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113 Cards in this Set
- Front
- Back
CHERRY RED SPOT
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TAY SACHS
SILIDOSIS NEIMAN PICS OTHERS |
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C.A.T.
FOR BIG HEAD |
CANAVANS
ALEXANDERS TAY SACHS |
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Most common SYMPTOMATIC tumor in Brain
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Gliomas
If broaken down to subtypes of Gliomas then Meningoma is answer |
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Most common NON SYMPTOMATIC tumor in Brain
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Meningoma
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1p19q deletion
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Oligodendroglioma
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Hemisphere-Adult
Vermis-Child |
Meduloblastoma
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Round Blue Cells
Neuroblastic Rossets |
Meduloblastoma
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Can spread via CSF like Ependemoma
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Meduloblastoma
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Steroids can make this Brain tumor biopsy non diagnostic
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Lymphoma
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This tumor can be DWI positive
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Lymphoma
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'Motor Oil Apperance'
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Crainopharyngioma
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Chromosome 22
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NF2
Meningoma |
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MS is mostly _____ cell mediated
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Th1 cell
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Urinary Hesitancy- Bethanacol
Urinary Urgency- Oxybutinin(Ditropan) |
Bethanacol for __________
Oxybutinin(Ditropan) __________ |
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Name the two B1a drugs
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Avonex
Rebiff |
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decrease MRI lesion burden with
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Betaseron B-1b
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progression relapse of MS decreased from 37% to 28%
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Avonex B-1a
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Rebiff vs Avonex
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Rebiff:
dec RR, MRI lesion and dec next remission. but NO change in EDSS |
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Trial using Inc Betaseron dosage shows
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No more effective
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Rebiff vs Copaxone trial
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No change
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Rebiff vs Copaxone trial shows
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No change
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S.E. of Copaxone
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Flushing, Dyspnea, Chest tightening, Palpitations, Anxiety
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Natalizumab
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anti VLA4 antibody
blocks lymphocute and monocyte adherence. 80% dec new/enhancing T2 lesions. 96% dec in enhancing lesions. |
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AFFIRM trial
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Tisabri dec 68% relapses.
PML, 2% anaphalaptic rxn |
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what crainal nerves run in Juglar foramen
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C.N. 9-11
ICA Internal Jugular Vein |
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Ototoxic Meds
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Aminoglycocydes
Loop Diuretics High dose ASA Cisplatin |
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Cavernous Sinus contents
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III IV V1 V2 VI ICA with symp fibers
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85% CP angle tumors are
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Schwanomas arising form VIII
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Optic canal
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Optic Nerve and Opthalmic artery
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Oral MS drug
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fampradine
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Alpha Motor Neuron: role of
Glutamate GABA Glycine |
Glumate stimulates
GABA Glycine inhibit |
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Only 5% ALS is familial.
Gene accounts for 15% of familial ALS |
SOD-1 gene
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A4Vgene in ALS
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Rapid form of ALS
Die usually within a year |
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GQ1b antibody is associated with ataxia, areflexia and opthalmoplegia and is classic in _________
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Miller Fisher variant GBS
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AD inheritance with TTR gene
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Amyloidosis
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Ptosis, sever tongue atrophy seen in
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anti MUSK antibodies
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striated muscle antibodies ass with
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thymomatous Myasthenia Gravis
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Voltage Gated K channel antibodies ass with
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Neuromyotonia
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P/Q antibodies ass with
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LEMS
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One difference between M.G. and LEMS is
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LEMS spares the eyes.
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what other ca other than lung ca can be associated with LEMS
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Lymphoma
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what % of patients don't get cancer in LEMS
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50%
more often tend to be male, younger |
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NCV difference between Botulism and LEMS
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LEMS has small CMAP with facilation and large CMAP. Botulism, the CMAP is small and very little facilation.
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Neuromyotonia
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voltage K channel antibodies
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Emerin-nuclear protein deficiency
Joint contractures Largely X-linked |
Emery Dreifuss
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Auto Dom Dystrophies
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FSHD
Myotonic I and 2 OculoFacial MD |
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Dysferalin defiency myopathy
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LGMD
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Difference between Myotonic MD
1 and 2 |
Type 2 Myotonic MD has no MR and no Cardiac conduction defects.
