Parkinson's Flashcards
Extrapyramidal Disorders
affect the regulation of movement: – initiation of movement – speed of movement – control or quality of the movement – tremor – whether movement is voluntary or not
Abnormal Movements
Tremor, Chorea, Athetosis, Dystonia, Balismus, Myoclonus and Tics
Types of Tremors
- Physiological: normal (6-12)
- Intention: during activity, cerebellar injury
- Resting: associated with PD (4-6)
Chorea
– Involuntary rapid, irregular muscle jerks
– Associated with lesions of the caudate or putamen
– When strong, facial and tongue movements are also observed
– Voluntary movements can be distorted.
Unilateral Chorea
Hemiballismus
Dystonia & Athetosis
– Slow, purposeless, writhing movements (athetosis)
– Dystonia refers to those movements which are more like or turn into postures
– Not present during sleep; affected by stress and intention
– Associated with perinatal anoxia, CP, Huntington’s & drug side effect (inherited or acquired)
Myoclonus
- Sudden, violent muscle jerks
- Can be spontaneous or brought on by sensory stimulation, arousal, or as part of a voluntary movement
Physiologic Myoclonus
nocturnal myoclonus & hiccups
Essential Myoclonus
isolated abnormality
Epileptic Myoclonus
manifestation of epilepsy
Symptomatic Myoclonus
Associated many neuro-degenerative and metabolic disorders (from cerebral cortex – brainstem –spinal cord)
Tics
– Sudden, recurrent, coordinated abnormal movements or verbalizations
• Occurs repeatedly and can be suppressed voluntarily for short periods
• Worsen with stress
• Diminished during voluntary movements or mental concentration, and disappear during sleep
Transient simple tics
common in children; usually go away within a year
Chronic simple tics
begin in childhood; benign but don’t go away
Tourette’s Syndrome
Chronic multiple motor and vocal tics
Involuntary Verbal Tics in Tourettes Syndrome
– Typical to include grunts, barks, hisses, or coughing.
– Coprolalia: vulgar or obscene utterances
– Echolalia: parroting of another’s speech
– Parilalia: repeating the same word over and over
Involuntary Motor Tics in Tourette’s Syndrome
– Blinking, grimacing, sniffing
• hopping, jumping, obscene gestures may develop
– Echopraxia: imitation of another’s movements
– Self mutilation (40-50%): biting nails, picking nose, pulling hair
Lesions of Basal Ganglia
• Decomposes behavior into isolated motor acts
• Decomposes intended motor acts into movements of inappropriate amplitudes
– Too little or too much amplitude
Idiopathic Parkinsonism
– Progressive, neurodegenerative disease
• Deficits due to loss of dopamine 2° degeneration of dopaminergic neurons in substantia nigra pars compacta
Two Major “Forms” of Parkinsonism
Tremor dominant and Akinetic
PIGD (Akinetic) Type Parkinsonism
more postural instability, gait difficulty -associated with a worse prognosis
Later Onset is more likely to cause
PIGD type
Risk Factors of Parkinson Disease
• Genetic, behavioral, and environmental factors are being examined
Genetic Risk Factors for Parkinson’s
– 5-10% with familial pattern of inheritance
– Role of alpha-synuclein protein due to change in
the gene SNCA (Lewy bodies)
Environmental Risk Factors for Parkinson’s
– Chemical toxins
Higher Risk: Rural Living
Lower Risk: Cigarette smoking, alcohol use, caffeine, physical activity, regular ibuprofen use, calcium channel blockers
Pathology of Parkinsonism
Widespread distribution of Lewy Bodies in the lower brainstem
Long Prodromal Phase: Early non-motor signs (NMS) prior to Dx
– Olfaction involvement (hyposmia)
– Constipation
– REM sleep behavior disorder
Cardinal Features of Sporadic PD
- Tremor – resting “pill rolling”
- Rigidity – cogwheel or lead pipe
- Bradykinesia – slow movement
- Postural instability
Supportive Criteria of Parkinson’s
- Unilateral onset with persistent asymmetry
- Progressive
- Response to dopamine therapy
- Olfactory dysfunction
Exclusion Criteria of Parkinson’s
- Lack of benefit from dopamine
- Current or recent use of dopaminergic blockers
- Documentation of alternative cause: (Hydrocephalus, encephalitis, trauma, toxins, vascular disorder)
- Physical exam
Physical Exam findings that exclude a pt from Parkinson’s dx
– Cerebellar signs (ataxia, dysmetria)
– Early dementia
– Supranuclear gaze palsy
– PD limited to the LE’s for more than 3 yrs.
• Likely Lower Body Parkinsonism
– Less responsive to L-dopa and higher incidence of HTN
– Cortical signs (aphasia, apraxia, astereo-agnosia)
Diseases that may look like Parkinson Disease
- Progressive supranuclear palsy: progressive and fatal
- Multiple system atrophy: progressive and fatal
- Lewy body disease
Progressive supranuclear palsy
– Idiopathic, degenerative, primarily affecting subcortical grey matter (dentate nucleus, pons, midbrain, BG)
– Failure of voluntary vertical gaze
– Postural instability
– Neck often assumes an extended posture
– Psuedopulbar palsy – facial weakness, dysarthria, dysphagia, increased jaw and gag reflexes
– Dementia - forgetfulness, slowed thought, changes in mood and personality
How is Progressive supranuclear palsy distinguished from PD?
