CASE PRESENTATION:

Dr.J.HOBART.
Dr.A.FAWAD.
 History:
 61.yr.old lady,Rtd house keeper.
 Presented on july.03. to G.MEDICS with c/o

1- polyurea, polydipsia. 6/52.

2- wt.loss, lethargy.
At that stage she was diagnosed D.M II.
Her chol. 8.0.
She was D/C home on insulin+statin.
 Readmission:
 Readmitted on Aug.03 under neurology with 1/52
h/0 following complaints.

 Back pain.
 B/L thighs pain.
 Numbness of arm+legs in glove/stocking.
 Weakness of legs. RT more than LT.
 At that stage G.P stop her statin in consideration
to high CK…..MYOSITIS ??
 PAST.HX:
 Long standing hx. Of dizziness, s/b E.N.T.
MRI .brain …N.
 Long standing Hx.of palpitation,S/B cardio.

MVP, confirm on echo

 MEDICATIONS: insulin, statin, aspirin,

stematil.
 Family Hx. Of D.M.(mother+sister)


 Examination:
 Vital signs stable.
 MOTOR SYST.
 Power L.L. RT. LT.
HIP 2/5 3/5.
knee 2/5 3/5.
ankle 4/5 3/5.
 Tone……N.
 Reflexes…..absent. Planters…..down going.
 SENSORY SYST: Diminished touch pin prick
Vibration sensation in glove and stocking fashion.
 Investigations:
 FBC,U.E,LFT,TFT…….N.
 Myeloma Screen….N.
 Autoantibody Screen….N.
 B12,Folate,ferritin…….N.
 ACE.levels……..N.
 Syphilus Serology…..neg.
 CXR………..N.
 Antiganglioside antibody…..neg.
 MRI. Lumber spine …………N.
 Lumber puncture…….. Unsuccessful.
NEUROPHYSIOLOGICAL:
Progress during rehabilitation:
5 day course of IVIG….No significant
change.
 TRIAL of steroids..
 Power improved.
 Reflexes still absent.
 Sensory symptoms improved.
 Walking with stick.
 Chronic inflammatory
Demyelinating
Polyradiculoneuropathy.

(CIDP)
 INTRODUCTION:
 CIDP is an Acq. Demyelinating.Ds.of
P.N.S, characterized by relapsing/prog.
Proximal and distal muscle weakness with
possible sensory loss.
 In fact CIDP. Considered the peripheral
counterpart of M.S. b/O relapsing /prog.
Course and focal demyelination , axonal
loss.
 PATHOGENSIS:
 Morphology: CIDP is a multifocal
predominantly proximal,inflammatory
affect spinal roots, spinal nerves, major
complexes,or nerve trunks.
 CIDP lesions extends in P.N.S including
I/M. nerves sympathetic trunks, and
autonomic nerves.
 Inflammation. Demyelination.


 Axonal loss. Remyelination.

 INFLAMMATION
epineurial +endoneurial
T CELLS: MACROPHAGES:
activated; upregulated MHC class ii expression.
 DEMYELINATION.
-macrophages mediated.
multifocal.esp with active demyelination.
- segmental.
-thin myelin sheath.
ONION bulb formation. In chronic cases (20%).
 Photograph of onion ring.
 ONION BULBS


Early onion bulb formations

Note the few extra layers of basal
lamina around several thinly
myelinated larger axons.

Electron micrograph: From Robert Schmidt MD

 Chronic Immune Demyelinating
Polyneuropathy (CIDP)


Thinly myelinated axons.

Axonal loss.

Thinly myelinated axons.

Patchy axonal loss.
 Myelin stripping
Phagocytic cell process (Arrow) extends
beneath a layer of myelin.
 REMYELINATION:
Few lamellae of loosely compacted
myelin.
schwann cell cytoplasm rich in organelles.
schwann cell mitosis.
AXONAL LOSS: variable vasicular
involvement.
patchy loss of axon.
axonal regeneration in 20%.
 EPIDEMIOLOGY:
 Male more as compared to female.
 Onset age ; average 50. Yr.
 Prevalence: 1/100,000
 Preceding event; increased frequency of
immunization or infection.
 Clinical features:
 ONSET:
slowly progressive weeks to mon.
 WEAKNESS: proximal+distal.
symmetric.
most disabling feature.
SENSORY LOSS: all modalities.
distribution, legs more than arm
PAIN: 20% do have a pain.
AUTONOMIC: micturation changes.
horner’s syndrome.
TENDON REFLEX: reduced /absent in 90%.
 ASSOCIATED DISORDER:
 Monoclonal antibodies.
 Diabetes mellitus.
 HIV.
 Hepatitis C infection.
 Sjogren syndrome.
 Inflammatory bowel .Ds.
 Diagnostic criteria

 Clinical HX.
 Neurological examination.
 Electrophysiological studies.
 Pathological appearance.
 C.S.F findings.
 Clinical criteria
 Pattern of clinical involvement:
motor/sensory dysfunction involving more
than one limb.

 TIME COURSE: at least 2/12.

 REFLEXES: areflexia or hyporeflexia

 Electrophysiological criteria:
 AT least 3 of 4 criteria must be met.
 1 - partial conduction block. Def,prob,possb must
be present in at least 1 motor.N.
 2 - conduction velocity must be abnormal in at
least 2 motor nerves.
 3 - distal latency must be abnormal in at least 2
motor nerves.
 4 - F- waves must be abnormal in at least 2 .N
 Pathological criteria:
 Sural.n biopsy:
mandatory: evidence of
demyelination/remyelination.
supportive: evidence of
perineurial/endoneurial,onion bulb
formation with mononuclear infiltration.
 C.S.F CRITERIA:

 mandatory: cell count less than 10/mm.

 Supportive: elevated protein.

 ThisAAN criteria allow the diagnosis of
CIDP into……….
 DEFINATE CIDP.
 PROBABLE CIDP.
 POSSIBLE CIDP.
 TREATMENT OPTIONS:
 1-Prednisone: 60-100mg/d,p/o. than taper.
 Start taper after 3-6 month or clinical
improvement begins.
 many pt. Will relapse if prednisone is
stopped without additional immunosuppres.
 2- methylprednisolone: 1 gm/d.than
additional i.v/p.o weekly to monthly.
 3- I.V Immune globulin:
 1.gm/kg/d for 2 days;repeat 3 to 8 week.
 0.4gm/kg 1 to 2 times per week for 8weeks.
 4- cyclosporine :
 5- plasma exchange:
 6- interferon alpha 2 A.
 REFERANCES:
 NEUROLOGY:

1- vol 59.no12.supplement 6. 2002.

2- vol 60.no 8 .supplement 3. 2003.
www.neuromuscular homepage.com.