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Flashcards in Shappy Final Deck (174):
1

Parkinson's Disease

Pathophysiology:

 

 

Pathophysiology:

  • Degeneration of Substantia Nigra, Locus Ceruleus, dorsal aspect of putamen, and brainstem.
    • –Neuroglial cells issues
      • –Lewy bodies ultimately get formed (IMPORTANT to KNOW!)
        • •Synuclein Filled
          • a chemical marker (an inflametory protein)

2

Congenital Malforations: Characteristics/Details (3)

 


•Congenital Malformations

  1. –Manifest early in life
    • –Example: maybe cerebellum maybe just didn’t form)
  2. –doesn’t progress (won’t get worse)
  3. –Variety of deficits is possible
     

2

What is ALS ?

 


•Motor neuron disease

  1. –Degenerative spinal cord, brain, brainstem motor neuron loss.
  2. –Typically develops in UE first, then progresses to lower
    • •One of few conditions where Diplegia is present in UE

3

What is Motor Control?

Dr Bringman:

Motor control is

  • the ability to maintain and change posture and movement
  • the result of a complex set of neurological and mechanical process.

Shappy Notes: Motor control

(she said we must have some knowledge of this side beyond this slide and that we got it from Bringman. She did not talk more about it really)

 

 


 

 

4

Multiple Sclerosis

Clinical Course (general):

 

 

 

Clinical Course (general):

  1. May be progressive or not, may have remissions and relapses. Very unpredictable.
  2. optic nerve, motor and sensory cortex- not limited to these areas.
  3. symptoms wax and wane which may or may not have an element of progression

5

what are some good questions to ask yourself when trying to treat movement disorders?

 

How can I control these movement disorders?

What does society say about what is appropriate or acceptable? (when deciding what to do to help pts with problems)

5

Multiple Sclerosis

Non-PT Treatment

What about for an exacerbation?

 

Non PT Treatment:

  1. Anti-inflammatory (steroid) at first to stop inflammatory response
  2. may use IVIG to attack anitbodies that are created.
  3. Oligodendrocytes can be repaired (remyelination can happen).

    1. Remyelination can stop at any point.

For an Exacerbation:

  1. Exacerbation (attack): symptoms get worse, treat immediately with anti-inflammatory (steroids), controls edema, after edema goes away the symptoms may also reduce. It is an argument to be as fit as possible before the attack

7

define hypotonia

flaccidity

7

define Nystagmus

 

  • –Rapid alternating eye movement (back and forth of the eyes; rapid alt of the eye movements)
  • –Involuntary
  • –Some people live in this state

7

Tremor: Basic definition


•Rhythmic alternating oscillatory movements of anything
 

7

Describe (or just read) more details about Romberg Test

 

From Wikipedia:

Romberg's test or the Romberg maneuver is a test used in an exam of neurological function, and also as a test for drunken driving. The exam is based on the premise that a person requires at least two of the three following senses to maintain balance while standing: proprioception (the ability to know one's body in space); vestibular function (the ability to know one's head position in space); and vision (which can be used to monitor [and adjust for] changes in body position).

A patient who has a problem with proprioception can still maintain balance by using vestibular function and vision. In the Romberg test, the standing patient is asked to close his or her eyes. A loss of balance is interpreted as a positive Romberg's test.

The Romberg test is a test of the body's sense of positioning (proprioception), which requires healthy functioning of the dorsal columns of the spinal cord.[1]

The Romberg test is used to investigate the cause of loss of motor coordination (ataxia). A positive Romberg test suggests that the ataxia is sensory in nature, that is, depending on loss of proprioception. If a patient is ataxic and Romberg's test is not positive, it suggests that ataxia is cerebellar in nature, that is, depending on localized cerebellar dysfunction instead.

It is used as an indicator for possible alcohol or drug impaired driving and neurological decompression sickness.[2][3] When used to test impaired driving, the test is performed with the subject estimating 30 seconds in his head. This is used to gauge the subject's internal clock and can be an indicator of stimulant or depressant use.

http://en.wikipedia.org/wiki/Romberg%27s_test

7

what are tremors? (what are some variables of tremors?)

 

•Tremors; rhythmic, non-rithmic, oscilating, regular
 

7

Can you see plaques on an MRI for someone with MS?

 

Scars plaque can be found anywhere in CNS

7

characteristics of Secondary Progressive MS: (4)

 

Secondary Progressive MS:

  1. •Develops from relaxing remitting
  2. •However is more progressive
  3. •Occasional relapses to differentiate it with minor remissions
    • •A lot of these people will plateau during the remission period (you will not see them return to full function)
  4. •pts are older at this age

7

ALS: prognosis

From onset of symptoms to death: 5-10 years (usually from respiratory problems)

7

Does ALS affect cognition?

 

Cognitive function is generally spared for most people, although some (about 5%) also develop frontotemporal dementia.

per wikipedia: http://en.wikipedia.org/wiki/Amyotrophic_lateral_sclerosis#Diagnosis

8

what is a postural tremor?

 

 

A type of Action tremor (happens when body is in movement).

  1. Postural tremor: maximal when a limb is maintained in a fixed position against gravity.
  2. large movments
     

8

What two ways the neuron can degenerate?

 

  1. Something wrong in the synapse
  2. Problem not in the synapse

8

Progressive Supranuclear Palsy:

Transmission:

 

 

Transmission:

seems to have a genetic component

8

Progressive Supranuclear Palsy:

Diagnosis: (3)

 

 

Diagnosis:

  1. MRI will not show until severe atrophy.
  2. so diagnosis must be by symptoms before it is severe
  3. Has protein depositis like other similar diseases, possibly part of scar tissue while body is trying to heal (so it doesn't show until that point)

9

define tremor (brief definition of it generally)

rhythmic ocillatitory movement

  • –“another garbage tern”
  • –Many types of tremors
  • –Rhythmic alternating oscillatory movements of anything
    • •Facial twitches

 

10

what is the center of motor cordination?

 

the cerebellum

10

what can both demyelination and synaptic degeneration lead to?

 

 

•Death of neuron

  • •Louie bodies
    • •Build up of protein and eosinophil
    • •Common in Alzheimer's and Parkinson's

10

Parkinson's Disease

Diagnosis, Non PT: (3)

 

 

Diagnosis, Non PT:

  1. –Looking for levels of dopamine, L-dopa, and markers that is Synuclein in lewy bodies
  2. –Ultimately, we can see degeneration on CT scan and MRI
    • •Degeneration of Substantia Nigra, Locus Ceruleus, brainstem, and dorsal aspect of putamen
    • •Results in the motor deficits we define as Parkinsons
  3. Try treating like parkinsons (L-dopa or other dopamine replacement therapy) to see if it responds

10

Multiple Sclerosis (everything below, but not detailed types and clinical manifestations):

What is it?

Epidemiology:

Etiology:

Pathophysiology:

Clinical Course (general):

Exacerbating Factors:

Non PT treatment:

PT Treatment:

 

What is it?

  • Chronic inflammatory demyelinating disease of CNS

Epidemiology:

  • Adult onset typically 20-40 years old
  • Race: white 2x more likely than African Americans
  • Sex: Females 2x more likely than males

Etiology:

  • Unknown
  • Best accepted theory is some sort of underlying viral disorder that causes autoimmune response
    • Some have suspected chalmydia, but this has not been proven and is debatable
  • Possible geneitc connection (15%)

Pathophysiology:

  • Autoimmune resonse sets of Immune system
  • •Something crosses BBB and causes demyelination (that can stop at any point)
    • attacks to myelin cause inflammation and then scar tissue develops,
    • oligodendrocytes are the glial cells that myelinate CNS axons and experience damage
      • shappy said they are the ones that are activated to respond (but microglial cells are like the phagocytes of the CNS, so I would think they have a large role in the innflammation and immue response - maybe oligodendrocytes try to regenerate?
    • You get inflammation first, then you will get the scar tissue after on the site (treat with inflammatory first)

  • •Slows conduction
  • •Decreasing transmission
  • •Results in weakness
  • Sort of like a CNS version of chronic Gillian Barre.

Clinical Course (general):

  • May be progressive or not, may have remissions and relapses. Very unpredictable.
  • optic nerve, motor and sensory cortex- not limited to these areas.

