Movement Disorders

1
Movement Disorders

• Refer to Chapter 7 of
• Clinical Neurology Textbook

2
Movement Disorders

• Background
• AKA Extrapyramidal Disorders
• These disorders impair the regulation of
  voluntary motor activity w/o affecting the
  strength, sensation, or cerebellar fcn.

3
Movement Disorders

• Background
• Result from dysfunction of the basal ganglia
• Caudate
• Putamen
• Globus Pallidus
• Subthalamic Nucleus
• Substantia Nigra
• Lentiform Nucleus
• Putamen Globus Pallidus
• Corpus Striatum
• Lentiform Nucleus Caudate Nucleus

4
Movement Disorders

• Basal Ganglia Circuitry (Fig 7-1)
• Corticocortical Loop

5
Movement Disorders

• Basal Ganglia Circuitry (Fig 7-1)
• Nigrostriatal Loop

6
Movement Disorders

• Basal Ganglia Circuitry (Fig 7-1)
• Striatalpallidal Loop

7
Movement Disorders

• Types of Abnormal Movements
• Tremor rhythmic movement characterized by when
  it occurs
• Postural Tremor
• During sustained posture
• Intention Tremor
• During movement absent at rest
• Resting Tremor
• At rest

8
Movement Disorders

• Types of Abnormal Movements
• Chorea irregular muscle jerks
• Florid Cases
• Fully developed
• Forceful movements of limbs, head, facial
  grimacing, tongue movements
• Mild Cases
• Characterized by
• Clumsiness
• Milkmaid grasp
• Absent in sleep

9
Movement Disorders

• Types of Abnormal Movements
• Hemiballismus
• Unilateral Chorea
• Involves the proximal muscles
• Vascular disease of contralateral subthalamic
  nucleus

10
Movement Disorders

• Types of Abnormal Movements
• Athetosis
• Continued slow, sinuous, writhing movements
• Dystonia sustained athetotic movements
• Segmental Dystonia
• Affects one or more limbs
• Focal Dystonia
• Affects localized muscle groups
• Palliative/Provocative
• Causes

11
Movement Disorders

• Types of Abnormal Movements
• Myoclonus
• Definition
• Classification
• Generalized widespread
• Physiological
• Essential
• Epileptic
• Symptomatic
• Segmental more localized

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Movement Disorders

• Types of Abnormal Movements
• Tics
• Definition
• Palliative/Provocative
• Types
• Transient Simple common in children, resolve w/I
  1 yr
• Chronic any age, no tx
• Persistent Simple or Multiple onset before 15
  yoa, resolve in adults
• Chronic Multiple Tourettes Sydrome

13
Movement Disorders

• Hypokinetic Movement Disorders
• Parkinsons Disease
• Hyperkinetic Movement Disorders
• Huntingtons Disease
• Wilsons Disease
• Tourettes Syndrome
• Restless Leg Syndrome

14
Movement Disorders

• Parkinsons Disease - Hypokinetic
• Defined as a syndrome consisting of variable
  combination of tremor, rigidity, bradykinesia,
  and characteristic disturbance of gait and
  posture
• Onset mid-late life mean age is 57 yrs
• Epidemiology
• Affects all ethnicities
• has equal M/F distribution
• occurs 1-2 per 1,000 people in general population
• occurs 1 per 100 people that are over 65 yrs
• 4th most common disease in the elderly

15
Movement Disorders

• Parkinsons Disease - Hypokinetic
• Cause unknown
• Pathophysiology
• Loss of dopaminergic cells in the substantia
  nigra
• Dopamines normal function
• Over excitation of the caudate putamen
• Over excitation of the corticospinal tracts
• Oscilation of feedback
• Decrease in thalamic excitation of the motor
  cortex

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Movement Disorders

• Parkinsons Disease - Hypokinetic
• Four Hallmark Signs
• Resting Tremor (Pill-Rolling)
• Rigidity (Lead-Pipe or Cogwheel)
• Bradykinesia
• Flexed Posture with shuffling gait (Festinating)

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Movement Disorders

• Parkinsons Disease - Hypokinetic
• Examination
• History
• Phsyical Findings
• Passive movement
• Muscle Strength
• Sensory
• Deep Tendon Reflexes
• Autonomic
• Myersons Sign
• Pull Test

