Busy. Please wait.
or

show password
Forgot Password?

Don't have an account?  Sign up 
or

Username is available taken
show password

why


Make sure to remember your password. If you forget it there is no way for StudyStack to send you a reset link. You would need to create a new account.
We do not share your email address with others. It is only used to allow you to reset your password. For details read our Privacy Policy and Terms of Service.


Already a StudyStack user? Log In

Reset Password
Enter the associated with your account, and we'll email you a link to reset your password.
Don't know
Know
remaining cards
Save
0:01
To flip the current card, click it or press the Spacebar key.  To move the current card to one of the three colored boxes, click on the box.  You may also press the UP ARROW key to move the card to the "Know" box, the DOWN ARROW key to move the card to the "Don't know" box, or the RIGHT ARROW key to move the card to the Remaining box.  You may also click on the card displayed in any of the three boxes to bring that card back to the center.

Pass complete!

"Know" box contains:
Time elapsed:
Retries:
restart all cards
share
Embed Code - If you would like this activity on your web page, copy the script below and paste it into your web page.

  Normal Size     Small Size show me how

USAT Neurology

Dr.Robinson, January 2015

QuestionAnswer
Three main stabilizing ligaments of the spine Ligamentum Flavum, anterior longitudinal ligament, posterior longitudinal ligament
First hit which ligament in the LP? Ligamentum Flavum
Where do you perform LP? L3-L5
Angle needle slightly _______ for LP Cephalad
While performing a Lumbar Puncture, have the patient Straighten their legs when measuring what? Pressure (performed lying down)
Increased pressure on Lumbar puncture seen in what condition especially? CNS tumors (the pressure is normally 1/10th the blood pressure (8-15 mmHG
Low glucose CSF Bacterial or fungal Infections and malignancy (Normal level is 60% of the blood glucose, about 48mg/dL)
Elevated proteins in CSF Infections or Malignancy (normal is 1% of blood levels up to 40mg/dL)
Presence of RBC in CSF Hemorrhagic stroke, trauma (nml is zero)
Presence of WBC in CSF Infection, autoimmune disease (Normal is zero or 1-2 per HPF)
Elevated lactate in CSF stroke, head trauma with hypo perfusion
Elevated LDH in CSF INTRACRANIAL hemorrhage, meningitis with cellular damage.
Positive immune studies in CSF antibodies in autoimmune disease such as Ankylosing spondylitis, MS, or Guillain-Barre syndrome.
Most significant complication of LP Headache-treat with blood patch (damage to cauda equine possible as well). Can present up to 5 DAYS LATER
Nml duration of EEG 20-30 min
The electrodes of EEG detect which neurons? Larger Pyramidal neurons close to the surface of the brain
EEG valuable for evaluating seizures, transient global amnesia
relaxation as in watching TV Alpha
Problem solving, active thinking Beta
Fast intense, conscious awareness. When we correlate sensory stimuli, thoughtful analysis and memory. Gamma
Sleeping waves Delta
Day dreaming, meditating Theta
EEG record which neurons? Pyramidal
Type of neurons found in active thinking including the cerebral cortex, hippocampus, amygdala Pyramidal neurons
Primary excitation units of the mammalian prefrontal cortex and corticospinal tract that innervates the muscle. Pyramidal Neurons
1 pyramidal cell receives______excitatory inputs and ________ inhibitory inputs 30, 0000 and 1700
Neurotransmitter of Pyramidal neurons for excitatory stimuli, Neurotransmitter for Inhibitory stimuli Glutamate for excitatory and GABA for inhibitory
How many neurons do we have? 100 Billion
EEG may be flat but person still alive. How? EEG do not capture BRAINSTEM activity. Also in hypnotic, sedative, hypothermia
Why use sterile wet brain EEG apparatus? More precise tracing
Electromyography evaluate: PERIPHERAL nerve and muscular disorders. Useful for entrapment syndromes such as carpal tunnel
Non con CT scan for hemmorhagic vs embolic stroke
85 % of strokes are this kind arterial thrombosis (1st three hours the CT looks Nml)
Which studies are more accurate for stenosis than Ultrasound? CTA and MRA
Scan that is better parenchymal disease of the lung, bone lesions, fractures CT