  • symptoms wax and wane which may or may not have an element of progression

Exacerbating Factors:

  • –Heat
  • –Stress

Non PT Treatment:

  • Anti-inflammatory at first to stop inflammatory response
  • may use IVIG to attack anitbodies that are created.
  • Oligodendrocytes can be repaired (remyelination can happen).

    • Remyelination can stop at any point.

  • For an Exacerbation:
    • Exacerbation (attack): symptoms get worse, treat immediately with anti-inflammatory (steroids), controls edema, after edema goes away the symptoms may also reduce. It is an argument to be as fit as possible before the attack

PT Treatment:

  1. –Help them avoid exacerbating factors
  2. –Exercise is okay, but not in hot environments (pools must be cool)
  3. –May need specialized outcome measures
    • Think of things that you can measure to show progress
      • Sensations (semmes weinstein, temperature, etc.)
    • •Need more research
      • •Including more short term and long term outcome measures
    • –Insurance companies don’t want to reimburse for maintenance
      • •Insurance companies like short term outcome measures
  4. Work on balanc and overall conditioning
  5. ”Strategic Weighting” Weighted belt can improve ability to move. There is certain spots for the weights on the belts. (sort of like jackets for scared dogs)

  6. Don’t forget wound care stuff

10

what does ALS stand for?

Amyotrophic Lateral Sclerosis

11

Progressive Supranuclear Palsy:

Treatment: (3)

 

Treatment:

  1. does not respond to dopamine replacement like parkinson's dz
  2. a fact sheet I found discussed treatment for symptoms (like difficulty swallowing), but said there was no treatment for the actual disease.
  3. PT's treat symptoms (I surmise)

11

What are tics?

 

•Tics: the urge to do it and the relief felt afterwards. “it” is whatever.
 

12

Movement Disorders, non-rhythmic: what is hemiballismus?

 


•Hemiballismus: non rhythmic rapid movements. Violent, flinging, non-suppressible.

dr Lake taught us that it would be contralateral to the legion, and it is usually the upper extremity. Hemi means one sided, so it would just be one arm. Ballisums also exists, which would be bilateral (make sure you read carefully when taking the exam!)
 

12

What are ways neurons can degenerate at the synapse?

 

why is it important to know what is wrong?

Problems with:

  1. receptors,
  2. reuptake,
  3. neurotransmitter production (amount of neurotransmitters)

if you know what is wrong, then you can give the correct meds to help with the synapse

12

Fragile X:

Transmission
 

 

Transmission:

Carrier is the female (daughters of males who have the disease) . dad can be symptomless, but pass it on to the daughter who will then give to her sons who will show the symptoms

13

What is Agillity?

 

1. the power of moving quickly and easily; nimbleness (http://dictionary.reference.com/browse/agility)

 

From Wikipedia, since we didn't discuss much in class:

Agility or nimbleness is the ability to change the body's position efficiently, and requires the integration of isolated movement skills using a combination of balance, coordination, speed, reflexes, strength, and endurance. Agility is the ability to change the direction of the body in an efficient and effective manner and to achieve this requires a combination of

balance – the ability to maintain equilibrium when stationary or moving (i.e. not to fall over) through the coordinated actions of our sensory functions (eyes, ears and the proprioceptive organs in our joints);
static balance – the ability to retain the centre of mass above the base of support in a stationary position;
dynamic balance – the ability to maintain balance with body movement; speed - the ability to move all or part of the body quickly; strength - the ability of a muscle or muscle group to overcome a resistance; and lastly,
co-ordination – the ability to control the movement of the body in co-operation with the body's sensory functions (e.g., in catching a ball [ball, hand and eye co-ordination]).

In sports, agility is often defined in terms of an individual sport, due to it being an integration of many components each used differently (specific to all of sorts of different sports). Sheppard and Young (2006) defined agility as a "rapid whole body movement with change of velocity or direction in response to a stimulus".[attribution needed]

Agility is also an important attribute in many role playing games, both computer games and as Dungeons and Dragons. Agility may affect the character's ability to evade an attack or navigate uneven terrain.

http://en.wikipedia.org/wiki/Agility

13

Movement Disorders: What is a  hyperkinetic movement disorder characterizd by?

 

increased movement

14

How many categories of MS are there?

 

6 categories of Primary Progressive MS

15

Movement Disorders: What are rhythmic movement disorder characterizd by?

 


–Rhythmic- regular alternating or oscillatory
 

16

define  Scanning Speech:

 

  • –Slow enunciation and a tendency for hesitation
    • •It’s there, but it is slow and interrupted (almost a stutter)
    • •Typically more at beginning of word or sylable

(slow speech, interrupted, hesitated)

16

What are the 5 theories of ALS Etiology?

 

 

  1. •Free radical build up
    • –Enzymes whose job it is to elimiate free radicals are dysfunctional (superoxide)
  2. •Neurotransmitter issues
    • –Glutamate is typically elevated
  3. •Motor neuron degeneration
  4. •Autoimmune
  5. •Unscheduled apoptosis++
     

17

what is friedreich's ataxia?

Friedreich's ataxia is an autosomal recessive inherited disease that causes progressive damage to the nervous system. It manifests in initial symptoms of poor coordination such as gait disturbance; it can also lead toscoliosis, heart disease and diabetes, but does not affect cognitive function. The disease progresses until a wheelchair is required for mobility. Its incidence in the general population is roughly 1 in 50,000

 

from wikipedia

18

Pathologic Tremor: Intention Tremor, two examples of causes/symptoms

 

 

  1. •Cerebellar lesions- low frequency, unilateral, ataxia, dysmetria, dysdiadochokinesia, dysarthria
  2. •History of drug use- tremor when stopping
     

19

Definition of Dysmetria:


–Inability to control ROM (hard to pick up specific objects)
 

19

What are Five types of Motor Coordination?

 

Coordination

  1. –Dexterity
  2. –Agility
  3. –Intra limb coordination
  4. –Inter limb coordination
  5. –Visual motor coordination
    • •Hand-eye coordination

19

What does POMA stand for?


–Performance-Oriented Mobility Assessment (POMA)
 

20

Movement Disorders: What is a  hypokinetic movement disorder characterizd by?

what are some goals for treatment?

 


–Hypokinetic- decreased or slow
•Treat: speed them up or prevent them from getting slower
 

21

Huntington's Disease

PT role in Treatment:

 

 

PT role in Treatment

  1. we might be the first medical professional  to recognize symptoms
  2. We can teach adaptations
  3. We can try to slow progression

22

What is Inter-limb coordination?

 

From Wikipedia:

Inter-limb coordination concerns how movements are coordinated across limbs. J. A. Scott Kelso and colleagues have proposed that coordination can be modeled as coupled oscillators, a process that can be understood in the HKB (Haken, Kelso, and Bunz) model.[13] The coordination of complex inter-limb tasks is highly reliant on the temporal coordination. An example of such temporal coordination can be observed in the free pointing movement of the eyes, hands, and arms to direct at the same motor target. These coordination signals are sent simultaneously to their effectors. In bimanual tasks (tasks involving two hands), it was found that the functional segments of the two hands are tightly synchronized. One of the postulated theories for this functionality is the existence of a higher, "coordinating schema" that calculates the time it needs to perform each individual task and coordinates it using a feedback mechanism. There are several areas of the brain that are found to contribute to temporal coordination of the limbs needed for bimanual tasks, and these areas include the premotor cortex(PMC), the parietal cortex, the mesial motor cortices, more specifically the supplementary motor area (SMA), the cingulate motor cortex (CMC), the primary motor cortex (M1), and the cerebellum.[14]

http://en.wikipedia.org/wiki/Motor_coordination#Intra-limb

24

Pontocerebellum (neocerebellum) is composed of which anatomical parts?

Lateral parts of the hemispheres

 

Picture: Cerebrocerebellum is the same as pontocerebellum

A image thumb
25

Physical therapy for ALS

from wikipedia

Physical therapy plays a large role in rehabilitation for individuals with ALS. Specifically, physical and occupational therapists can set goals and promote benefits for individuals with ALS by delaying loss of strength, maintaining endurance, limiting pain, preventing complications, and promoting functional independence.

