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113 Cards in this Set

  • Front
  • Back
CHERRY RED SPOT
TAY SACHS
SILIDOSIS
NEIMAN PICS
OTHERS
C.A.T.
FOR BIG HEAD
CANAVANS
ALEXANDERS
TAY SACHS
Most common SYMPTOMATIC tumor in Brain
Gliomas
If broaken down to subtypes of Gliomas then Meningoma is answer
Most common NON SYMPTOMATIC tumor in Brain
Meningoma
1p19q deletion
Oligodendroglioma
Hemisphere-Adult
Vermis-Child
Meduloblastoma
Round Blue Cells
Neuroblastic Rossets
Meduloblastoma
Can spread via CSF like Ependemoma
Meduloblastoma
Steroids can make this Brain tumor biopsy non diagnostic
Lymphoma
This tumor can be DWI positive
Lymphoma
'Motor Oil Apperance'
Crainopharyngioma
Chromosome 22
NF2
Meningoma
MS is mostly _____ cell mediated
Th1 cell
Urinary Hesitancy- Bethanacol

Urinary Urgency- Oxybutinin(Ditropan)
Bethanacol for __________

Oxybutinin(Ditropan) __________
Name the two B1a drugs
Avonex
Rebiff
decrease MRI lesion burden with
Betaseron B-1b
progression relapse of MS decreased from 37% to 28%
Avonex B-1a
Rebiff vs Avonex
Rebiff:
dec RR, MRI lesion and dec next remission.
but NO change in EDSS
Trial using Inc Betaseron dosage shows
No more effective
Rebiff vs Copaxone trial
No change
Rebiff vs Copaxone trial shows
No change
S.E. of Copaxone
Flushing, Dyspnea, Chest tightening, Palpitations, Anxiety
Natalizumab
anti VLA4 antibody
blocks lymphocute and monocyte adherence. 80% dec new/enhancing T2 lesions. 96% dec in enhancing lesions.
AFFIRM trial
Tisabri dec 68% relapses.
PML, 2% anaphalaptic rxn
what crainal nerves run in Juglar foramen
C.N. 9-11
ICA
Internal Jugular Vein
Ototoxic Meds
Aminoglycocydes
Loop Diuretics
High dose ASA
Cisplatin
Cavernous Sinus contents
III IV V1 V2 VI ICA with symp fibers
85% CP angle tumors are
Schwanomas arising form VIII
Optic canal
Optic Nerve and Opthalmic artery
Oral MS drug
fampradine
Alpha Motor Neuron: role of
Glutamate
GABA
Glycine
Glumate stimulates
GABA Glycine inhibit
Only 5% ALS is familial.
Gene accounts for 15% of familial ALS
SOD-1 gene
A4Vgene in ALS
Rapid form of ALS
Die usually within a year
GQ1b antibody is associated with ataxia, areflexia and opthalmoplegia and is classic in _________
Miller Fisher variant GBS
AD inheritance with TTR gene
Amyloidosis
Ptosis, sever tongue atrophy seen in
anti MUSK antibodies
striated muscle antibodies ass with
thymomatous Myasthenia Gravis
Voltage Gated K channel antibodies ass with
Neuromyotonia
P/Q antibodies ass with
LEMS
One difference between M.G. and LEMS is
LEMS spares the eyes.
what other ca other than lung ca can be associated with LEMS
Lymphoma
what % of patients don't get cancer in LEMS
50%
more often tend to be male, younger
NCV difference between Botulism and LEMS
LEMS has small CMAP with facilation and large CMAP. Botulism, the CMAP is small and very little facilation.
Neuromyotonia
voltage K channel antibodies
Emerin-nuclear protein deficiency
Joint contractures
Largely X-linked
Emery Dreifuss
Auto Dom Dystrophies
FSHD
Myotonic I and 2
OculoFacial MD
Dysferalin defiency myopathy
LGMD
Difference between Myotonic MD
1 and 2
Type 2 Myotonic MD has no MR and no Cardiac conduction defects.
Auto Ressive MDs
LGMD
other rare Fukiamya and Walker-Warberg
Congenital myopathies
NOT all are Benign or Nonprogressive.
-Relativly normal CPK. Hypotonia.
Calcium channel abnormality
HYPOkelemic Periodic Paralysis
Sodium Channel Abnormality
HYPERkalemic periodic paralysis
Paramyotonia Congenita
Chloride Channel abnormality
Myotonia Congenita
Myopathy with Hearing loss- think
Mitochondrial
preponderence of CD4 and B cells myopathy
Dermatomyositis
preponerence of CD 8 cells myopathy
Polymyositis
Dermatomyositis histology
perifasicular atrophy
Polymyositis histology
Endomesial inflamation
features of a mucopolysacchridosis
coarsening facial features, corneal clouding, organomegaly, poor growth and dysostosis multiplex
Peroxisomes are involved in the metabolism of these fatty acids. most common peroxisomal disorder are
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.
Her dry eyes and dry mouth is clue to
Sjögren Syndrome.
Sjögren Syndrome antibodies are
Anti-RO (SSA) or anti-La (SSB) antibodies
Anterior ischemic optic neuropathy refers to ischemia involving the
optic nerve head, which is evidenced, as in this case, by optic nerve head swelling.
T.A. causes ischemia of anterior or posterior optic nerve?
anterior
Multiple system atrophy (MSA) is a condition
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.
Describe Concession Grade 1:
* Transient confusion;
* NO loss of consciousness;
* Concussion symptoms clear in less than 15 minutes.
Describe Concession Grade 2:
* Transient confusion;
* NO loss of consciousness;
* Concussion symptoms or mental status abnormalities last longer than 15 minutes.
Describe Concession Grade 3:
Any loss of consciousness, either brief (seconds) or prolonged (minutes).
NO sports for one week.
The most common pathological finding in HD is
the presence of intranuclear and cytoplasmic aggregates of the medium spiny neurons.
Tufted astrocytes
progressive supranuclear palsy
midbrain atrophy typically seen on MRI.
progressive supranuclear palsy
for Dystonic reaction give
Anticholinergics
Ingestion of a heavy carbohydrate meal or cold exposure may produce weakness with
hypokalemic periodic paralysis.
Prolonged fasting precipitates weakness with
hyperkalemic paralysis
symptom complex, including altered mental status, headache, visual complaints, and cardiovascular collapse, is suggestive of
pituitary apoplexy
episodic ataxia
an cause symptoms similar to basilar artery migraine.
autosomal-dominant condition
ADEM involves
DEEP GRAY STRUCTURES
white matter
Does Meniere’s disease have progressive sensorineural hearing loss?
YES
Refsum’s disease
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
Windmill pitcher’s(ie Baseball) radial neuropathy
Involves Triceps.
The lesion is in the radial nerve as it crosses the axilla since it involves triceps.
RBD has been associated with
including Lewy body dementia, Parkinson disease, and multiple system atrophy
synucleinopathies include
Lewy body dementia, Parkinson disease, and multiple system atrophy
hematin is used to treat________
PORPHYRIA:
-Auto Dom
-mutation in the enzyme porphobilinogen (PBG) deaminase gene
“hummingbird sign” seen on mid-sagittal MRI is characteristic of
progressive supranuclear palsy.
RLS symptoms may be worsened by
antipsychotic agents, and this includes the newer generation antipsychotics that are both selective dopamine antagonists and partial dopamine agonists.
interventional procedures such as angioplasty or intraarterial vasodilators should be considered in
vasospasam from SAH after hemodynamic augementation(fluids and possible vasopressors) fails.
Friederich’s ataxia protein
frataxin
patient autonomy
allow patients to participate in decisions about their medical care
Meningitis in Immunocomprised
Listeria, Nocardia, TB, Crytococcus
Meningitis in Neonates
group B Strep, E. coli, Listeria
Skull Fracture
S. Pneumonia, Staph, Cornyebacterium
Rifampin
Meningococcal contacts
Spinal epidural abscess
Usually thorasic level
Tender on percussion
Lymphocytic pleocytosis, normal glucose and usually normal protein
viral meningitis
Viral agent with often Polymorphonuclear pleocytosis
treat with Gancyclovir
CMV
Polio like viral infection
viral yet Neurtophile predominance
West Nile
Nephrogenic systemic fibrosis
with gadolinium in renal dysfunction
associated with a mutation at codon 178.
fatal familial insomnia
mutation at Codon 129 is involved in
CJD.
Use of an MAOI will do what to sleep?
Decrease the amount of REM sleep

