Neuro 17.2: Spinal Cord Lesions Flashcards
What is the mutation in Friedreich Ataxia?
unstable trinucleotide repeat (GAA) in the frataxin gene
What is Friedreich Ataxia?
- a degenerative disorder of the cerebellum and spinal cord tracts
- *** “Friedreich is Fratastic (frataxin): he’s your favorite frat brother, always staggering and falling but has a sweet, big heart.”
Where does sensory info for pain/temperature ascend?
in the spinothalamic tract
Why does dysfunction of the frataxin gene cause Friedreich Ataxia to occur?
- normal = essential for mitochondrial iron regulation
- damage = increased risk of free radicals via the Fenton reaction –> free radical damage of neurons
What is Riluzole? What is it for? How does it work?
- tx for ALS/Lou Gehrig disease
- decreases glutamate excitotoxicity (via unknown mech.)
- *** “For Lou Gehrig disease, give rilouzole”
Dx?
- unstable trinucleotide repeat (GAA) in the frataxin gene
Friedreich Ataxia
Where in the spinal cord is the the hypothalamic spinal tract found?
in the lateral horns
What does syringomyelia arise in assoc. with?
- trauma
- Arnold-Chiari malformation
Who classically gets Amyotrophic Lateral Sclerosis (ALS)?
middle-aged adults
Where does sensory info for pain/temperature decussate?
at the anterior white commissure
This is the name for the cystic degeneration of the spinal cord at the anterior white commissure.
syringomyelia
Dx?
- a degenerative disorder of upper AND lower motor neurons (the corticospinal tract)
Amyotrophic Lateral Sclerosis (ALS)
How is polio transmitted?
fecal oral
Which spinal cord lesion presents with a “cape-like” distribution of symptoms?
syringomyelia
O2- + superoxide dismutase = _______
H2O2
What does a negative Babinkski sign look like?
downgoing toes
What are the initial s/s of a poliovirus infection?
- fever
- sore throat
- ab pain
- nausea/vomiting
How can Amyotrophic Lateral Sclerosis (ALS) be distinguished from syringomyelia?
there is NO sensory impairment in Amyotrophic Lateral Sclerosis (ALS)
At what level of the spinal cord does syringomyelia classically occur?
C8-T1
If a syringomyelia lesion expanded, it would knock out the anterior horns and then the ______ of the spinal cord.
lateral horns
On which chromosome is the frataxin gene located?
chromosome 9
What are the 3 classic findings of Horners syndrome?
ptosis, myosis, and anhydrosis
Where does sensory info for pain/temperature synapse?
in the posterior horn of the spinal cord
What is the prognosis for Werdnig-Hoffman disease?
death w/i a few years after birth (median = 7 mos)
What disease presents like poliovirus?
Werdnig-Hoffman disease
What is Amyotrophic Lateral Sclerosis (ALS)?
a degenerative disorder of upper AND lower motor neurons (the corticospinal tract)
Where in the body does poliovirus initially infect?
oropharynx and small bowel
Dx?
- a degenerative disorder of the cerebellum and spinal cord tracts
- ataxia
- loss of vibration sense
- loss of proprioception
- muscle weakness in lower extremities
- loss of DTRs
Friedreich Ataxia
How does poliomyelitis present?
- lower motor neuron signs:
- muscle weakness and atrophy
- decreased tone
- impaired reflexes
- flaccid paralysis
- negative Babinksi sign (downgoing toes)
What info. does the dorsal column of the spinal cord carry?
fine touch/position sense
If a syringomyelia lesion expanded, it would first knock out the _____ of the spinal cord.
anterior horns
What runs thru the lateral horn of the spinal cord?
the hypothalamal spinal tract- sympathetic input to the face