MS, Myasthenia Gravis, Guillain Barre and so much more

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Multiple sclerosisGuillain-Barré syndrome

Things you should know

  • Autoimmune disease, triggered by an antigen and resulting in an attack on the peripheral nervous system
    • Campylobacter jejuni
    • Cytomegalovirus
    • Influenza
  • Ascending paralysis - weakness of the feet and hands going upward, towards the trunk

I came in to see my physician assistant today because of…

  • Bilateral ascending paralysis – begins in the lower limbs and rapidly moves up.
    • Difficulty walking with rubbery legs or legs that buckle.
  • Tingling or numbness
  • Muscle tenderness or cramp-like pain
  • Orthostatic hypotension
  • Abnormal heart rate – Palpitations can occur
  • Blurred vision and double vision
  • Difficulty moving facial muscles

Emergency symptoms:

  • Breathing temporarily stops
  • Difficulty breathing
  • Difficulty swallowing
  • Drooling
  • Fainting
  • Feeling light-headed when standing

Labs, Studies and Physical Exam Findings

  • Physical Examination
    • Presence of muscle weakness
    • Loss of tendon reflexes in the arms and legs – Areflexia
    • Loss of proprioception
    • Loss of autonomic function
      • Orthostatic hypotension
      • Changes in rate and rhythm
      • Labile blood pressure
    • Decreased breathing – caused by gradual respiratory paralysis
  • Lumbar puncture –  presence of albuminocytological dissociation, an elevated protein level, but with no increased cell count
  • EMG ( electromyography) and Nerve conduction velocity test – show prolonged distal latencies, conduction slowing, conduction block, and temporal dispersion of compound action potential in demyelinating cases
  • ECG – may show arrhythmias
  • Pulmonary function tests

Treatment

  • Supportive care is the primary treatment.  About 30% of patients will require breathing support
  • Plasmapheresis – in an effort to filter the antibodies
  • Gammaglobulin (IVIG)
  • Rehabilitation – 60- 80% of patients recover completely within a year.

Multiple sclerosis

Things you should know

  • It is a demyelinating disease, however the cause is uncertain
    • Autoimmune destruction of myelin
    • Failure of myelinating cells
  • This is a progressive disease
  • Risk Factors?
    • Geography – more common to those who live farther from the equator and in the northern Europe regions.  People who move after age 15 retain the risk factor from their country of origin.
    • Some genetic variations may be at an increased risk
    • Infections?
    • Smoking
  • The course is usually relapsing and remitting

I came in to see my physician assistant today because of…

  • Symptoms are relapsing and remitting and often move from one area of the body to another during each exacerbation
  • Visual disturbances
    • Double vision, nystagmus, blindness, blurry vision
  • Focal weakness
  • Gait difficulties – Ataxia
  • Fatigue and weakness
  • Numbness or tingling
  • Dizziness and Vertigo
  • Pain
  • Emotional Changes
  • Bladder dysfunction
  • Tremor
  • Headache
  • Seizures

Labs, Studies and Physical Exam Findings

  • Physical Exam
  • Lumbar puncture – may show evidence of chronic inflammation in the CNS including mild protein elevation, mild increase in leukocytes
  • MRI with gadolinium shows areas of demyelination
  • Evoked Potential Tests – measure electrical signals sent by the brain in response to stimuli. It can be visual stimuli or electrical stimuli.  Lesions and nerve damage to the optic nerves, brainstem or spinal cord can be detected.
  • Blood tests – to rule out some infectious diseases that have similar symptoms with MS

Treatment

  • To Treat attacks
    • Corticosteroids high dose
      • oral prednisone
      • intravenous methylprednisolone (Solu-Medrol)
    • Plasmapheresis has been shown to be beneficial
  • To Slow progress of the disease
    • Beta interferons
    • Glatiramer acetate (Copaxone)
    • Fingolimod (Gilenya)
    • Natalizumab (Tysabri)
    • Mitoxantrone
    • Teriflunomide (Aubagio)
  • To Treat Symptoms
    • Physical therapy – will involve stretching and strengthening exercises and the use of devices that can make it easier to perform daily tasks.
    • Dalfampridine (Ampyra) an oral medication may improve walking speed
  • Baclofen and Tizanidine (Zanaflex) help with spasticity
  • Amantadine helps with fatigue
  • Medications also may be prescribed for depression, pain, and bladder or bowel control problems that may be associated with multiple sclerosis.

