Bells Palsy

Guillain-Barre Syndrome (GBS)

Myastenia Gravis (MG)
Gangguan Nervi Perifer Lain

Fakultas Kedokteran – UMY

Dr. dr. TW. Yuliati, Sp.S, M. Kes
 Bells Palsy
Definisi : Kelumpuhan fasialis perifer akibat proses
non supuratif, non neoplasmatik, non degeneratif
primer namun sangat mungkin akibat edema jinak pd
bagian nervus fasialis di foramen stilomastoideus
atau sedikit proksimal dari foramen tersebut, yang
mulainya akut dan dapat sembuh sendiri tanpa tx

Epidemiologi : Prevalensi Bells Palsy cukup tinggi,

- Inggris 22,4/100.000 penduduk / tahun
- Amerika 22,8/100.000 penduduk / tahun
Dan dapat menyerang pria / wanita disetiap usia
 Bells Palsy

Etiologi : belum diketahui secara pasti.

Kongenital : anomali kongenital (sindr. Moebius)
trauma lahir (fraktur tengkorak,
pendarahan intrakanial, dll )
Didapat : trauma
penyakit tulang tengkorak (osteomielitis)
proses intrakranial (tumor, radang,prdrhn)
proses di leher yang menekan daerah
prosesus stilomastoideus
infeksi tempat lain(otitis media, herpe)
sindroma paralisis N. Fasialis familial
 DIAGNOSA

1. Anamnesa
Bagian atas dan bawah dari otot wajah seluruhnya
lumpuh. Dahi tidak dapat dikerutkan. Fisura
palpebral tidak ditutup dan pada usaha untuk
memejam mata terlihatlah bola mata yang berbalik
ke atas. Sudut mulut tidak bisa diangkat. Bibir tidak
bisa dicucurkan dan platisma tidak bisa digerakkan.
Karena lagoftalmos, maka air mata tidak bisa
disalurkan secara wajar sehingga tertimbun di situ.
PENGOBATAN

1. kortikosteroid, misalnya Prednison harus

diberikan dalam waktu tidak lebih dari 2 hari setelah
timbulnya gejala dan dilanjutkan sampai 1-2 minggu
Dosis : 1mg/kg bb / hari atau 60mg p.o diturunkan
Sec tapp off.
2. Vitamin B1, B6 & B12 dosis tinggi
3. Botox / Botolinum toxin type A atau yang lebih
dikenal dengan botox merupakan alternatif terapi
yang dapat digunakan dan berfungsi untuk relaksasi
otot otot wajah.
4. Aciclovir 400mg 5 kali sehari, selama 7 hari
5. Fisioterapi
Saran
- Istirahat terutama pada keadaan akut
- Tiap malam mata diplester
- Hindari sentuhan langsung dengan angin
KOMPLIKASI
1. Fenomena air mata buaya (waktu makan keluar air
mata (regenerasi saraf otonom yg salah arah)
2. Kontraktur otot wajah
3. Sinkinesis – gerakan sadar menutup mata terjadi
pengangkatan sudut mulut, kontraksi otot platisma /
kerutan dahi(regenerasi saraf mencapai otot yg salah)
4. Spasme otot wajah
5. Ptosis alis
6. Bells palsy rekuren
Georges Guillain
 Guillain-Barre Syndrome

● Classic GBS / acute inflamatory demyelinating polyneuropathy / AIDP

= Acute Ascending Paralysis
= Landry Guillain Barre Strohl Syndrome
= Polineuritis pasca infeksi

● Merupakan kondisi polineuropati akut, dimana trjd paralisis ascenderen,

akibat proses autoimun dengan respon inflamasi pada radiks dan saraf
tepi ( poliradikulopati dan polineuropati ) - (Rowland,1995)

● Variants :
♣ Acute motor axonal neurophaty (AMAN) = pure motor involvement
♣ Acute motor & sensory axonal neurophaty (AMSAN) = mixed sensorimotor
♣ Miller Fesher synd.=clinical triad of extraocular weakness, areflexia, ataxia
♣ A pharyngo – cervico – brachial variant
♣ Pure sensory GBS
♣ Acute pandysautonomia
 Neuropati

