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CNS degen diseases

PD, MS, ALS, tremors, etc.

QuestionAnswer
what are movement disorders characterized by? excessive or reduced movement, disorganization of movement, abnormal tone
T/F movement disorders are usually characterized as weakness and spasticity FALSE
What structures are typically involved in movement disorders? basal nuclei, cerebellum, and supplementary motor cortex
What classification of tremor affects the body part at rest and supported against gravity? resting tremor
What classification of tremor affects the body part held in a fixed position against gravity or actively moved? action tremor
What are two types of kinetic tremors? terminal tremors (at end range reaching target) and task-specific (handwriting)
What type of tremor occurs in isolation, not associated with other neurological symptoms or disease? postural tremors
Which type of tremor is present in all normal persons at a very low amplitude and is frequently invisible? physiological tremor
What is an "enhanced physiological tremor" and what causes it? fatigue, anxiety, foods, and drugs and usually no treatment is required
What medication can be prescribed to a person with enhanced physiological tremor? propronolo
T/F medical conditions can result in a tremor TRUE
What is the most common movement disorder? Benign essential tremor
What percent of older persons have a benign essential tremor? 5%
Is an essential tremor usually bilateral or unilateral? bilateral
T/F An essential tremor is usually relieved by rest TRUE
What body parts are most liekly going to be affected by an essential tremor? head, tongue, voice, hands (fine movements)
What is the number one cause of parkinsonism syndrome? Parkinson's Disease
What are characteristic of parkinsonism syndrome? resting tremor, brandykinesia/akinesia, rigidity, impaired postural reflexes
What is histologically lost in a pt with PD? loss of pigmented neurons in the substantia nigra that produces dopamine
What are neuronal cytoplasmic inclusions? Lewy bodies seen in PD pt's cellular cytoplasms
T/F the most common causes of PD are familial and genetic FALSE most are idiopathic and sporadic
What is the clinical presentation of PD? flexed forward posture, short shuffling steps, masked expression, drool, rigid spine and trunk, slight hip and knee and elbow and wrist flexion, and reduced arm swing, resting tremor asymmetrical (spares the head), cogwheel rigidity
What structures does the lack of dopamine affect? decrease of dopamine causes an inc of activity on the GP int and ext leading to greater inhibition of the thalamus leading to less excitiation of the motor cortex leading to less movement
What is the typical onset age for PD? 50-60s
T/F a person with PD usually presents with a asymmetrical resting tremor that involves the head FALSE spares the head
What is generally the first symptom in PD? tremor
Desribe the tremor in a PD patient. unilateral, distal limb, pill-rolling, pronation-supination of forearm, resting tremor and may be associated with an action tremor
What is the pharmacological management for PD? attempt to replace the dopamine in the system, but can lead to excessive movement, dopamine agonist, levodopa, amantadine, etc.
T/F PD medication can slow the progression of the disease FALSE- only manages sxs
What are possible surgical treatments to manage PD? DBS, stereotactic pallidotomy, removal the GP internus
T/F DBS works best is PD pts who are responsive to LDopa TRUE
What structure does DBS target? STN
What disease is symmetric in onset of parkinsonism, absence of resting tremor, early autonomic dysfunction, prominent dystonia, significant early cognitive impairment, and prominent early falls Secondary Parkinson's Syndrome
What disease is asymmetric, resting tremor, lateral cognitive impairment, and generally no early falls? Parkinson's Disease
What disease has freezing gait, ataxia, dysarthria, dysphagia (choking), occular involvement, blepherospasm, apathy, cognitive impairments? Progressive supranuclear palsy
What disease has onset 5th - 7th decades, autonomic dysfuntion, varying degrees of involvement in the barinstem, cerebellum, spinal cord, and peripheral nerves? Diffiuclty swallowing, choking, and falling. Very poor prognosis? Multiple System Atrophy
What disease has neuronal loss with gross atrophy concentrted in the pons, olives, and cerebellum. Results in ataxia, rigidity, spasticity, and oculomotor disturbances? olivoponotcerebellar atrophy
What disease has progressive hepatolenticular degeneration of basal ganglia realted to depositions of copper, cysts of necrosis, and enlargement of the lateral ventricles? Wilson's Disease
Restless leg syndrome is related to what? reduced iron store in the basal ganglia
T/F restless leg syndrome is related to dopominergic neurons FALSE
T/F Shakes in older people are due to PD FALSE
