GBS

Question 1

Guillain-Barré Syndrome is an acute, ___-mediated, inflammatory, demyelinating disorder with potential for chronic implications. The __ system attacks __ cells in PNS. Syndrome has multiple variant forms, the most common is ___ (ADIP). Also GBS is the most common type of __ __ __. Sensory, autonomic, and __ abnormalities may occur, but GBS is characterized by rapidly progressive ___, SYMMETRICAL motor weakness AND __ reflexes.

Answer 1

1) immune
2) immune system
3) Schwann cells
4) Acute Demyelinating Inflammatory Polyneuropathy
5) Acute paralytic neuropathy
6) brainstem
7) ascending
8) diminished

Question 2

GBS peaks in frequency in young adults AND ____ decades. Incidence rates ___ with age (20% for every 10 year age increase), and is highest >__ years. Infections are associated w/ a ___ relationship and incidence is higher in the ___ gender.

Answer 2

1) 5-8th decades (40s-70s)
2) increase
3) >60 y/o
4) seasonal
5) male

Question 3

The triggering cause can be __ but more than 1/2 of cases occur after a bacterial or viral infection. ___ is found in undercooked food, esp. poultry. More than 1/2 of pt have a PMHx of URI or GI viral infection w/in the last month (4 wks). Other viral etiologies in __ virus, Epstein-Barr virus, and ____.
Other connections include __ disease, allergic response, post-trauma (surgery, post-natal), and __ which are VERY rare.

Answer 3

1) idiopathic
2) Campylobacteriosis
3) Zika virus
4) COVID-19
5) autoimmune disease
6) vaccinations

Question 4

Progression of S&S occurs from __ hours to __ days before plateau is reached. “__” (plateau) phase is __ wks then recovery occurs __ → __. Cardinal S&S include __ __, __ motor weakness with progression distal → proximal & __ weakness before __ weakness, and __reflexia

Answer 4

1&2) 12 hours to 28 days (rapid progression)
3) nadir
4) 2-4 weeks (days to 1 month)
5&6) proximally → distally
9&10) rapidly progressive, symmetrical
11) leg → arm weakness
12) hypo or areflexia

Question 5

CN involvement is common, with CN __ most frequently involved. This CN’s involvement manifest as bilateral ___ weakness. Other CNs involved that manifest double vision are CNs __, __, & __. Dysphagia and laryngeal paralysis also can occur d/t CN __ and __ involvement.

Answer 5

1) Facial N (CN 7)
2) Facial
3-5) CN 3, 4, & 6 (oculomotor, trochlear, abducens Ns)
6&7) CN 9 & 10 (glossopharyngeal & vagus Ns)

Question 6

Another common S&S is BRUTAL ___ or __ pain. Autonomic dysfunction can cause orthostatic hypotension (BP fluctuation), __cardia, and arrhythmias. Respiratory difficulties can occur but are not common. Sensory paresthesias and hyperesthesia can occur but will be seen much __ than motor impairments. __/__ ctrl remain intact.

Answer 6

1&2) neuropathic or msk pain
3) tachycardia
4) much LATER than motor
5&6) Bowel/Bladder

Question 7

NINDS dx criteria for GBS required what S&S for dx? (2)

Answer 7

1) Progressibe, symmetrical weakness of legs initially and arms (from mild weakness, to total paralysis)
2) Areflexia or Hyporeflexia in affected limbs
our cardinal symptoms

Question 7

Components used to dx GBS include clinical exam, MRI which shows ___ of spinal N roots, NCVs (N conduction block, decr. amplitude or absent distal motor AP, decr. CV, incr. latency), and CSF exam showing ___ elevation by the 2nd week.

Answer 7

1) thickening/swelling of spinal N roots
2) protein

Question 8

5 Supportive features part of NINDS criteria for GBS.
these are not required for dx, but support dx

Answer 8

1) Progression of symptoms <4 weeks (reach nadir in <4 wks)
2) Symmetry
3) Mild sensory symptoms or signs
4) Cranial nerve involvement, especially bilateral facial nerve weakness
5) Recovery starting 2-4 wks after nadir STARTS
6) Autonomic dysfunction
7) Pain
8) No fever at the onset
9) Elevated protein in CSF
10) Electrodiagnostic abnormalities consistent with GBS

Question 9

According to NINDS, these 5 features make GBS dx DOUBTFUL

Answer 9

1) Sensory loss > motor loss
2) asymmetry of weakness
3) Bowel and bladder dysfunction (at onset or severe/persistent)
4) Severe pulmonary dysfunction with little/no limb weakness at onset
5) Fever at onset (infection should be long gone by the time GBS presents)

Question 10

3 other variants of GBS besides AIDP are __, __, & __. __ presents with ophthalmoplegia, ataxia, & arreflexia. ___ (AMAN) and ___ (AMSAN) have axonal involvement & will be more severe in presentation. AMAN presents w/ muscle weakness, occasionally preserved ___, & sensory is SPARED. AMSAN impacts both __ and ___ nerves.