early falls, problems of downward gaze, extended neck posture, tremor uncommon in PSP and PD drugs are less effective in PSP
Multiple system atrophy
– Wide-spread neuro degeneration
– MSA-P – neuronal loss in striatum and GP
• Bradykinesia; rigidity
– MSA-C – cerebellar degeneration
– Shy-Dragersyndrome: autonomic involvement
• Postural hypotension, anhidrosis, disturbance of sphincter control and impotence
How is Multiple system atrophy distinguished from PD?
symmetric presentation and multi-system involvement
Lewy body disease
– Form of dementia
– Cognitive changes lead to dementia and usually precede or occur shortly after the appearance of PD-like deficits
– Cognitive function may fluctuate markedly within a 24 hour period
– Postural hypotension and syncope
How is Lewy Body disease distinguished from PD?
early cognitive involvement, symmetric deficits, and less likely to see tremor
PD Voluntary Movement
• Akinesia
– No movement or
– Delay in initiation
• Bradykinesia
– Slow movement
• Hypokinesia
– Low amplitude movement
Involuntary Movement PD
• Tremor
– Pill Rolling
– Resting
4-6 times/sec
• Rigidity
– Different than spasticity
PD Walking
- Flexed posture
- Anterior displaced CM
- Short, shuffling steps
- Loss of associated movement
- Festinating gait
Standing Balance; Static - PD
Postural predisposition to loss of balance
Dynamic Standing Balance- PD
– Impairments in preparatory postural adjustments
– Loss of righting and protective responses
Mental Status in PD
- Decline in cognitive function (usually significant declines occur later in the disease process)
- Visual disturbances (hallucinations)
- Depression
Precursor of Dopamine
L-dopa
3-5 year max benefit
What is the goal of taking medication with PD?
restoring balance between Ach & Dopamine
What can brain stimulation help with?
- dyskinesias
- may reduce rigidity
Huntington’s Disease (chorea)
- A progressive degenerative (of striatum) disorder
- A hereditary disorder which manifests itself in early to mid adult life
- Each child of an affected parent has a 50% chance of inheriting the gene that causes HD
- Progressive and fatal: average lifespan is 15 years after clinical onset
Clinical Findings of HD
- Chorea: starts as restlessness, can’t suppress blinks, but progresses to choreiform movements
- Motor impersistence (drop objects, cant hold tongue out, minor car accidents)
- Dystonia
- Dementia and emotional disturbances
- With OCD and chorea, weight loss is a significant problem in HD
Atypical or Westphal variant form and subtypes of HD
– Can show rigidity, akinesia (like PD)
– Juvenile form (before age 20) is rare but can show rigidity, akinesia along with other neurobehavioral symptoms
Dementia and emotional disturbances in HD
– Earliest changes consist of irritability, moodiness, and antisocial behavior
– Later – OCD, impairment of attention and executive function with
eventual psychosis, consistent with frontostriatal pathology
– Depression (potentially many years)
– Personality issues
Hemiballismus
- Sudden (acute) onset of flinging – choreic movements (implicating proximal muscle involvement)
- Typically thought to be due to a lesion of the subthalamic nucleus in the basal ganglia (usually as a result of a CVA)
Tardive Dyskinesia
- Choreo-athetoid movements of the face, mouth, jaw and tongue
- Grimacing, pursing the mouth, writhing movements of the tongue, and “fish-gaping”
- Associated with prolonged use of psychotropic drugs
Role of Cerebellum in Movement
- Balance and Eye Movements
- Skilled Voluntary Movement
- Locomotion
- Muscle Tone (hypotonia is common with damage)
Typical signs and symptoms of damage to the cerebellum
- Incoordination
- Delays in movement initiation and termination
- Hypotonia
- Intention tremor
- Dysequilibrium and vertigo
Ataxia (Incoordination)
• Loss of ability to control and coordinate movements
Ataxia is often associated with
– Delays in initiation
– Dysmetria: errors in the range of movement (Hypometria or hypermetria)
– Dysdiadochokinesia (Errors in rate and regularity of movement)
Etiologies of Damage to Cerebellum
- Toxins
- Genetic Diseases (Friedreich’s Ataxia)
- System Degeneration (Olivopontocerebellar atrophy)
- Structural Diseases (Tumors)
- Demyelinating Disease (MS)
Friedreich’s Ataxia
- One of the most common hereditary disorders of the nervous system
- Disrupts normal assembly of amino acids into proteins
- Progressive degeneration of – Dorsal root ganglia, large myelinated axons of peripheral sensory nerves, Corticospinal tracts, Dentate nuclei
Friedreich’s Ataxia average age of onset
13 years
Friedreich’s Ataxia Sxs
- Ataxia begins in lower limbs (gait ataxia) but progresses up
- Nystagmus and dysarthria
- Impairment of sensation; later LE weakness (UMN and LMN)
- Scoliosis
Olivopontocerebellar Atrophy
Combined degeneration of the cerebellum, pons, and
inferior olives
Olivopontocerebellar Atrophy Sxs
• Progressive ataxia with a later onset than Friedreich’s ataxia
• Gait is usually affected first with resultant decreased movement speed and balance deficits
• Dysarthria, muscle spasms, weakness and autonomic
disorders are also common: Urinary incontinence; orthostatic
hypotension
B1 (thiamine) deficiency
Wernicke-Korsakoff
oculomotor abnormalities; mental status; ataxia of stance and gait