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Auto Ressive MDs
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LGMD
other rare Fukiamya and Walker-Warberg |
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Congenital myopathies
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NOT all are Benign or Nonprogressive.
-Relativly normal CPK. Hypotonia. |
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Calcium channel abnormality
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HYPOkelemic Periodic Paralysis
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Sodium Channel Abnormality
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HYPERkalemic periodic paralysis
Paramyotonia Congenita |
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Chloride Channel abnormality
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Myotonia Congenita
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Myopathy with Hearing loss- think
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Mitochondrial
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preponderence of CD4 and B cells myopathy
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Dermatomyositis
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preponerence of CD 8 cells myopathy
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Polymyositis
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Dermatomyositis histology
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perifasicular atrophy
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Polymyositis histology
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Endomesial inflamation
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features of a mucopolysacchridosis
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coarsening facial features, corneal clouding, organomegaly, poor growth and dysostosis multiplex
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Peroxisomes are involved in the metabolism of these fatty acids. most common peroxisomal disorder are
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adrenoleukodystrophy, is an X-linked disorder, presenting in early to mid childhood with behavioral change and spastic diplegia. Signs of a leukodystrophy are present on imaging of the brain, most commonly affecting the more posterior white matter first. In adults, either males with a milder course, or heterozygous females, the disease can be restricted to the spinal cord and peripheral nerves, termed adrenomyeloneuropathy.
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Her dry eyes and dry mouth is clue to
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Sjögren Syndrome.
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Sjögren Syndrome antibodies are
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Anti-RO (SSA) or anti-La (SSB) antibodies
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Anterior ischemic optic neuropathy refers to ischemia involving the
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optic nerve head, which is evidenced, as in this case, by optic nerve head swelling.
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T.A. causes ischemia of anterior or posterior optic nerve?
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anterior
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Multiple system atrophy (MSA) is a condition
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ie Shy Drager
in which parkinsonism is accompanied by variable cerebellar, autonomic, and other variable features. MSA-autonomic subtype manifests as parkinsonism with autonomic failure. This symptomatic orthostatic hypotension is frequently associated with the absence of reflex tachycardia upon standing. Erectile dysfunction represents the most common, and often earliest feature in male MSA patients. In addition, patients may notice constipation and hypohydrosis or anhydrosis. Urologic features are common and include urinary urgency, frequency, nocturia and urge incontinence. MSA is often minimally levodopa-responsive but some patients have a moderate response to levodopa. |
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Describe Concession Grade 1:
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* Transient confusion;
* NO loss of consciousness; * Concussion symptoms clear in less than 15 minutes. |
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Describe Concession Grade 2:
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* Transient confusion;
* NO loss of consciousness; * Concussion symptoms or mental status abnormalities last longer than 15 minutes. |
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Describe Concession Grade 3:
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Any loss of consciousness, either brief (seconds) or prolonged (minutes).
NO sports for one week. |
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The most common pathological finding in HD is
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the presence of intranuclear and cytoplasmic aggregates of the medium spiny neurons.
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Tufted astrocytes
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progressive supranuclear palsy
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midbrain atrophy typically seen on MRI.
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progressive supranuclear palsy
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for Dystonic reaction give
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Anticholinergics
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Ingestion of a heavy carbohydrate meal or cold exposure may produce weakness with
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hypokalemic periodic paralysis.
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Prolonged fasting precipitates weakness with
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hyperkalemic paralysis
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symptom complex, including altered mental status, headache, visual complaints, and cardiovascular collapse, is suggestive of
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pituitary apoplexy
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episodic ataxia
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an cause symptoms similar to basilar artery migraine.
autosomal-dominant condition |
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ADEM involves
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DEEP GRAY STRUCTURES
white matter |
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Does Meniere’s disease have progressive sensorineural hearing loss?
