CIDP

Bentuk kronik dari acquired demyelinating

neuropathies
Bentuk akut dan kronik mungkin sulit
dibedakan saat awal. Identifikasi biasanya
dari perjalanan klinisnya
Immune mediated.
CIDP
Gambaran klinis awal dapat seperti anak
dengan distrofi atau miopati yang lain
Kadang-kadang perjalanan klinisnya
lambat , sakit (-), kelumpuhan distal atau
proksimal.
Terjadi pada semua usia (2-70 tahun)
Penting diketahui secara dini, karena
respons terhadap kortikosteroid atau
imunoglobulin sangat baik.
 Guillian-Bare Syndromes

Acute Acute Fisher

Inflammatory Motor axonal syndrome
Demyelinating Neuropathy
polyneuropathy (AMAN)

Increasing
Severity
Of the immune
attack AIDP with Acute motor-
Secondary Sensory axonal
Axonal Neuropathy
degeneration (AMSAN)

Fig. 22-3. Proposed interrelationships of the forms of GBS. (Reprinted with permission
From Griffin et al., Pathology of the motor-sensory axonal Guillian-Barre syndrome,
Ann Neurol 39:17 28, 1996 [41].)
 Kriteria diagnostik (AAN91)
Disfungsi motor dan sensori pada lebih
dari satu ekstremitas yang progresif atau
relaps, dan berlangsung lebih kurang 2
bulan.
Arefleksia atau hiporefleksia, biasanya
pada ke 4 ekstremitas
Temporal courses of CIDP

SLOWLY
PROGRESSIVE

STEPWISE
PROGRESSIVE

SLOWLY
MONOPHASIC

MONOPHASIC
RELAPSING

PROGRESSIVE
RELAPSING

RELAPSING
REMITTING

MONTHS / YEARS
 DISTINGUISHING FEATURES BETWEEN
ACUTE GUILLAIN BARRE AND CIDP

Features Typical GBS Typical CIDP

Antecedent event 70 % 20-30 %

Onset to maximal deficit 4 wk 6 mo to several years
Fluctuations Uncommon Common
Ophthalmoplegia 10-20 % <5%
Respiratory failure requiring 30 % Seldom
ventilation
Autonomic dysfunction 50 % Seldom
Normal CSF protein level 10 % 90 %
Early electromyographic Proximal conduction Marked slowing of motor nerve
(EMG) findings block conduction
HLA association None A1 : B8 DRw3
 Table 10-7 OUTCOME IN LARGE CIDP SERIES

Author No. Follow - Up Outcome

Dyck et al..310 1975* 53 Mean 7.4 yr Complete recovery 4%

Moderately disabled 68 %
Wheetchair-bound 28 %
Prineas and McLeod.957 1976 23 Mean 3 yr (range 0.5-31 yr) Complete recovery 26 %
Moderately disabled 74 %
McCombe et al..773 1987 76 10 yr (range 1-41 yr) Complete recovery 30 %
Minimal Impairment 43 %
Moderate Impairment 18 %
Wheelchair-bound 2%
Died 7%
Barohn et al..80 1989 60 34 mo (range 6-142 mo) Responded to treatment 95 %
Died 3%

Twelve percent died (6 related to CIDP.3 to other diseases)

 Guillain-Barre symdrome
Is an acquired desease of the peripheral
nervus system
Acute inflammatory demyelinating
polyradiculoneuropathy; infectious neuronitis;
acute infectious polyneuritis
Is an immune-mediated disease directed
against the peripheral nervous system
The most serious complications are
respiratory failure and autonomic
disturbances
 Guillain-Barre syndrome
Commonest cause of acute generalised
paralysis
0.6 1.1 per 100,000 (<15 yrs)
Any time during childhood (4 and 9 yrs)
Core symptom
Progresivesymmetric weakness
Cease by 4 weeks
areflexia
Considerable clinical variability
Untreated mortality 15% (at least)
 Clinical Characteristics of 56
children with GBS
Antecedent infection 70%
Distal weakness predominantly 44%
Cranial nerve weakness 43%
Paresthesia and pain 43%
Meningeal irritation 17%
CSF protein > 45 mg/dl 88%
Asymmetry of involvement 9%
Full recovery or mild impairment 77%
Relapses 7%
Mortality 4%
 Diagnostic Features of Guillain-Barre Syndrome
Features required for diagnosis :
Progressive motor weakness of more than one limb
Areflexia or marked hyporeflexia

