Spinal cord lesions

Question 1

Where does poliomyelitis affect the spinal cord?

Answer 1

Destruction of anterior horn

Question 2

Where does Werdnig-Hoffman disease affect the spinal cord?

Answer 2

Spinal Muscular Atrophy - destruction of anterior horn.

Question 3

What are the characteristics of poliomyeltis and Werdnig Hoffman disease?

Answer 3

LMN lesions only due to destruction of anterior horn.

Question 4

What causes poliomyelitis?

Answer 4

Poliovirus (fecal oral).

Question 5

Where does poliomyelitis replicate?

Answer 5

Oropharynx and small intestine, then spreads via bloodstream to CNS.

Question 6

What are the lab findings associated with poliomyelitis?

Answer 6

CSF with incr WBCs and slight icnr of protein w/ no change in glucose. Virus recovered from stool or throat.

Question 7

What is the pathogenesis of Werdnig Hoffmans?

Answer 7

Congenital deterioration of anterior horns of spinal cord –> LMN lesion. Floppy baby, hypotonia, tongue fasciulations. Median age of death 7 months. AR inheritance.

Question 8

Where does MS affect the spinal cord?

Answer 8

Demyelination sort of randomly; mostly affects white matter of cervical region.

Question 9

Where does amyotrophic lateral sclerosis affect the spinal cord?

Answer 9

Lateral corticospinal tracts/anterior horns.

Question 10

What is the presentation of ALS?

Answer 10

Combined UMN and LMN deficits with no sensory or oculomotor deficits. Both UMN and LMN signs.
Commonly presents as fasciculations with eventual atrophy and weakness of hands.

Question 11

What enzyme deficiency can cause ALS?

Answer 11

superoxide dismutase 1.

Question 12

What is the medication that is helpful?

Answer 12

RiLOUzole. (Lou Gherig). Glumatate antagonist. Mostly increases survival by decreasing presynaptic glutamate release.

Question 13

What does occlusion of the anterior spinal artery cause?

Answer 13

2:00-10:00 of the spinal cord is knocked out. Spares dorsal columns and lissauer tract.

Question 14

Where is the watershed area vulnerable to anterior spinal artery infarct?

Answer 14

Upper thoracic above T8. Below T8, artery of adamkiewicz supplies ASA territory.

Question 15

What causes tabes dorsalis?

Answer 15

Tertiary syphilis. Degeneration/demyelination of dorsal columns and roots.

Question 16

What are the sx of tabes dorsalis?

Answer 16

Impaird sensation and proprioception, progressive sensory ataxia, inability to sense or feel the legs, poor coordination.

Question 17

What are findings are associated with tabes dorsalis?

Answer 17

Charcot joint, shooting pain, Argyl–Robertson pupils.

Question 18

What will exam findings of tabes dorsal be?

Answer 18

Absence of DTRs and +romberg sign.

Question 19

What will exam findings of tabes dorsal be?

Answer 19

Absence of DTRs and +romberg sign.

Question 20

What is syringomelia?

Answer 20

Syrinx (central canal) expands and damages anterior white commissure of spinothalamic tract (2nd order neurons).

Question 21

What are the sx of syringomelia?

Answer 21

Bilateral loss of pain and temperature sensation (usually C8-T1) in cape-like distribution.

Question 22

What congential condition is syringomelia associated with?

Answer 22

Chiari I malformation.

Question 23

How does B12 deficiency affect the spinal cord?

Answer 23

Subacute combined degeneration; demyelination of dorsal columns, lateral corticospinal tracts, and spinocerebellar tractsl ataxic gait, paresthesia, impaired position and vibration sense.

Question 24

What does hemisection of spinal cord cause?

Answer 24

Brown-Sequard syndrome:
Below Lesion:
- Ipsilateral UMN signs below level of lesion.
- Ipsilateral loss of tactile/vibration/proprioception below level of lesion.
- Contralateral pain and temp loss below level of lesion.
At level of lesion:
- Ipsilateral loss of all sensation at level of lesion.
- Ipsilateral LMN signs.

Question 25

What happens w/ hemisection above T1?

Answer 25

Horner syndrome due to loss of oculosympathetic pathway.

Question 26

What congenital syndrome causes degeneration of multiple spinal cord tracts, presents w/ kyphoscoliosis, and is autosomal recessive?

Answer 26

Fredreich ataxia.

Question 27

What is the pathogenesis of Fredreich ataxia?

Answer 27

Autosomal recessive trinucleotide repeat disorder (GAA) on Ch 9 in gene that encodes frataxin, an iron binding protein.

Question 28

What sx are seen w/ Fredreich ataxia?

Answer 28

Loss of mitochondrial function.
Muscle weakness, loss of DTRs, dorsal columns.
Staggering gait, frequent falling, nystagmus, dysarthria.
Systemic:
DM, HCM, pes cavus, hammer toes. kyphscoliosis.