26

Huntington's Disease

Cause:

 

 

Cause:

gene mutations

 

27

What are three parts of the CNS that influence voluntary movement?

  1. Corticospinal Tract
  2. Basal Ganglia
  3. Cerebellum

29

what is ataxia?

 

•Ataxia: really wide based gait.

(I think it is broader than just gait, but go with what shappy said)
 

31

what is "decomposition of movement"?


-inability to correctly sequence fine coordinated movements/acts

 

33

Movement Disorders, non-rhythmic: what are Tics?

 

Tourette's is the example. Rapid repetitive non-rhythmic. Pts usually have some urge to do it and get some sort of relief after they do it. There is some thought that urge can be suppressed.

34

what is chorea?

 

 

•Chorea: non-rhythmic jerky rapid movements. Non-suppressible, involuntary. Distal muscles, facial muscles are commonly involved.

34

ALS Epidemiology

Epidemiology

  1. –Men more common than women (2:1)
  2. –10-15% seem to have familial link
  3. –30K cases in USA (15 diagnosed per day)

35

Parkinson's Disease

Treatment: (Non PT and PT)

 

Treatment:

  • Treat even if MRI is negative
    1. Dopamine replacement therapy (L-dopa is common)
    2. PT:
      • shappy told us about BIG therapy; DO NOT CALL IT "BIG" therapy in your notes unless certified!!!
      • Walker with laser beam that can help break akinesia in pt
      • Treat symptoms
      • Don't give up, our brains are the limit on treatment options!  

36

Clinical Manifestations of ALS affecting Bulbar tract neurons (4)

 

 

  1. •Face
  2. voice
  3. swallowing
  4. speaking

37

what is an action tremor?


–Happens when body is in movement. It may or may not change when the target is reached.

there are at least three types
 

37

Parkinson's Disease (everything):

What is it?/Cause/Progression:

Pathophysiology:

S/S:

Diagnosis, Non PT:

DIagnosis, PT:

Treatment:

 

 

What is it?/Cause/Progression:

  • Idiopathic, slow progressive, degenerative disorder
  • Can be caused by drugs, trauma, strokes, lots of stuff. May be a familial connection

Pathophysiology:

  • Degeneration of Substantia Nigra, Locus Ceruleus, and brainstem,
    • dorsal aspect of putamen is also involved
  • –Neuroglial cells issues
  • –Lewy bodies ultimately get formed (IMPORTANT to KNOW!
    • •Synuclein Filled
      • a chemical marker (an inflametory protein)

S/S (but not a check-list; pt may not have all symptoms):

 

  1. –Festinating gait
  2. –Resting tremor
  3. –Pill rolling
  4. –Stooped posture
  5. –Bradykinesia
  6. –No arm swing
  7. –Rigidity
  8. –Dementia
  9. –Akinesia
  10. –Sleep disorder issues (typically)
  11. –Flat affect

Diagnosis, Non PT:

 

  • –Looking for levels of dopamine, L-dopa, and markers that is Synuclein in lewy bodies
  • –Ultimately, we can see degeneration on CT scan and MRI
    • •Degeneration of Substantia Nigra, Locus Ceruleus, brainstem, and dorsal aspect of putamen
    • •Results in the motor deficits we define as Parkinsons
  • Try treating like parkinsons (L-dopa or other dopamine replacement therapy) to see if it responds

DIagnosis, PT:

 

  • PT tests
    • reflexes -  positive babinski (so hyperreflexive)
    • Speed dependent tone
    • •Finger to nose,
    • tone and rigidity
    • diadodyskenisia
    • •Postural reflexes
    • •Analyze gait
    • balance
    • facial expressions (flat affect)
    • lack of blinking
    • Try treating like parkinson's to see if it improves condition

Treatment:

  • Treat even if MRI is negative
    • Dopamine replacement therapy (L-dopa is common)
    • PT:
      1. shappy told us about BIG therapy; DO NOT CALL IT "BIG" therapy in your notes unless certified!!!
      2. Walker with laser beam that can help break akinesia in pt
      3. Treat symptoms
      4. Don't give up, our brains are the limit on treatment options!

 

 

 

38

ALS DIagnosis

 

ALS is a very difficult disease to diagnose. To date, there is no one test or procedure to ultimately establish the diagnosis of ALS. It is through a clinical examination and series of diagnostic tests, often ruling out other diseases that mimic ALS, that a diagnosis can be established. A comprehensive diagnostic workup includes most, if not all, of the following procedures:

  1. electrodiagnostic tests including electomyography (EMG) and nerve conduction velocity (NCV)
  2. blood and urine studies including high resolution serum protein electrophoresis, thyroid and parathyroid hormone levels and 24-hour urine collection for heavy metals
  3. spinal tap
  4. x-rays, including magnetic resonance imaging (MRI)
  5. myelogram of cervical spine
  6. muscle and/or nerve biopsy
  7. thorough neurological examination

http://www.alsa.org/about-als/diagnosing-als.html

40

Multiple Sclerosis

Etiology:

 

 

Etiology:

  • Unknown
    • Best accepted theory is some sort of underlying viral disorder that causes autoimmune response
      • Some have suspected chalmydia, but this has not been proven and is debatable
    • Possible geneitc connection (15%)

41

Etiology of Cerebellar Disorders: 3 categories

 

  1. Congenital Malformations
  2. Hereditary Ataxias
  3. Acquired Conditions

43

What are three things that an MD considers when making a medical diagnosis for cerebellar disorders?

 

 

  1. •Family history
  2. •Neuroimaging/MRI
  3. •Genetic testing
     

44

Managment of ALS

 

 

 

  1. •We usually go straight to power chair with multiple different concepts on how to get it to move, partly because UE goes first
    • •Sip and puff and eye devices
  2. •Computerized technology for speech
    • –Lots of very expensive technology for very short period of time
       

Wikepedia says:

Management of ALS attempts to relieve symptoms and extend life expectancy. This supportive care is best provided by multidisciplinary teams of health care professionals working with the person and their caregivers to keep them as mobile and comfortable as possible

Then lists the following:

  1. medications
  2. breathing support
  3. Therapy
    1. physical
    2. occupational
    3. speech
  4. nutrition
  5. pallitiave care (care meant to keep pt comfortable but not improve condition as they approach death)

45

What are three types of Pathologic Tremors?

  1. Action or postural tremor
  2. Resting tremor
  3. Intention Tremor

45

Progressive Supranuclear Palsy:

 

S/S: (8 examples)

 

 

S/S:

  1. bradykinesia,
  2. rigidity,
  3. eye movement,
    • Cant look down (can look side to side but the eyes can not go down; if the head is moved up and down, the eyes stay forward- compared pt to a doll’s eyes
  4. progressive,
  5. pseudo-bulbar palsy (facial),
  6. dementia,
  7. From Video: gait is wide and staggering (not like parkinson's with the shuffling)
    • I think they lean backwards too (not forwards like parkinson's)
    • pt seems apethetic to their unsteadyness and "plunges ahead"
  8. From Video: Characteristic speech pattern: spastic speech in combo with ataxic speech
    • strained and slow
    • syllables grouped into unnatural groups with unnatural pauses
    • this speech pattern occurs in almost no other condition
  • The video of the guy in his underwear had Progressive Supranuclear Palsy ("don't worry about his underwear!")

46

what are the defining functions of the pontocerebellum?
 

quick finely controlled limb movement (mainly UE coordination)

 

48

Dr. Shappy spent a lot of time talking about an example of Dystonia, Spasmodic Torticollis. We should probalby watch the videos she posted.