Myasthenia Gravis

Things you should know

  • An autoimmune disorder where acetylcholine receptors are blocked by antibodies
  • Acetylcholine normally causes an excitatory effect at the neuromuscular junctions

I came in to see my physician assistant today because of…

 

  • Fatigability – Increasing weakness which improves with rest

 

    • Ptosis (Droopy eyelids)
    • Diplopia (Double vision)
    • Difficulty chewing and swallowing
    • Change in the voice
    • Difficulty breathing, leading to respiratory failure (myasthenic crisis)

Labs, Studies and Physical Exam Findings

  • Physical and Neurologic Exam
    • Often Normal
    • Easy Fatigability, weakness improving after rest and worsening again on repeat exertion
    • Normal reflexes and feeling of sensation
  • Blood test
    • Acetylcholine receptor antibody test
  • Ice test – applying ice causes an increase in strength
  • Edrophonium is a short acting anticholinesterase which may aid in the diagnosis
  • CXR / CT / MRI – to identify a tumor or thymoma
  • Nerve Conduction Studies
  • Electromyography – results will present muscle fibers to be unresponsive to stimuli
  • Pulmonary function tests
  • Muscle biopsy

Treatment

  • Medication
    • Acetylcholinesterase inhibitors
      • Neostigmine and pyridostigmine – slows down the natural enzyme cholinesterase that degrades acetylcholine in the motor end plate.
    • Immunosuppressive drugs
      • Prednisone, cyclosporine, mycophenolate, and azathioprine
      • Use in combination with an acetylcholinesterase inhibitor.
  • Plasmapheresis and IVIG – in an emergency to remove antibodies
  • Surgery
    • Thymectomy

PANCE Tips

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The Final Step

Key Terms and Ideas

Matching section answer choices are MS, MG, GB.

  • Ascending paralysis                                                   GB
  • Easy fatigability which improves with rest          MG
  • Relapsing and remitting symptoms                       MS
  • Has an issue with acetylcholine receptors           MS
  • Loss of tendon reflexes                                              GB

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Concussions, Dementia, Delerium

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300px ConfusionConcussion

Things you should know

  • Also known as minor or mild brain injury
  • Caused by head trauma leading to blunt trauma of the brain

I came in to see my physician assistant today because of…

  • Typically, symptoms have an immediate onset
  • Headache or a feeling of pressure in the head, and worsens over time
  • Temporary loss of consciousness
  • Confusion or feeling as if in a fog
  • Amnesia surrounding the traumatic event
  • Dizziness or “seeing stars”, lasting and recurrent
  • Tinnitus – ringing in the ears
  • Nausea and vomiting, repeatedly
  • Slurred speech
  • Delayed response to questions
  • Fatigue
  • Difficulty with concentration and memory
  • Irritability and crankiness
  • Loss of balance and unsteady gait
  • Crying excessively
  • Change in eating or sleeping patterns
  • Seizure
  • Vision or eye issues like blurred or double vision.  Light sensitivity.
  • Large head bumps or bruises on areas other than the forehead

Labs, Studies and Physical Exam Findings

  • Rule out Intracranial hemorrhage
  • Neurologic Examination
    • Eyes
      • Unequal pupils is a concerning sign
    • Hearing
    • Strength and sensation
    • Balance
    • Coordination
    • Reflexes
  • Cognitive Test
    • Glasgow coma scale with a score of 13-15
    • Memory
    • Concentration
  • Imaging Tests
    • CT Scan / MRI Scan – If hemorrhage is suspected

Treatment

  • Prevention is best!!
  • Pain medications – Ibuprofen, acetaminophen
  • Sleep aids may be necessary
  • Physical and cognitive rest for about a week
  • Monitoring  for 24 to 72 hours

Post-concussion Syndrome

Things you should know

  • Symptoms may last for years but typically resolve over a few months.

