3- Hyperkinetic Movement Disorder Flashcards

1
Q

Define tremor. How is tremor classified in terms of clinical characteristics? 🔑🔑

A

💡 Rhythmic involuntary movement of a body part. Happens at rest, while moving or posturing.

1. Resting Tremor

Involuntary movement at rest, usually affecting hands and fingers.

Seen in Parkinson’s disease (PD).

2. Intentional - Action Tremor

Occurs with movement from one point to another, “finger-to-nose” test.

Amplitude of tremor increases as extremity approaches target

Seen in cerebellar dysfunction.

Treatment: Wrist weights

3. Essential - Postural Tremor

Posture-maintaining tremor associated with sustained muscle contraction or stress

Accentuated by goal-directed activities, i.e. holding a cup of tea.

Occurs during sustained contraction of muscles to maintain the position of a body part against gravity, resulting in flexion-extension movement of the hand.

Treatment: Beta-blockers (e.g., propranolol).

Most common type of tremor, Benign (patients should be reassured about this)

Cuccurollo 4th Edition Chapter 12 Movement Disorder pg867-868

Braddom 6th Edition Chapter 45 Movement Disorder pg973

1. Postural Tremors

Physiologic - Essential - Basal Ganglia Disease - Cerebellar Disease - Peripheral Neuropathy - Post-Traumatic - Alcoholic

2. Kinetic Tremors

Cerebellar Disease

3. Task-specific Tremors

Writing - Vocal - Orthostatic

PMR Secrets 3rd Edition Chapter 56 Movement Disorders pg471-472 Box 56-3

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2
Q

List 3 Characteristics of resting tremor. 🔑🔑

A
  1. Appears within the body part completely at rest → resting tremor
  2. Subsides with action → its not intentional
  3. Subsides with assuming a posture → its not porstural

Braddom 6th Edition Chapter 45 Movement Disorder pg974

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3
Q

List 4 Characteristics of essential tremor - How it is treated? 🔑🔑

A

Characteristics

  1. It is a postural tremor → it’s essential to keep the posture
  2. Flexion-extension movement of the hand
  3. Accentuated by goal-directed activities
  4. Can interfere with hydration and nutrition
  5. Slowly progressive with age.
  6. Worsen with anxiety, stress, and stimulants

Treatment

  1. Propranolol remains the most effective medication for essential tremor
  2. Less effective but occasionally useful medications are lorazepam, clonazepam, diazepam, alprazolam, gabapentin, and topiramate.

Neurology Secrets 6th Edition Chapter 12 pg150

Braddom 6th Edition Chapter 45 Movement Disorder pg973

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4
Q

List 6 Causes of Physiologic tremor 🔑

A
  1. Stress induced
  2. Anxiety
  3. Caffeine (coffee, tea)
  4. Stimulants (amphetamines, cocaine)
  5. Dopaminergic drugs (levodopa, dopamine agonists)
  6. Alcohol withdrawal
  7. Exercise
  8. Fatigue
  9. Thyrotoxicosis
  10. Hypoglycemia
  11. Beta Agonist

Neurology Secrets 6th Edition Chapter 12 pg149 Table 12-1

Braddom 6th Edition Chapter 45 Movement Disorder pg973

PMR Secrets 3rd Edition Chapter 56 Movement Disorders pg471

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5
Q

Provide 4 strategies to treat intention tremors. 🔑🔑

A

Non-Pharmacological:

  1. Avoiding triggers (stimulants and coffee)
  2. Physical therapies – strengthening exercises
  3. Balance and coordination
  4. Weighted tools (wrist splints, leg, walkers, utensils)
  5. Proximal splinting for feeding and dressing

Pharmacological:

  1. Beta – blockers (propranolol)
  2. Anticonvulsants: Keppra, Carbamazepine, gabapentin
  3. Benzodiazepines (clonazepam)
  4. Topiramate (topamax)

Surgical:

  1. Thalamic DBS.
  2. Thalamotomy (stereotactic).

Ref: Braddom pg 1245; 2011 – symptomatic management of multiple sclerosis article; J Neurol (2007) 254:133–145.

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6
Q

List 8 features of an essential tremor that differentiates it from Parkinson tremor:

A

Think the opposite

  1. Decreases with rest
  2. Decreases with alcohol
  3. Increases with movement (intention).
  4. Increases with stress (PD increases with stress too… Delisa pg 646)
  5. 4-8 Hz (PD = 3.5 Hz).
  6. Familial inheritance strongly present (autosomal dominant).
  7. Involves head and voice
  8. Onset 10-80 yrs old as opposed to after 50

Ref: Movement disorders in clinical practice textbook pg 6-7.