T/F PD can occur at any age but generally occurs in an older population TRUE
What is the most dibilitating part of PD? bradykinesia, inhibition of postural protective reactions
Who is more likely to be affected by MS? women > men, peak 25-30 (15-40), white, genetic
What are the most common initial symptoms of MS? focal sensory loss, optic neuritis, focal weakness, paresthesias
T/F most pts with MS will gradually accumulate permanent neurological deficits TRUE
What are the 4 types of MS? Which is most common? relapsing-remitting (most common), secondary, primary, progressive relapsing
What si required to diagnose MS? 2 or more lesions afecting the CNS white matter, two or more separate episodes occuring at least one month apart or gradual progression of at least 6 months, MRI montor progression
What is difference between acute and chronic MS? Acute: inflammation present, Chronic: inflammation gone but scarring present
T/F MS results in poor transmission due to the destruction of myelin. TRUE
What is the geographic thought behind MS? the first 15 yrs of life in northern places are related to a higher incidence of MS (distances north and south of the equator)
What is the current thinking behind the cause of MS? early childhood exposure of some virus and person with the appropraite genetic background will later in life be exposed to a triggering agent
T/F there is no cure for MS TRUE, the drugs aim at reducing the frequency of attacks thus reducing the permanent changes
T/F some medication for MS are oral TRUE - 3 oral, the rest injectable
T/F corticosteroids are beneficial for chronic use FALSE acute exacerbations
Is ALS a degeneration of UMN and/or LMN? both. LMN- Muscle atrophy, muscle cramps, insidious asymmetrical weakness (distal --> proximal) UMN- Spasticity, speech & swallowing issues, fasiculations
T/F ALS affects the motor tracts in the SC, brainstem, and cotex TRUE
What is the most common form of adult onset progressive motor neuron disease ALS
When is the onset of ALS typcially 50s
T/F ALS can have a genetic component TRUE, FALS 5-10% cases
What system is preserved in ALS? DCML
When does dementia typically set in in ALS patients? later stages
What levels are histologically elevated in ALS? glutamate
How is ALS diagnosed? clinical signs and EMG
What is generaly the timeframe between bulbar, UE, and LE dx? UE 15 mo, bulbar 17 mo, and LE 21 mo
What is frequently the first symptom of ALS? insidious, asymmetric weakness of distal aspect of one limb
T/F Gognitive impairments are usually more significant with initial UE onset FALSE, bulbar
What body structure is usually preserved in ALS? the eyes
What usually causes death in ALS? And when? respiratory failure and inability to eat, 2-5 yrs
T/F medication only attemps to slow the progression of ALS, there is no cure TRUE
T/F Riluzole is the only FDA approved medicaiton for ALS TRUE
Which disease is a progressive, hereditary disorder of the CNS that causes abnormal movements, personailty disturbances, and dementia? Huntington's Disease
Which disease can be identified through gene therapy? Huntington's Disease
What occurs in the brain in a person with HD? striatum atrophy, decrease in brain volume, cortical atrophy, disruption of NT
What movement disorders and other clinical presentations present in HD? chorea, abnormal eye movements, dysarthria, dysphagia, sleep disorders, UI, speaking
What is the prognosis of HD? slow advancing death usually 15-20 yrs after onset, increasing disability, inc suicide incidence
What are the incidence of benign essential tremors? Prevalance increases with age, females>males --> often affects hands and voice
How is benign essential differentially dx? Usually is bilateral tremor, affects fine motor skills, usually affects tongue/voice, BY RULING EVERYTHING ELSE OUT!
What are the characteristics of "parkinsonism disorder"? Resting tremor, bradykinesia, rigidity, impaired postural reflexes
How many characterisitics of parkisionism disorder does the pt need to be classified with parkinsonism syndrome? 2 out of 4
What is the # 1 cause of parkisonism syndrome? Parkinson's Disease
In Parkinson's disease what NT is affected & is this NT increased of decreased? Dopamine is decreased
What causes the decrease in dopamine in the basal ganglia? There is a decrease of dopamine producing cells in the substantia nigra.
Briefly explain the pathophysiological process of PD. Decrease in dopamine (normally inhibits GP int) --> Increased in GP int activity --> Increased INHIBITION of thalamus --> Decreased motor output --> Decreased movement
What type of rigidity is common with PD? cogwheel rigidity
What are some early S&S of PD? shuffling gait, masked expression, difficulty getting out of bed, micrographia, slurred speech, focal dystonias, resting tremor
T or F:  PD pts can have both a resting tremor and action tremor True but they are caused by different things, they are two separate issues
Created by: Leeli.PT