Better for mediastinal and hilar disease of the chest and musculoskeletal disorders MRI
Better for bony detail CT
Better for soft tissues MRI
No MRI for patients with metal implants, metal based tattoos, pacemaker, piercings
MRI where WATER appears DARKER and FAT brighter. Used to obtain fine contrast in tissues such as Rotator Cuff tear T 1 MRI
MRI where Fat shows darker and water lighter. Highlighting EDEMA and inflammation (INFLAMMATION in frozen shoulder). MS or ALS. T2 MRI
Which type of MRI is used for angiograms (lights up the blood) T1 OR T2 T2 (LESS COMMON) stick with #1 (T1 is used more often)
Two drugs that cause neuropathy isoniazid and streptomycin
Tendency of water to diffuse along the line of a nerve axon Anisotropy
Diffusion MRI shows what and is good in working up which disease? Brain maps of fiber directions to see where certain connections of the brain are disrupted or demyelinated. Good in MS workup
most popular contrast gadolinium-highlights inflammation or tumor growth, neovascularization
SE of gadolinium renal toxicity
PET Scan-must administer RADIOISOTOPE 1st which emits positrons which collide with electrons and cause the emission of gamma rays. it shows the greatest brain activity in a given patient at a certain point in time.
Radioisotope given for which study? PET scan
PET in Alzheimers disease. Pt given which radioactive dye to image Beta Amyloid Plaques? Florbetapir
SPECT SCAN (only in teaching hospitals) similar to CT and give off single gamma ray photon (PET give off double gamma ray) Show precise: Anatomy
SPECT Scan good for Epilepsy, study blood flow in diseased conditions.
Good for solid tissue PET scan
show where tissues are taking up nutrients SPECT (can also create 3-D images of the brain based on blood flow)
Measures brain activity by detecting associated changes in blood flow. fMRI
Uses blood oxygen level dependent contrast technique (bold) contrast technique fMRI
Used in lie detection fMRI
We need this part of the brain more when we lie prefrontal cortex
When you recognize someone which part of the brain lights up? visual cortex
shows how deeply a brain-injured person is in a coma, can help determine if person is brain dead or not fMRI
takes advantage of scattering properties of neurons themselves. Uses infrared lights EROS (Event related optical signal)
measurement of blood to active areas of the brain, accurate to 1-2 seconds fMRI
limited to evaluating only a few centimeters deep, limits imagining to the cerebral cortex EROS-provides a direct measure of cellular activity
Agiography need contrast onto artery then takes several x-rays
Uses standard x-rays Angiography
Gold standard of dx of vascular disease angiography
Cerebral Angiography good for the diagnosis of what? Cerebral Vascular Disease
Catheter through the femoral artery into aortic arch and directly into the carotid artery-contrast then injected. cerebral Angiography
Vertebral Artery rarely get atherosclerosis, more commonly the carotids and cerebral vessels.
Hemorrhagic Stroke make up what percent of stroke? 15 % (think berry aneurysms)
HTN, vasculitis, bleeding d/o. Usually in older people. What type of stroke is this? Intracerebral stroke
young people, congen berry aneurysms, congenital AVM. Sub arachnoid
Trauma (not stroke) Kind of brain bleed of people who fall often in the bathroom, slow VENOUS bleed. Show signs 2-3 days later Subdural-crescent shaped lesion on CT. Bridging veins between dura and arachnoid. Think elderly age related cerebral atrophy pulls on veins
Young people, severe trauma. Sharp blow to head over middle meningeal artery. Stunned then Lucid then N/V and Dilated PUPIL on the side of injury Epidural
Reduces the metabolic rate of the brain tissue and cerebral blood flow. Decreases swelling avoiding brain damage Barbiturate induced coma with Propofol
confusion, slurred speech, dysarthria, aphasia, inability to speak, dizzy stroke is on the dominant side
The dominant side of the brain for most people is which side? Left (even if you are left handed)
Catalyzes plasminogen to plasmin TPA (Alteplase). Lyses fibrin
TPA needs to be administered within 3 hours
Treating frost bite, MI, PE, DVT TPA, only used for significant deficit even in stroke and not used if patient is improving.
