Neurology Case
Omar Jawdat, MD
9/14/2012
CC and HPI
• CC: Elderly woman with weakness in all 4
extremities
• Initial complaint: right biceps myalgia followed
by right shoulder and hand weakness
• 2 wk later right foot drop
• 2 months later: weakness all 4 extremities,
can not walk, numbness on feet and fingers
• Rx with IVIG with good response for 5 wks
• 2-3 relapses, Rx with IVIG in 3-5 wk intervals
HPI, PMH, FHx and SHx
• C-Spine MRI shows severe central stenosis C6C7  Surgical decompression 1/2011
• Post op, patient is worse, IVIG, Decadron, PLEX
and then transferred to KU on 2/2011
PMH:
• HTN, Hypothyroidism, GERD, right IJ venous
thrombus on Coumadin
FHx/SHx:
• No family history of neurologic conditions,
homemaker, quit smoking 2001, drinks
occasionally, No recreational drugs
Examination
• MS: A & O x 4
• Speech: Fluent without dysarthria
• CN: RERRLA, Full Fields, EOMI, normal sensation,
palpebral fissure 8/9mm, no ptosis, Orb Oculi 4,
Orb Oris 5-. Palatal elevation is symmetric.
Sternomastoid and trapezii are 5. Normal tongue
with no fasciculation, power 5.
• Motor:
SAb
EF
EE
WF
WE
FE
FF
FAb
HF
KF
KE
ADF
APF
TE
1
3
3
3
3
1
2
2
2
3
3
2
2
2
2
4
4-
4-
4-
2
3
3
3
4
4
3
2
3
• Normal tone, bulk, no atrophy or fasciculation.
Neck Flexion 3+
Examination
• Sensory: diminished LT/PP in feet
Vibration: R/L toes 0, ankles 3/0, lower tibia 3/2
sec fingers 3 sec. Proprioception intact
• DTR: 0 all over, Flexor planter response, -ve
Hoffman, -ve jaw jerk.
• Coordination and gait: too weak to perform.
Work Up
Serum
Serum
Serum
CSF
HgbA1C
5.5
ESR
1
SPEP
-ve
WBC
2
B12
351
CRP
0.16
HIV
-ve
RBC
10
Homocysteine
16.7
ANA
>80
HBsAg
-ve
Prot
157
MMA
0.14
ds ANA
<10
Anti HBs
142
Gluc
68
MCV
96
Anti SSA
-ve
Anti HBc IgM -ve
OCB
-ve
CK
90
Anti SSB
-ve
Anti HCV
Lyme
-ve
F Cyto
-ve
TSH
8.58
RF
<20
F T4
1.3
GM1 Ab
-ve
Copper
1.31
cANCA
-ve
Ceruloplasmin
25.8
pANCA
-ve
Zinc
0.83
C3
nl
RPR
-ve
C4
nl
ACE
8
CH50
nl
-ve
Cryoglobulin -ve
• CT Chest: -ve for masses or LAP
• CT Abd: -ve for masses or LAP
• Where?
• What?
NCS/EMG
1. Severe axonal demyelinating sensorimotor
neuropathy. There is marked demyelination
2. Right peroneal
neuropathy at the knee
Differential Dx
•
•
•
•
•
•
MADSAM (Multifocal acquired demyelinating sensory motor neuropathy)
MMN (multifocal motor neuropathy)
POEMS
Mononeuritis muliplex
Classic CIDP
Infectious and infiltrative processes such as HIV,
Lyme, sarcoidosis
• Neoplastic invasion of spinal roots by lymphoma
• HNPP
Clinical Spectrum of CIDP
• CIDPs form a heterogeneous group of NP that
share one common feature, demyelination of
peripheral nerves
• First reported by James Austin, MD in 1958,
over 50 years after Guillan, Barré and Strohl
described Acute ascending paralysis
• Classification is based on clinical features, lab
findings and associated systemic disease
Classification by clinical pattern
Clinical feature
Classic CIDP
DADS
MADSAM
MMN
Weakness and
Sensory loss
Symmetric,
distal and
proximal
Symmetric,
mild distal
Asymmetric,
mostly distal
Asymmetric,
purely motor,
mostly distal
Elevated CSF
Protein
Yes
Yes
Yes
No
M Protein
Uncommon,
IgG or IgA
Usually IgM,
rarely IgG
Uncommon
Uncommon,
rarely IgM
Antineural
Antibodies
Uncommon
Anti MAG in
50% of patients
Uncommon
Anti GM1
Motor NCS
Demyelination
Demyelination
Demyelination
Demyelination
Sensory NCS
Absent or small
SNAPs
Absent or small
SNAPs
Absent or small
SNAPs
Normal
Treatment
Prednisone,
IVIG, PLEX,
Immunosuppre
ssants
Possibly
Rituximab, IVIG
for patients
without AntiMAG
IVIG,
IVIG, possibly
Prednisone,
Cyclophospham
Immunosupress ide
ants
Variants of CIDP
• Temporal variants
- SIDP (subacute presentation)
• Concurrent illness variants
HIV, Lymphoma, POEMS, MGUS, Hep C, IBD,
connective tissue disease
• Distribution variants
DADS neuropathy, MADSAM neuropathy, MMN
and classic CIDP
MADSAM
• Also called Lewis-Summer syndrome
• Unlike MMN but similar to CIDP, CSF protein is
elevated
• Asymmetric pattern of sensory and motor
deficits and like MMN, it starts distal.
• Electrophysiologic evidence of sensory and
motor nerve involvement, motor
demyelination, TD and motor conduction
block
Treatment
• Responds to IVIG
• Responds to Corticosteroids as opposed to
MMN patients
• Our patient responded very well to PLEX
acutely with IVIG and IV methylprednisone
• Currently on Prednisone, IVIG q4wk and
CellCept
Conclusion
• This case reports a patient with chronic
progression of asymmetric numbness and
weakness in four extremities.
• MADSAM neuropathy was diagnosed after clinical
and laboratory evaluations and to look for other
possible differential
• It is very important to distinguish between CIDP,
MADSAM neuropathy, and MMN by clinical,
laboratory, and electrodiagnostic features
because of different effective therapeutic
strategies