular dystrophy is caused by a defective gene on the X chromosome responsible for producing dystrophin.
Duchenne Muscular Dystrophy
How is Becker muscular dystrophy different from duchenne muscular dystrophy?
Those affected with BMD make some dystrophin where as in DMD none is produced.
What is the classic triad of Emery-Dreifuss Muscular Dystrophy?
Contractures of elbows, ankle plantar flexors, and spine
What is the most common form of muscular dystrophy in whites?
Myotonic dystrophy
What type of muscular dystrophy affects shoulder girdle and/or hip girdle?
Limb-Girdle Muscular Dystrophy
which our muscular dystrophies is autosomal dominant?
Facioscapulohumeral muscular dystrophy
Which muscular dystrophy will have tongue atrophy and facial muscle weakness?
Oculopharyngeal muscular dystrophy