I came in to see my physician assistant today because of…

  • Headache – most common
  • Dizziness and Vertigo
  • Nausea
  • Double, or blurred, vision
  • Hearing loss and Tinnitus
  • Reduced sense of smell and taste
  • Problems tolerating light and noise
  • Easily irritable
  • Feeling anxious easily
  • Depression
  • Sleep disturbance
  • Reduced sex drive
  • Loss of appetite
  • Personality changes such as showing socially or sexually inappropriate behavior
  • Lack of energy and interest on things
  • Having sudden outbursts of emotion
  • Difficulty remembering things
  • Concentration and attention problems
  • Slowed reaction times
  • Problems processing information and problem solving
  • Difficulty learning new things

Labs, Studies and Physical Exam Findings

  • Physical Examination
    • Muscle strength
    • Coordination and Sensation
    • Mental Processes
      • Stroop Color Test
      • 2 &7 Processing Speed Test
      • Rivermead Post Concussion Symptoms Questionnaire
      • Hopkins Verbal Learning A Test
  • CT Scan
  • MRI Scan

Treatment

  • Medication
    • Amitriptyline and Dihydroergotamine combined with metoclopramide (Reglan) for headache relief
  • Psychotherapy
    • Cognitive behavioral therapy
    • Educate the patient on how to deal with impairments
  • Neurotherapy – an operant conditioning test where patients are given conditional audio/visual rewards after producing particular types of brainwave activity

Dementia

Things you should know

  • Loss of the ability to problem solve significant enough to affect the patient’s activities of daily living
  • Chronic slowly progressive loss of function
  • Causes
    • Non reversible causes
      • Alzheimer’s Disease is the most common cause making up about 75% of all dementias
      • Brain injury
      • Lewy body dementia
      • Vascular dementia
    • Reversible causes
      • Hypothyroidism
      • Vitamin B12 deficiency
      • Lyme disease
      • Neurosyphilis
      • Hydrocephalus

I came in to see my physician assistant today because of…

  • Loss of the ability to think clearly and problem solve
  • Impulsivity
  • Anxiety
  • Depression
  • Agitation
  • Balance and coordination problems
  • Tremor
  • Difficulty with speech and language
  • Eating and swallowing difficulty
  • Delusions
  • Hallucinations
  • Memory distortions
  • Wandering and restlessness

Labs, Studies and Physical Exam Findings

  • Medical History
  • Cognitive Testing
  • Laboratory tests
    • VItamin B12
    • Folic Acid
    • TSH
    • C-reactive protein
    • Complete Blood Count
    • Electrolytes
    • Calcium
    • Renal Function
    • Liver Tests
  • CT or MRI – may suggest normal pressure hydrocephalus
  • SPECT or PET – to assess long-standing cognitive dysfunction

Treatment

  • Treatable causes may be dealt with appropriately
  • Psychological Therapies
  • Medication
    • Acetylcholinesterase inhibitors – Donepezil
    • Antipsychotic drugs
    • Antidepressants

Delirium

Things you should know

  • Acute state of confusion
  • Causes
    • Electrolyte imbalance
    • Advancing age
    • Fever and acute infection, particularly in children
    • Previous delirium episodes
    • Visual or hearing impairment
    • Poor nutrition or dehydration
    • Severe, chronic or terminal illness
    • Multiple medical problems or procedures
    • Treatment with multiple drugs
    • Alcohol or drug abuse or withdrawal
    • Infection
    • Psychological stress
    • Medications
      • Especially anticholinergics, opioids