 


•http://www.youtube.com/watch?v=pfDYLYyoUyw
•http://www.youtube.com/watch?v=28kMNZdNHaw
•http://www.youtube.com/watch?v=P6XhHb90ciQ
•http://www.youtube.com/watch?v=ElSYsIQMJZQ
•httphttp://www.youtube.com/watch?v=u_-UO2upGW8
•http://www.youtube.com/watch?v=I1bD5Dun7Ss
 

48

Characteristics of Benign MS? (2)

 

.Benign MS

  • (about 20% of MS
  • •Mild disease with full function greater than 15 years)

50

Fragile X:

Treatment

Treatment:

There are some med that can help, but only slow the progression/ does not fix (behavioral, speech, balance, etc). IEPs fdeveloped for education at school

51

9 sysmptoms to evaluate for differential diagnosis

 

  1. •Chorea: non-rhythmic jerky rapid movements. Non-suppressible, involuntary. Distal muscles, facial muscles are commonly involved.
  2. •Athetosis: like slow chorea. Impaired inhibition of thalamocortical neurons by the basal ganglia. Excess dopamine activity?
  3. •Hemiballismus: severe chorea. Unilateral rapid, non-rhythmic, wild flinging movements. Lesion in or around the contralateral subthalamic nucleus.
    • –Tx: antipsychotic medications.
  4. •Ballismus: bilateral version of hemiballisums, but less common.
  5. •Myoclonus: restless leg syndrome falls into this category. Brief shock-like muscle contractions.
  6. •Tics: the urge to do it and the relief felt afterwards. “it” is whatever.
  7. •Ataxia: really wide based gait.
  8. •Tremors; rhythmic, non-rithmic, oscilating, regular
  9. •Dystonias: sustained abnormal posturing movements. Or jerky, twisting movements.
     

52

Fragile X:

Signs and symptoms
 

Signs and symptoms:

s/s: Progressive neural dysfunctional diseases: tremors, dystonia, mood issues (impatience, hostility), autonomic nervous system issues (sweating, bowel, bladder, ect), Parkinson like, and eventually dementia

53

Movement Disorders, non-rhythmic: what is athetosis?

 

movements that are non-rhythmic slow and wringing. Primarily distal muscles (Dr. Lake: common in CP pts). Tend to produce a flowing stream of movement.

 

Dr. lake gave us a youtube video of an adult man with CP with his fingers moving in his affected (and dysfunctioning) hand. I think he looked like he was from India or some similar country. His hand looked spastic or rigid with difficult continued movement of fingers.
 

54

Huntington's Disease (everything):

Type of Disorder:

Pathophysiology:

Cause:

S/S:

Progression/Prognosis:

Diagnostics:

Non PT treatment options:

PT role in Treatment:

 

 


Type of Disorder:

  • Autosomal dominant disorder

•Pathophysiology:

  • Caudate nucleus from a pathophysiologic standpoint
    • Spiny neurons in corpus striatum degenerate and there is a
    • decrease in neurotransmitters,
      • substance P and GABA neurotransmitters specifically

Cause:

  • gene mutations

•Signs and symptoms:

  • List of s/s is large. There are similarities to schizophrenia, bipolar, antisocial, dementia, depression, apathy, irritability, and then obvious movement disorders (Chorea, gross movement disorders – see video): puppet like gait, huntington’s dance, tongue protrusion, mouth movements, head thrusting, quick eye movements,

•Progression/Prognosis:

  • –Very severe – ends in long term care and maybe psych ward.
  • – Usually live 13-15 years from onset of symptoms.

•Diagnostics

  • –Some CT MRI imaging on caudate nucleus and frontal lobe.
    • Usually starts to show up at 40-50 years old on a CT scan.
      • must have a certain number of neurons degenerate in order to pick up dz on scan
  • –Can be genetically tested for
  • Often diagnosed by presence of symptoms

Non PT treatment options:

  • there are some medications that can help with symptoms a bit

PT role in Treatment

  • we might be the first medical professional  to recognize symptoms
  • We can teach adaptations
  • We can try to slow progression

55

Multiple Sclerosis

PT Treatment:

 

PT Treatment:

  1. –Help them avoid exacerbating factors
  2. –Exercise is okay, but not in hot environments (pools must be cool)
  3. –May need specialized outcome measures
    • Think of things that you can measure to show progress
      • Sensations (semmes weinstein, temperature, etc.)
    • •Need more research
      • •Including more short term and long term outcome measures
    • –Insurance companies don’t want to reimburse for maintenance
      • •Insurance companies like short term outcome measures
  4. Work on balanc and overall conditioning
  5. ”Strategic Weighting” Weighted belt can improve ability to move. There is certain spots for the weights on the belts. (sort of like jackets for scared dogs)
  6. Don’t forget wound care stuff

 

57

What is the difference between physiologic and pathologic tremor?

  • –Physiological just means it’s there, but its not interfering or getting worse. Not sign of an underlying condition.
    • •Typically barely perceivable until stress provokes it or ability to inhibit it are down
  • –Pathologic is a sign of an underlying condition
     

58

What is the pathophysiology of tremor? (three main)

 


•Pathophysiology-

  1. lesions of brain stem,
  2. extrapyramidal,
  3. cerebellum

–Is complex, lots of stuff.
 

59

Describe Pyramidal tracts and what they do


•Pyramidal tracts- pass through the medullary pyramids

  • –Connect cerebral cortex to brain stem and SC lower motor centers

 

More from wikipedia:

The pyramidal tracts (pyramides)[citation needed] include both the corticospinal and corticobulbar tracts. These are aggregations of upper motor neuron nerve fibres that travel from the cerebral cortex and terminate either in the brainstem (corticobulbar) or spinal cord (corticospinal) and are involved in control of motor functions of the body.

The corticobulbar tract conducts impulses from the brain to the cranial nerves.[1] These nerves control the muscles of the face and neck and are involved in facial expression, mastication, swallowing, and other functions.

The corticospinal tract conducts impulses from the brain to the spinal cord. It is made up of a lateral and anterior tract. The corticospinal tract is involved in voluntary movement. The majority fibres of the corticospinal tract cross over in the medulla, resulting in muscles being controlled by the opposite side of the brain. The corticospinal tract also contains Betz cells (the largest pyramidal cells), which are not found in any other region of the body.

The pyramidal tracts are named because they pass through the pyramids of the medulla.

http://en.wikipedia.org/wiki/Pyramidal_tracts

61

Parkinson's Disease

DIagnosis, PT: (11 examples of things to test)

 

 

DIagnosis, PT:

PT tests

  1. reflexes -  positive babinski (so hyperreflexive)
  2. Speed dependent tone
  3. •Finger to nose,
  4. tone and rigidity
  5. diadodyskenisia
  6. •Postural reflexes
  7. •Analyze gait
  8. balance
  9. facial expressions (flat affect)
  10. lack of blinking
  11. Try treating like parkinson's to see if it improves condition

61

ALS Pathophysiology

 


•Pathophysiology

  1. –Motor neuron progressive degeneration
  2. progression
    1. – from cranial nerves
    2. –Can progress to sensory
    3. –Bowel and bladder
  3. –Typically develops in UE first, then progresses to LE
    • •One of few conditions where Diplegia is present in UE
  4. –Distal to proximal
  5. –Ultimately can affect breathing
     

63

what is athetosis?

 

•Athetosis: like slow chorea. Impaired inhibition of thalamocortical neurons by the basal ganglia. Excess dopamine activity?

(this is the one Dr. Lake showed as part of CP)

64

Three Treatments for Dystonias

 

 

  • –Physical measures
  • –Botulinum toxin injections or medication
  • –Surgery to release (like in dorsal rhizotomy)
     

65

Huntington's Disease

Progression/Prognosis:

 

 

Progression/Prognosis:

  • –Very severe – ends in long term care and maybe psych ward.
  • – Usually live 13-15 years from onset of symptoms.

 

66

what is the defining functions of the vestibulocerebellum?


•Maintaining equilibrium and coordinating eye, head, neck movement.
 

67

Describe the system that are usually involved in movement disorders (because they are part of motor control)?