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7
Q

Dystonia: Definition, Types, Aggravating & Alleviating Factors, Treatment 🔑🔑

A

DYSTONIA

Sustained muscle contractions, causing twisting repetitive movements, which may progress to prolonged abnormal postures

Primary dystonia (autosomal dominant) is the most common

Course can be very variable with spontaneous remissions and exacerbations

TYPES

💡 Most common dystonia: Cervical dystonia (focal) > Blepharospasm (focal)

1. Focal dystonia

One part of the body is involved

Blepharospasm, oromandibular dystonia, cervical dystonia and writer’s cramp

2. Segmental dystonia

Two or more contiguous parts involved

Meige syndrome (blepharospasm + oromandibular)

Brachial dystonia and crural dystonia

3. Multifocal dystonia

Two or more noncontiguous parts are involved.

4. Hemidystonia

One side of the body is affected.

5. Generalized dystonia

FACTORS

  • Aggravated by anxiety, stress, and fatigue
  • Relieved by rest and/ or sleep or tactile stimuli

TREATMENT

Conservative → Focal dystonias

  1. Massage therapy
  2. Relaxation techniques.
  3. Physiotherapy – strengthening, stretching.

Medication

  1. Baclofen (cranial dystonia)

S/E local weakness: patients with blepharospasm may have ptosis, whereas dysphagia is a potential complication of treatment for cervical dystonia → resolve spontaneously after 2 to 4 weeks.

  1. Benzodiazepines: Clonazepam (Rivotril) (blepharospasm)
  2. Levodopa (Sinemet) for dopa-responsive dystonia (DRD), 10% of children
  3. Botulinum toxin injections. (Most effective for focal dystonia)

Surgical

  1. DBS of the globus pallidus internus
  2. Intrathecal baclofen infusions (lower limb dystonia and spasticity)

Cuccurollo 4th Edition Chapter 12 Movement Disorder pg869

Braddom 6th Edition Chapter 45 Movement Disorder pg973

PMR Secrets 3rd Edition Chapter 56 Movement Disorders pg473 Box 56-4

Neurology Secrets 6th Edition Chapter 12 pg155

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8
Q

Define Athetosis & List 4 causes.

A

Slow, non-rhythmic, writhing (twisting) involuntary movements and inability to maintain the position.

Cuccurollo 4th Edition Chapter 12 pg867

DeLisa 5th Edition Table 26.8

Etiologies:

  1. Stroke.
  2. Tumor.
  3. Cerebral palsy (CP)
  4. Idiopathic.
  5. Basal ganglia disease (Parkinson Disease)
  6. Wilson’s disease.
  7. Huntington’s disease.
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9
Q

Define choreoathetoid.

A

Athetoid movements, slow involuntary twisting movements with superimposed faster or jerky chorea type movements.

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10
Q

Define chorea. List 4 causes of chorea.

A

Fast, non-rhythmic, unpredictable, incoherent, purposeless, jerky movement of limbs

Usually interfere with purposeful activity.

Cuccurollo 4th Edition Chapter 12 pg867

Causes: any chorea + extrapyramidal

  1. Huntington’s disease (HD)
  2. Sydenham chorea (SC)
  3. Levodopa (Sinemet)
  4. Typical Antipsychotics (ExtraPyramid)
  5. Anti-emetics (ExtraPyramid)
  6. Hyperthyroid
  7. SLE
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11
Q

Define Hemiballismus

A

Sudden, violent, involuntary movements on one side of the body

Lesion in subthalamic nucleus (regulates globus pallidus)

Cuccurollo 4th Edition Chapter 12 pg867

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12
Q

Myoclonus Definition, Types 🔑 , Treatment

A

Sudden, jerky, irregular, or periodic involuntary contractions of a muscle or group of muscles

Types

  1. Spontaneous: stimulus sensitive, occurs with muscle contraction, muscle stretch, or superficial stimuli like touch
  2. Non-Spontaneous: non-stimulus sensitive, occurs at rest
  3. Positive: Involuntary contraction of the muscle.
  4. Negative: Involuntary relaxation of the muscle.
  5. Physiologic: After exercise
  6. Hypnagogic jerks: During sleep

Treatment

  1. Anti-epileptic (Keppra)
  2. Botox Injection

Cuccurollo 4th Edition Chapter 12 Movement Disorder pg868

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13
Q

Define Akathisia. What medications is it often associated with? 🔑

A

Reversible motor restlessness, with unpleasant sensation of inner tension or anxiety

20-40% of patient on antipsychotic drugs (extra-pyramidal reaction to dopamine blockage).

Cuccurollo 4th Edition Chapter 12 Movement Disorder pg867

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14
Q

Define Tic - Types - Reliving factor

A

Tic

Abnormal movements (motor tics) or abnormal sounds (phonic tics) that are brief, involuntary, rapid, and non rhythmic.

Irresistible urge to move prior to the tic, resulting in a tension that builds and is subsequently relieved by execution of the tic.

