Movement Disorders Flashcards
What are the Hoehn and Yahr stages of Movement disorder?
I. Unilateral involvement only, minimal or no functional impairment
II. Bilateral or midline involvement, without impairment of balance
III. First sign of impaired righting reflex, mild to moderate disability
IV. Fully developed, severely disabling disease; patient still able to walk and stand unassisted
V. Confinement to bed or wheelchair unless aided
What are the components of the extrapyramidal system?
- Caudate
- Putamen
- Globus palidum
- Substantia nigra
- Sub thalamic nucleus
What is the pathology of Parkinson’s disease?
Loss of pigmented cell in the substancia nigra pars copacta.
Deposition of alpha synuclein into inclusion bodies- lewy bodies in neurons
Tyrosine hydroxylase diminishes and reduced production of Dopamine
Loss of dopaminergic input from SN to striatum.
Increased activity indirect pathway.
Decreased activity direct pathway.
Increased activity GPi
Increased inhibition thalamocortical and midbrain tegmental.
Hypokinesia
What is the clinical presentation of Parkinson’s disease?
Chronic, slow progressing, asymmetrical rigidity
Bradykinesia (slowness)
Hypomimia
Cogwheel rigidity
Gait disturbance-shuffling, freezing, festinations, turns “en block”
Postural instability
Tremor: Rest 4-5 Hz (pill rolling), hand, chin, tongue
Reduced eye blink rate
Voice – monotonous , dysphonic (hoarse), dysprosodic (droning).
Retropulsion
Cog wheel rigidity and noike
Dopa responsive
What are the differential diagnoses for Parkinson’s Disease?
P.S.P – progressive supra nuclear palsy
D.L.B.D - Dementia with Lewy bodies
Corticobasal degeneration
Multiple system atrophy
N.P.H – normal pressure hydrocephalus
Multi infarct parkinsonism
Drug induced parkinsonism
Essential tremor
What are the clinical findings of progressive supranuclear palsy (PSP)?
6th decade
Recurrent falls-even at the beginning.
Slurred speech, dysphagia.
Axial rigidity - dystonia
Retropulsion
Paralysis of vertical gaze.
From book: no tremor, no ophthalmoplegia
Quicker progression than Parkinson’s
What are the clinical findings in D.L.B.D - Dementia with Lewy bodies?
Synucleinopathy
Visual hallucinations (increased with L-dopa)
Diurnal fluctuations
Early cognitive deterioration but less than AD
Parkinsonism less than in Parkinson’s
Most important cause of subcortical dementia-Not rare- about 25% of Dementias
What are the clinical findings in Corticobasal degeneration?
Progressive extrapyramidal rigidity+ pyramidal signs (hyperreflexia).
Fast progression
Asymmetrical clumsiness & apraxia.
some have anosognosia (don’t recognize disability even if complete hemiplegia)
“Alien hand”
What are the clinical signs in multiple system atrophy - striato nigral degeneration
Parkinsonism( rigidity, stiffness, akinesia)
More severe than PD.
Relative symmetry.
No classical rest tremor.
Autonomic dysfunction
No L. DOPA improvement.
All systems affected, can be Shy-drager syndrome – autonomic insufficiency with Parkinsonianism; Or olivo ponto cerebellar- more cerebellar signs.
Cerebellar signs – 50%
Pyramidal signs -60%
How is Parkinson’s treated and what are complications of treatment?
Carbadopa and levodopa, Cinemet (combo)- lots of side effects so treat with others first
D2 agonists
COMT inhibitors
MAO B inhibitors
Apomorphine
Deep brain stimulation
Palidothomy
Honey moon 4-5y
Fluctuations (end of dose off,on-off)
Drug related dyskinesias
Hallucinations, cognitive deterioration
High dependency 10-15y
What are non-motor features of Parkinson’s disease?
Cognitive dysfunction and dementia- 40-80%
Psychosis and hallucinations - 20-40% usually visual, Dopa makes worse
Mood disorders including depression, anxiety, and apathy/abulia
Sleep disturbances
Fatigue
Autonomic dysfunction (orthostatic hypotension, constipation)
Olfactory dysfunction
Pain and sensory disturbances
Dermatologic findings (seborrhea)
What is the Hakim triad?
- Lower body parkinsonism
- Cognitive deterioration
- Urinary incontinence
Large pressure on ventricles
More diameter and less pressure at same tension
What is the differential diagnosis for “lower-body-Parkinsonism”?
Normal Pressure Hydrocephalus (NPH-Hakim triad)
Multi-infarct Parkinsonism (Pyramidal signs, frontal signs, Pseudobulbar palsy-laughing and crying without control, often seen around ventricles)
Drug induced Parkinsonism (antipsychotics, neuroleptics, fast progressing, symmetrical, no pill-rolling)
What are the differential diagnoses of hyperkinetic movement disorders?
Chorea(Hereditary-Huntington (treat with Hallidol), Acancytosis; Rheumatic-Sydenham’s; Chorea gravidarum; drug induced-neuroleptic/contraceptive; systemic)
Athetosis (more subtle than chorea, disinhibition of subthalamic nucleus)
Dyskinesia/ dystonia
Hemibalismus (contralateral subthalamic nucleus lesion, tx: neuroleptics)
Tic disorder
Wilson disease
What are the types of dystonia?
What are the differential diagnoses?
What is the treatment of dystonia?
Generalized – DYT1 torsion dystonia-common in Aschkenazi Jews
Segmental
Focal – blepharospasm of the eyelid, torticollis,hand – musician or writer’s cramp.
DD:
Idiopathic - genetic
Secondary - Drug induced, post infectious, trauma, perinatal
Tx: Medication – BZD, Baclofen (GABA agonist), anti cholinergic, Botulinum toxin-few side effects when used focally. Deep brain stimulation.
