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Flashcards in Movement Disorders Deck (44)
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1

4 features of parkinsonism

1) Rest tremor - usually a pill rolling type tremor when in the hands. Present only at rest

2) Bradykinesia - overall slowness of motor tasks

3) Rigidity - increased tone in muscles independent of velocity which may have a cog-wheeling or lead-pipe component

4) Postural instability - Gait disturbance leading to falls. Usually with small, shuffling steps and decreased arm-swings

2

Important causes of parkinsonism

1) Parkinson Disease

2) Drug-induced

3) Parkinson's Plus syndromes

4) Vascular

3

Parkinson Disease

Begins asymmetrically with prominent tremor. Gait instability occurs later and is associated with a typical flexed posture. Dementia develops LATE in a minority

Tx = Levodopa is mainstay of treatment, but dopamine agonists, anticholinergics, selegeline and amantadine are effective as well. Always give levodopa with carbadopa

4

Drug-induced parkinsonism

Usually caused by a neuroleptic even for a short time.

May be indistinguishable from PD. Gait instability less frequent

TX = withdrawal of offending agent if possible. Clozapine does not cause this syndrome and is an alternative

5

Parkinson's Plus syndromes

1) Progressive supranuclear palsy - falling all the time within 1st year of disease, vertical gaze issues

2) Multiple System Atrophy - autonomic dysfunction early in disease

3) Dementia with Lewy Bodies - cognitive impairment in 1st year with parkinsonism, visual hallucinations

4) Cortical basal degeneration - asymmetric, highly asymmetric rigidity, apraxia, alien limb phenomenon, levitating arm

Important to distinguish from PD. Consider one if you see early dementia (DLB), early falls or eye movement problems (PSP) and early autonomic instability (MSA)

Tx = infrequent and variable response to levodopa. DLB does respond to central acetylcholinesterase inhibitors like donepezil

6

Vascular parkinsonism

Usually involves legs more than arms. Due to multiple small strokes in basal ganglia

Tx = control progression with secondary stroke ppx. Generally poor response to levodopa

7

Rest tremor

Usually pill rolling when in hands. Present only in rest

Low freq, high amp

Classic condition is Parkinsonism

8

Postural tremor

Present when limbs are voluntarily maintained against gravity

high freq, low amp

Classic conditions:
1) Physiologic tremor
2) Essential tremor
3) Enhanced physiologic tremor

9

Kinetic tremor

Occurs during voluntary movement. AKA intention tremor.

cerebellar component

Classic condition: Cerebellar tremor

10

Essential tremor

Autosomal dominant. Mixed penetrance.

Can be in head and voice.

Predominantly postural tremor. Usually starts in middle age and worsens over time. Family history is typical. Improves with alcohol

Tx: If it interferes with daily tasks, beta blockers (propranolol) and primidone (pro-drug of phenobarbital) can be effective. Eliminate caffeine.

Make sure it's not PD

DBS if don't respond to drugs (Thalamus is the target)

11

Wilson Disease

Though rare, it is vital to diagnose as this devastating disease can be prevented from progressing.

Classic triad: Movement disorders (tremors, parkinsonism, chorea, dystonia), psychiatric disturbance and hepatic failure

Can present in any order. Look for Kayser-Fleischer Rings Check serum ceruloplasmin and 24hr urine copper

Tx: Limit dietary copper intake. Penicillamine, Zn, and dimercaprol have all been used with success

12

Focal dystonia

Blepharospasm (involuntary closure of eye), torticollis (involuntary contraction of one SCM) and writer's cramp are the common types

Tx: Focal dystonias respond well to local injection of botox into involved muscle

13

Huntington's Disease

Class triad is chorea, dementia, and positive family history

Tx: Symptomatic therapies exist for the chorea, but unfortunately the disease relentlessly progresses until death

14

basal ganglia function

Planning of movement

Lesion will show too slow movement or extra movements they didn't want

15

Cerebellum function

Gets motor plan and also sensory feedback

If you veer off course it will adjust you

Trouble enacting the motor plan if there is a lesion

16

Chorea

Dance like (chore-ographer)

Damage to caudate/putamen

17

Ballism

Classically a loss of the indirect pathway - stroke in contralateral STN

Violent flailing (ballis-tic missile)