Tourette syndrome

Both motor tics and phonic tics are presented

Types

  1. Simple Tics
  • Simple Motor: eye blink, facial grimace, shoulder shrug, or head jerk.
  • Simple Vocal: throat clearing, grunting, coughing, snorting, or animal sounds
  1. Complex Tics
  • Complex Motor: touching, grooming, scratching, kicking, hand shaking,
  • Complex Vocal: words, phrases

Worsen by

  • Stress, anxiety, and fatigue

Relieved by

  • Concentrating on a task or activities such as playing a musical instrument or reading.

Cuccurollo 4th Edition Chapter 12 pg867

Braddom 6th Edition Chapter 45 Movement Disorder pg974

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15
Q

What is the classic triad of Huntington’s Disease (HD)? 🔑🔑

Cause of death? Treatment?

A

💡 Huntington is the abnormal protein product of HD gene that accumulates in brain cells.

Triad

  1. Family history (dominant inheritance)
  2. Chorea/choreoathetosis

Involuntary, continuous, abrupt, rapid, brief, unsustained, irregular movements that flow randomly from one body part to another.

  1. Dementia and personality changes

Loss of recent memory, impairment of judgment, concentration, and acquisition

Apathy, social withdrawal, agitation, impulsiveness, depression, mania, paranoia, delusions, hostility, hallucinations, and psychosis

Caudate and putamen atrophy on neuroimaging studies is another feature supportive of the diagnosis of HD.

Other motor symptoms include

  • Dysarthria, dysphagia
  • Postural instability, ataxia
  • Myoclonus, and dystonia

Death

  • 15–20 years after onset (aspiration pneumonia)

Treatment

  1. Neuroleptics/Dopamine Receptor Blockers
  2. Presynaptic Dopamine Depleters

Cuccurollo 4th Edition Chapter 12 Movement Disorder pg874 Table 12-4

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16
Q

Define Ataxia. List 3 Types of Ataxia 🔑🔑

A

Lack of normal coordination between different joints

Resulting from abnormal contractions of agonist and antagonist muscles

Seen in

  1. Vestibular ataxia: Vertigo, nausea, and vomiting
  2. Cerebellar ataxia: Dysmetria - Dsynergia - Dysdiadochokinesia (3D)
  3. Sensory ataxia: loss of proprioception (dorsal columns)

Cuccurollo 4th Edition Chapter 12 pg867

17
Q

Define Dysmetria - Dyssynergia - Dysdiadochokinesia 🔑🔑

A

Dysmetria

  • Difficulty placement of a body part during active movement
  • Hypometria (undershooting of the target) or hypermetria (overshooting of the target).

Dyssynergia

  • Difficulties within the sequence and speed of the components of movement

Dysdiadochokinesia

  • Difficulty with repetitive or fine movement.

Cuccurollo 4th Edition Chapter 12 pg867

18
Q

Friedreich’s ataxia: Presentation - Examination - Non-Neurological signs 🔑

A

Presentation

  1. Oculomotor abnormalities, nystagmus, optic atrophy, reduced visual acuity
  2. Sensorineural hearing loss
  3. Dysarthria
  4. Progressive ataxia
  5. Areflexia of the lower limbs
  6. Impaired vibration, or position sense.
  7. Extensor plantar reflexes
  8. Progressive weakness of extremities

Non-Neurologic Signs

  1. Scoliosis
  2. Pescavus
  3. Hypertrophic Obstructive Cardiomyopathy (HOCM)

Cuccurollo 4th Edition Chapter 12 Movement Disorder pg875

19
Q

Restless leg syndrome (RLS) Definition, Treatment 🔑🔑

OSCE station: TBI with fatigue due to poor sleep secondary to RLS.

A

Restless leg syndrome (RLS)

Abnormal unpleasant sensation in the legs that arises during prolonged rest or when the patient is drowsy and trying to fall asleep, especially at night

Dysesthetic, bothersome, painless sensations

Sensations are partially or completely relieved by movement such as walking or stretching

Types

  1. Primary RLS: TBI or CNS disorder
  2. Secondary RLS: Iron deficiency or peripheral neuropathy

Also: diabetes, multiple sclerosis, Parkinson’s disease and pregnancy, caffeine, Deficiency of vit B12, folate, and Mg, Hypothyroidism, Fibromyalgia

First line Treatment

  • Long-acting dopaminergic compounds
  • Iron supplements, particularly in patients with low serum ferritin (<50 to 80 mg)

Second line Treatment

  1. Anticonvulsants: gabapentin, pregabalin, or carbamazepine.
  2. Benzodiazepines: Clonazepam (Rivotril)

Cuccurollo 4th Edition Chapter 12 Movement Disorder pg869-870

Braddom 6th Edition Chapter 45 Movement Disorder pg1077

https://www.researchgate.net/figure/Some-secondary-causes-of-restless-legs-syndrome-RLS_fig1_24398887