Week 6.2 Flashcards
What are the features of parkinsonism?
- bradykinesia/akinesia
- rigidity
- postural instability
The most common cause of parkinsonism is what?
Parkinson’s disease
Parkinson’s disease affects which neuron population?
dopaminergic neurons in the substantia nigra
What are the clinical features of Parkinson’s disease?
- resting tremor
- parkinsonism
- autonomic dysfunction
- neuropsychiatric disturbances
What are some examples of autonomic dysfunction experienced by those with Parkinson’s disease?
- orthostatic hypotension
- constipation
- drooling
- excessive sweating
- sexual dysfunction
What are some types of neuropsychiatric disturbances experienced by those with Parkinson’s disease?
- depression
- dementia
- psychosis
The average age of onset for Parkinson’s disease is what?
60 years old
What clinical findings may suggest a non-Parkinson’s disease cause of parkinsonism?
- symmetry at onset
- early dementia
- abrupt onset or rapid progression
- early or severe autonomic dysfunction
What are the features of Shy-Drager Syndrome?
- autonomic failure
- parkinsonism
- pyramidal tract signs
- peripheral neuropathy
- cerebellar signs
What sort of damage leads to Shy-Drager syndrome?
damage to both the substantia nigra and striatum
What sort of drugs can cause drug-induced parkinsonism?
those that block dopamine signaling
What is the primary cause of vascular parkinsonism?
ischemic damage to the striatum
What are the risk factors for vascular parkinsonism?
anything that increases one’s risk to striatal ischemia
What is MPTP?
a drug that induces parkinsonism
Name four pharmacologic therapies for Parkinson’s disease.
- levodopa
- COMT inhibitors
- MAO-B inhibitors
- dopamine agonists
How does carbidopa enhance the effect of levodopa?
it inhibits COMT in the periphery, increasing the amount of levodopa that makes it into the CNS
What are the three primary complications of levodopa in the long-term?
- motor fluctuations
- dyskinesias
- mental status change
What is MAO-B?
an enzyme that metabolizes dopamine in the synaptic cleft
What are selegiline and rasagiline?
MAO-B inhibitors
What are entacapone and tolcapone?
COMT inhibitors
Deep brain stimulation for Parkinson’s disease targets what structure?
the sub-thalamic nucleus
What is a tremor?
a rhythmic involuntary movement
What is a tic?
a suppressible involuntary movement
What is a dystonia?
a sustained involuntary movement
What is a chorea?
a ballistic involuntary movement
What is a myoclonus?
a non-rhythmic, non-sustained, non-ballistic, non-suppressible involuntary movement
What are three common causes of tremor?
- Parkinson’s disease
- essential tremor
- familial tremor
Tics are a common feature of which diseases?
- Tourett’e syndrome
- adult onset tic disorder
How do we treat tics?
by blocking DA
What is athetosis?
a type of dystonia that appears as a writhing movement
What is the underlying problem that leads to dystonia?
a lack of reciprocal inhibition
How do we treat focal dystonia?
botulinum toxin
What is a ballistic movement?
an agonist, antagonist, agonist sequence
What is tardive dyskinesia?
involuntary movements seen after the use of dopamine blocking agents
What are three common etiologies for myoclonus?
- idiopathic
- primary generalized epilepsy
- post-anoxic
How do we prevent tardive dyskinesia?
with anticholinergics
The most common cause of parkinsonism is what?
Parkinson’s disease
Parkinson’s disease affects which neuron population?
dopaminergic neurons in the substantia nigra
What are the clinical features of Parkinson’s disease?
- resting tremor
- parkinsonism
- autonomic dysfunction
- neuropsychiatric disturbances
What are some examples of autonomic dysfunction experienced by those with Parkinson’s disease?
- orthostatic hypotension
- constipation
- drooling
- excessive sweating
- sexual dysfunction
What are some types of neuropsychiatric disturbances experienced by those with Parkinson’s disease?
- depression
- dementia
- psychosis
The average age of onset for Parkinson’s disease is what?
60 years old
What clinical findings may suggest a non-Parkinson’s disease cause of parkinsonism?
- symmetry at onset
- early dementia
- abrupt onset or rapid progression
- early or severe autonomic dysfunction
What are the features of Shy-Drager Syndrome?
- autonomic failure
- parkinsonism
- pyramidal tract signs
- peripheral neuropathy
- cerebellar signs
What sort of drugs can cause drug-induced parkinsonism?
those that block dopamine signaling
What are three common causes of tremor?
- Parkinson’s disease
- essential tremor
- familial tremor
How do we treat tics?
by blocking DA
What is tardive dyskinesia?
involuntary movements seen after the use of dopamine blocking agents
The most common cause of parkinsonism is what?