  • •Pyramidal tracts- pass through the medullary pyramids
    • –Connect cerebral cortex to brain stem and SC lower motor centers
  • •Extrapyramidal system- Basal ganglia
    • –Caudate nucleus, putamen, globus pallidus, subthalamic nucleus and substantia nigra
    • –Deep to the forebrain, direct output rostrally through thalamus to cerebral cortex
    • –Movement disorders typically occur in this system
       

Dr. Shappy basically skipped over this slide. She said When we have Disordered movement, we are looking at movement disorders that are cause by problems in this system (didn’t specify if she meant both or one or the other)

69

List 8 functional balance tests

 

  1. –Romberg test: (I noted that I should look it up)
    • •Often done when pulled over for DUI
    • •Stand on one leg, put head back, close eyes (or not) and touch nose alternating with fingers from outstretched arms.
  2. –Functional Reach and Multidirectional Reach Tests
  3. –Berg Balance Scale
  4. –Performance-Oriented Mobility Assessment (POMA)
  5. –Timed get up and go test (TUG)
  6. Tinetti Assessment Tool
  7. 4-Stage Balance Test
  8. 30-Second Chair Stand Test

(see hand-outs)

71

Fragile X:

Diagnostic testing
 

 

Diagnostic testing:

Diagnostic testing: Genetic testing can identify it, MRI, EKG can show the deficiencies in the brain.

 

72

What are the things the PT can identify and evaluate in a pt with a cerebellar disorder? (9 examples)

 

The PT assists in diagnosis, outcome measures, assessment

  1. Ataxia
  2. Decomposition of Movement
  3. Dysarthria
  4. Dysdiadochokinesia
  5. Dysmetria
  6. Hypotonia
  7. Nystagmus
  8. Scanning Speech
  9. Tremor

72

Multiple Sclerosis

What is it?

What is it?

Chronic inflammatory demyelinating disease of CNS

74

Movement Disorders: What are non-rhythmic movement disorder characterizd by?

 

can be slow, sustained, or rapid

Many types!

75

What are complex tremors?

A combination of any kind of tremor

76

What are three classifications of movement disorders?

and some examples

 

  1. •Hypokinetic disorders- Decreased or slow movement
    • –Parkinson’s disease
  2. •Hyperkinetic disorders- Increased movement
    • –Tremors, myoclonus, dystonia, chorea, hemiballismus, athetosis, tics
  3. •Overlap
    • like tremors in Parkinson’s disease

Shappy said all of these terms were defined in last class.

77

Movement Disorders, non-rhythmic: what is chorea?

 


•Chorea: non-rhythmic jerky and rapid. Distal muscles or the face are most common.

[My note: This is typical in Huntington's Disease (aka Huntington's Dance)]
 

78

Multiple Sclerosis

Exacerbating Factors:

 

 

Exacerbating Factors:

–Heat
–Stress

80

what is an example of a pathology that involves the spinocerebellum?

Friedreich's ataxia

82

what is dystionia?

symptoms?


Dystonia: Sustained involuntary muscle contractions of antagonistic muscle groups

Symptoms: Abnormal posturing or jerky, twisting, intermittent spasms
 

83

Vestibulocerebellum (Archicerebellum) is composed of which anatomical parts?

Flocculonodular lobe (flocculus and nodule)

 

Picture: cerebrocerebellum is the same as pontocerebellum

A image thumb
83

What are two diseases that Progressive Supranuclear Palsy is easily confused with?

 

Easily confused with Parkinson's and Alzheimer's

84

Two types of Hereditary Ataxias

 

  1. Freidreich Ataxia
  2. Spinocerebellar Ataxia

86

In general, what does the cerebellum do in regards to voluntary movement?

 

Cerebellum- center of motor coordination

  • Complex interaction to ensure smooth, purposeful movement without extraneous muscular contractions

88

will most movement disorders show up on scans?

Can we treat them if they dont?

 

A lot of these conditions will not show up on scans until they are very severe.

Too hard to test in the basal nuclei down to the neuron level (to see it degenerating). Most of these disorders are identified post mortem.

 

So we may not have a specific diagnosis, but we can treat the symptoms

 

 

90

Neuron degeneration: what are some problems that can occur outside of the synapse?

 


•Demyelination is the main one

  • •Progressive degenerative process that causes abnormal responses



 

92

Describe extrapyramidal system and what it does

 


•Extrapyramidal system- Basal ganglia

  • –Caudate nucleus, putamen, globus pallidus, subthalamic nucleus and substantia nigra
  • –Deep to the forebrain, direct output rostrally through thalamus to cerebral cortex
  • –Movement disorders typically occur in this system
     

I found this confusing without explanation, so below is a helpful section from wikipedia:

In anatomy, the extrapyramidal system is a neural network that is part of the motor system that causes involuntary reflexes and movement, and modulation of movement (i.e. coordination). The system is called "extrapyramidal" to distinguish it from the tracts of the motor cortex that reach their targets by traveling through the "pyramids" of the medulla. The pyramidal pathways (corticospinal and some corticobulbar tracts) may directly innervate motor neurons of the spinal cord or brainstem (anterior (ventral) horn cells or certain cranial nerve nuclei), whereas the extrapyramidal system centers on the modulation and regulation (indirect control) of anterior (ventral) horn cells.

Extrapyramidal tracts are chiefly found in the reticular formation of the pons and medulla, and target neurons in the spinal cord involved in reflexes, locomotion, complex movements, and postural control. These tracts are in turn modulated by various parts of the central nervous system, including the nigrostriatal pathway, the basal ganglia, the cerebellum, the vestibular nuclei, and different sensory areas of the cerebral cortex. All of these regulatory components can be considered part of the extrapyramidal system, in that they modulate motor activity without directly innervating motor neurons.

The extrapyramidal system is very old and three of the four tracts of the human extrapyramidal system are clearly present in salamanders.[1][2]

The extrapyramidal tracts include parts of the following:[3]

rubrospinal tract
pontine reticulospinal tract
medullary reticulospinal tract
lateral vestibulospinal tract
tectospinal tract

 

http://en.wikipedia.org/wiki/Extrapyramidal_system

93

Movement Disorders, non-rhythmic: what are Tremor?

(general)

 


•Definition: Rhythmic alternating oscillatory movements of anything
many types (go back to other slides/flashcards for full story)

95

Is the cerebellum isolated?

No It has conneections the basal ganglia, etc.

95

What is Dexterity?

 

From Wikipedia, since we didn't explicity discuss it:

Fine motor skills (or dexterity) is the coordination of small muscle movements—usually involving the synchronization of hands and fingers—with the eyes. The complex levels of manual dexterity that humans exhibit can be attributed to and demonstrated in tasks controlled by the nervous system. Fine motor skills aid in the growth of intelligence and develop continuously throughout the stages of human development.

http://en.wikipedia.org/wiki/Fine_motor_skill

Other definitions:

skillfulness in the use of one's hands or body.

Fine Motor Control: Ability to perform delicate manipulations with the hand requiring steadiness, muscle control, and simultaneous discrete finger movements.
Synonym(s): dexterity, fine coordination, fine motor control.

http://medical-dictionary.thefreedictionary.com/dexterity

96

does damage to the three regions of the cerebellum occur in isolation?

doesn't have to

can have injuries that inovle one, two, or three of the areas

98

Dystonia: diagnosis

 


Diagnosis by Exclusion of other diseases, such as:

  • •Tardive dyskinesia
  • •Basal ganglia and other CNS infections
  • •Neck infections or tumors
     

99

What is intra-limb coordination?

 

 

From Wikipedia:

Intra-limb coordination involves the planning of trajectories in the Cartesian planes.[4] This reduces computational load and the degrees of freedom for a given movement, and it constrains the limbs to act as one unit instead of sets of muscles and joints. This concept is similar to "muscle synergies" and "coordinative structures." An example of such concept is the Hogan and Flash minimum-jerk model,[15] which predicts that the parameter that the nervous system controls is the spatial path of the hand, i.e. the end-effector (which implies that the movement is planned in the Cartesian coordinates). Other early studies showed that the end-effector follows a regularized kinematic pattern[16] relating movement's curvature to speed and that the central nervous system is devoted to its coding.[17] In contrast to this model, the joint-space model postulates that the motor system plans movements in joint coordinates. For this model, the controlled parameter is the position of each joint contributing to the movement. Control strategies for goal directed movement differ according to the task that the subject is assigned. This was proven by testing two different conditions: (1) subjects moved cursor in the hand to the target and (2) subjects move their free hand to the target. Each condition showed different trajectories: (1) straight path and (2) curved path.[18]

http://en.wikipedia.org/wiki/Motor_coordination#Intra-limb

101

Huntington's Disease

Diagnostics:

 

 

 

Diagnostics

  • –Some CT MRI imaging on caudate nucleus and frontal lobe.
    • Usually starts to show up at 40-50 years old on a CT scan.
    • must have a certain number of neurons degenerate in order to pick up dz on scan
  • –Can be genetically tested for
  • Often diagnosed by presence of symptoms

102

what is ballismus?

treatment?