Ischemic stroke control Hypertension with IV Sodium Nitroprusside
Blockage of the vertebral or basilar artery-ischemic infarct of the Brain Stem locked in syndrome
total locked in syndrome eyes are also paralyzed
6 organs that we harvest cornea, lungs, heart, liver, pancreas, kidneys
simian posture, degenerative disorder of the midbrain parkinsons
resting tremor, bradykinesia, slow gate, frozen facial expression parkinsons
Parkinson dz involves extra pyramidal syndrome (substantia nigra develops Lewy bodies) neuro transmitter that is involved is: dopamine depletion
What part of the brain degenerates with parkinson? substantia nigra
Fewer nerve connections at which part of the brain in park? basal ganglia-less dopamine in parkinsons
Lewy bodies with large eccentric nucleus found in which part of the brain in parkinson? Substantia Nigra
Micrographia seen in parkinsons
Extrapyramidal tracts coordinate body mvmt, balance, posture Reticulospinal tracts
Control body position and balance readjustments with motion Vestibulospinal tracts
Coordination of head and eye movement, re-position of the head when movement occurs Tectospinal tracts
Fine tuning of smooth large muscle movement and fine control of small muscle movement Rubrospinal Tracts
differentiate between early and late signs of parkinson Early:resting tremor, difficult buttoning, micrographia. Later:can't get out of car or do ADL's, parkinson dementia (Lewy body form)
Parkinsons dementia occurs from Lewy bodies T/F? T
No dx markers Parkinsons
Pesticide that interferes with the ETC and causes Park sx Rotenone
What diabetes drug stabilizes the mitochondria? Actos (pioglitazone)-activates part of the PGC-1 alpha pathway, stabilizing the mitochondria. Maybe CoQ10 (not a diabetic drug though)
Ach competes with ____ what in the basal ganglia DA
Most common Anticholinergic for parkinson Biperiden (Akineton)
Anticholinergic and dopaminergic commonly with dopamine Amantadine HCL (Symmetrel)
Good in drug for Parkinson tremors induced by Phenothiazine drugs Amantadine HCL (Symmetrel) an anticholinergic and dopaminergic commonly used with dopamine.
Drugs that decrease the breakdown of DA and help parkinsons patients MOA's Nardil and Marplan
Complication seen in over half of the patients given levodopa/carbidopa for five years Tardive Dyskinesia, vivid nightmares, auditory and visual hallucinations.
What did we add to levodopamine to prevent it from being decarboxylated too soon? Carboxylase inhibitors
Effective in small doses and inhibits the enzyme aromatic-L-amino-acid decarboxylase that converts L-dopa to dopamine in the peripheral tissues, not in the brain Carbidopa, effective in small doses
Standard therapy for Parkinson's is what drug? Comes in various combinations of levodopa and carbidopa. Sinemet
Must first cross the blood-brain barrier and then be decarboxylated to dopamine after it enters the brain. Levodopa**Dopamine which is the active compound CAN NOT enter the blood brain barrier.
