I was struck and aggrieved by Snakelegs passing yesterday--
I'll be doing 10 of each today to make up for yesterday.
we are on pharm (Neurology) and Endocrine diagnostics
let's start with Pharm
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45 year old woman evaluated because of painful contracted muscle. There is marked hypertonicity and hyperreflexia.
Baclofen and Tizanadine can be used for spasticity. Diazepam and dantrolene can also be used but are less effective
Vaclofen is an analog of GABA. the precise mechanism of relieving spasticity isn't known. Tizanadine is centrally acting alpha adrenergic agonist but does not lower pressure. Tinazadine inhibits pre-synaptic motor neurons.
The adverse effects of baclofen are dizziness, lighteheadedness, drowsiness, and weakness. Tinazidine causes dizziness, lighthededness and dry mouth
the rate of progression of MS can be slowed with beta interferon and copolymer I (glatirimer)
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A man is evaluated for a motor neuron dz in which he has fasciculations, muscle wasting, hyperreflexia, weakness, and upgoing toes, but cognition is intact
ALS is a combination of upper and lower motor neuron dysfunction. There is weakness, wasting, hyperreflexia, and fasciculations, but intact cognition, bowel, bladder, and sexual function. Als progression can be slowed down by riluole.
Riluzole inhibits glutamate release presynaptically b blocking sodium channels.
Riuluzole can case nausea, vomiting, weakness, and spasticity.
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Lamotrigine
used to treat the following conditions
Seizure disorder, generalized or partial
Peripheral neuropathy
mood disorders, such as bipolar disorder
Lamotrigine blocks the repetitive firing neurons by inactivating voltage dependent sodium channels. It may selectively influence neurons that synthesize glutmate and aspartate.
Lamotrgine can cayse very severe skin reactions, such as steven's Johnsons syndrome.
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A man comes to the ER because of sudden, severe, sharp, pain in his face while he was eating. It started near the side of the mouth and progressed to the ear. It resolved after a few seconds and then recurred. Currently his sensory exam is normal
caebamazepine is the best initial therapy for trigeminal neuralgia. it should be effective in more than 90% of cases. for those not responding. Phenytoin, baclofen or gabapentun maybe effective, surgery is performed if all meds fail
carbamazepine works in a similar fashion to phenytoin. It blocks the sodium channels at therapeutic concentrations. and inhibits high frequency repetitive firing in the neurons in culture. It also acts presynaptically to decrease synaptic transmission.
carbamazepine is also effective as first line medication for seizures and for peripheral neuropathy.
carbamazepine can cause diplopia, ataxia and hyponatremia, rarely it brings on aplastic anemia.
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Levetricetam
Levetricetam is effective in the treatment of seizures.
it modifies the synaptic release of glutmate and GABA throigh action on vesicular function
levitricetam has virtually no adverse effects and does not require drug monitoring. Some pts feel weak, sleepy and dizzy.
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pt with generalized recurrent seizure. there have been several short seizures over a few months that resolved spontaneously. There are no focal deficits, and all lab testing is normal.
these meds are acceptable as initial treatment for generalizes seizures.
it isn't clear which is superior
Valproate oxcarbzepine
Carbamazepine Phenobaribital
Phenytoin Topiramate
Lamotrigine
the adverse effects are
Valproate : weight gain, tremor, hepatotoxicity
oxcarbzepine: vertigo, Ataxia, hyponatremia
Carbamazepine : Hyponatremia, lethargy, neutropenia
Phenobaribital: sedation
Phenytoin : Gingival hyperplasia
Topiramate : Glaucoma
Lamotrigine : Stevens-Johnsons Syndrome
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62 year old man with diabetes comes to the office, he has been experiencing numbness and tingling in his feet for the last few months
either gabapentin or pregabalin is the best initial rx for peripheral neuropathy, especially from diabetes or HIV. Both of these meds can be used for seizure as well.
