quiz #3- degenerative diseases & toxic/metabolic disorders
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What are different inherited degenerative disorders?
- charcot marie tooth disease
- friedreich's ataxia
- spinal muscular atrophy
- muscular dystrophy
- huntington's chorea
What nerve does charcot marie tooth deal cause atrophy of?
What are symptoms of charcot marie tooth?
- foot drop
- steppage gait
- gradual onset in adolescence
- bilarteral symptoms
How many people are affected and what genes are affected with charcot marie?
- autosomal dombinant- various genes
- 1/2500 people
What is fredreich's ataxia (basic)?
- degenerative changes in the spinal cord
- the cerebellum isn't getting infro from the spinocerebellar tracts so it can't do its job
How do you get fredreich's ataxia?
- autosomal recessive, gene 9
- both parents have to be carriers
When is the onset of fredreich's ataxia?
- 5-15 years
- w/c bound by 10 years
What are symptoms of fredreich's ataxia?
- ataxic gait
- sensory loss
What are different acquired degenerative disorders?
- amyotrophic lateral sclerosis
- multiple sclerosis
Why does syringomyelia usually occur?
a cyst in the spinal cord- usually over the cervical cord
What are symptoms of syringomyelia and when do they start?
- pain and weakness in arms
- onset is 25-40 yo
What are different metabolic disorders?
- cerebral annoxia
- hyper/hypo glycemia
- hyper/hypo natremia
- hyerp/hypo kalemia
- hyper/hypo thyroid
- renal insufficiency
- liver failure
- vitamin deficiency
What are the different levels of anoxia and their significance?
- 50 mmHg: confusion, decreased visual acuity, uncoordinated
- 40 mmHg: very ataxic
- 32 mmHg: loss of consciousness
- <32 mmHg: decorticate, decerebrate, death
- (normal is 90-100)
What is affected first in cerebral anoxia, gray or white matter?
- gray matter
- cell bodies are attacked first
What is the recovery for cerebral anoxia?
- 10% of victims recover completely
- partial recovery w/ myoclonus, intellectual defecits, seizures, motor/sensory defecits
- persistent vegetative state- no cortical activity
What is more serious in cerebral anoxia- rapid or chronic?
rapid is more serious
What are the levels for hyper/hypo glycemia
- hyperglycemia: >120
- hypoglycemia: <80
What are characteristics of hyperglycemia?
- occurs in diabetics- extra thirsty
- can produce encephalopathy/coma
- can be reversed
What are characteristics of hypoglycemia?
- usually due to insulin overdose
- progromal symptoms- makes you feel just a little off
- seizures, delerium, coma
- can be reversed if treated quickly
What are the levels for hyper/hypo natremia?
- hypernatremia: >145 mEq/L
- hyponatremia: <135 mEq/L
What are characteristics of hypernatremia?
- dehydration- can have electrolyte imbalance from vomitting
- produces myoclonus, chorea, stupor
- mortality high in elderly
What are characteristics of hyponatremia?
- causes- kidney failure, CHF, execessive water, medication
- produces fatigue, nausea, hypotension, coma
What is more severe acute or chronic hypo-hypernatremia?
What are the levels for hyper/hypo kalemia?
- hyperkalemia: > 4.8 mEq/L
- hypokalemia: < 3.6 mEq/L
What are causes and symptoms of hyperkalemia?
- causes: renal failure, crush injury, meds
- symptoms: cardiac arrhythmia
What are causes and symptoms of hypokalemia?
- causes: diuretics, renal failure, diarrhea/vomitting
- symptoms: weakness, constipation, cardiac arrhythmia, muscle cramps
What are characteristics of renal insufficiency (uremic encephalopathy, dialysis dementia, uremic neuropathy, intracranial bleed)?
- affects central and peripheral nervous system
- uremic encephalopathy: causes asterixis, hallucinations, reversible by dialysis
- dialysis dementia: can occur after 3-4 years of dialysis (dialysis will eventually cause brain damage)
- uremic neuropathy: peripheral axons die, restless leg syndrome, parasthesia
- intracranial bleed: 25% of patients on dialysis
Diabetes puts you at increased risk for what?
- CVA: because more likely to get atherosclerosis which increases risk of CVA
- peripheral neuropathy: 50% of pts have peripheral neuropathy after 25 years of DM
What does hyperthryoidism cause?
- proximal muscle weakness
- grave's disease
Who does hyperthyroidism effect most often?
middle age women
What is graves disease?
- auto-immune disease
- doesn't get signal you're producing too much from the pituitary gland
- is hyperthyroid, but when treating graves may cause hypothyroid
What are symptoms of hypothyroidism?
- nerve entrapment
- mild dementia
- increased risk of stroke
- decreased mental function
- 25% of eldelry have hypothyroid
What can liver failure cause (acute and chronic)?
- hepatic encephalopathy
- acute: asterixis, hyperreflexia, seizures, confusion
- chronic: mood disturbances, asterixis
What type of liver failure do alcoholics develop?
- chronic liver failure
- (mood disturbances, asterixis)
What vitamin complex causes significant neurological changes?
What are some different toxic agents causing disorders?
- heavy metals (mercury and lead)
What does methanol cause?
damage to optic nerve
What does ethanol cause (acute and chronic)?
- acute: >100 mg/dl, alcohol poisoning
- chronic: wernicke-krosakoff syndrome, polyneuropathy, withdrawal
What does mercury cause and where can it be found?
- chronic exposure leads to nervousness, personality changes, confusion
- IN FISH
What does lead exposure cause and where can it be found?
- decreased intellect, lethargy, hyperactivity, ab pain, irritability, insomnia
- paint/drinking water
What are different drugs that can cause problems and their associated problem?
- vincristine: tingling, sensory loss in fingers and feet
- lasix: cranial nerve VIII dysfunction
- flagyl: sensory loss
- antiviral: sensory loss (if have shingles or AIDS)
- cholesterol reducing: sensory loss
- anti-seizure: sensory loss
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