Spinal Cord Lesions Flashcards
A 42 year old patient presents to your neuro clinic with a sensory loss of pain and temperature in her upper extremities. Fine touch and position sense is intact. What does she have and what caused it?
She has syringomyelia which presents due to involvement of the anterior white commissure of the spinothalamic tract with sparing of the dorsal column.
What is synringomyella? What causes it and where does it occur?
Cystic degeneration of the spinal cord, arises with trauma or in association with a type 1 Arnold-Chiari malformation. Usually occurs at C8-T1.
What happens due to syrinx expansion in syringomyelia?
- Muscle atrophy and weakness with decreased muscle tone and impaired reflexes due to damage to lower motor neurons of the anterior horn
- Horner syndrome with ptosis (droopy eyelids), miosis (constricted pupil), and anhydrosis (decreased sweating) due to disruption of the lateral horn of the hypothalamospinal tract.
A patient presents with flaccid paralysis with muscle atrophy, fasiculations, weakness with decreased Muscle tone. His reflexes were impaired, but Babinksi sign is negative. What could lead to this presentation?
He might have damage to the anterior motor horn due to poliovirus infection which is known as POLIOMYELITIS.
You see a floppy baby at the pediatric hospital. What is his prognosis? What disease does he have and how is it inherited?
He has Werdnig-Hoffman disease which is an autosomal recessive inherited degeneration of the anterior motor horn. Death occurs a few years after birth.
What mutation is associated with amyotrophic lateral sclerosis? What does it do?
Zinc-copper superoxide dismutase (SOD1) mutation is present in some familial cases. Leads to free radical injury in neurons.
Is sensory impairment present in ALS or syringomyelia?
Syringomyelia
What is ALS?
Degenerative disorder of upper and lower motor neurons of the corticospinal tract.
If a patient presents with flaccid paralysis with muscle atrophy, fasiculations, weakness with decreased muscle tone, impaired reflexes and a negative Babinksi sign, what does he have?
Anterior motor horn degeneration due to lower motor neuron signs
If a patient presents with spastic paralysis with hyperreflexia, increased muscle tone and positive Babinksi sign, what does he have?
Lateral corticospinal tract degeneration which leads to upper motor neuron signs.
What is Friedrich ataxia? How is it inherited and which gene is responsible? What is it associated with and When does it present?
Degenerative disorder of the cerebellum and spinal cord autosomal recessive due to expansion of an unstable trinucleotide repeat (GAA) in the frataxin gene. The frataxin gene is essential for mitochondrial iron regulation. Loss results in iron buildup with free radical damage. Presents in early childhood with patients being wheel chair bound within a few years. Associated with hypertrophic cardiomyopathy.
