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Neurology > Movement Disorders > Flashcards

Movement Disorders Flashcards

1
Q

4 features of parkinsonism

A

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

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2
Q

Important causes of parkinsonism

A

1) Parkinson Disease
2) Drug-induced
3) Parkinson’s Plus syndromes
4) Vascular

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3
Q

Parkinson Disease

A

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

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4
Q

Drug-induced parkinsonism

A

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

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5
Q

Parkinson’s Plus syndromes

A

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

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6
Q

Vascular parkinsonism

A

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

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7
Q

Rest tremor

A

Usually pill rolling when in hands. Present only in rest

Low freq, high amp

Classic condition is Parkinsonism

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8
Q

Postural tremor

A

Present when limbs are voluntarily maintained against gravity

high freq, low amp

Classic conditions:

1) Physiologic tremor
2) Essential tremor
3) Enhanced physiologic tremor

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9
Q

Kinetic tremor

A

Occurs during voluntary movement. AKA intention tremor.

cerebellar component

Classic condition: Cerebellar tremor

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10
Q

Essential tremor

A

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)

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11
Q

Wilson Disease

A

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

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12
Q

Focal dystonia

A

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

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13
Q

Huntington’s Disease

A

Class triad is chorea, dementia, and positive family history

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

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14
Q

basal ganglia function

A

Planning of movement

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

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15
Q

Cerebellum function

A

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

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16
Q

Chorea

A

Dance like (chore-ographer)

Damage to caudate/putamen

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17
Q

Ballism

A

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

Violent flailing (ballis-tic missile)

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18
Q

Dystonia

A

1 = drug-induced

Fixed muscle position

19
Q

Myoclonus

A

1 = renal failure

Rapid muscle jerk

20
Q

Tics

A

Involuntary but suppressible - the motor version of a compulsion

21
Q

Akathisia

A

Inner sense of restlessness relieved by movement

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

22
Q

Complications of PD treatment

A

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
Q

Tourette Syndrome

A

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
Q

Evaluating multifocal myoclonus

A

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
Q

Physiological tremor

A

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
Q

Enhanced physio tremor

A

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

27
Q

What is the most common movement disorder?

A

Essential tremor

28
Q

Essential tremor timing, tx, and diagnosis

A

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
Q

Latency time in ET

A

Time of onset for tremor to appear while holding arms out

PD: 9s
ET: 1-2s

30
Q

Athetosis

A

Twisting and writhing often associated with chorea

31
Q

Huntington genetics

A

CAG triplet repeat within Huntintin gene.

Auto dominant with 100% penetrance

Chromosome 4

Number of repeats correlates with manifestations of disease

More than 40 repeats almost always linked to clinical disease

Repeats of 26-30 sometimes

Less than 25 almost never

Paternal transmission leads to more repeat length increasing.

Anticipation seen (esp in paternal transmission)

32
Q

Opisthotonos

A

Great rigid spasm of the body with the back fully arched and the heels and head bent back

33
Q

Primary generalized dystonia

A

DYT-1 is early onset torsion dystonia caused by mutation in the torsion A gene of chromosome 9

DYT-5 is dopa-responsive dystonia

DYT-1 is most common. Starts in limb. Associated with GAG deletion in exon 5 of DYT1 (TOR1A). Large phenotypic variation.

Progressive, disabling disorder that usually begins in childhood and is linked to several genetic loci. Many inherited as autosomal dominant traits cause by a deletion in torsion A gene.

Begins as focal action dystonia nefore the middle of the third decade with most cases beginning in childhood. 65% progress to multifocal or generalized dystonia. 10% become segmental. 25% stay focal.

Most primary dystonias have normal routine imagine. In primary torsion dystonia FDG-PET has been shown to see some metabolic differences

Dx: Rule out secondary dystonia first

  • History of trauma or exposure to drugs, infections, cerebral anoxia, etc
  • Dystonia at rest rather than with action at its onset
  • Atypical site for age of onset (leg onset in an adult, cranial onset in a child)
  • Early onset of speech abnormality
  • hemidystonia
  • presence of abnormalities other than dystonia on neuro exam or general med exam
  • Nonphysio findings suggesting a psychogenic basis
  • Abnormal brain imaging
  • Abnormal labs

Remember for primary dystonia, dystonia is the ONLY symptom.