 

 

•Ballismus: bilateral version of hemiballisums, but less common.

probably has same treatment ast hemiballisums: antipsychotic meds

103

what are Lewy Bodies?

 

Wikipedia:

Lewy bodies are abnormal aggregates of protein that develop inside nerve cells in Parkinson's disease (PD), Lewy body dementia, and some other disorders. They are identified under the microscope when histology is performed on the brain.

Lewy bodies appear as spherical masses that displace other cell components. The two morphological types are classical (brain stem) Lewy bodies and cortical Lewy bodies. A classical Lewy body is an eosinophilic cytoplasmic inclusion consisting of a dense core surrounded by a halo of 10-nm-wide radiating fibrils, the primary structural component of which is alpha-synuclein. In contrast, a cortical Lewy body is less well defined and lacks the halo. Nonetheless, it is still made up of alpha-synuclein fibrils. Cortical Lewy bodies are a distinguishing feature of dementia with Lewy bodies (DLB), but may occasionally be seen in ballooned neurons characteristic of Pick's disease and corticobasal degeneration,[1] as well as in patients with other tauopathies.[2] They are also seen in cases of multiple system atrophy, particularly the Parkinsonian variant.[3]

http://en.wikipedia.org/wiki/Lewy_body

104

What are Four big categories of movement disorders?

  1. Hypokinetic
  2. Hyperkinetic
  3. Rhythmic
  4. Nonrhythimc

105

what is something that distigueshis Progressive Supranuclear Palsy from Parkinson's?

 

Progressive Supranuclear Palsy doesn't respond to dopamine replacement therapy

105

What are three types of motor neurons that ALS affects?

 

  1. LMN
  2. UMN
  3. Bulbar neurons

106

Multiple Sclerosis: Four autonomic functions affected

 

Autonomic changes

  1. –Cardiovascular
  2. –Bladder
  3. –Bowel
  4. –Sexual dysfunction

107

What is an essential tremor? (2)

 


•Essential tremor-

  1. familial condition typically;
  2. rhythmic shaking of almost any body part
     

107

ALS Etiology

 

 

  • Unkown
  • –A number of theories
    1. •Free radical build up
      • –Enzymes whose job it is to elimiate free radicals are dysfunctional (superoxide)
    2. •Neurotransmitter issues
      • –Glutamate is typically elevated
    3. •Motor neuron degeneration
    4. •Autoimmune
    5. •Unscheduled apoptosis
       

108

Characteristics of Progressive Relapsing MS: (2)

 

Progressive Relapsing

  1. •Intervals between relapses actually show disease progression (so in between exacerbations you are still getting worse)
  2. •Occurs with aging

110

Parkinson's Disease

What is it?/Cause/Progression:

 

 

What is it?/Cause/Progression:

  1. Idiopathic, slow progressive, degenerative disorder
  2. Can be caused by drugs, trauma, strokes, lots of stuff. May be a familial connection

112

What are the three main parts of the cerebellum called by their phylogenic names (matched with thier functional names in parentheses)?

archicerebellum (vestibulocerebellum)

paleocerebelum (spinocerebellum)

neocerebellum (pontocerebellum)

 

113

what is an alternate name for ALS?

 

Lou Gheric's Disease

114

what is a intention tremor?

A type of Action tremor (happens when body is in movement).

  1. occurs during the movement towards the target.
  2. Might go away when target is reached.
     

115

Multipule Sclerosis: Clinical Manefestations/What does MS affect? (9 examples)

 

 

Anywhere CNS nerves are

  1. –Sensory Cortex
  2. –Pain
  3. –Visual Changes (optic nerve)
  4. –Motor dysfunction (motor cortex)
  5. –Speech/swallowing
  6. –Cognitive
  7. –Depression
  8. –Affective
  9. •Autonomic changes
  • –Cardiovascular
  • –Bladder
  • –Bowel
  • –Sexual dysfunction


•Basically everything in CNS

116

What is Friedreich ataxia?
 

 

Shappy Notes: Genetic mutation; Progressive disorder

(see video:  https://www.youtube.com/watch?v=ThMH3WCUU4A)

(also is the crossfit guy vidio: https://www.youtube.com/watch?v=rHlC1W9r704)

Wikipedia:

Friedreich's ataxia is an autosomal recessive inherited disease that causes progressive damage to the nervous system. It manifests in initial symptoms of poor coordination such as gait disturbance; it can also lead to scoliosis, heart disease and diabetes, but does not affect cognitive function. The disease progresses until a wheelchair is required for mobility. Its incidence in the general population is roughly 1 in 50,000.

http://en.wikipedia.org/wiki/Friedreich%27s_ataxia

117

what is dystonia

 

•Dystonias: sustained abnormal posturing movements. Or jerky, twisting movements.

(spasmotic torticolis was an example she gave)

119

What is a kinetic tremor?

what amplitude?

 

A type of Action tremor (happens when body is in movement).

  1. tend to appear in the last part of the movement toward the target.
  2. Typically small amplitude

121

what is an example of a pathology that involves the pontocerebellum?

Chorea

(as in Huntington's Dance or Huntington's Chorea)

122

What does TUG stand for?


–Timed get up and go test (TUG)
 

123

Pathologic Tremor: Resting Tremor, two diseases that commonly have resting tremor

 

 

Resting Tremor

  1. •Parkinson’s- pill rolling, chin, leg; shuffling gait, bradykinesia, cogwheel rigidity, micrographia
  2. •Progressive supranuclear palsy- coarse or jerky tremor with eye gaze issues (typically difficulty looking down)
     

124

characteristics of Primary Progressive MS (the little version of the big category of Primary Progressive MS): (2)

 

Primary Progressive (the little version of the big category of all of this)

  1. •Disease progression without or with progression
  2. •If you don’t fit into one of the other 5 categories, so you just say that it is Primary Progressive

125

What is visula motor coordination (hand-eye coordination)?

 

the ability to coordinate vision with the movements of the body or parts of the body. (http://medical-dictionary.thefreedictionary.com/visual-motor+coordination)

From Wikipedia:

Eye–hand coordination (also known as hand–eye coordination) is the coordinated control of eye movement with hand movement, and the processing of visual input to guide reaching and grasping along with the use of proprioception of the hands to guide the eyes. Eye–hand coordination has been studied in activities as diverse as the movement of solid objects such as wooden blocks, sporting performance, music reading, computer gaming, copy-typing, and even tea-making. It is part of the mechanisms of performing everyday tasks; in its absence most people would be unable to carry out even the simplest of actions such as picking up a book from a table or playing a video game. While it is recognized by the term hand–eye coordination, without exception medical sources, and most psychological sources, refer to eye–hand coordination.[citation needed]

http://en.wikipedia.org/wiki/Eye%E2%80%93hand_coordination

126

what is hemiballisums?

what is a non-pt treatment?

 

  • •Hemiballismus: severe chorea. Unilateral rapid, non-rhythmic, wild flinging movements. Lesion in or around the contralateral subthalamic nucleus.
    • –Tx: antipsychotic medications.

 

127

Dystonia Pathophysiology

 

Progressive neuron degeneration of the basal nuclei and cerebellum

What does degeneration of the neuron mean?

  • •Something wrong with the synapse
    • •She listed several things that could go wrong at the synapse (I think it is from previous lectures)
  • •Not in the synapse
    • •Demyelination
      • •Progressive degenerative process that causes abnormal responses
    • •Death of neuron
      • •Louie bodies
        • •Build up of protein and eosinophil
        • •Common in Alzheimer's and Parkinson's

128

Huntington's Disease

Non PT treatment options

 

 

Non PT treatment options:

there are some medications that can help with symptoms a bit

129

what is myclonus?

 

 

•Myoclonus: restless leg syndrome falls into this category. Brief shock-like muscle contractions.