DA receptor AGONIST that can cause valvular heart disease (slide 203) Pergolide, (Permax)
DA receptor AGONIST may enhance mitochondria function Pramipexole (Mirapex)
DA receptor AGONIST that can cause vomiting, given with domeperidone Apomorphine (Apokyn)
DA receptor AGONIST works best for parkinsons for the first 5 years (Still the STANDARD therapy) Sinemet (Carbidopa/levodopa)
Blocks re uptake of N EPI and EPI Cocaine
Too much acetylcholine in Basal Ganglia and it COMPETES with dopamine in: Parkinsons Disease
What neurotransmitter overstimulates the muscles causing tremor and rigidity in Parkinsons disease? Ach
Most popular Anticholinergic agent to treat Parkinson Biperiden (Akineton)
Drug used with Dopamine which is an Anticholinergic and Dopaminergic-commonly used with dopamine Amantadine HCL (Symmetrel)
Many Parkinsons patients are started with Sinemet 10-100 and Symmetrel. What age group tolerates this regimen the best? Tolerated best in patients under the age of 65
Most popular ANTICHOLINERGIC agent used for Parkinsons Biperiden
Two drugs that can cause NEUROPATHY Streptomycin and Isoniazid
Study that has dominated brain mapping research because it does not require people to ingest or inject or be exposed to radioactive radiation markers. It measures the changes in magnetization between oxygen rich and oxygen poor blood as its basic measure. fMRI-VERY SENSITIVE
Language RECEPTION area of the brain Wernicke's
Speech Production area of the brain Broca's speech production area
Type of stroke associated with small punched out lesion which is isolated Lacunar infarct
Multiple small infarcts may lead to what dementia
Master regulator gene involved with maintenance and repair of mitochondria PGC-1alpha
Average patient has lost what percent of their dopamine producing neurons by the time of diagnosis? 70%
Early warning signs of Parkinson's tremor, micrographia, loss of smell (bannanas, licorice, pickles), trouble sleeping, stiffness, constipation, soft voice, hunching, dizzy
Pallidotomy is neurosurgery performed for Parkinson's disease and is helpful in 30% of cases. Which part of the brain is severed? Basal ganglia outflow in the globus pallidus
Surgical implantation of normal _____________ from a cadaver or fetal tissue in the treatment of Parkinson's. Basal Ganglia
Surgically implanted, battery-operatered neurostimulator blocking abnormal nerve signals that cause tremor and symptoms. Deep brain stimulation
Targeted areas of the brain in Deep Brain Stimulation (2 areas) which when stimulated, decrease effects of Parkinson's disease. 1. Globus Pallidus 2. Subthalamic nucleus
Most common cause of Dementia Alzheimer's
Dementia that is closely related to PARKINSON'S Disease, but WITHOUT the symptoms of Parkinson's disease. Lewi Body Dementia
Drugs causing dementia in elderly Anticholinergic, Digoxin, H2 receptor blockers, beta blockers, steroids
Dementia caused by mad cow disease (Prions) Creutzfeldt-jacob disease
heavy metals causing dementia Mercury, arsenic, Lead
On Histology you will see intracellular protein debris plaques. LARGE TEAR DROP shaped cells with eccentric nucleii. Lewy Body
Degeneration of the Cerebral Cortex, Hippocampus, and entorhinal cortex Alzheimers disease
Located in MEDIAL TEMPORAL LOBE and functions as a 'Hub" in a widespread network for memory and cerebral navigation. Entorhinal cortex
MAIN interface beween the HIPPOCAMPUS and the CORTEX Entorhinal cortex
Which "system" in the brain plays important role in autobiographical/declarative memories, memories of the past, spatial memories, and orientation? Entorhinal cortex-hippocampus system
Vital "system" in the brain for conscious processing in the ongoing narrative of our life. Entorhinal cortex-hippocampus system
Functions primarily in MEMORY FORMATION/CONSOLIDATION and MEMORY OPTIMIZATION in sleep Entorhinal cortex
Accumulations of plaques and tangles seen in which disease? Alzheimer's
General term for fragments or waste products the body normally produces. Amyloid
Protein fragment from an AMYLOID PRECURSOR PROTEIN seen in Alzheimer's Beta Amyloid-in a normal brain they are broken down, in ALZ they accumulate and form insoluble plaques
Neurofibrillary tangles are insoluble twisted fibers found in the brain cells in which disease? Alzheimers
Neurofibrillary tangles of Alzheimers disease are primarily what type of proteins? Tau protein-normally forms part of microtubule
Cellular structure that transports nutrients from one part of a nerve cell to another and are important in mitosis. Microtubule
What specifically happens to the tau protein in Alzheimers? It becomes hyper-phosphorylated and the microtubules collapse in twisted masses called tangles.