18
Movement Disorders

• Parkinsons Disease - Hypokinetic
• Diagnosis
• Four Hallmark signs
• Tremor is absent in 30 of patients
• Differential Diagnosis
• Involuntary tremor vs. Intentional tremor
• Depression
• Wilsons Disease
• Huntingtons Disease

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Movement Disorders

• Huntingtons Disease Hyperkinetic
• A neurodegenerative disorder which predominately
  has behavioral, cognitive, or movement disorders
  signs
• Onset Usually begins during adult life
• Epidemiology
• 5-10 per 100,000 in the US
• 50 chance to pass on the disorder
• Anticipation
• Paternal Descent

20
Movement Disorders

• Huntingtons Disease Hyperkinetic
• Cause Autosomal Dominant Disorder
• Pathophysiology
• Mutation on chromosome 4 CAG repeats
• CAG Normal Function codes for glutamine
• Over-expression of the gene i.e. excess
  glutamine
• Uncertainty?

21
Movement Disorders

• Huntingtons Disease Hyperkinetic
• Cause Autosomal Dominant Disorder
• Pathophysiology
• Pathological Changes
• Atrophy neuronal degeneration of cortex
• Hallmark caudate atrophy
• Projected Conclusion?
• Over activity
• Under activity

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Movement Disorders

• Huntingtons Disease Hyperkinetic
• Examination
• Physical Findings
• Initial Findings
• Gradual onset
• Slowed saccadic movements 1st sign
• In 85 chorea is predominate movement disorder
• Juvenile Form
• AKA The Westphal Variant
• Rigidity bradykinesia
• Tremors, Dystonic postures, Ataxia
• Mental retardation, Seizures, myoclonus

23
Movement Disorders

• Huntingtons Disease Hyperkinetic
• Examination
• Physical Findings
• Adult Onset
• Prominent chorea
• Bradykinesia
• Postural reflex compromise
• Terminal Phase
• Dysarthria, dysphagia, respiratory difficulties
• General
• Cognitive impairment
• Depression
• Psychiatric disorders

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Movement Disorders

• Wilsons Disease Hyperkinetic
• Onset
• Hepatic Dysfunction 11 yoa
• Neurological Dysfunction 19 yoa
• Epidemiology
• Rare
• 1 in 40,000 people
• Cause Autosomal Recessive Disorder

25
Movement Disorders

• Wilsons Disease Hyperkinetic
• Pathophysiology
• Abnormal copper metabolism
• Deposition of copper in tissues

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Movement Disorders

• Wilsons Disease Hyperkinetic
• Examination
• Physical Findings
• Children hepatic dysfunction predominates
• Sardonic Smile
• Behavioral problems
• Adults neurological dysfunction predominates
• Parkinsonian features
• General
• Hallmark Kayser-Fleischer Rings
• 1/3 experience psychiatric symptoms
• Other ocular abnormalities

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Movement Disorders

• Gilles de la Tourette Syndrome Hyperkinetic
• Diagnosed when childhood onset tics are
  multifocal, motor or vocal, lasting longer than 1
  yr and naturally wax and wane
• Cause unknown
• Onset 2-21 yoa
• Male predilection

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Movement Disorders

• Gilles de la Tourette Syndrome Hyperkinetic
• Examination
• Physical Findings
• Simple Tics
• Motor blinking, facial grimacing, shoulder
  shrugging
• Vocal throat clearing, grunting, snorting,
  barking
• Complex Tics
• Motor hopping, skipping, Echopraxia
• Vocal Coprolalia, Echolalia, Palilalia

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Movement Disorders

• Restless Legs Syndrome Hyperkinetic
• Common movement disorder
• Diagnostic Criteria
• Desire to move limbs which is associated with
  unpleasant sensations
• Restlessness
• Worsening of symptoms _at_ rest w/ temporary relief
  w/ movement
• Worsening of symptoms _at_ night

30
Movement Disorders

• Restless Legs Syndrome Hyperkinetic
• Common Descriptions
• Always unpleasant, but not necessarily painful
• Need to move
• Crawling
• Tingling
• Itching
• Restless