Spinal Cord Lesions

Question 1

What is syringomyelia?

Answer 1

Cystic degeneration of the spinal cord

Question 2

What are the two major causes of syringomyelia?

Answer 2

Trauma

In association with Arnold-Chiari malformation

Question 3

What are the spinal levels that are classically affected by syringomyelia? What are the s/sx here? What type of sensation is spared?

Answer 3

• C8-T1
• Sensory loss of pain and temp
• Sparing of fine touch and proprioception

Question 4

True or false: there is sparing of fine touch and position sense in the UE with syringomyelia

Answer 4

True

Question 5

Where do neurons of the spinothalamic tract cross?

Answer 5

At the level, in the anterior white commissure

Question 6

What area of the spinal cord in particular is affected with syringomyelia? What may also be affected?

Answer 6

• Anterior white commissure

- Anterior horns of the spinal cord (LMNs)

Question 7

LMN or UMN s/sx with syringomyelia?

Answer 7

LMN

Question 8

What is the tract that is in the lateral horn of the spinal cord? What is the significance of this in syringomyelia?

Answer 8

Hypothalamospinal tract–carries sympathetic input to the face (Horner’s syndrome)

Question 9

Which usually has more advanced muscle atrophy: UMN or LMN lesions?

Answer 9

LMN

Question 10

What part of the spinal cord is damaged with Poliomyelitis? Does this result in UMN or LMN s/sx?

Answer 10

Anterior horn

LMN

Question 11

What is the viral family of poliomyelitis?

Answer 11

Picornavirus

+ssRNA

Question 12

What is Werdnig-Hoffman disease? Inheritance pattern?

Answer 12

• Inherited degeneration of the anterior motor horn

- AR

Question 13

What is the usual presentation of Werdnig-Hoffmann disease? Prognosis?

Answer 13

• Floppy baby

- Death occurs within a few years after birth

Question 14

UMN and LMN s/sx = ?

Answer 14

ALS

Question 15

What, generally, is ALS?

Answer 15

Degeneration of the upper and lower motor neurons (corticospinal tract)

Question 16

What part of the spinal cord is damaged in ALS to produce the LMN s/sx?

Answer 16

Anterior horn

Question 17

What part of the spinal cord is damaged in ALS to produce the UMN s/sx?

Answer 17

Corticospinal tract

Question 18

What is the key distinguishing feature of ALS from syringomyelia?

Answer 18

Lack of sensory impairment in ALS

Question 19

When does ALS usually present?

Answer 19

Middle age

Question 20

What is the familial mutation that causes ALS? How does this cause disease?

Answer 20

• Zn-Cu superoxide dismutase mutation

- Inability to reduce O2- to H2O2, causing ROS damage in the neurons

Question 21

What is Friedreich-Ataxia? How does it present?

Answer 21

• Degenerative disorder of the cerebellum and spinal cord tracts
• Ataxia (cerebellum) with loss of vibratory sense and proprioception, muscle weakness in LE, and loss of DTRs

Question 22

What causes Friedreich ataxia?

Answer 22

AR, GAA expansion in the frataxin gene

Question 23

When does Friedreich ataxia usually present?

Answer 23

Early Childhood

Question 24

What is the heart defect associated with Friedreich ataxia?

Answer 24

Hypertrophic cardiomyopathy

Question 25

What is the purpose of the Frataxin gene?

Answer 25

Essential for mitochondrial Fe regulation