Neronal tangles and plaques think Alzheimers
Plaques in ALZ made of Beta Amyloid
Neurofibrillary tangles in Alzheimers are a result of what Tau Protein T=T (tangles=tau)
On autopsy which two areas of the brain do you see severe shrinkage in Alzheimers? Shrinkage of cerebral cortex and HIPPOCAMPUS. ENLARGEMENT of LATERAL VENTRICLES
An example of a gene containing 2 SNPs (Slide 244) in Alzheimer's disease which result in 3 possible alleles for this gene ApoE is the gene, three possible alleles are E2, E3, E4. Each allele differs by 1 DNA base-protein product of each gene differs by 1 AA
One of two or more forms of the gene is the definition for what? Allele
Each person inherits a maternal copy and paternal copy of ApoE. If you inherit at least one _____allele your chances of getting Alzheimer's increase greatly. E4 (4 is higher than 2 so you have a higher risk with 4)
Inheriting the _____ or ______ allele indicates the person is less likely to develop the disease. E2, E3
Can have 2 E4 alleles and NEVER develop Alzheimers T/F? T
E4 also has a relationship to what and can be measured/monitored with CRP, ESR which are usually elevated in Alzheimers disease. Inflammation
One causation theory of alzheimers is that it due to the REDUCED synthesis of the neurotransmiter _________ Ach
Amyloid hypothesis postulated that Alz is caused by beta-amyloid deposits. The gene for amyloid precursor protein is gene #______ 21
Trisomy 21 patients exhibit Alzheimers by the age of 40 why? they have an extra copy for the amyloid precursor protein
Normal tau does what for microtubules? stabilize the microtubules in cells
In the tau hypothesis for ALZ the hyperphosphorylated tau begins to PAIR with other threads of tau forming what? neurofibrillary tangles inside nerve cell bodies
When the neurofibrillary bodies form in the nerve cell bodies, the microtubules disintegrate collapsing__________ the neuron's transport system. Communication between neurons is lost and cell death results.
Which virus has been thought to make susceptible versions of Apo E gene causing Alzheimers HSV type 1
the 4 D's for the work-up of dementia Delirium, disorientation, depression, dementia
Perfect score for MMSE 30
MMSE 5 PARTS Language 9pt, Orientation time and place 10pt, Recall 3pt, Immediate recall 3 pts, Attention and calculation 5 pts
What is the standard time you put on the clock face test? 11:10
Sulci and ______ become more accentuated in Alzheimers gyri
fMRI imaging show decreased ________ and _________ activity in Alzheimer's disease when encoding new info hippocampal and parahippocampal activity
Amyloid imaging using the Pittsburgh Imaging compound__________ B ligand (florbetapir)-PET scan shows up take of Florbetapir in the brains of those with Alz disease
Best scan for Alz Disease with the Pittsburgh imaging material PET Scans
Amyloid plaques are seen extracellularly or intracellularly in autopsies of those with Alzheimer's disease? Extracellularly
Which dementia may improve with time? Multi-infarct
Alz drug that inhibits acetylcholinesterase to INCREASE Ach and IMPROVE neural transmission. Tacrine
Which receptors deal with glutamate and are OVERSTIMULATED in Alzheimer's disease? NMDA
Four cholinesterase inhibitors for Alzheimer's disease Tacrine, ARICEPT, Exelon and Remenyl
One anti NMDA drug for Alzheimers disease Memantine (Namenda)
Non of the Alz disease drugs should be taken if the patient also suffers from: Ulcers, COPD, Asthma
What initial drug is tried in Alz disease? Aricept (Namenda later)
Antipsychotics such as _______ can be used to treat symptoms of Alz disease with some good results but are also dangerous causing strokes and Park-like symptoms Resperidone
New research shows in Alzheimers it MIGHT be a problem of ______beta amyloid, not too much production of beta amyloid Can't clear beta amyloid
stop beta amyloid build-up, enhance mitochondrial function, stabilize cholesterol metabolism and reduce autoimmune response are research topics in: Alzheimers disease
Autoimmune d/o affecting neuromuscular junction Myasthenia Gravis
Antibodies attack Ach receptors of motor end plate on muscles so that nerve conduction to muscle is interrupted. What kind of neuron dysfunction is seen? LMN, progressive weakness seen in Myasthenia Gravis
MG auto antibodies most commonly act against ______________ acetylcholine receptors Nicotinic
Some forms of the antibody in MG impair the ability of _____ to bind to receptors. ach
MG antibodies different MOA destroy receptors by complement or by inducing muscle cell to eliminate the receptor via endocytosis.
