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41 Cards in this Set

  • Front
  • Back
What is the clinical presentation of Huntington's?
Chorea, difficulty with breathing and swallowing, progressive dementia
What would a person with Huntington's CT and MRI look like?
Enlarged Lateral ventricles

cell death in basal ganglia (gross degeneration of the caudate nucleus)
What is the gene defect that causes Huntington's?
36+ repeats of CAG on Chromosome 4 in the region of the HTT gene.

Celine
Always
Goes
Huntintin (the protein)
4
How is Huntington's inherited?
Autosomal dominant. SUCKS! Person always has 50% chance of getting it
Why did the Hungtington's patient get a lung infection or have lung problems?
Due to moderate aspiration during swallowing, something probably got in his lung
What does a serum ceruloplasmin/copper study check for?
Wilson's diease
What is the main difference between Wilson and Huntington's disease?
Wilson's disease usually is accompanied by liver problems
What functions are associated with the caudate nucleus?
Inhibition of nigrostriatal pathway ( destruction causes chorea) via GABAeric neurons of the nigrostriatal pathway which is the feedback system for movement
What types of neurotransmitter is involved with the nigrostriatal pathway?
DOPE comes from Nigga

GABA goes from striatum to Substania nigra
What mood changes are involved with a Huntington's patient?
Depression

Perhaps even grandiosity and egoism
What does increased CAG repeat correspond with?
Age of onset in Huntington's patient (90 CAG's = 20 yrs of age onset)
What would chronic alcoholism cause in the brain?
Anterior Lobe Syndrome (of cerebellum); loss of lower limb coordination

alcohol is unlikely to cause only ipsilateral effects
What structures would a right anterior cerebellar lesion affect?
It could affect all brainstem levels; in our patient:

CN 5, 7, 8, 9 near pontomedullary junction
Medial Lemniscus
What would cause unilateral anterior lob syndrome and cafe au lait spots?
Neurofibromas especially Type I
What gene defect causes neurofibromas and how is it inherited?
NF1 gene which is AD inherited
What does a patient with right hemiparesis, speech difficulty, and right homonymous hemianopia have?
MCA infarct that affected FPOT left lateral cortex
What would ACA occlusion cause?
leg weakness and sensory loss with face spared
What would PCA occlusion cause?
CN 5-11 defects, horner's syndrome, vision loss
What does a MRI of a occluded MCA patient look like?
abnormal tissue/lack of gyri, occlusion of MCA
How did the neruocystercercosis patient relapse into non-communicating hydrocephalus?
larvae formed a cyst by his shunt, thereby blocking it
What caused unconsciousness in the hydrocephalus patient?
Pressure on cardiorespiratory centers in the medulla.
Why should a lumbar puncture not be taken in someone with increased intracranial pressure?
could cause brain stem herniation
What is SSEP? What does it tell you?
Somatoseonsory evoked potential - helps pinpoint location of lesion
What disease:

High IgG, nystagmus, weakness/tingling?
Multiple sclerosis
What is Eaton Lambert?
Autoantibodies that attack Ca channels on the presynaptic axon at the NMJ so ACh cannot be released.
Why would administration of anesthesia to an Eaton-Lambert patient cause paralyzed respiration?
The anesthesia exacerbated her condition and had an anticonvulsant and inhibitory affect of musculature.
What is paraneoplasmic syndrome?
When antibodies in cancer start attacking non-cancer host cells :(
What is the treatment for Eaton Lambert's and how does it work?
3,4-diaminopyridine; blocks K channels to increase AP potential duration and thus greater ACh release
What does an EMG help assess?
NMJ function and differentiation of pre and post synaptic diseases
What is the action of botulism?
Cleaves vesicle docking proteins in the presynaptic neuron of the NMJ so ACh is not released.
Where did the patient with occlusion of MCA have ictal EEG waves? What kind of seizure did he have?
Seizure onset was in anterior left temporal lobe.

Aura indicates limbic system functioning and so does his loss of conscience which means this was a complex partial seizure
What about seizures does SPECT show?
SPECT shows perfusion and thus areas of high metabolic activity to indicate site of origin and spread of seizure
What other disorder did the kid with petit mal have to rule out?
ADHD
How do anti epileptic drugs work?
GABA agonists, Na or CA channel blockers to hyperpolarize cells.
What might febrile convulsions indicate?
both the possibility of kindling and the genetic predisposition to be more likely to have seizures.
Which MRI weight is dark?
T1
What scanning method mesaures glucose untilization?
PETS love sugar
What detects cerebral blood flow and intense tissue metabolism
SPECT, much like in our seizure case
What is the best scanning method for spatial resolution?
MRI>CT>PET>SPECT

Mr. CT Pets snakes
What is the best for temporal resolution
EEG
What are CT scans good for detecting
STV - strokes, tumors, ventricles, bone