Spinal Cord Lesions Flashcards
What is syringomyelia?
Cystic degeneration of the spinal cord
How does syringomyelia arise?
Arises with trauma or in association with Arnold-Chiari malformation
At what level of the spinal cord does syringomyelia usually occur?
C8 - T1
Clinical presentation of syringomyelia
Sensory loss of pain and temperature with sparing of fine touch and position sense in the upper extremities
The cyst initially only involves the anterior white commissure of the spinothalamic tract while sparing the dorsal column
Syrinx expansion results in involvement of spinal tracts other than the spinothalamic tract leading to:
- Muscle atrophy and weakness with decreased muscle tone and impaired reflexes
- Due to damage to LMN of the anterior horn
- Horner syndrome
- Ptosis
- Miosis
- Anhidrosis
- Due to disruption of lateral horn of the hydrothalamospinal tract
What it poliomyelitis?
Damage to the anterior motor horn due to poliovirus infection
Clinical presentation of poliomyelitis
LMN signs:
- Flaccid paralysis with muscle atrophy
- Fasciculations
- Weakness with decreased muscle tone
- Impaired reflexes
- Negative Babinski sign
What is Werdnig-Hoffman Dz?
Inherited degeneration of the anterior motor horn
Autosomal recessive
Clinical presentation of Werdnig-Hoffman
Floppy-baby
Death occurs within a few years
What is amyotrophic lateral sclerosis (ALS)?
Degenerative disorder of upper and lower motor neurons of the corticospinla tract
Anterior motor horn degeneration leads to what motor damage?
LMN damage
What are the LMN signs?
Flaccif paralysis with muscle atrophy
Fasciculations
Weakness with decreased muscle tone
Impaired reflexes
Negaive Babinski signs
Degeneration of the lateral corticospinal tract leads to damage of what neurons?
UMN damage
What are the UMN damage signs?
Spastic paralysis with hyperreflexia
Increased muscle tone
Positive Babinski sign
What are the signs of ALS?
Atrophy and weakness of hands is an early sign
How do you disntiguish ALS from syringomyelia?
ALS has a lack of sensory impairment
What mutation is present in some familial cases of ALS?
Zn-Cu superoxide dismutase mutation (SOD1)
Leads to free radical injury in neurons
What is Friedrich Ataxia?
Degenerative disorder of the cerebellum and spinal cord
Clinical presentation of Friedrich Ataxia
Degeneration of the cerebellum leads to ataxia
Degeneration of multiple spinal cord tracts leads to loss of vibratory sense and proprioception, muscle weakness in the lower extremities and loss of deep tendon reflexes
What gene disorder causes Friedrich Ataxia?
Expansion of an unstable trinucleotide repeat (GAA) in the frataxin gene
Autosomal recessive
What is the role of the frataxin gine?
Essential for mitochondrial iron regulation
Loss of the frataxin gene results in iron buildup wiht free radical damage
At what age does Friedrich Ataxia present?
Early childhood with patients wheelchair bound within a few years
What condition is associated with Friedrich Ataxia?
hypertrophic cardiomyopathy
What signals are carried on the spinothalamic tract?
Pain and temperature sensation
