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CNS degen diseases
PD, MS, ALS, tremors, etc.
| Question | Answer |
|---|---|
| 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
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