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
What is referred to as “floppy baby syndrome:?
Congenital muscular dystrophy
Which our muscular dystrophies will have scapular winging as a symptom?
Facioscapulohumeral muscular dystrophy
When it comes to concussions, what does secondary injury refer to?
Occurs in minutes, hours, days AFTER the initial insult
can be intracellular swelling, electrolyte imbalances, inflammatory response
What are classic signs of a basilar skull fracture?
- Battle’s sign
- Raccoon eyes
- CSF rhinorrhea or otorrhea
- Hemotympanum
What is second impact syndrome?
Diffuse cerebral swelling –> increased cranial pressure
Rare, but fatal
What guidelines do we follow when evaluating whether an athlete is allowed to participate post concussion?
Stepwise return to play:
- No activity and rest until asx
- Light aerobic exercise
- Sport-specific training
- Non-contact drills
- Full-contact drills
- Game play
Can only progress if Asx
Do we give narcotics for pain with concussion?
NO
Put these in the right order:
- Perception
- Transmission
- Transduction
- Modulation
- Transduction: noxious stimuli are converted to electrical signals in sensory nerve endings
- Transmission: neural events which relay the information from the periphery to the cortex
- Modulation: the nervous system can selectively inhibit the transmission of pain signals
- Perception: subjective interpretation by the cortex of the noxious stimulus
Pain occurring in the absence of detectable ongoing tissue-damaging process is a clinical feature of what type of pain?
Neuropathic
What were opioids originally used for?
Diarrhea – dysentery
what are the three cloned opioid receptors?
mu, delta, and kappa
opioid analgesics can work at several levels, what are they?
- peripheral receptors
- spinal cord
- supraspinal sites
What is a desirable effect of opioids?
Analgesia
What are some undesirable effects of opioids?
Nausea, vomiting, constipation, and respiratory depression
What are some mixed desirability effects of opioids?
Sedation, decreased bowel motility, and euphoria
What is the process in which previous exposure to a drug results in diminution of an effect or the need for a higher dose to maintain an effect?
Tolerance
What is the most life-threatening of the side-effects associated with opioid analgesics?
Respiratory depression
What drug reverses the depressant effects of most opioids?
Narcan
What drug is used in the treatment of heroin addiction and managing opioid withdrawal?
Methadone
What drug is marketed as a compound with efficacy similar to codeine with less side-effects?
Tramadol
What drug is indicated for opioid-induced constipation in patients with advanced illness and who are not responding to conventional laxative therapy?
Methylnaltrexone
Describe a resting tremor
Body part affected is supported against gravity, no muscle contraaction
An action tremor is produced by _________ muscle contraction
Voluntary
What type of tremor is present during visually-guided, target-directed motion?
Intentional tremor
What is the most common movement disorder worldwide?
Benign Essential Tremor
95% of benign essential tremors start with postural ________ ____ ______
distal arm tremor
The amplitude of a benign essential tremor increases with?
stress, fatigue, CNS stimulants
The amplitude of a benign essential tremor decreases with?
EtOH, beta-blockade, rest
Treatment options for benign essential tremor?
Primidone, beta-blockade – propranolol
3 take home points of benign essential tremor
- active tremor
- neurological exam NORMAL
- slow onset
4 cardinal signs of Parkinson’s. Go.
- Tremor
- Rigidity
- Bradykinesia
- Postural impairment/gait
true or false
patients with parkinson’s will have weakness and decreased DTRs.
False!
No weakness, and no change in DTRs
What part of the brain is affected with Parkinson’s?
Substantia nigra
When do you start treatment for Parkinson’s?
When functional disability starts
What symptoms do anticholinergics help with for Parkinson’s patients?
tremor
Take home points of Parkinson’s?
- pill rolling, gait disturbance, start hesitation
- slow onset
- treat when FUNCTIONAL disability begins
- Dopamine
What is Huntington’s triad?
Motor, cognitive, and psychiatric symptoms
Tell me about the levels of the following neurotransmitters in Huntington’s
- GABA
- Acetylcholine
- Dopamine
GABA decreases
Acetylcholine decreases
Dopamine decreases
What chromosome is Huntington’s associated with?
4
How many repeats of the glutamine “CAG” sequence results in Huntington’s?
40+
Take home points of Huntington’s?
- Disease of the YOUNG
- Symptomatic treatment
- Multi-care specialty care needed
- Genetic counseling
- Watch for SI
What is an inherited neurobehavioral disorder characterized by sudden involuntary repetitive muscle movements and vocalizations?
Tourette’s
Symptoms need to begin before this age to be diagnosed with Tourette’s?
18
How long does a person need to have Tourette symptoms before being diagnosed?
1 year
What is echopraxia?
meaningless repetition or imitation of the movements of others
What word is used to describe repeating one’s own words?
Palilalia
What are other conditions associated with Tourette’s?
OCD, ADD, rage, anxiety
Tourette’s is caused by excessive _______ in the basal ganglia.
glutamate
For Tourette’s patients, what should we try before starting medications?
Habit reversal training
Take home points of Tourette’s?
- Treatment goal = functional preservation
- Many will have other psychiatric issues
- Meds can be useful but don’t forget about behavior modification therapy
What is a movement disorder in which involuntary sustained or intermittent muscle contractions cause twisting and repetitive movements, abnormal postures or both?
Cerebral palsy
What element may prevent cerebral palsy?
Magnesium
What is compression at the superior thoracic outlet resulting from excess pressure placed on a neurovascular bundle passing between the anterior scalene and middle scalene muscles?
Thoracic Outlet Syndrome
What branches of the trigeminal nerve is usually affected with trigeminal neuralgia?
2nd and 3rd
What are triggers for trigeminal neuralgia?
Light touch – breeze, kiss, shaving, chewing, washing, talking
Does firm pressure elicit pain with trigeminal neuralgia?
no
What drugs do we use to treat trigeminal neuralgia?
anti-convulsants –> carbamazepine, phenytoin
Take home points of trigeminal neuralgia?
- extremely painful
- consider alternative diagnosis above age 50
- exam normal except for severe pain with light touch
What site on the body is most at risk for developing post herpetic neuralgia?
Trigeminal and brachial plexus