(I think it is just one symptom of restless leg - shock-like symptoms are not always present, but go with what shappy said)
 

131

what is an example of a pathology that involves the vestibulocerebellum?

Torticolis (specifically spasmotic torticolis which is a form of dystonia - cervical dystonia)

 

132

What are Spinocerebellar ataxias

 

Shappy's Notes:

  • •Ataxia, parkinsonism, dystonia, facial twitching
    • –Will go into more detail with some of these later

Inherited

 

Wikipedia:

Spinocerebellar ataxia (SCA) or also known as Spinocerebellar atrophy or Spinocerebellar degeneration, is a progressive, degenerative,[1] genetic disease with multiple types, each of which could be considered a disease in its own right. An estimated 150,000 people in the United States are diagnosed with Spinocerebellar Ataxia. SCA's are the largest group of this hereditary, progressive, degenerative and often fatal neurodegenerative disorder. There is no known effective treatment or cure. Spinocerebellar Ataxia can affect anyone of any age. The disease is caused by either a recessive or dominant gene. In many times people are not aware that they carry the ataxia gene until they have children who begin to show signs of having the disorder.[2]

http://en.wikipedia.org/wiki/Spinocerebellar_ataxia

133

Characteristics of Malignant MS? (3)

 

Malignant MS

  1. also called Marburg Disease ) – malignant does not mean cancer, just that it is fast and hard
  2. •Rapid onset, continued progression.
  3. Significant disability or death

134

Movement Disorders: what are seven types of non-rhythmic movement disorders?

 

 

  1. •Athetosis:
    • movements that are non-rhighmic slow and rihging. Primarily distal muscles (Dr. Lake: common in CP pts). Tend to produce a flowing stream of movement.
  2. •Chorea:
    • non-rhythmic jerky and rapid. Distal muscles or the face.
  3. •Dystonias:
    • sustained muscle contractions.  Typically alter posture or body position.
  4. •Hemiballismus:
    • non rhythmic rapid movements. Violent, flinging, non-suppressible.
  5. •Myoclonus:
    • rapid jerky. Shock like twitches. Restless leg falls under this category.
  6. •Tics:
    • Tourets is the example. Rapid repetitive non-rhythmic. Pts usually have some urge to do it and get some sort of relief after they do it. There is some thought that urge can be suppressed.
  7. •Tremor: 
    • go back to other definitions she gave

(these will have a seperate flashcard for each also)

135


•Dysdiadochokinesia: 
 


–Inability to produce rapid alternating movement (Examples: flipping hands, moving leg up and down shin)

 

136

what is Fragile X?

Who and what structures it affects

Transmission
Signs and symptoms
Diagnostic testing
Treatment
 

 

It is a genetic Disorder (involves  the X chromosome), and the most common intellectual disability in males. Affects cerebellar peduncles, but may impact other areas- need more research. May occur before school age, so IEP (individualized education plan) are developed for education in the school setting.

Carrier is the female (daughters of males who have the disease) . dad can be symptomless, but pass it on to the daughter who will then give to her sons who will show the symptoms

s/s: Progressive neural dysfunctional diseases: tremors, dystonia, mood issues (impatience, hostility), autonomic nervous system issues (sweating, bowel, bladder, ect), Parkinson like, and eventually dementia

Diagnostic testing: Genetic testing can identify it, MRI, EKG can show the deficiencies in the brain.

Treatment: There are some med that can help, but only slow the progression/ does not fix (behavioral, speech, balance, etc). IEPs fdeveloped for education at school

137

What are the 6 Categories of Primary Progressive MS?

 

 

  1. .Benign MS (about 20%)
    • •Mild disease with full function greater than 15 years)
  2. 2Malignant MS (also called Marburg Disease ) – malignant does not mean cancer, just that it is fast and hard
    • •Rapid onset, continued progression. Significant disability or death
  3. Relaxing Remitting MS (70%) – she likes to test on this one
    • •Acute episodes followed by remission/improvement without disease progression
  4. Secondary Progressive MS
    • •Develops from relaxing remitting
    • •However is more progressive
    • •Occasional relapses to differentiate it with minor remissions
      • •A lot of these people will plateau during the remission period (you will not see them return to full function)
    • •pts are older at this age 
  5. Progressive Relapsing
    • •Intervals between relapses actually show disease progression (so in between exacerbations you are still getting worse)
    • •Occurs with aging
  6. Primary Progressive (the little version of the big category of all of this)
    • •Disease progression without or with progression
    • •If you don’t fit into one of the other 5 categories, so you just say that it is Primary Progressive
       

138

Clinical Manifestations of ALS affecting LMN (3)

 

 

  1. •UE tend to be first LE second
  2. •Distal to proximal
  3. •Can affect reflexes (typically flaccid)
     

140

Huntington's Disease

S/S: (6 movement examples, 7 other examples)

Signs and symptoms:

List of s/s is large.

  • There are similarities to
    1. schizophrenia,
    2. bipolar,
    3. antisocial,
    4. dementia,
    5. depression,
    6. apathy,
    7. irritability, and then
  • obvious movement disorders (Chorea, gross movement disorders – see video):
    1. puppet like gait,
    2. huntington’s dance,
    3. tongue protrusion,
    4. mouth movements,
    5. head thrusting,
    6. quick eye movements,

 

141

characteristics of relaxing Remitting MS: (2)
 

 


Relaxing Remitting MS

  1. 70% of all MS – she likes to test on this one
  2. •Acute episodes followed by remission/improvement without disease progression

142

What are three  things you can test when evaluating movement disorders?

 

 

  1. •Sitting balance
  2. •Standing balance
  3. •Gait
     

143

Progressive Supranuclear Palsy:

What is it/what does it damage? (3)

What is it/what does it damage?

  1. Rare degenerative CNS disorder
  2. Affects Basal ganglia and brainstem
  3. Has protein depositis like other similar diseases

144

Movement Disorders, non-rhythmic: what is dystonia?


 

 


•Dystonias: sustained muscle contractions.  Typically alter posture or body position.
 

Youtube gave an example where spasmotic torticolis is a type of dystonia. Other examples mentioned a dystonic storm that looks similar to a grand mal seizure. Some videos make it look like contortions or random contrations of the body. I reccomend looking up videos to get the idea.

From Wikipedia (Because I still didn't understand; Probably more than you need to know):

Dystonia is a neurological movement disorder, in which sustained muscle contractions cause twisting and repetitive movements or abnormal postures.[1] The movements may resemble a tremor. Dystonia is often initiated or worsened by voluntary movements, and symptoms may “overflow” into adjacent muscles.[2]

The disorder may be hereditary or caused by other factors such as birth-related or other physical trauma, infection, poisoning (e.g., lead poisoning) or reaction to pharmaceutical drugs, particularly neuroleptics.[1] Treatment must be highly customized to the needs of the individual and may include oral medications, botulinum neurotoxin injections, physical therapy and/or other supportive therapies, and/or surgical procedures such as deep brain stimulation.

http://en.wikipedia.org/wiki/Dystonia

145

Multiple Sclerosis

Epidemiology:

 

 

Epidemiology:

  1. Adult onset typically 20-40 years old
  2. Race: white 2x more likely than African Americans
  3. Sex: Females 2x more likely than males

146

how will we determine the difference between dementia and alzheimer's?

The pathophysiology will help us know.

147

What is Motor Coordination?

 

from Wikipedia:

Motor coordination is the combination of body movements created with the kinematic (such as spatial direction) and kinetic (force) parameters that result in intended actions.

 

More if you feel like it:

Motor coordination is achieved when subsequent parts of the same movement, or the movements of several limbs or body parts are combined in a manner that is well timed, smooth, and efficient with respect to the intended goal. This involves the integration of proprioceptive information detailing the position and movement of the musculoskeletal system with the neural processes in the brain and spinal cord which control, plan, and relay motor commands. The cerebellum plays a critical role in this neural control of movement and damage to this part of the brain or its connecting structures and pathways results in impairment of coordination, known as ataxia.

http://en.wikipedia.org/wiki/Motor_coordination

(We did not talk of it in class I don't think)

148

Huntington's Disease

Type of Disorder:


Type of Disorder:

Autosomal dominant disorder

 

150

what is Tardive dyskinesia?