Klinis:
- Pada proses demielinisasi gangguan fungsi motorik
akan lebih nyata dibandingkan fungsi sensorik
- Serabut saraf sensorik sebagian besar tak bermielin
 Guillain-Barre Syndrome
♣ GBS - commonest cause of acute flaccid paralysis in the West
♣ Incideces 1 to 4 per 100.000
The syndrome typically :
♣ Rapidly progressive, symetrical
♣ Ascending limb weakness consistent with a polyradiculonrpt
♣ Often with associated cranial nerve involvement
♣ Motor signs and symtoms usually predominate
♣ Loss of tendon reflexes is almost ubiquitous
♣ 2/3s have identifiable preceding event
♣ 50% begin with paresthesias followed by weakness in legs;
10% begin with arm weakness; rarely begins in face
♣ Ophthalmoplegia: partial 15%, total 5%
♣ Autonomic dysfunction in 65%, arrhythmias,
hypotension,urinary retention in 10-15%, pupillary inequality
 Guillain-Barre Syndrome
● Progresses for days to 4 weeks
● Recovery is good, Persistent disabillity 20 %,
15% with severe disability, Death 4-15 %
● CSF: protein may be normal early, elevated in
90% by clinical nadir, cells< 10 in 95%, >50
suggests HIV
● EDX: prolonged F & distal motor latencies,
conduction block 30-40% in routine studies
 Guillain-Barre Syndrome
Diagnosis :
● Electro-clinical diagnosis
● Well established clinical criteria- Asbury’s
● ENMG study should always be done
● Confirm the diagnosis
● Alternate diagnosis
● Electrophysiological classification
● Prognostic
♣ Anti ganglioside antibodies - limited Dx application
♣ CSF exam. – albumin dissociation is useful mainly
to exclude alternative diagnoses (infections)
 Dissosiasi sitoalbumin

Inflamasi dan edema kompresi

Transudasi protein serum ke ruang subarachnoid dan LCS

Protein dalam LCS naik

Hiperemi dan dilatasi pembuluh darah

pasokan protein lebih besar ke LCS

Lumbal Pungsi dan LCS
 KRITERIA DIAGNOSTIK SGB (ASBURY&CORNBLATH,1990)
Tanda yang dibutuhkan untuk menegakkan diagnosis
- kelemahan kedua lengan dan tungkai
- Arefleksia
Tanda yang menyokong diagnosis
- Progresifitas gejala dlm beberapa hari sampai 4 minggu
- Gejala relatif simetri, ggn sensorik ringan, disfungsi autonomik
- Gangguan saraf kranial, terutama saraf fasialis bilateral
- Perbaikan dalam 2 – 4 minggu setelah masa progresif
- Saat awitan tanpa panas
- Kenaikan protein LCS tanpa kenaikan sel ( < 10 sel / mm 3 )
- Gangguan elektrofisiologik yang tipikal
Tanda yang mengacaukan diagnosis
- Gangguan sensorik sesuai dermatom
- Tanda dan gejala bersifat asimetri
- Disfungsi urine et alvi berat dan persisten
- Ditemukan sel > 50 sel / mm 3 cairan otak
Tanda yang menyingkirkan diagnosis
- Terbukti botulisme, miastenia gravis, poliomyelitis atau neuropati toksik
- Kelainan metabolisme porfirin, Sindrom sensorik murni tanpa kelemahan
- Difteria
Specific antecedent infection associated with GBS
 Patofisiologi GBS
(Seneviratne, 2000)

• Molecular mimicry - Presence of lymphocyte of

small vessels in the endoneurium and perineurium
• Lymphocyte infiltration of the nerve
• Macrophage mediated segmental demyelinitation
 Mekanisme GBS (Berger, 2000)

•Generally accepted that GBS has an autoimmune pathogenesis

•Involving both cellular and humoral mechanisms
•Cytokines have an important pathogenetic role
•Immune response in GBS may be directed against glycolipid
components of the axolemma and myelin sheath
•Antibodies to peripheral nerve constituents may activate the
complement cascade and macrophages
•Preceding Camplyobacter jejuni infection may initiate an
antibody response due to molecular mimicry between
carbohydrate epitopes present in the axolemma and the
bacteria's lipopolysaccharide coat
•The immunologic response initially directed at the bacteria also
attack the myelin sheet because they look alike
 Overview of Adaptive Immunity
● Lymphocytes: “command & control,” identify
antigen components, respond specifically,
mobilize other elements and direct the attack
c memory for each antigenic assault
● Antibodies: specialized immunoglobulin
molecules directly neutralize and remove antigen
 Self-tolerance

● The process of self recognition

● T & B cells learn self tolerance during maturation
● Autoimmunity occurs when the mechanisms of
self protection are defective
 Mechanisms of Autoimmunity

