Extrapyramidal diseases Flashcards
How do we recognize Parkinson’s disease?
Asymmetric movement disorder
Akinesia
Rest tremor
Rididity
Postural instability
Esencial tremor. Differential diagnosis with Parkinson
Symmetric upper limb tremor
Action and positional (not at rest)
No other parkinsonian signs
Dyskinesia
Tremor
Dystonia
Chorea
Tics
Myoclonus
Asymmetric atrophy of the parietal lobe on head MRI
Corticobasal degeneration
How do we treat a young patient with signs of moderate Parkinson’s disease
Dopamine agonist
How to treat Parkinsonism
L-dopa
Anticholinergics
Amantadine
Deep brain stimulation
MAO inhibitors
Dopamine agonists
68 years old with Parkinson’s disease who is motor regulated, presents symptoms of psychosis. Adjustment of dopaminergic has not improved the condition. Which neuroleptic would you use
Clozapine, because it has least extrapyramidal effects
Type of gaze palsy expected in Steel-Richardson-Olszewski syndrome
Vertical gaze palsy
Basis of symptomatic treatment for Parkinson’s disease is based on deprivation
Dopamine
In Parkinsonism an excess of ___ is found in the striatum
acetylcholine
Dopamine what stimulates and inhibits
Dopamine stimulates the direct pathway via D1 receptors and inhibits the indirect pathway via D2 receptors, inhibiting cholinergic neurons in the putamen
Motor dysfunction of the basal ganglia due to dopamine deficiency in the striatum manifests as
Akinetic-rigid syndrome
After long-term dopamine deficiency
Hypersensitivity of striata neurons
Depletion of dopaminergic cells in the nucleus reduces dopamine levels in
Striatum
Uncoordinated movements of the tongue and lips (dyskinesias) are characteristic of
Tardive dyskinesia due to neuroleptics
Essential tremor therapy
Propanolol
Treatment of Huntington’s chorea
Haloperidol
Is myoclonus common in Creuzfeldt-Jacob disease
Yes
The most effective treatment for focal dystonias is
botulinum
Treatment of dystonias
Focal dystonias: botulinum
Levodopa
Anticholinergics
Stereotactic brain surgery
Deep core stimulation
Young patient with Wilson’s disease clinical
Dystonia
Parkinsonism
Dysarthria
Tremor
DYT1 dystonia
Starts in childhood, affects the lower limb first and then generalizes
AD inherited. DYT1 is the gene that encodes the protein torsion A.
Tremor in Wilson disease is
Fluttering
Dyskinesias are the result of
Elevated levodopa concentrations in plasma
Progressive supranuclear palsy name
Steel-Richardson-Olszewski syndrome
Fall back in
Progressive supranuclear palsy
38 years old. Right hand trembles at rest. Bradykinetic without rigidity incipient Parkinson’s disease. Treatment
Anticholinergics
78 years old. Long-standing Parkinsons’s disease and severe motor complications. Treatment
Stimulation of the subthalamic nucleus
Myoclonus and cortical sensitivity disturbances are expected in
Corticobasal degeneration
Corticobasal degeneration
rare sporadic cause of adult-onset Parkinsonism. movement disorder.
Unilateral upper limb fixed dystonia, akinesia, rigidity, myoclonus, apraxia, cortical sensory loss.
Later, dementia and supranuclear gaze problem
Corticobasal degeneration MRI
Asymmetric atrophy of the parietal lobe
Dystonia in a child who later develops idiopathic torsion dystonia sill start at
Legs
Young patient with signs of moderate Parkinson’s disease drug
Dopamine agonist
In Parkinson’s disease what happens in the striatum
Increased acetylcholine tone in the striatum
Hemibalism in hyperglycemia?
Yes
Hemibalism in hypoglycemia?
No
Hemibalism in hypokalemia?
No
Hemibalism in hyperkalemia?
No
Hemibalism in hypernatremia?
No
Treatment of focal dystonias
Botulinum injections
55 years old with apparent Parkinson. Treatment
Dopamine agonists
Pathology in Parkinson is in
substancia nigra pars compacta
MRI Huntington’s disease
Enlargement of the lateral ventricle
Essential tremor treatment
Primidone
Deep brain stimulation of thalamus
Propanolol
Benzodiazepines
Levodopa enters the central nervous system by
diffusion
In Parkinson, the dopaminergic-cholinergic imbalance in the striatum is causes by
increased released (liberación) of acetylcholine from cholinergic interneurons due to dopamine deficiency in the striatum
Wilson’s diseases caused by a defect in
ceruloplasmin function
Huntington’s disease type of disease
An autosomal dominant disorder caused by a mutation in the Huntington gene on chromosome 4
Diagnostic procedures for Parkinson’s disease from the most important to the least important
Clinical presentation, levodopa test, MRI
Therapeutic options for blepharospasm from those to be tried first to those to be tried last
Botulinum toxin injection, pharmacological treatment, surgery
Which motor effects which are side effects of L-dopa treatment
Dystonias
Chorea form movements
motor fluctuations
How does Levodopa work
precursor of dopamine
L-dopa uptake % into the brain without dopa carboxylase inhibitors is
1%
Substance located in striatum is
dopamine
Amantadine
Increases the release of endogenous dopamine
Can cause neuroleptic malignant syndrome
Abrupt cessation of dopamine agonist
Apomorphine claims
postsynaptic dopamine receptor agonist
Wilson’s disease what type of disease
Inheriting. autosomal recessive
Wilson’s disease. Clinical
Dystonia, Parkinson, dysarthria, tremor
B12 deficiency, uremia, hepatic
Keyser-Fleisher ring
Basal ganglia damage (more the putamen)
MSA (atrofia multisistémica) Clinical
Parkinsonism, ataxia, urinary incontinence, paraparesis
Defect in the dopamine pathway in multiple system atrophy (MSA) is in
Pre and postsynaptic
Treatment of chromatic joints in Huntington’s disease
Haloperidol
How do we distinguish Parkinsonism plus from Parkinson’s disease
Levodopa test
Focal dystonias. Clinical
Spastic torticolis
Blefarospasm
Meigs syndrome
L-dopa-responsive dystonia
Wilson’s disease type of illness
Extrapyramidal, inherited. Chromosome 13
Defect basal ganglia-thalamus-cortex pathway. Which impairment in Parkinson’s disease
Affective
Parkinson’s disease is caused by presynaptic or postsynaptic
Presynaptic
Premotor symptoms in Parkinson
Decreased sense of smell, depression, night-time sleep disturbances, mood and gastrointestinal problems
How to treat chromatic dyskinesias after levodopa
Taking lower doses of levodopa more often
Treatment for painful leg dystonia that occurs in the early morning after several years of Levodopa treatment
Botulinum injections into the affected muscle
Gene mutation associated with the onset of early Parkinson’s disease
alfa-synuclein
Progressive supranuclear palsy is
pre and postsynaptic impairment of dopaminergic synapses
System excised in Parkinson’s disease
Glutaminergic
Parkinsonism plus response to dopamine agoinst
Respond poorly to treatment with dopamine agonists
Glial inclusions stained for alfa-synuclein is found in
Multiple system atrophy
Sensory trick
physical genture or posotion that a patient with dystonia can adopt to relieves or eliminates dystonic symtoms
How to worsen Parkinson
Inhibition of the nigrostrial pathway
Wilson’s disease treatment
Penicillamine
Test in a young patient with Parkinsonism
Measure copper in urine