 Bentuk kronik dari acquired demyelinating neuropathies  Bentuk akut dan kronik mungkin sulit dibedakan saat awal. Identifikasi biasanya dari perjalanan klinisnya  Immune mediated.

 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 Inflammatory Demyelinating polyneuropathy Acute Motor axonal Neuropathy (AMAN) Fisher syndrome AIDP with Secondary Axonal degeneration Acute motor- Sensory axonal Neuropathy (AMSAN) Increasing Severity Of the immune attack Fig 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].)

 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

SLOWLY PROGRESSIVE STEPWISE PROGRESSIVE SLOWLY MONOPHASIC RELAPSING PROGRESSIVE RELAPSING REMITTING MONTHS / YEARS

DISTINGUISHING FEATURES BETWEEN ACUTE GUILLAIN –BARRE AND CIDP Features Typical GBS Typical CIDP Antecedent event 70 % % Onset to maximal deficit4 wk6 mo to several years Fluctuations UncommonCommon Ophthalmoplegia10-20 %< 5 % Respiratory failure requiring30 %Seldom ventilation Autonomic dysfunction 50 %Seldom Normal CSF protein level10 %90 % Early electromyographic Proximal conduction Marked slowing of motor nerve (EMG) findings block conduction HLA associationNoneA1 : B8 DRw3

AuthorNo.Follow - UpOutcome Dyck et al * 53 Mean 7.4 yr Complete recovery 4 % Moderately disabled 68 % Wheetchair-bound 28 % Prineas and McLeod Mean 3 yr (range yr)Complete recovery26 % Moderately disabled74 % McCombe et al yr (range 1-41 yr)Complete recovery30 % Minimal Impairment43 % Moderate Impairment 18 % Wheelchair-bound 2 % Died 7 % Barohn et al mo (range mo)Responded to treatment 95 % Died 3 % Twelve percent died (6 related to CIDP.3 to other diseases)

 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

 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 › Progresive symmetric weakness › Cease by 4 weeks › areflexia  Considerable clinical variability  Untreated mortality 15% (at least)

 Antecedent infection70%  Distal weakness predominantly44%  Cranial nerve weakness43%  Paresthesia and pain43%  Meningeal irritation17%  CSF protein > 45 mg/dl88%  Asymmetry of involvement 9%  Full recovery or mild impairment77%  Relapses 7%  Mortality 4%

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 Features required for diagnosis : Progressive motor weakness of more than one limb Areflexia or marked hyporeflexia

weeks yearsweeks Months/years weeks Months/years Figure Possible temporal courses following acute GBS ACUTE MONOPHASIC GBS ACUTE MONOPHASIC GBS WITH LIMITED RELAPSE RELAPSING ACUTE MONOPHASIC GBS CIDP STARTING AS GBS ACUTE GBS FOLLOWED BY CIDP

 Transverse myelitis  Acute spinal cord compresion  Botulism  Tick paralysis  Myastenia gravis  Periodic paralysis  Poliomyelitis  Acute inflammatory myopathies

 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

 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

Severity of Illness Mild  Able to walk  No cardiovascular dysautonomia Moderate  Unable to walk, but lifts limbs from bed or chair  Oropharyngeal weakness but swallows safety Severe  Unable to lift limbs  Aspiration risk  Blood pressure fluctuations Treatment  Observe : treat with plasma exchange if still worsening  Active physical therapy exercises as tolerated  Begin plasma exchange/IVIG  Passive physical therapy  Plasma exchange/IVIG if hemodynamically stable  Passive physical therapy and splinting

 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

 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

 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.