17.2 Spinal cord lesions

Question 1

syringomyelia

• assoc with what congenital disorder
• other main cause

Answer 1

1. Chiari I malformation (tonsils and vermis into foramen magnum)
2. trauma

Question 2

ALS

• what affected?
• clinical presentation
• what is a classic early sign of ALS?

Answer 2

Amyotrophic Lateral Sclerosis

• degenerative disorder of UMN and LMN of corticospinal tract
  1. LMN: muscle weakness, atrophy, etc
  2. UMN: spastic paralysis, hyperreflexia, etc
• early sign: atrophy and weakness of hands (without pain/temp sensory impairment, which is seen in syringomyelia)

Question 2

Friedreich ataxia

-clinical presentation (4)

Answer 2

degenerative disorder of:

1. cerebellum-ataxia
2. spinal cord–multiple tracts, so:
   1) loss of vibration/proprioception sense
   2) muscle weakness in lower extremities
   3) loss of deep tendon reflexes

Question 3

Friedreich ataxia

-assoc with what cardiac problem

Answer 3

Hypertrophic cardiomyopathy

Question 3

Which neural degenrative disorder affects both cerebellum and spinal cord?

Answer 3

Friedrich ataxia

Question 5

Poliomyelitis

-clinical presentation, neurological

Answer 5

Fecal/Oral, spreads to CNS by blood

• affects ventral horns of spinal cord, so LMN dysfunction
• paralysis, weakness, etc

Question 5

Baby is born a “Floppy baby,” dies few years after birth.

-think what neurological problem?

Answer 5

Werdnig-Hoffman disease

(similar to polio)

auto reces

Question 6

Syringomyelia

• mech
• usual affected location
• clinical presentation (3)

Answer 6

• cystic degeneration of spinal, usu at C8-T1
  1. Affects spinothalamic tract but not Dorsal column, so:
• loss of pain/temp, sparing of fine touch/vibration/proprioception
  2. Anterior horn–LMN: extremity weakness
  3. Symapthetic lateral horns (affecting T1)– Horner’s syndrome

Question 7

How to distinguish ALS from syringomyelia?

Answer 7

No sensation loss (pain/temp) in ALS. Syringomyelia may have LMN motor weakness in addition to loss of pain/temp sensation (spinothalamic)

Question 9

Horner’s syndrome

-presentation (3)

Answer 9

loss of sympathetic trunk

1. miosis (constricted pupil)
2. ptosis (droopy eyelid)
3. anhydrosis (no sweating)

Question 11

Wednig-Hoffman disease

-presentation

Answer 11

• similar to Polio
• Auto recess inherited degeneration of ventral motor horns. LMN weakness
• Presents as “floppy baby,” dies few years after birth

Question 11

Friedreich ataxia

• etiology/mech (HY)
• what population, what is prognosis?

Answer 11

• auto recess expansion of GAA unstable trinucleotide repeat in Frataxin gene
• Frataxin is important for mitochondrial Fe regulation. Loss results in Fe buildup and free radical damage with Fenton reaction.
• presents in early childhood, patients are wheelchair bound in a few years

Question 13

ALS:

• causes:
  1. most common (what population)
  2. rare, HY cause

Answer 13

1. sporadic, in middle aged adults
2. Zinc-copper SOD1 (superoxide dismutase) mutation leads to free radical injury

Remember:

O2- –> H2O2

SOD

Question 14

Pt presents with hands that are weak and have atrophy, but without loss of sensation.

Think what?

Answer 14

ALS–classic early presentation

(and not syringomyelia b/c no loss of pain/temp sensation)

Question 15

Pt presents with hands that have lost sensation to pain/temp, but not touch and vibration.

think what?

Answer 15

syringomyelia

Question 16

Hypertrophic cardiomyopathy

-assoc with what neural disorder?

Answer 16

Friedrich ataxia