The auto antibodies are elaborated by plasma cells in MG which are triggered by the _______ cell function T4
MG is more common in which gender? female
Which gland is 75% of the time a culprit in MG? Thymus (10% have a thymic tumor but usually benign)
Eye symptoms of MG: drooping eyelids aka Blepharoptosis (usually affects 1 eye). Weak eye muscles with double vision and strabismus.
15 % of patients with MG have ONLY what kind of symptoms? eye
85% of patients with MG develop what? Muscle weakness throughout the body
other symptoms of MG speech/swallowing, easy fatigue of muscles, WEAK HAND GRIP strong at 1st but then becomes weak after 20-30 seconds
Are there any changes in sensation with MG? NO
Tensilon test for MG-inject an anti-cholinesterase to INCREASE Ach and improve strength temporarily if the person has MG. What is injected? Edrophonium
In MG conduction blocks are seen on Electromyography
Thymus tumor must always by looked for on what dx test in the workup for MG? MRI
Most common drug used in MG treatment Pyridostigmine (Mestinon)-an anticholinesterase
Treatments for MG Steroids, Methotrexate, Imuran, CellCept, Anti TNF (Enbrel)
For severe MG crisis what treatment can be given? Plasmaphoresis. Thymectomy if tumor
Inflammatory disease affects nerves in the CNS, most likely autoimmune. MS-DOES NOT AFFECT PERIPHERAL NERVES
#1 MOST COMMON neurological disease in the USA Parkinsons#1
#2 Most COMMON neurological disease in the USA MS
MS affects women ____x more than men 3
Disease that Mostly occurs in developed, non tropical countries MS-fewer cases near the equator
Lifespan of MS patient shortened by ___ years 6
Most common symptom of MS Sensory loss in upper or lower extremity
Second most common symptoms of MS Disturbance of gait
MS vision loss in one or both eyes. Also eyes don't tract together. one eye
What abnormal reflexes do you see in MS? Hyper-reflexia positive Babinski, Spasticity
What kind on tremor is seen with MS? Intention (resting in Parkinsons)
T/F Memory loss and Trigeminal neuralgia seen in MS T
Scan used to Dx MS MRI-95% positive, gadolinium injected and bright areas of inflammation seen.
LP in MS postive for immunoglobulins
On MRI when you see "Dawson Fingers" this is pathoneumonic for what disease? MS
Antispasmotic agent for MS Baclofen (Liofen)-GABA agonist
Surgical rhizotomy for sever pain in MS but results in: Numbness (laser posterior nerve roots)
Anti cholinesterase, Amantadine, is helpful in MS for fatigue but what unwanted side effect does it have? Weight gain
Injections given 3x week up to year to modify course of MS Beta Interferons (Avonex and Betaseron)
Other agents used for MS Methotrexate (immune suppression) and methylprednisolone for exacerbations.
New Drug (monoclonal antibody therapy) for MS given once a month causing 66% fewer relapses: Tysabri (Natalizumab)
Blocks IL 2 (Monoclonal antibody drug) causing T cell levels to go down (t cells are the ones that attack the nerve cells) and NK went up showing improvement in patients. Zenapax (daclizumab)
Where in the spinal cord does ALS affect? Lateral corticospinal tracts
ALS is a rapidly progressing disease with average death ____ years from time of diagnosis 3
Degeneration of ______ and ______ in ALS UMN and LMN
affects ONLY the muscles of the face, jaw and throat. CN that originate off the bulbar area (medulla, pons, and cerebellum) AND emotional outbursts Pseudobulbar palsy
Neuro muscular disease that affects the Glossopharyngeal, Vagus, and Hypoglossal nerves. Trouble talking, drooling, swallowing. RAPID PROGRESSION to Death in 1-3 years. Patients present at ages 50-80 y/o Progressive Bulbar Palsy
Neuro muscular disease, SLOW progression can live up to 25 yrs after diagnosis. LMN involvement so NO spasticity. Progressive Muscular Atrophy
Neuro muscular disease progresses over 2-3 decades. +Spasticity. Affects VOLUNTARY muscles. WEAKNESS and GAIT issues. PRIMARY LATERAL SCLEROSIS
Motor neuron disease with UMN and LMN degeneration ALS
2 Motor Neuron diseases with ONLY UMN involvement Primary lateral sclerosis AND Pseudobulbar palsy
2 Motor Neuron diseases with ONLY LMN involvement Progressive Muscular Atrophy and Progressive BULBAR PALSY
Most common of the 5 motor neuron diseases (ALS, PLS, PMA, PBP, or pseudo bulbar palsy) ALS
Atrophy and spasticity at the same time. Bowel and urinary sphincters spared until very late in the disease. Issues with talking/eating seen early on. ALS
In _____ sensory and autonomic nerves are spared. Still hear/see/taste/smell and cognition is pretty well preserved. ALS
In ALS 5-10% cases are autosomal dominant and many have defective _____ which normally would deactivate free radicals that build up in the nervous system. Super-oxide dysmutase 1 (SOD1)
One theory of progressive spread of ALS is misfolded proteins and that a defective SOD1 has ________ properties which cause template directed mis-folding. Prion
Coercion of one protein by another protein to change shape and accumulate in large complexes in a similar way to the process underlying prion disease. Template-directed mis-folding
Where do you first experience weakness in ALS? hands 1st, then feet, then loss of grip, tripping and falling.