18

Dystonia

Fixed muscle position

#1 = drug-induced

19

Myoclonus

Rapid muscle jerk

#1 = renal failure

20

Tics

Involuntary but suppressible - the motor version of a compulsion

21

Akathisia

Inner sense of restlessness relieved by movement

Fe deficiency can present with restless leg syndrome (Basal ganglia has Fe receptors)

22

Complications of PD treatment

Levodopa is best treatment. It is converted to dopamine in the brain and corrects the lack of dopamine that causes the disease

Levodopa leads to complications in 3-5 years though so young patients are often started on other meds. Levodopa can cause ortho hypo, hallucinations, psychosis. on-off phenomenon, wearing-off phenomenon, dyskinesias

Dopamine agonists (pramipexole) are less effective but have less motor side effects. Can cause edema though.

MAOIs (rasagaline) have mild symptomatic benefits and may slow disease progression

Anticholinergics (trihexyphenidyl) are effective at treating the tremor but are quite sedating

Amantadine can also be effective

23

Tourette Syndrome

neurological disorder characterized by repetitive, stereotyped, involuntary movements and vocalizations called tics

Early symptoms of TS are almost always noticed first in childhood, with avg age of onset of 7-10 years.

First sx's usually occur in the head and neck area and may progress to include muscles of the trunk and extremities. Motor tics generally precede the development of vocal tics and simple tics often precede complex tics.

Most patients experience peak tic severity before the mid-teen years with improvement for majority of patients in late teen years and early adulthood

Some patients can suppress, hide or otherwise manage their tics in an effort to minimize their impact on functioning. They often report huge buildup of tension though when suppressing their tics to the point that they feel like tic must be expressed

TS is inherited. Some forms of ADHD and OCD are genetically related to TS

Tx: Medical therapy when tics interfere with social interactions, school performance, or activities of daily living. Goal for tics is not complete elimination but rather control of tics to alleviate the social embarassment or discomfort due to the tic.

D2 blockers (neuroleptics) are the most effective meds for treating tics, but the side effect profile (EPS,TD) is a limit to first line treatment. Haldol and pimozide are approved for TS. Haldol has 80% response rate for tic suppression

24

Evaluating multifocal myoclonus

Note: seizure is unlikely to present in all limbs with a patient who is described as only mildly confused.

Ddx is renal failure, benign nocturnal myoclonus, CJD, meds, other metabolic derangements like liver failure and certain forms of epilepsy

Order serum lytes, renal function, LFTs. In an ICU patient with multifocal myoclonus, an EEG is ordered to exclude seizures. Brain MRI is usually unimpressive. CSF should only be obtained if there are associated signs and symptoms of meningitis or encephalitis

Tx: Correct metabolic derangements associated with RF. Levetiracetam, piracetam and valproic acid can suppress myoclonic jerks but this is not needed unless it is very disturbing to family members

25

Physiological tremor

Very low-amp, fine tremor (btw 6-12 Hz) that is barely visible to naked eye. Present in every normal person during maintaining a posture or movement. Neuro exam results of patients with physiologic tremor are usually normal

26

Enhanced physio tremor

High-freq, low-amp visible tremor that occurs primarily when a specific posture is maintained. Drugs and toxins induce this form of tremor

27

What is the most common movement disorder?

Essential tremor

28

Essential tremor timing, tx, and diagnosis

Usually middle age, but can be earlier

Pronounced when patient engages in voluntary movement such as drinking a glass of water, writing, threading needle

Fatigue, anxiety and temp extremes make it worse

usually disappears at rest or while asleep

Lose doses of alcohol helps in half of patients

It's a clinical diagnosis

Tx: beta blockers or primidone (an anti-seizure drug)

B-blocker side effects: dizziness, confusion, memory loss in older adults so they are better choice for younger patients

Also avoided in asthma, diabetes, certain heart problems

Primidone side effects: flu-like, drowsy

Tranquilizers like xanax (alprazolam) or valium (diazepam) can help when tremors are worsened by anxiety or tension. Side effects are confusion, memory loss

BoTox can help when head or voice are involved, Used for up to 3 months at a time. When used to treat hand tremors it may cause weakness

DBS to thalamus if severe and if meds aren't effective

29

Latency time in ET

Time of onset for tremor to appear while holding arms out

PD: 9s
ET: 1-2s

30

Athetosis

Twisting and writhing often associated with chorea