Continuum PNS and MND: GBS + CIDP

Question 1

History and Epidemiology

Answer 1

Eichorst 1890 - Dyck coined - 3 /100k

Question 2

Dx of CIDP : Exclude …

Answer 2

other forms of DM (MMN/Contactin/Neurofascin/IFE/VEGF/MAG/CMT De/TTR Amyloid)

Question 3

Name 7 types of CIDP

Answer 3

CIDP typical (50% the rest is 5-15%), Acute CIDP, Sensory CIDP, CISP, Motor CIDP, MADSAM, DADS

Question 4

Clx Features: Acute CIDP

Answer 4

nadir by 8 weeks

Question 5

Clx Features: Sensory CIDP

Answer 5

sensory predominant : sensory ataxia : hyporeflexia

Question 6

Clx Features: CISP

Answer 6

pure sensory ataxia - NCS normal (SSEP or CSF analysis)

Question 7

Clx Features: Motor CIDP

Answer 7

predominant motor (okay to have some NCS changes)

Question 8

Clx Features: MADSAM

Answer 8

Asymmetric Distal predominant motor/sensory

Question 9

Clx Features: DADS

Answer 9

Symmetric Distal sensory>motor (anti MAG >1:1000)

Question 10

Clx Criteria for typical CIDP

Answer 10

symmetric weakness in 4 limbs and proximal in at least one limb

Question 11

CIDP EDX criteria definite (1+)

Answer 11

CMAP DL >50% in two nerves other than median
CV >30% in two nerves
F-wave absence or prolongation (further modifiers)
CB in two nerves >50% (other modifiers)
TD in two nerves >30% duration increase

Question 12

IVIG response times

Answer 12

6 weeks before switching and 12 weeks for max benefit.

Question 13

SCIG vs IVIG

Answer 13

IV works quicker (OPTIC protocol) but SC is easier (weekly dosing .2 or .4 g/kg)

Question 14

Steroids don’t work for

Answer 14

GBS, MMN and Motor CIDP

Question 15

Clx criteria for GBS

Answer 15

symmetric progressive (2-4 weeks) muscle weakness >1limb with hyporeflexia

Question 16

CSF may not be positive in

Answer 16

first week (20%)

Question 17

CN involvement

Answer 17

50%

Question 18

Mechanical ventilation

Answer 18

1/4

Question 19

GBS Variants

Answer 19

AMAN - AMSAN - Miller Fisher (r/o bickerstaff - pure small fiber - pure autonomic -

Question 20

Serial NCS may be required

Answer 20

AMAN vs AIDP CB verification

Question 21

Antiganglioside antibodies are

Answer 21

IGG1 and IGG3 and implicated in AMAN (Gq1b strongest for MF)

Question 22

Triggerring event can be

Answer 22

within 8 weeks

Question 23

2 infections noted to produce molecular mimicry

Answer 23

Mycolplasma pneumoniae and Campylobacter

Question 24

GBS DDx (6)

Answer 24

cord injury - anterior horn cell (WNV/Entero) - poly radic - periodic paralysis - myasthenia crisis - acute myopathies

Question 25

Toxic ddx

Answer 25

hexacarbons (occupational/abuse) organophosphates