● Molecular mimicry - microbe cell surface Ag

resembles self protein.
● Excessive cytokine release due to profound
immune stimulus may awaken self tolerant T cells.
● Self Ags bound to drugs may lose tolerated status.
Main pathologic events of a distal axonal
degeneration or axonopathy (Potts, 2001)
Main pathologic events of a sensory
neuronopathy (Potts, 2001)
 Main pathologic events of primary segmental
demyelination in immune-mediated inflammatory
polyneuropathies (Potts, 2001)
Normal peripheral motor nerve anatomy and
responses to injury (Quan, 1999)
 Clinical Picture of Polyneuropahty (Valenstein, 2000)

● Lower before upper extremity; distal first (feet)

● Decreased ankle jerks
● Stocking, then glove sensory loss
● Distal more severe than proximal (Impairment of neuro
transmission is more severe in long nerve, because of the
confrontation from greater number of demyelinated
segments (Wilkinson, 1997)
ENMG demielinisasi dan aksonopati (Hughes, 2002)
 TREATMENT

√ Class I and II evidence that PE = IVIG = steroids

√ IVIG is usually considered as first line therapy
because of side effect profile but this needs to be
based on avilability, cost and patient profile
√ Combination tx: - Respiratory failure
- Autonomic dysfunction
- Pain
- Positioning & Skin care
- Physical therapy
- Nutrition / supportive tx
 Respiratory Failure
● Oropharyngeal weakness in 25% with impaired
swallowing of secretions & aspiration
● Mechanical respiratory failure- mainly due to
diaphragmatic weakness(Phrenic nerves)
 Respiratory Failure
● ~33% require intubation
● Time to intubation is 1 week & these pts.
have substantially longer recovery time
● Need is unlikely if patient does well for 2
wks. post onset of paresthesiaes
Psychological
● Fear
● Helplessness
● Communication
● Pain
● Sleep deprivation & hallucinosis
● Depression
● Visits from other GBS patients
Corticosteroids
● IV Methylprednisilone 500 mgm/day x 5.
● Ineffective
● May cause relapse (Lancet 1993)
 Plasma Exchange
● Removal of the blood’s liquid soluble
components including complement,
immunoglobulin, immune complexes,
cytokines and interleukins
● A typical session removes about 60% of the
body mass of plasma proteins which is
replaced c saline, albumin
 Intravenous Immune Globulin
IVIG
● Dosage: 0.4 gms/kgm/day x 5 c each dose given
over 3-4 hours preceded by IV diphenhydramine and
or po ibuprofen
● Caution c renal insufficiency or IgA deficiency
● 38 Center trial in 1997
● Equal to plasma exchange
Prognosis GBS

Secara Umum – Px Baik

Kematian : 25% minggu I, 50% bulan I
Sebab : cardiac arrest (20%-30% kasus),
infeksi pulmo,
emboli paru, dan
gagal nafas

(Seneviratne, 2000)
 COMPLICATIONS

♣ Respiratory failure
♣ Autonomic dysfunction
♣ Thromboembolism
MYASTENIA GRAVIS
 Statistics