An increase in monoamines is associated with decrease in REM.
Bilateral medial frontal injury ie. bilateral ACA infarcts
Akinetic Mutism
Gelastic seizures (laughing seizures)
are often associated with hypothalamic hamartomas.
difference between 'Phase contrast MRA' and 'Time of Flight MRA'
Phase contrast uses Gadolinium
Time of flight: No Gadolinium
Progressive External Ophthalmoplegia (PEO)
a Mitochondrial myopathy
The most common clinical features include adult onset of weakness of the external eye muscles (ophthalmoplegia) and exercise intolerance.
Hyperekplexia, a congenital startle syndrome is due to a mutation in:
Glycine receptor
In a patient with central nervous system injury the following syndrome will cause hyponatremia with excessive sodium excretion in the urine.
Cerebral Salt wasting.
It usually resolves spontaneously after a few weeks. Unlike in SIADH, the urine in cerebral salt wasting is dilute.
Moro and Grasp reflex begin to dissapear at about
5-6 months
AED: T-type calcium channel blocker
Ethosuximide
Dilantin, Tegretal, mechanism of action
block frequency-, use- and voltage-dependent neuronal SODIUM channels
Depokote mech of action
It may augment the action of GAD (glutamic acid decarboxylase), a GABA-synthesizing enzyme. And T-type Calcium channels
Gabatril mech of action
blocks the neuronal and glial reuptake of gamma aminobutyric acid (GABA) after its release from postsynaptic GABA receptors,
Street Drugs associated with development of dystonia
PCP, Cocaine
May present early in HIV
Aseptic meningitis