In ALS weakness presents _______ and progresses ______ distally, progresses up the arm or leg
In ALS there is NO ________ loss and NO pain sensory loss (NO SENSORY LOSS IN ALS)
T/F ALS can affect one side of the body more than the other True
Serum creatinine kinase is usually what in ALS Normal or slightly elevated
Nerve muscle studies in ALS show what? partial denervation of muscle groups, reduced # of functioning muscle groups, 3 out of 4 extremities at least must be involved to confirm DX
Nerve conduction velocity is _______ in ALS Normal
Only drug that shows some improvement for ALS patients. Increase survival approx 3-5 months by moderating excess Ca+ release in nerve damaged cells. Riluzole (Rilutek)
Progressive degenerative disease of athletes with multiple concussions. Reduction in brain weight, atrophy of frontal and temporal cortices. Enlarged lateral and 3rd ventricles Chronic Traumatic Encephalopathy
Which proteins are found to be elevated in Chronic Traumatic Encephalopathy? Tau and TDP-43
Protein that interferes with normal transcription of RNA and may be major feature of many degenerative diseases. TAR DNA binding Protein 43 (TDP-43)
Tau deposition results in ______ tangles, and glial cell tangles made of astrocytes and other glial cells. Neurofibrillary (Seen in Chronic Traumatic Encephalopathy)
Acute paralysis of the peripheral nervous system (LMN's) and progresses over hours. Guillain-Barre Syndrome
GB syndrom pathogenesis autimmune->triggered usually by compylobactor, mycoplasma, CMV or influenza. The antibodies for the infx get misdirected and attack GM 1 gangliosides of schwann cells.
Antibodies destroy schwann cells of peripheral nervous system, breaking them down and destroying the myelin sheath of the peripheral nerves. Guillain Barre Syndrome-DOES NOT AFFECT CNS
ASCENDING PARALYSIS, beginning in the feet going up to the truck (in 5% it can start in the eyes and go down the body). "Rubbery Legs" works up and affects ability to breath. GBS-also feels NUMBNESS AND ACHING PAIN
Is sensory involvement in present in GBS it usually affects _____ Proprioception causing ataxia
GBS-can affect up to the cranial nerves (facial weakness) and breathing. ____ % require a ventilator 30
In SEVERE cases of GBS loss of autonomic function occurs causing Blood pressure issues, orthostatic Hypotension, Arrhythmias
Variant of GBS involving 5% of cases with DESCENDING PARALYSIS. Eye muscles 1st-OPHTHALMOPLEGIA, ATAXIA, AREFLEXIA. Sudden blurred vision, act drunk. Miller Fisher Syndrome
Most common culprit that causes GBS Campylobacter jejuni
Many cases of ____ have occurred after flu virus inoculation GBS
CSF in GBS shows high protein titers of Anti GM1 (also in blood stream)
80 % of GBS have only ______ loss, 20 % have _____ damage as well. 80% have only myelin loss, 20 % have axon damage as well.
