Spinal Cord Lesions Flashcards
What is syringomyelia?
Cystic degeneration of the spinal cord
What are the two major causes of syringomyelia?
Trauma
In association with Arnold-Chiari malformation
What are the spinal levels that are classically affected by syringomyelia? What are the s/sx here? What type of sensation is spared?
- C8-T1
- Sensory loss of pain and temp
- Sparing of fine touch and proprioception
True or false: there is sparing of fine touch and position sense in the UE with syringomyelia
True
Where do neurons of the spinothalamic tract cross?
At the level, in the anterior white commissure
What area of the spinal cord in particular is affected with syringomyelia? What may also be affected?
- Anterior white commissure
- Anterior horns of the spinal cord (LMNs)
LMN or UMN s/sx with syringomyelia?
LMN
What is the tract that is in the lateral horn of the spinal cord? What is the significance of this in syringomyelia?
Hypothalamospinal tract–carries sympathetic input to the face (Horner’s syndrome)
Which usually has more advanced muscle atrophy: UMN or LMN lesions?
LMN
What part of the spinal cord is damaged with Poliomyelitis? Does this result in UMN or LMN s/sx?
Anterior horn
LMN
What is the viral family of poliomyelitis?
Picornavirus
+ssRNA
What is Werdnig-Hoffman disease? Inheritance pattern?
- Inherited degeneration of the anterior motor horn
- AR
What is the usual presentation of Werdnig-Hoffmann disease? Prognosis?
- Floppy baby
- Death occurs within a few years after birth
UMN and LMN s/sx = ?
ALS
What, generally, is ALS?
Degeneration of the upper and lower motor neurons (corticospinal tract)
What part of the spinal cord is damaged in ALS to produce the LMN s/sx?
Anterior horn
What part of the spinal cord is damaged in ALS to produce the UMN s/sx?
Corticospinal tract
What is the key distinguishing feature of ALS from syringomyelia?
Lack of sensory impairment in ALS
When does ALS usually present?
Middle age
What is the familial mutation that causes ALS? How does this cause disease?
- Zn-Cu superoxide dismutase mutation
- Inability to reduce O2- to H2O2, causing ROS damage in the neurons
What is Friedreich-Ataxia? How does it present?
- 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
What causes Friedreich ataxia?
AR, GAA expansion in the frataxin gene
When does Friedreich ataxia usually present?
Early Childhood
What is the heart defect associated with Friedreich ataxia?
Hypertrophic cardiomyopathy
What is the purpose of the Frataxin gene?
Essential for mitochondrial Fe regulation
