| Parkinson disease medications | Neurovascular anatomy | NCS/EMG | Myopathy | Fascioscapulohumeral muscular dystrophy |
|---|---|---|---|---|
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90 min
Small intestine
What is the half-life of Levodopa-Carbidopa?
Where is being absorbed? |
M1- proximal
M2 -sylvian M3- distal
List 3 parts of MCA
|
Temp 32 C decreases latency and duration
How does temperature influence NCS?
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Disorders in which there is primary functional or structural impairment of skeletal muscle with no evidence of denervation
What are myopathies? (per Dr. Bassam)
|
Asymmetric weakness of face, shoulder, girdle, upper extremities
Asymmetric scapula winging is highly characteristic
What is the characteristic pattern of weakness in FSH?
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Nausea/vomiting, orthostatic hypotension, somnolence, dyskinesias, rarely, hallucinations
What are the most common side effects of Sinemet?
|
Cervical
Petrous Cavernous Supraclinoid
List four parts of ICA
|
>50% drop in amplitude from distal to proximal site
What is a conduction block?
|
Hereditary (muscular dystrophies, channelopathies, mitochondrial and metabolic myopathies) and acquired (inflammatory, drug-induced, toxic)
How would you classify myopathies? Give examples.
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Autosomal dominant for the majority of cases with variable penetrance in 30% of cases
What is a mode of inheritance for FSH?
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Inhaled Levodopa-Carbidopa formulation, used for off states, starts to work as soon as 10 min
What is Inbrijia? What is being used for and when it starts to work?
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Superior thyroid artery
Ascending pharyngeal artery Lingual artery Facial artery Occipital artery Posterior auricular artery Maxillary artery Superficial temporal artery
List the main branches of the external carotid artery
|
In axonal neuropathy decreased amplitude predominates (less axons->less voltage)
In demyelinating neuropathy increased latency and slowed velocity predominate (less myelin->slower signal travel and later arrival)
Characteristics of axonal vs. demyelinating neuropathy?
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Myalgias, cramps, stiffness, myoglobinuria, muscle hypertrophy
What are the "positive" clinical symptoms of myopathy?
|
- no respiratory or bulbar muscle involvement
- no associated cardiomyopathy - supraspinatus and infraspinatus muscles are spared - can cause restrictive lung disease, retinal vasculopathy, sensorineural hearing loss
What are the clinical key points which help to distinguish FSH from other types of muscular dystrophies?
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COMP inhibitor which allows for decreased L-dopa peripheral degradation, prolonging the half-life of L-dopa and increasing its plasma concentration
Metabolized by liver
What is Entacapone and how does it influence Levodopa-Carbidopa metabolism? How is being metabolized in the body?
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Lateral lenticulo-striate branches of MCA Medial striate (recurrent artery of Huebner) from ACA Posterior limb supplied by anterior choroidal artery
Describe the internal capsule vascular supply
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Insertional activity ( increased in neuropathic and myopathic processes, decreased in muscle necrosis)
Resting activity ( normal when absent) Voluntary activation
List 3 components of the EMG study and when do we see abnormalities in these components?
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Duchenne's, Becker's. Emery-Dreifuss
List X-linked hereditary myopathies
|
A combination of
-depression due to wasting of trapezius -bulge due to jutting of the superior angle of the scapula -second depression from the wasted trapezius -bony prominence d/t displacement of the acromioclavicular joint -wasting of the proximal part of the deltoid and biceps
What is a Poly-hill sign?
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Carbidopa-levodopa, Trihexyphenidyl, MAO inhibitors, Amantadine, dopamine agonists
List PD medications that can be used as an initial monotherapy for this condition
What factors have to be considered when we choose initial medication? |
head of the caudate nucleus, medial portion of globus pallidus, anterior limb of the internal capsule, anterior hypothalamus, nucleus accumbens, parts of the uncinate fasciculus, diagonal band of Broca, basal nucleus of Meynert
What is supplied by the recurrent artery of Huebner?
List all structures |
Increased amplitude
Increased duration Polyphasia Decreased recruitment (normal ratio 5:1)
Signs of chronic denervation on EMG
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Cholesterol-lowering agents, Chloroquine, Colchicine, Emetin, Labetalol, Cyclosporine, Vincristine, Isoretinoic acid, alcohol
List medications that can cause necrotizing myopathy
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FSHD1 (~95% of FSHD). A heterozygous pathogenic contraction of the D4Z4 repeat array in the chromosome 4q35
FSHD2 (~5% of FSHD). Hypomethylation of the D4Z4 repeat array as a result of a heterozygous SMCHD1 pathogenic variant or a heterozygous DNMT3B pathogenic variant
What genes are involved in FSH1 and FSH2?
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