Neurology: Spinal Cord Disease Flashcards Preview

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Flashcards in Neurology: Spinal Cord Disease Deck (20):
1

Describe Werdnig Hoffman Disease.

Spinal Muscular atrophy:
-degeneration of anterior horn due to chromosome 5q deletion preventing "survivor motor protein" from being synthesized.
-no UMN signs

Usually occurs in babies (floppy baby), weak intrauterine kicking, suckling, and respiratory failure

2

Describe Kugelberg Welander Disease.

Spinal Muscular atrophy:
-degeneration of anterior horn due to chromosome 5q deletion preventing "survivor motor protein" from being synthesized.
-no UMN signs

Oropharyngeal muscles spared, kids usually live longer that WHD patients.

3

Name two biochemical causes of Lou Gehrig Disease (amyotrophic lateral sclerosis)

1. Gain of function mutation in superoxide dismutase

2. Decrease in VEGF function leading to spinal cord ischemia

4

Name two motor regions spared in ALS.

1. Extraocular muscles
2. Urinary sphincter muscles

!All sensory is spared!

5

What is bulbar palsy?

Brainstem palsy: affects talking, swallowing, breathing, etc.

6

How can ALS be differentiated from:
1. Cervical spondylosis
2. Syringomyelia

Both have sensory deficits, ALS does not

7

How is ALS diagnosed on EMG?

There are 4 axes measured on a full body EMG. Cranial, cervical, thoracic, and lumbar.

If a decreased conductance is detected in at least 3 axes, the EMG is positive for ALS.

8

What is the general prognosis for ALS.

Poor, patients usually die within 5 years from respiratory failure.

9

What is an Arnold-Chiari malformation.

Malformation in the cerebellum where the tonsil herniates thru the foramen magnum. This decrease in the space of the foramen can lead to syringomyelia.

10

Describe Subacute Combined Degeneration.

B12 deficiency leads to pernicious anemia which can affect both CNS and PNS.

The main lesion is demyelination in posterior columns.

Leads to UMN signs, ataxia, and somatosensory loss.

11

How can you diagnose and treat Subacute Combined Degeneration?

Check homocyteine and methylmalonic acid levels. Methylmalonic acid is a product that only accumulates in a B12 deficiency.

Treat with intramuscular B12 shots

12

Treatment for Arnold-Chiari malformation.

Surgical expansion of the foramen magnum

13

Which pregnant patients are at risk of having babies that develop spina bifida?

Those on anti-seizure medications which act as folic acid antagonists.

Also women on Vitamin A acne medication can cause this due to Vit. A toxicity.

14

What the hell is nevus flammeus?

A vascular anomaly consisting of superficial and deep dilated capillaries in the skin which produce a reddish to purplish discoloration of the skin. Sometimes a benign symptom of spina bifida

15

Describe Friedreich Ataxia.

Deficiency in the protein frataxin (protein involved in mitochondrial homeostasis) that leads to degeneration of the posterior funiculus, lateral cortical spinal tracts, spinocerebellar tracts, dorsal roots, AND clarke's nucleus.

16

How do patients often present with Friedreich Ataxia?

Babinksi sign with NO reflexes even after spinal shock (posterior roots destroyed).

Pes Cavus and maybe scoliosis

17

Describe cervical spondylosis w/ myelopathy.

Degeneration of vertebral discs leads to compression of the spinal cord.

Reflexes are bouncy.

18

Describe Conus Medullaris Syndrome.

Patients present with:
-urinary and fecal incontinence
-no erection or ejaculation
-pelvic floor paralysis
-REFLEXES SPARED

19

Describe Brown-Sequard Syndrome.

IL weakness, vibratory, discriminative touch, proprioception loss

CL-pain and temp loss

20

What is a distinguishing factor to diagnose anterior spinal artery syndrome?

Paralysis (comlete motor loss), pain and temperature sensation are lost but everything in the posterior columns are fine.