Both meds are analogs of GABA
the most common adverse effects are somnolence, dizziness, ataxia, headache and tremor.
peripheral neuropathy. Tricyclic antidepressants are also effective against neuropathy but can cause dry mouth, urinary retention and constipation because of their anti-cholinergic effect
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tricyclic antidepressants
Amitriptyline
Imipramine
Nortriptyline
Desipramine
Protriptyline
these are indicated for
Major depressive disorder
obsessive-compulsive disorder, phobia, anxiety
Attention deficit disorder
chronic pain syndrome, and peripheral neuropathies
nocturnal enuresis
migraine headache prophylaxis
TCA block the reuptake of amine neurotransmitters norepinephrine, and sertonin, leaving more of the neurotransmitters in the synapses.
TCA can cause many anticholinergic adverse effects, even at therapeutic doses such as dry mouth, urinary retention,, constipation, and orthostasis. At toxic level they can cause QT prolongation, arrhythmias and seizures.
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Catechol-Omethyltransferase (COMT)inhibitors:
Tolcapone
Entacapone
Al capone (kidddddddddddding) just checking to see if you are awake hehe
Tolcapone and entacapone are COMT inhibitors that extend the duration of levodopa replacement treatment for parkinsons' dz.
COMT inhibitors prevent the degradation of levodopa. Levodopa is normally inactivated by COMT. COMT inhibitors keep levodopa levels higher for longer. Entcapone andtolcapone can't be used alone, they must accompany Levodopa replacement.
this is for use in pts whose sx are progressing in spite increasing levodopa/carbidopa use.
Adverse: Orthostatic hypotension, dyskinesias, confusion and fatal hepatic failure.
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72 year old female comes to office with tremor difficult walking, she is able to take care of herself at home but foesn't like her increasing slowness. She has mild cogwheel rigidity ...
Mild parkinson's disease is best treated with monoamine oxidase inhibitors MAOis selegeline, rasagilene tremor only
<70 year olds, the anticholinergics trihexphenidyl and benztropine
>70 yrs of age Amantadine
Moderate disease
Dopamine agonist: prmipexole and ropinerole: these have less postent, less adverse effects than levodopa replacement.
Severe parkinsons
Kevodopa/carbidopa (sinemet); if levodopa replacement doesn't control SX, a catechol-o-methyltransferase (COMT) inhibitor is added. Severe is defines as the inability to take care of oneself or to be employed.
levodopa: carbidopa is added to bloc peripheral metabolism so more levdopa enters the brain
selegeline and rasagiline. These MAO inhibitors block dopamine metabolism.
Amantadine, increases dopamine release from the substantia nigra
the adverse effects of these treatments is as follows
levodopa: dyskinesia, the on'off phenomenon
selegiline/rasagiline confusion, also, these medications interact with tricyclic antidepressants and SSRI's
anticholinergics dry mouth, constipation and urinary retention.
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Diagnostics we'll finish endocrine and move on to GI
24 hour urine for metanephrine, vanillylmandelic acid (VMA) and catecholamines
24 hour urine collection for metanephrine, VMA, and the catecholamines is the best initial test for pheochromocytoma.
if VMA is elevated, then do a CT or an MRI of the adrenal glands as the next best thing for pheo
if ct/MRI find nothing, a metaiodobenzylguaniidine (MIBG) scintigrapy would be the next best option. This is a norepinephrine analog that concentrates in the adrenals an pheochromocytomas.
Pheochromocytomas is associated with MEN II and MEN III.
look for a case of episodic HTN, particularly with flushing and diarrhea.
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diagnostics GI
this is an abdominal x-ray
the abnormality is a small bowel obstruction, there are multiple air-fluid levels seen throughout the bowel
the most accurate test for small bowel obstruction is a combo od abdominal x-ray and CT scan. there is no blood test to diagnose obstruction, an there are no findings on endoscopy
pt usually has abdominal pain and distension, hypoactive or absent bowel sounds, possible electrolyte abnormalities such as potassium, ca2+, mg 2+ disorders, and failure to pass stoll or flatus. this is normal after abdominal surgery.