Tx: High-freq stimulation of globus pallidus, pars interna through DBS
Symptom treatment with levodopa, benzos, baclofen, anticholinergics (trihexyphenidyl)

34
Q

Pathologic feature of PD

A

Loss of pigment in substantia nigra

Remaining neurons may show intracytoplasmic eosinophilic inclusions called Lewy Bodies

Marked striatal dopamine depletion

35
Q

Autosomal dominant cerebellar ataxia clinical classification

A

Hardin system. For late onset ADCA. Also, sporadic cerebellar syndromes include idiopathic forms of obscure etiologies marked by progressive ataxia, autonomic failure, and extrapyramidal features (like MSA)

ADCA I - optic nerve, cerebral cortex, peripheral nerves

ADCA II - pigmentary macular dystrophy

ADCA III - pure late onset cerebellar syndrome

Since this time, we refer to them as spinocereballar ataxias (SCAs) and they are more characterized based on genetic loci

36
Q

ADCA genetics

A

Most are caused by expansions, most are trinucleotides (SCA 1-3, 6-10, 12, 17)

SCA 8 - CTG repeated

SCA 10 - ATTCT repeated

SCA 4 - no trinucleotide repeat

37
Q

ADCA age of onset

A

Most are 20-30

SCA 6 40-50

SCA 8 late 30s

38
Q

ADCA clinical features

A

Most common is SCA 3 - chromosome 14 (Ataxin 3 gene) CAG repeat.

SCA 1: 6% - Ataxia, dysarthria, pyramidal signs, peripheral neuropathy, hyperreflexia, cognitive impairment. Nystagmus, hypermetruc saccades, slow saccades, ophthalmoparesis. CAG chromosome 6

SCA 2: 14% - Ataxia, dysarthria, peripheral neuropathy, hyporeflexia, dementia, myoclonus. Slow saccades. Ophthalmoplegia. CAG chromosome 12.

SCA 3** 21% - Ataxia, dysarthria, spasticity, parkinsonism, amyotrophy. Lid retraction, nystagmus, saccade, dysmetria, ophthalmoparesis, square wave jerks. CAG chrom 14.

SCA 6: 15% - Ataxia, dysarthria, sometimes episodic ataxoa, very slow progression, lack of FHx. Nystagmus (usually downbeating), saccadic pursuit. CAG chrom 19

SCA 7: 5% - Ataxia, dysarthria, retinopathy, peripheral neuropathy, pyramidal signs, infantile phenotypes. Saccadic smooth pursuit, slow saccades. CAG chrom 3

SCA 8: 2-5% - ataxia, dysarthria, mild sensory neuropathy. Nystagmus, saccadic pursuit. CTG chrom 13

Exhibit anticipation

39
Q

Dx of an SCA

A

Initially suspected by the adult-onset of symptoms.

MRI or CT of brain can detect atrophy (wasting) of the cerebellum and a variety of other subcortical structures.

Molecular genetic test to determine gene that has the repeat can quickly ID other carriers in the family. Many of them can be confirmed by DNA testing.

Once genetic defect is characterized, test family members

40
Q

SCA-3

A

Machado-Joseph Disease

Most common SCA

Most variable phenotype

Pure cerebellar ataxia, familial parkinsonism, hereditary spastic paraplegia, herediatry neuropathy, and restless leg syndrome.

Impaired temp discrimination in all limbs and even trunk and face is very specific for SCA3 but also pretty rare.

Pseduoexophthalmos (bulging eyes caused by lid retraction)

Faciolingual myokyma

Dystonia

41
Q

Treatment of SCAs

A

No cure

Supportive treatment

Ultimately fatal. SCA 8 and 11 have normal lifespans. 6 and 11 are less severe.

42
Q

Treatment of TD

A

Benzos, baclofen, vitamin E

Dopamine depleting agents like reserpine or tetrabenazine that do not block receptors have not be shown to cause TD.

43
Q

TD

A

Most common cause of TD is use of chronic dopamine blocking agents like typical antipsychotic agents. Note that all antipsychotics can do it. Also metoclopramide and other anti-nausea drugs.

Generally develops months after beginning dopamine receptor blocking drugs

Most freq causes Sterotypical movements of the mouth and surrounding regions

Treatment is usually less than optimal and so the best course is to avoid TD altogether - constantly reassess need for and amount of dopamine blocking drugs

Backward arching movement of neck (retrocolis) believed to strongly suggest TD

44
Q

Simple vs complex tics

A

Simple motor tics involve single groups of muscles. This causes jerk movements in cases of clonic tics, or briefly sustained posture in cases of dystonic or tonic tics

Simple clonic - blinking, head/limb jerks, nose twitching

Simple dystonic - oculogyric deviation, bruxism, blepharospasm, torticlois-like posturing

Most common tonic tics are abdominal and other muscle tensing

Simple phonic tics - coughing, sneezing, throat clearing, grunting, others

Complex motor tics include coordinated movements which involve multiple muscle groups and often resemble normal movements. They vary from head shaking to touching and hitting.

A complex tic should be considered a compulsion if it is preceded by obsessive thought, anxiety or fear. Complex tics are often hidden by incorporating them into seemingly planned and purposeful movement.

Complex phonic tics include linguistically meaningful verbalizations. Although rare, coprolalia (bad words) can be seen. More common is repetition of someone elses or own words (echolalia or palilalia). Bad motor tic equivalent (middle finger) is copropraxia

Neurology (14 decks)

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