2 treatments for GBS 1. Plasmapheresis (filters out the antibodies)-hastens recovery. start within 4 weeks of symptoms. 2. IV Ig-neutralizes antibodies
Glucocorticoids in GBS are effective in treatment of Guillain Barre T/F F
Treatment for GBS started within 2 weeks of symptoms and fewer complications than plasmapheresis IVIg
1st line treatment for GBS. Do not use more than 5 days or may cause hepatitis or renal failure. IVIg
What week does recovery usually start taking place after onset of GBS? 4th week
What percent of patients recover from GBS? 80-85% (some patients have life long deficits such as loss of reflexes)
Death rate of GBS in US 2-3%
Relapse of GBS is called Chronic inflammatory demyelinating polyneuropathy
Post polio syndrome affects 25-50% of people who had polio. Symptoms occur ______ years later. 20-30 years
Increased muscular weakness, muscle atrophy, PAIN, and fatigue seen in: Post-polio syndrome (periods of stability then decline and can affect the ability to breath)
Decreased tolerance for cold temperatures and extreme debilitating fatigue. Post-polio syndrome
What theory for post polio syndrome states that polio destroys ant horn cells and death of motor neurons. Surviving neurons sprout new but enlarged motor units. Neural Fatigue Theory-gradually the new large sprouted neural fibers become too great of a metabolic demand and the whole neuron then dies.
2 main symptoms of post-polio syndrome 1. Muscle weakness and 2. Paralysis
Treatment for Post-polio syndrome Palliative, rest analgesia, powered wheel chairs. NO EFFECTIVE MEDS
T/F post polio syndrome is not a reoccurrence of the original polio True (no polio virus is excreted in feces)
rhythmic or repetitive neural activity in the central nervous system. Neural Oscillation
Which brain waves are seen in 1.concentration and 2. intense concentration? 1. beta 2.gamma
More than ___ % of cases of epilepsy occur in underdeveloped and third world countries. 80
New episodes of epilepsy occur in which 2 age groups? infants, people over 65 y/o
Epilepsy might be a result of defective _______ channels Sodium
Too much of this neurotransmitter might cause seizures Glutamate-excessive ca++ release, massive uncontrolled action potentials.
Brain waves awake, alert, normal consciousness Beta (14-30 Hz)
Relaxed, calm, lucid but not actively thinking Alpha (9-13 Hz)
Deeply relaxed, Meditation, mental imagery Theta (4-8 Hz)
Deep, DREAMLESS sleep Delta (1-3 Hz)
seizures in infants, children, no pathology febrile
Seizure where you stay awake-lights flashing, smells, aud hallucin, sweating, pupil dilation, jacksonian seizure start in hand or foot and progress up the body to other areas. Simple partial (HAS AUTONOMIC SYMPTOMS)
Altered consciousness seizure, AURA, pt not aware that it happened. Mumble meaningless sounds (psychomotor epilepsy), pull out their hair, staring, stupor, purposeless movement of arms and legs. Complex partial
Petit mal seizures, momentarily unconscious. CHILDREN 5-15 y/o, staring, fluttering eyes, twitch, LOC but child maintains posture. FEW SECONDS. Child unaware it happened. Absence seizures
Involuntary jerking when awake. NO AUTONOMIC symptoms myoclonic seizures
Drop attacks-children, temp LOC, child falls to ground. Atonic seizures
Classic FULL Seizure, may begin with LOUD CRY, generalized shaking of entire body. TONGUE BITING , APNEA, cyanosis. INCONT, NO memory of seizure, usually lasts 2-3 min. H/A body pain after Grand mal Seizure
Unusual manifestations seen in what type of seizures? Involves amygdala and hippocampus usually only involves sensations not muscle spasms (deja vu) or a waking dream (memories flood your brain) Temporal lobe (simple partial seizure subtype) Feelings of grandeur, visual sensations, out of body feelings)
Term "simple" when used to describe seizures means what? Consciousness is not altered
What diet has helped children with seizures founded by the amish? Ketogenic diet
Drugs to treat seizures Phenytoin, Carbamazepine (Tegretol), Klonapin (clonazepam)
Treatment for Alcoholic seizures Valium, IV Thiamine
Two drugs that can cause neuropathy 1. ISONIAZID 2. Streptomycin
Created by: kad53pa