Answer 10

1-3) Miller-Fisher Syndrome, AMAN, & AMSAN
4) Miller-Fisher Syndrome
5) Acute Motor Axonal Neuropathy
6) Acute Motor-Sensory Axonal Neuropathy
7) DTRs
8&9) motor and sensory Ns

Question 11

The earlier tx starts, the better! There is NO cure for GBS. Primary management goal is ___ __ __. Intravenous Immunoglobin (IVIg) uses plasma products made of __ extracted from blood and administers blood products to boost ___ production, blocking __ and antibody binding. IVIg shows benefits for more than 1/2 of pts w/ GBS. It can ___further myelin loss and axonal loss, also can aid in sustained remission.

Answer 11

1) Control Inflammatory response
2) antibodies
3) antigen
4) macrophage
5) PREVENT

Question 12

Plasma Exchange (__) removes blood plasma, separates it into plasma and cells, and transfuses __ back into bloodstream. Tx is 5 exchanges over 2 wks. This medical tx ___ N damage and shows faster clinical improvement. What pt population is this tx recommended for (be specific)?

Answer 12

1) Plasmapheresis
2) cells
3) reduced
4) Pts who CANNOT walk 10 M w/out ast.

Question 13

Acute phase of GBS is the rapid progression of S&S, within __ week(s) 50% of impairment shows and pts reach “nadir” within __ weeks. Pts are usually in hospital. Plateau phase can last from ___ up to ___. PT will be in the recovery phase where most pts regain ___ 2-4 wks after “nadir”. However, __ impairments and __ can persist for years.

Answer 13

1) 1 week
2) 2-4 weeks
3&4) days up to 1 month
5) strength
6) sensory
7) fatigue

Question 14

Potential complications includes resp. issues, pneumonia, prolonged hosp./immobility complications, and relapses. Name 2 more potential complications.

Answer 14

1) autonomic instability
2) pain

Question 15

Most pts recover the ability to ___ within 6 months. Lingering S&S that may be difficult to manage involve mod/severe __, persistent __, and motor deficits like __ __. GBS mortality rate is ___. Total recovery time can take up to ____.

Answer 15

1) ambulate
2) pain
3) fatigue
4) foot drop (or paresthesia)
5) LOW
6) up to 2 years

Question 16

NEGATIVE prognostic indicators include: ___ age at onset, rapid onset (<7 days) ___ admission, hx of ___ illness prior to onset, and an avg distal motor response amplitude reduction to ____ of normal. Name 1 more (-) Prognostic indicator.

Answer 16

1) >60 y/o
2) BEFORE
3) GI (presence of diarrhea)
4) <20% of normal
5) Need for ventilatory support

Question 17

T/F: More than 1/2 of pts w/ GBS will require ventilatory support.

Answer 17

False, 1/3 of pts will need it though!

Question 18

What is the 2 measures are part of the International GBS Outcome Scale (IGOS) to estimate prognosis? Which one evaluates a pt’s risk of developing reps. failure in 1st week of admission? What 3 components does the measure assess to evaluate the risk?

Answer 18

1) Erasmus GBS Resp. Insufficiency Score (EGRIS) and Erasmus GBS Outcome Score (EGOS)
2) EGRIS
3) days b/w onset of weakness and hosp. admission, facial/bulbar weakness at admission, and UE/LE strength at admission

Question 19

EGOS estimates pts ability to ___ at __ months and can be used at 1 AND 2 weeks after admission. At week 1, mEGOS assesses ___ at day 7 of admission.
At week 2, the EGOS assesses GBS __ score after hosp. admission. What 2 components are assessed at week 1 AND week 2?

Answer 19

1&2) ambulate at 6 mo.
3) UE/LE strength
4) GBS disability score
5) age at onset & preceding diarrhea in the last 4 weeks

Question 20

There are 2 GBS Specific OMs. Which one is a 7-pt scale of global disability with 0 = healthy and 6 = death?

Answer 20

GBS Disability Scale

Question 21

Overall Disability Sum Score (ODSS) scores __ tasks on range of 0 (healthy) to 12 (severe disability). It can be scored during the __ __ or by the __. ODSS has a SIGNIFICANT association w/ pt’s ___ of clinical condition.

Answer 21

1) functional UE/LE
2&3) pt interview or by the individual
4) own perception

Question 22

Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) presents similar to GBS but takes a chronic course. CIDP has gradual onset of S&S over the course of ___ weeks before any signs of plateau. S&S are motor AND sensory and can be symmetric or __. ___ are common, as CIDP does NOT improve after plateau. Onion bulb changes occur with CIDP as well. What med does CIDP respond to?

Answer 22

1) >8 weeks of S&S progression
2) asymmetric
3) Relapses
4) glucocorticoids