 

 

 

Wikipedia:

Tardive dyskinesia /ˈtɑrdɨv ˌdɪskɨˈniːʒə/ is a difficult-to-treat and often incurable form of dyskinesia, a disorder resulting in involuntary, repetitive body movements. In this form of dyskinesia, the involuntary movements are tardive, meaning they have a slow or belated onset.[1] This neurological disorder most frequently occurs as the result of long-term or high-dose use of antipsychotic drugs,[Note 1] or in children and infants as a side effect from usage of drugs for gastrointestinal disorders.[Note 2][2]

151

Ataxia definition


shappy: reeling wide-based movements related to gate
 

(I dissagree that it is mainly just gait. Ataxic gait is mainly gait, but ataxia seems to just mean uncoordinated movments from my experience outside of shappy's class. Wikipedia does seem to make special effort to point out the gait aspect though)

Wikipedia:

Ataxia (from Greek α- [a negative prefix] + -τάξις [order] = "lack of order") is a neurological sign consisting of lack of voluntarycoordination of muscle movements that includes gait abnormality. Ataxia is a non-specific clinical manifestation implying dysfunction of the parts of the nervous system that coordinate movement, such as the cerebellum. Several possible causes exist for these patterns of neurological dysfunction. Dystaxia is a mild degree of ataxia. Friedrich's ataxia has gait abnormality as the most common presenting symptom.[1]

153

Movement Disorders, non-rhythmic: what is myoclonus?

 

Myoclonus: rapid jerky. Shock like twitches.

This is related to restless leg syndrome (RLS)

(I think it is a symptom that can be present but is not always present with RLS)

154

what are the defining functions of the spinocerebellum?


•Coordinated truck and LE movements
 

156

define dysarthria:


–Inability to articulate words (motor function). “speech problems” per Dr. Shappy
 

 

157

What are some symptoms of Progressive supranuclear palsy? (2)

 


•Progressive supranuclear palsy-

  1. coarse or jerky tremor with
  2. eye gaze issues (typically difficulty looking down)
     

158

Huntington's Disease

Pathophysiology:

Pathophysiology:

  • Caudate nucleus from a pathophysiologic standpoint
    • Spiny neurons in corpus striatum degenerate and there is a
    • decrease in neurotransmitters,
      • substance P and GABA neurotransmitters specifically

159

what are the classifications of tremors based on? (at least the classifications that Dr. shappy gave us)

 

based on when the tremor occurs

160

Parkinson's Disease

S/S: (11 examples)

 

 

S/S (but not a check-list; pt may not have all symptoms):

  1. –Festinating gait
  2. –Resting tremor
  3. –Pill rolling
  4. –Stooped posture
  5. –Bradykinesia
  6. –No arm swing
  7. –Rigidity
  8. –Dementia
  9. –Akinesia
  10. –Sleep disorder issues (typically)
  11. –Flat affect

161

What are some examples of aquired conditions? (9)

 

 

  1. –Cerebellar strokes,
  2. systemic disorders,
  3. MS,
  4. repeated TBI,
  5. toxins,
  6. idiopathic,
  7. heat stroke,
  8. alcohol abuse (the withdrawal part)
  9. brain tumor,
  10. etc.

162

Progressive Supranuclear Palsy (everything):

What is it/what does it damage?

Transmission:

S/S:

Diagnosis:

Prognosis:

Treatment:

 

What is it/what does it damage?

  • Rare degenerative CNS disorder
  • Affects Basal ganglia and brainstem
  • Has protein depositis like other similar diseases

Transmission:

  • seems to have a genetic component

S/S:

  • bradykinesia,
  • rigidity,
  • eye movement,
    • Cant look down (can look side to side but the eyes can not go down; if the head is moved up and down, the eyes stay forward- compared pt to a doll’s eyes
  • progressive,
  • pseudo-bulbar palsy (facial),
  • dementia,
  • From Video: gait is wide and staggering (not like parkinson's with the shuffling)
    • I think they lean backwards too (not forwards like parkinson's)
    • pt seems apethetic to their unsteadyness and "plunges ahead"
  • From Video: Characteristic speech pattern: spastic speech in combo with ataxic speech
    • strained and slow
    • syllables grouped into unnatural groups with unnatural pauses
    • this speech pattern occurs in almost no other condition
  • The video of the guy in his underwear had Progressive Supranuclear Palsy ("don't worry about his underwear!")

Diagnostic Testing:

  • MRI will not show until severe atrophy.
    • so diagnosis must be by symptoms before it is severe
  • Has protein depositis like other similar diseases, possibly part of scar tissue while body is trying to heal (so it doesn't show until that point)

Prognosis:

  • degenerative,
  • most live 6-8 years (according to video Dr. Shappy posted)
    • death is usually from related problems, not the actual condition

Treatment:

  • does not respond to dopamine replacement like parkinson's dz
  • a fact sheet I found discussed treatment for symptoms (like difficulty swallowing), but said there was no treatment for the actual disease.
  • PT's treat symptoms

163

What are the three main parts of the cerebellum callded by their functional names?

 


vestibulocerebellum
spinocerebellum
pontocerebellum or cerebrocerebellum
 

A image thumb
164

Multiple Sclerosis

Pathophysiology:

 

Pathophysiology:

  • Autoimmune resonse sets of Immune system
  • •Something crosses BBB and causes demyelination (that can stop at any point)
    • attacks to myelin cause inflammation and then scar tissue develops,
      • oligodendrocytes are the glial cells that myelinate CNS axons and experience damage
        • shappy said they are the ones that are activated to respond (but microglial cells are like the phagocytes of the CNS, so I would think they have a large role in the innflammation and immue response - maybe oligodendrocytes try to regenerate?
    • You get inflammation first, then you will get the scar tissue after on the site (treat with inflammatory first)
      • •Can see Plaque build up
      • •Plaque is scar tissue
      • •Related to death of neuron
  • •Slows conduction
  • •Decreasing transmission
  • •Results in weakness
  • Sort of like a CNS version of chronic Gillian Barre.

165

Spinocerebellum (Paleocerebellum) is composed of which anatomical parts?

Vermis and intermediate parts of the hemispheres ("paravermis")

 

Picture: the cerebrocerebellum is another name for pontocerebellum

A image thumb
166

Tremmor Occurance: What is the

Pattern?

Severity?

acuity?

 


•Occurrence
–Pattern- could be intermittent or constant (any type of tremor)
–Severity- large or small oscillations, duration, etc. Amplitude
–Acuity- gradual onset  or abrupt onset
 

167

What are some symptoms of Parkinson's Dz? (5)

 

 

  1. Resting tremor
    • pill rolling,
    • chin
    • leg
  2. shuffling gait,
  3. bradykinesia,
  4. cogwheel rigidity,
  5. micrographia

169

Progressive Supranuclear Palsy:

Prognosis: (2)

 

 

Prognosis:

  1. degenerative,
  2. most live 6-8 years (according to video Dr. Shappy posted)
    • death is usually from related problems, not the actual condition

170

What is a resting tremor?


–Happens at rest or when body part is in gravity supported position., but goes away with intentional movment


 

171

Fragile X:

Who and what structures it affects

 

 

It is a genetic Disorder (involves  the X chromosome), and the most common intellectual disability in males. Affects cerebellar peduncles, but may impact other areas- need more research. May occur before school age, so IEP (individualized education plan) are developed for education in the school setting.

Carrier is the female (daughters of males who have the disease) . dad can be symptomless, but pass it on to the daughter who will then give to her sons who will show the symptoms

172

Clinical Manifestations of ALS affecting UMN (1)


•Spasticity if affects reflexes
 

173

Is MS easy or hard to diagnose?

 

Manifestations can be hard to put a label on because the symptoms are vague. Easy to miss the diagnosis

174

Pathologic Tremor: Action or Postural Tremor details/causes (3)

Action or Postural Tremor:

  1. •Essential tremor (non-familial)
  2. •Alcohol or drug withdrawal- DT’s (Delerium Tremins - what happens during alcohol/drug withdrawal)
  3. •Endocrine, metabolic or toxic conditions-
    • encephalopathy,
    • renal,
    • hepatic,
    • hypoglycemia,etc.