Clinical Case: ALS, GBS

Question 1

describe CN dysfunction seen in ALS

Answer 1

• V (motor): weakness of jaw opening, dysarthria
• IX, X: weakness of soft palate, increased gag reflex, dysarthria
• XII: atrophy and fasciculations of tongue, difficulties in protruding tongue bilaterally

Question 2

describe what an EMG in ALS would look like

Answer 2

Question 3

what functions are spared in ALS?

Answer 3

• there are UMN signs (loss of corticospinal tract) and LMN signs (damage to spinal cord motor neurons) but sensory and bowel/bladder functions are spared, as well as extraocular muscles

Question 4

describe bulbar manifestations seen in ALS

Answer 4

• bulbar motor nuclei are affected; difficulty in swallowing, coughing, speaking

Question 5

describe the pseudobulbar affect seen in ALS patients

Answer 5

• patients have “emotional lability”; uncontrollable laughter, crying, smiling
• degeneration of UMN leads to exaggeration of motor expressions and emotions

Question 6

most Guillain-Barre syndrome patients previously had ____

Answer 6

most Guillain-Barre syndrome patients previously had an infection (e.g. virus or bacteria)

Question 7

describe measurement of normal nerve sensory and motor conduction velocities and normal values

Answer 7

• D/ (T2 - T1)
• normal = 55-60 ms

Question 8

describe what happens to nerve conduction velocity in GBS patients

Answer 8

nerve conduction velocity significantly decreases

Question 9

describe normal CSF values and abnormal values in GBS

Answer 9

• normal CSF protein = 15-50
• GBS patient CSF protein = ~85

Question 10

acute weakness/paresthesias isolated to ____ should arouse suspicions of GBS

Answer 10

acute weakness/paresthesias isolated to legs should arouse suspicions of GBS