Parkinson’s disease
Parkinson’s disease affects which neuron population?
dopaminergic neurons in the substantia nigra
What are the clinical features of Parkinson’s disease?
- resting tremor
- parkinsonism
- autonomic dysfunction
- neuropsychiatric disturbances
What are some examples of autonomic dysfunction experienced by those with Parkinson’s disease?
- orthostatic hypotension
- constipation
- drooling
- excessive sweating
- sexual dysfunction
What are some types of neuropsychiatric disturbances experienced by those with Parkinson’s disease?
- depression
- dementia
- psychosis
What clinical findings may suggest a non-Parkinson’s disease cause of parkinsonism?
- symmetry at onset
- early dementia
- abrupt onset or rapid progression
- early or severe autonomic dysfunction
What are the features of Shy-Drager Syndrome?
- autonomic failure
- parkinsonism
- pyramidal tract signs
- peripheral neuropathy
- cerebellar signs
What sort of drugs can cause drug-induced parkinsonism?
those that block dopamine signaling
What are three common causes of tremor?
- Parkinson’s disease
- essential tremor
- familial tremor
How do we treat tics?
by blocking DA
How do we generally treat hyper-kinetic movement disorders?
dopaminergic and cholinergic antagonists
What is tardive dyskinesia?
involuntary movements seen after the use of dopamine blocking agents
Tardive dyskinesia is most likely to involve which kinds of involuntary movement?
oral, lingual, or buccal chorea or dystonia
What is the most common CNS demyelinating disease?
multiple sclerosis
Which populations are most affected by MS?
women between 15 and 45 years of age
What course of MS is most common?
chronic relapsing-remitting
What CSF findings are consistent with MS?
elevated protein, lymphocytes, and oligoclonal bands
What MRI findings are consistent with MS?
periventricular T2-hyperintense lesions
Describe the pathogenesis of MS.
- CD4 cells react against self-myelin and secrete cytokines
- cytokines activate macrophages and other inflammatory mediators
Which genes are linked to multiple sclerosis?
- HLA-DR15 allele
- IL-2RA and IL-7RA
MS preferentially affects which white matter tracts?
- periventricular
- optic nerve and chiasm
- superficial pons and spinal cord
How does an acute MS lesion differ from an inactive one?
- there is no white mater in an inactive one
- macrophages are only part of acute lesions
How can we differentiate a stroke lesion from an MS lesion?
MS lesions will stain for neurofilament since the white mater has been destroyed by the axons have not
What is the Marburg MS variant?
an acute form of MS with an earlier age of onset and rapid course unresponsive to treatment
What is neuromyelitis optica?
an aggressive demyelinating disorder that preferentially affects the optic nerve and spinal cord
Creutzfeldt cells are a feature of which demyelinating disease?
neuromyelitis optica
Neuromyelitis optics is mediated by what pathogenesis?
antibodies directed against aquaporin-4
What is aquaporin-4?
an astrocytic water channel targeted by antibodies in neuromyelitis optica
What is acute disseminated encephalomyelitis?
an acute demyelinating disease that affects younger people in the setting of viral infection and characterized by lesions of the same age
How do we treat encephalomyelitis?
- IVIG
- plasmapheresis
- steroids
What are the symptoms of acute hemorrhagic encephalomyelitis?
- rapid progression
- fever
- neck stiffness
- seizures
- recent upper respiratory infection
What is the characteristic feature of central pontine myelinolysis?
a basis pontis lesions
Central pontine myelinosis most commonly follows what?
rapid sodium correction
How does central pontine myelinolysis present?
with rapid onset quadriplegia
What is progressive multifocal leukoencephalopathy?
a demyelinating disease due to viral reactivation of JC virus in an immunosuppressed or compromised host
JC virus infects what cell type?
oligodendrocytes
What are the symptoms of progressive multifocal leukoencephalopathy?
progressive visual, motor, dementia, and sensory disturbances
What are leukodystrophies?
inherited, progressive disorders of abnormal synthesis of myelin due to lysosomal or peroxisomal enzyme deficiencies
Most leukodystrophies have what mode of genetic transmission?
autosomal recessive
The characteristic feature of Alexander’s disease is what?
the presence of rosenthal fibers
What is Alexander’s disease?
an autosomal dominant leukodystrophy caused by a mutation in GFAP
Adrenoleukodystrophy has what mode of genetic transmission?
X-linked recessive
What causes adrenoleukodystrophy?
a mutation in ABCD1, a peroxisomal membrane transporter, that cause defects in VLCFA metabolism
What are the symptoms of adrenoleukodystrophy?
- progressive intellectual and behavior problems
- often preceded by adrenal dysfunction
