ALS & GBS - 405

Question 1

ALS

Answer 1

a relentlessly progressive, presently incurable neurogenerative disorder that causes muscle weakness, disability & eventually death
median survival is 3-5 years

Question 2

ALS CM

Answer 2

-asymmetric limb weakness (upper = hand weakness or shoulder girdle, lower = foot drop)
-fatigue
-dysarthia/dysphagia
-muscle atrophy & spasticity
-fasciculations (twitching) of face or tongue
-respiratory muscle weakness
cognitive function stays in tact but body wastes away

Question 3

drug therapy for ALS

Answer 3

riluzole: only FDA approved for ALS, not curative but extends survival time

Question 4

exercise & ALS

Answer 4

guide pt through moderate intensity endurance type exercises for trunks / limbs to reduce spasticity

Question 5

care of ALS

Answer 5

-consults (slp & pt)
-NPO
-pain assessments
-minimize risk for injury
-facilitate patient and family anticipator grieving

Question 6

Guillain Barre Syndrome

Answer 6

acute, immune mediated polyneuritis - a progressive (ascending) fairly symmetric muscle weakness accompanied w/ absent or depressed deep tendon reflexes
can range from mild difficulty walking to nearly complete paralysis

Question 7

w/ GBS where does paresthesia usually occur

Answer 7

hands & feet

Question 8

w/ GBS where is pain usually located

Answer 8

back & extremities

Question 9

GBS dysautonomia

Answer 9

-tachycardia
-labile blood pressure
-orthostatic hypotension
-ileus
-urinary retention
autonomic nervous system

Question 10

what usually precedes GBS

Answer 10

a viral infection, immunization, trauma or surgery

Question 11

dx of GBS

Answer 11

-H&P
-cerebrospinal fluid (only after one week of onset): will show inc protein & normal WBC
-nerve conduction studies & needle EMG

Question 12

GBS plan of care

Answer 12

-astute, routine physical assessment (disease can progress rapidly and pt can end up on a vent)
-assess gag & corneal reflexes
-frequent VS
-possible dysphagia diet if needed
-pain mgt (neuropathic)
-monitor for urinary retention

Question 13

plasmapheresis

Answer 13

removes circulating antibodies, complement & soluble biological response modifiers
-4 to 6 treatments over 8 to 10 days

Question 14

GBS main modalities of therapy

Answer 14

-plasmapheresis
-IVIG
administer within 4 wks of onset

Question 15

GBS prognosis

Answer 15

-95% survive
-75% completely recover
-25-30% have residual weakness after 3 years
-3% may suffer relapse of muscle weakness & tingling many years post initial attack