Features strongly supportive of the diagnosis :

Progression over days to a few weeks
Relative symmetry
Mild sensory loss
Onset with extremity pain or discomfort
Cranial nerve involvement
Onset of recovery 2 to 4 weeks after halt of progression
Autonomic dysfunction
Initial absence of fever
Elevated CSF protein level after 1 week of symptoms
Abnormal electrodiagnosis with slowed conduction or prolonged
F Waves
 ACUTE
MONOPHASIC GBS

weeks
ACUTE MONOPHASIC
GBS WITH
LIMITED RELAPSE
weeks

RELAPSING ACUTE
MONOPHASIC GBS

weeks years weeks

CIDP STARTING
AS GBS

weeks Months/years

ACUTE GBS
FOLLOWED BY CIDP

weeks Months/years
Figure 10-1.Possible temporal courses following acute GBS
 Differential diagnosis
Transverse myelitis
Acute spinal cord compresion
Botulism
Tick paralysis
Myastenia gravis
Periodic paralysis
Poliomyelitis
Acute inflammatory myopathies
CSF protein content
 EMG
Diagnostic aids in detecting GBS
To determine and to differentiate axonal or
myelin disorder
Not pathognomonic, when present strongly
suggest the diagnosis
Present in 50% during the first 2 weeks and
80% the third week
Motor conduction, sensory conduction, F
waves and needle electromyography
 Approach to treatment
Severity of illness
Mild(able to walk)
Moderate (unable to walk, but lift limbs)
Severe (unable to lift limb)
Corticosteroids are not helpful, they tend to
prolong the course and possibly
contraindicated
Plasma exchange (plasmapheresis)
Intravenous immune globulin (IVIG)
physioterapi
 Table 16-1 SUMMARY OF SURVEY LANCE AND
APPROACH TO TREATMENT IN ACUTE STAGES OF GBS

Severity of Illness Treatment

Mild
Observe : treat with plasma
Able to walk
exchange if still worsening
No cardiovascular dysautonomia
Active physical therapy
exercises as tolerated
Moderate
Unable to walk, but lifts limbs
Begin plasma exchange/IVIG
from bed or chair Passive physical therapy
Oropharyngeal weakness but
swallows safety
Severe Plasma exchange/IVIG if
Unable to lift limbs
hemodynamically stable
Aspiration risk
Passive physical therapy and
Blood pressure fluctuations
splinting
Case 1
10 yrs old girl had a 3-day history with increasing
difficulty walking. Two weeks earlier, she had
URTI.
On examination. Alert, no facial weakness and no
trouble with chewing/swallowing. Lower
extremities demonstrated flaccid paraplegia. Arm
and hand strength were decreased. Sensory
examination was intact to touch.
EMG findings compatible with moderate -severe
demyelinating polyneuropathy. No lumbal
puncture was performed
Case 1
She had IVIG (400 mg/kgBB/day) for 3
consecutive days. Headache was noted on the first
day of administration.
Improvement were seen after the first dose of
IVIG and much better after the third.
On days 9, she could walk with slight assistance.
On days 16, she walked unsupported
Case 2
A 4-year-old boy was well until the evening before he was
seen when he suddenly had problem with walking.
There was unilateral ptosis, and facial weakness. No bulbar
dysfunction or respiratory insufficiency. Absent deep tendon
reflexes, and reflexes in upper extremities were slightly
diminished
Lumbal puncture was not done. The diagnosis of GBS was
confirmed by the EMG 6 days after onset of symptoms.