Movement Disorders

Question 1

Define hypokinetic

Answer 1

decreased/absence of movement

Question 2

Define hyperkinetic

Answer 2

increased amount of movement

Question 3

etiology of parkinson’s

Answer 3

degeneration and loss of pigmented dopamine neurons of the substantia nigra regions of the brain

Question 4

mean age of onset of parkinson’s

Answer 4

55

prevalence increases sharply after the age of 50 and peaks around about 75

Question 5

strongest predictor of increased risk of PD

Answer 5

age

Question 6

Parkinson’s disease: presentation (TRAP)

Answer 6

T: tremor (pill rolling)
R: rigidity (cogwheel/lead pipe)
A: akinesia/bradykinesia
P: postural (instability, retropulsion)

Question 7

Parkinson’s disease: common clinical presentation

Answer 7

forward head tilt
resting tremor
bradykinesia
>55y/o

Question 8

Common imaging results for Parkinson’s disease

Answer 8

CT is normal
MRI may have slight degeneration of substantia nigra
SPECT/PET may show reduced dopamine transporter density (less dopamine things)

Question 9

pharmacologic treatment for Parkinson’s

Answer 9

• levodopa
• dopamine agonists
• MAOI’s
• anticholinergic

Question 10

Non-pharmacologic treatments for Parkinson’s

Answer 10

• PT
• Tx comorbid conditions
• Caregiver support
• Deep brain stimulation

Question 11

pathophys for Huntington’s

Answer 11

causes cells in specific parts of the brain to degenerate: caudate, putamne, cerebral cortex

Question 12

Function of caudate and putamen that is affected in Huntington’s

Answer 12

These brain locations have functions that project pathways into different parts of the brain and help to control body movement, emotions, thinking, behavior, and perception of the world.

Question 13

Genetic component of Huntington’s

Answer 13

genetic disorder caused by faulty gene on chromosome 4

Autosomal dominant disorder

Question 14

Primary ages of Huntington’s

Answer 14

30-45

early as 5 and late as 70s

Question 15

Common clinical presentation of Huntington’s patients

Answer 15

nervous twitching
hyperactivity
clumsy
problems performing ADL's
forgetful, worsening memory
irritable, impulsive

Question 16

Essential tremor characteristics

Answer 16

• tremor reduced with EtOH ingestion
• tremor of hands/forearms (not a resting tremor)
• isolated head tremor (constantly saying yes or constantly saying no)
• usually have a FHx (family hx)

Question 17

Tourette’s common presentation

Answer 17

child between 6 & 11
performs obscene gestures they cannot control
feel impulsed to perform tic and feel better when they have completed the tic

Question 18

pharmacological treatments for Tourettes

Answer 18

antidopinergics: haloperiodol, pimozide, aripiprazole

Question 19

Cerebral palsy

Answer 19

permanent, nonprogressive motor dysfunction due to abnormalities of the developing fetal/infantile brain resulting from a variety of causes

Question 20

cerebral palsy is MC in what type of infants

Answer 20

premature!

Question 21

myasthenia gravis defintion

Answer 21

fluctuating weakness, exacerbated by exertion, fatigue, and improved at rest

Question 22

maysthenia gravis common presentation

Answer 22

droopy eyelids
double vision
slurred speech
facial weakness
head drop

Question 23

myasthenia gravis patho

Answer 23

autoimmune disorder of postsynpatic NMJ characterized by fluctuating weakness involving variable combinations of ocular, bulbar, limb, and respiratory muscles

autoABs against acetylcholine receptors

Question 24

myasthenia gravis treatment

Answer 24

immunomodulatory: IV immunoglobulin; plasma exchange
immunotherapy: pernisone

Question 25

Multiple sclerosis

Answer 25

autoimmune inflammatory demyelinating disease of idiopathic origin associated with axon degeneration of white matter of brain, optic nerve, and spinal cord

Question 26

multiple sclerosis presentation

Answer 26

uhthoff's phenomenon: temporary worsening of MS symptoms with heat L'hermitte's sign: flexion of neck causes lightning-shock type pain radiating from spine down leg trigeminal neuralgia optic neuritis (unilateral eye pain worse with EOM) marcus-gunn pupil: during swinging flashlight test, the affected eye does not dilate/constrict as much as it's supposed to so the eyes do not have symmetrical dilation/constriction AKA relative afferent pupil defect (RAPD)

Question 27

multiple sclerosis diagnostics

Answer 27

MRI with gadolinium: - shows white matter plaques - "open ring sign" - can also see "Dawson's fingers" of active inflammation and breakdown of the blood brain barrier LP shows increased IgG oligoclonal bands in the CSF

Question 28

treatment for multiple sclerosis: acute

Answer 28

- IV corticosteriods (solumedrol) | - plasmapheresis if not responsive

Question 29

treatment for multiple sclerosis: chornic

Answer 29

beta interferon | injectable glatiramer acetate to decrease number/severity of relapses

Question 30

ALS is a disease of...

Answer 30

upper and lower motor neurons

Question 31

ALS upper motor neuron symptoms

Answer 31

spasiticity hyperreflexia pathological reflexes

Question 32

ALS lower motor neuron symptoms

Answer 32

weakness atrophy hyporeflexia fasciculations

Question 33

ALS presentation

Answer 33

loss of ability to initiate and control motor movements SPARES: sensation, urinary sphincter, and voluntary eye movements

Question 34

ALS treatment

Answer 34

riluzole to slow progression baclofen for spasticity benzos for anxiety/depression

Question 35

neurofibromatosis 1

Answer 35

MC tumor seen in NF1 - peripheral nerve sheat tumor with malignant transformation in less than 10% of patients

Question 36

neurofibromatosis 1 presentation

Answer 36

optic glioma (pilocytic astrocytoma - MC tumor) cafe au lait freckling of axilla/groin learning disorders

Question 37

neurofibromatosis 2

Answer 37

autosomal dominant that results in bilateral vestibular schwannomas

Question 38

tuberous sclerosis

Answer 38

inherited neurocutanoeous disorder that is characterized by pleomorphic features involving many organ systems - brain, eyes, heart, lung, liver, kidney, skin

Question 39

presentation of tuberous sclerosis

Answer 39

most have epilepsy, ash-leaf macules, shagreen patches 1/2 or more have cognitive deficits/learning disabilites commonly have autism, behavioral problems

Question 40

con-hippel lindau disease

Answer 40

inherited autosomal dominant syndrome manifested by a variety of benign/malignant tumors MC manifestation: menangioblastomas - have mass effects also gives renal cell carcinomas, pheochromocytomas