I came in to see my physician assistant today because of…

  • Changes in alertness, usually more alert in the morning
  • Changes in feeling, sensation and perception
  • Altered level of consciousness
  • Changes in movement
  • Sleep disturbances
  • Drowsiness
  • Disoriented to time or place
  • Decrease in short-term memory and recall
    • Anterograde Amnesia – Unable to remember events since delirium began
    • Retrograde Amnesia – Unable to remember events before delirium
  • Disrupted or wandering attention
    • Inability to think or behave with purpose
    • Problems concentrating
  • Disorganized thinking
  • Emotional or personality changes
    • Anger
    • Agitation
    • Anxiety
    • Apathy
    • Depression
    • Euphoria
    • Irritability
  • Incontinence

Labs, Studies and Physical Exam Findings

  • Neurologic examination, to include tests of sensation, cognitive, and motor
    • Mental status test
  • Neuropsychological studies
  • Blood and Chemical tests:
    • Ammonia levels in the blood
    • Blood chemistry (comprehensive metabolic panel)
    • ABG
    • Creatine kinase level in blood
    • Drug, alcohol levels (toxicology screen)
    • Liver function tests
    • Serum magnesium
    • Thyroid function tests
    • Urinalysis
    • Vitamin B1 and B12 levels
    • Cerebrospinal fluid (CSF) analysis
  • Radiology
    • Chest X-Ray
    • Head CT scan
    • Head MRI scan
  • Electroencephalogram (EEG)

Treatment

Treatment involves:

  • Treating underlying causes:
    • Anemia
    • Hypoxia
    • Heart failure
    • Hypercapnia
    • Infections
    • Kidney failure
    • Liver failure
    • Nutritional disorders
    • Psychiatric conditions
    • Thyroid disorders
  • T-A-DA method – Tolerate, anticipate, don’t agitate
  • Medications – depends on the cause
    • Haloperidol, Olanzapine
    • Benzodiazepine – if delirium is due to alcohol withdrawal, benzodiazepine withdrawal, or if antipsychotic is contraindicated to the condition causing the delirium
    • Trazodone
  • Non-pharmacologic treatments
    • Avoiding unnecessary movements
    • Having recognizable faces at the bedside, such as family members
    • Providing means of orientation (Calendar or clock)
    • Verbal and non-verbal de-escalation techniques – to offer reassurance and calm the patient

 

Delirium VS Dementia

 

     DELIRIUM DEMENTIA
ONSET      Abrupt Usually insidious
COURSE      Fluctuates Slow decline
DURATION      Hours to weeks Months to years
ATTENTION      Impaired Intact early; often impaired late
SLEEP-WAKE      Disrupted Usually normal
ALERTNESS      Impaired Normal
ORIENTATION      Impaired Intact early; impaired late
BEHAVIOR      Agitated, withdrawn or depressed Intact early
SPEECH      Incoherent, rapid/slowed Word-finding problems
THOUGHTS      Disorganized, delusions Impoverished
PERCEPTION      Hallucinations/illusions Usually intact early

 

Study Tip

7-9 hours of sleep.  I am totally serious.  This may be the best study tip I can give.  Your brain is not only your most important asset it is your only asset.

 

Key Terms and Ideas

  • You are trying to determine between dementia and delirium.
    • A patient presents with very poor attention is.  Is this likely dementia or delirium?
      • Delirium
    • Patient is telling a story in the appropriate order, but is really struggling with word finding.  Is this likely dementia or delirium?
      • Dementia
  • Name two cognitive tests that are used for diagnosing dementia.
  • A patient is in a car accident.  He appears uninjured, but is having difficulty standing and isn’t making a lot of sense.  He suddenly throws up.  What is the likely diagnosis?
    • Concusion
  • name two reversible causes of dementia
    • Hypothyroidism
    • Vitamin B12 deficiency
    • Lyme disease
    • Neurosyphilis
    • Hydrocephalus
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