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YES
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Refsum’s disease
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autosomal recessive disorder is caused by a deficiency of the protein phytanoyl-CoA hydroxylase, which catalyzes the first step in alpha oxidation of phytanic acid. The accumulation of phytanic acid in the nervous system leads to the chief manifestations of the disorder including: night blindness, progressive polyneuropathy, sensorineural hearing loss and occasionally cerebellar ataxia. The visual symptoms are attributed to pigmentary degeneration of the retina, and are often the first feature of the disorder.
Dx: markedly elevated serum phytanic acid and confirm by oxidative ability of phytanic acid by skin fibroblasts. Rx:restriction of dietary phytanic acid intake |
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Windmill pitcher’s(ie Baseball) radial neuropathy
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Involves Triceps.
The lesion is in the radial nerve as it crosses the axilla since it involves triceps. |
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RBD has been associated with
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including Lewy body dementia, Parkinson disease, and multiple system atrophy
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synucleinopathies include
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Lewy body dementia, Parkinson disease, and multiple system atrophy
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hematin is used to treat________
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PORPHYRIA:
-Auto Dom -mutation in the enzyme porphobilinogen (PBG) deaminase gene |
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“hummingbird sign” seen on mid-sagittal MRI is characteristic of
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progressive supranuclear palsy.
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RLS symptoms may be worsened by
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antipsychotic agents, and this includes the newer generation antipsychotics that are both selective dopamine antagonists and partial dopamine agonists.
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interventional procedures such as angioplasty or intraarterial vasodilators should be considered in
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vasospasam from SAH after hemodynamic augementation(fluids and possible vasopressors) fails.
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Friederich’s ataxia protein
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frataxin
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patient autonomy
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allow patients to participate in decisions about their medical care
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Meningitis in Immunocomprised
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Listeria, Nocardia, TB, Crytococcus
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Meningitis in Neonates
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group B Strep, E. coli, Listeria
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Skull Fracture
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S. Pneumonia, Staph, Cornyebacterium
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Rifampin
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Meningococcal contacts
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Spinal epidural abscess
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Usually thorasic level
Tender on percussion |
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Lymphocytic pleocytosis, normal glucose and usually normal protein
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viral meningitis
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Viral agent with often Polymorphonuclear pleocytosis
treat with Gancyclovir |
CMV
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Polio like viral infection
viral yet Neurtophile predominance |
West Nile
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Nephrogenic systemic fibrosis
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with gadolinium in renal dysfunction
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associated with a mutation at codon 178.
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fatal familial insomnia
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mutation at Codon 129 is involved in
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CJD.
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Use of an MAOI will do what to sleep?
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Decrease the amount of REM sleep
An increase in monoamines is associated with decrease in REM. |
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Bilateral medial frontal injury ie. bilateral ACA infarcts
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Akinetic Mutism
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Gelastic seizures (laughing seizures)
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are often associated with hypothalamic hamartomas.
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difference between 'Phase contrast MRA' and 'Time of Flight MRA'
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Phase contrast uses Gadolinium
Time of flight: No Gadolinium |
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Progressive External Ophthalmoplegia (PEO)
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a Mitochondrial myopathy
The most common clinical features include adult onset of weakness of the external eye muscles (ophthalmoplegia) and exercise intolerance. |
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Hyperekplexia, a congenital startle syndrome is due to a mutation in:
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Glycine receptor
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In a patient with central nervous system injury the following syndrome will cause hyponatremia with excessive sodium excretion in the urine.
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Cerebral Salt wasting.
It usually resolves spontaneously after a few weeks. Unlike in SIADH, the urine in cerebral salt wasting is dilute. |
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Moro and Grasp reflex begin to dissapear at about
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5-6 months
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AED: T-type calcium channel blocker
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Ethosuximide
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Dilantin, Tegretal, mechanism of action
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block frequency-, use- and voltage-dependent neuronal SODIUM channels
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Depokote mech of action
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It may augment the action of GAD (glutamic acid decarboxylase), a GABA-synthesizing enzyme. And T-type Calcium channels
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Gabatril mech of action
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blocks the neuronal and glial reuptake of gamma aminobutyric acid (GABA) after its release from postsynaptic GABA receptors,
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Street Drugs associated with development of dystonia
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PCP, Cocaine
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May present early in HIV
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Aseptic meningitis
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