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colon cancer on barium enema

diverticulosis on barium enema
Barium Enema creates contrast picture to outline the lining of the rectum and colon. BE can be used in detecting colorectal cancer of the rectum and colon. BE can be used in detecting colorectal cancers, polyps, diverticulosis, and bowel obstruction and to evaluate the extent of inflammatory bowel disease.
BE is inferior to colonoscopy if you are suspecting a lesion in the colon. Colonoscopy is also superior to enema in that it can directly biopsy lesions or remove polyps.
Pts who may have peritonitis or bowel perforation must NOT undergo this test because the contrast material can leak into the peritoneum through the perforation. BE is also contraindicated in diverticulitis, in which it in creases the risk of perforation.
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this is an upper endoscopy showing a Barrett's esophagus
this occurs when the distal stratified squamous mucosa is replaced by metaplastic columnar epithelium containing goblet cells. The metaplasia results as a form of defense from the persistent acid refluc that cells get exposed to. Barrett's esophagus is not reversible but is preventable with PPIs
complications are ulcers, strictures and adenocarcinoma.
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this is a capsule with a camera and a small transmitter. once swallowed it begins transmitting images of the inside GI to a receiver worn by the patient. the doctor can review 6 hrs with of pictures for any abnormality.
this is the procedure of choice for suspected small bowel bleeding that was not detected on upper endoscopy.
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Amylase/Lipase levels
those are initial tests in the diagnosis of acute pancretitis. Lipase is more specific for the pancreas. Amylase can be elevated from damage to the salivary glands, esophagus or small bowel.
the patient presenting with the acute onset of severe epigastric pain radiating to the back. such pts may have a history of alchol abuse or gallstones.
most accurate test for acute pancreatitis is an abdominal CT scan is more accurate than Ranson's criteria. Pancreatic necrosis on a CT scan is extremely specific for severe pancreatitis and is the main indicator of the need for pancreatic biopsy.
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Antiendomysial
antigliadin antibodies
serum assay for endomysial and antigliadin antibodies are made against wheat or gluten (gliadin) antigens and the villous (endomysial) linning of the small intestine. They are used to confirm dx of celiac disease
celiac dz, presents with oily, greasy diarrhea, foul smelling stool witout evidence of cheonic pancreatitis.
they are the tests to answer to confirm gluten sensitive enteropathy after sudan black stain has confirmed a fat malabsorpption
small bowel biopsy is the most accurate test for celiac dz. it must be done to excluse lymphoma as well to dx the disease.
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Anti-mitochondrial antibodies (AMA)
serum antimitochondrial antibodies is the best test to make a specfic diagnosis of primary biliary cirrhosis (PBC)
look for a middle aged female presenting with itching (pruritus) and elevated alkaline phosphatase
with a normal bilirubin.
the most accurate test however is PBC is a liver biopsy.
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Anti-smooth muscle antibodies
serum assay detection of anti-smooth muscle antibodies (ASMA) is the best initial test for autoimmune hepatitis.. you know I personally always forget this one too so many anti this and anti that, so remember liver is smooth and you'll remember anti smooth lol
this is the test for a young woman with lover disease who doesn't drink or have inflammatory bowel disease or infectious hepatitis.
Auto-immune hepatitis is associated with antinuclear antibodies (ANA) and antibodies to liver/kidney microsome type 1 (anti-LKM1)
again liver biopsy is the most diagnostic .
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Barium esophagus
this is the best initial test for dysphagia. this is not in scenarios where there are clear signs of obstruction, for which upper endoscopy would be best initial. For achalasia barium esophagram shows a bird beak

at the esophageal junction. in diffuse esophageal spasm, barium esophagram shows a corkscrew

at the time of spasm.
Barium esophagram is the most accurate test for esophageal rings, webs, and diverticuli.
in achalasia, esophageal spasm, and nutcracker esophagus, the most accurate test is manometry. with cancer the most accurate is endoscoy for a biopsy.
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