● Male or female
● Every ethnic group
● Every geographic region
● Any age
 Myasthenia Gravis
❖ Myasthenia Gravis is a chronic disorder of the
skeletal muscles characterized by weakness and
easy fatigability due to autoimmune destruction of
the acetylcholine receptor in the postsynaptic
membrane of the neuromuscular junction
❖ Characterized by remissions & exacerbation's
 Myasthenia Gravis
● Myasthenia gravis is an autoimmune disease in which the
number of acetylcholine receptors at the skeletal muscle
motor endplate is reduced. This receptor deficiency is
caused by circulating antireceptor antibodies that block the
acetylcholine binding sites and accelerate the turnover rate
for the receptor (its internalization and destruction).
● The role of the thymus gland is not known, but 66% of
patients have thymic hyperplasia & 10-15% have thymoma
● Myasthenia gravis can occur at any age, but peak
incidence in women occurs in the third and fourth decades
and in men in the sixth and seventh decades. Among the
elderly, myasthenia gravis is more common in men.
Causes of Myasthenia Gravis
● Drug Related
● Viral / Bacterial
● Neonatal MG
● Adult Onset
 Diagnosis
● Symptoms and confirmed by certain tests
● Anticholinesterase test, a double-blind comparison
of edrophonium 5 mg IV vs. placebo is carried out
while the patient is monitored by an ECG. During the
procedure, IV atropine and resuscitative equipment
are kept available
● Repetitive stimulation testing often shows a
decremental response 1 to 2 min after exercise
 CLINICAL FEATURE
♠ Fluctuating weakness & fatigabillity of voluntary muscles
♠ Namely levator palpebrae, extraocular, bulbar, limb and
respiratory muscles
♠ Usually present with unilateral or bilateral
♠ Symptoms typically fluctuate; they are most pronounced at
the end of the day and are relieved by rest
♠ Initial symptoms include drooping of eyelid (ptosis), double
vision (diplopia), or blurred vision (from extraocular muscle
involvement) in > 50% of patients; generalized weakness
and fatigue in about 10%; and difficulty in swallowing
(dysphagia), facial weakness, or slurred nasal speech in
about 5% and proximal muscle weakness
♠ Symptoms remain limited to the extraocular muscles in
about 15% of patients and become generalized in 85%,
usually within the first year. Symptoms reach maximum
severity in the first year in 50% of patients and within 5 yr in
almost all patients.
 Symptoms
● Droopy eyes
● Flattened smile
● Slow light response in pupils
● Slurred speech
● Nasal speech
● Irregular posture
● Weak expiratory muscles
● Localized fatigue
● Difficulty swallowing or chewing
 Physiology
● Acetylcholine is released into the synaptic cleft
● Ach binds to Ach receptors on the motor end plate
● Influx of Na+ into sarcolemma creates action potential
● Action potential initiates the chain of events leading to
the muscle contraction+
Biosintesa dan katabolisme ACh

Kolin + Asetil-Ko A
Ach. transferase
Asetilkolin (Ach)
Asetilkolinesterase
Kolin + Asetat
What’s Wrong?
Acetylcholine Receptor Antibodies

Acetylycholine receptor blockade

What’s Wrong?

Acetylcholine Receptor Antibodies

Crosslinking
What’s Wrong?
● Damage to Neuromuscular Junction
● Antibody-Negative Myasthenia Gravis
Treatment of Myasthenia
● Medications
● Anticholinesterases
● Corticosteroids
● Immunosuppressants
● ***Important to administer on time to maintain
blood levels and maintain muscle strength
● Plasmapheresis- remove autoantibodies
● Surgery- removal of thymoma / Thymectomy
 Treatment
● First-line therapy = anticholinesterase drug = pyridostigmine
bromide = initial dose of 30 mg orally; the dose should be
repeated as necessary at intervals of at least 3 h. The dosage
must be individualized. Concomitant use of ephedrine 25 mg
bid or tid may have a synergistic effect.
● In the elderly, the most severe symptoms typically occur in the
extraocular and bulbar muscles, which are especially resistant
to anticholinesterase treatment; thus additional tx is usually
necessary. Immunosuppressant(azathioprine) & corticosteroid
(prednisone) are the mainstays of long-term management.
● Management of myasthenia gravis rarely causes cholinergic
crisis, characterized by increasing muscle weakness and
increasing cholinergic side effects. If it occurs, treatment
consists of withholding medications, intubating the patient,
and providing ventilatory support.
 TREATMENT
♠ A number of studies have shown that IVIG is
equivalent to PE in the theraphy of :
* Exacerbation of myastenia gravis
* Myastenic crisis
♠ Insufficient evidence for long term use of IVIG in
reducing steroid / other immuno suppressive dosage
♠ Evidence from a number of studies that lower dose
IVIG (1g/kg) is equally efficacious as higher (2g/kg)
Anticholinesterase Drugs
● Increase the response of muscles to nerve impulses -
muscle strength improved.
● Neostigmine 7.5 mg- 45 mg q 2-6 h PO
● Pyridostigmine (Mestinon) 60 mg - 180 mg BID-QID
● Ambenonium (Mytelase) 5 - 25 mg q 3-4 h
● Expect day-to-day variations
● S/E - cholinergic crisis, TX with atropine
 Anticholinesterase Drugs
● S/E - GI upset - eat meal 45 min. - 1 h after taking
the meds.
● Drugs to avoid: magnesium, morphine, curare,
quinine, quinidine, procainamide, & sedatives and
hypnotics because may inc. weakness.
● Antibiotics such as kanamycin, streptomycin,
tetracyclines may impair transmitter release & inc.
myasthenic symptoms.
 Anticholinesterase Drugs
● Increase the response of muscles to nerve
impulses -> muscle strength improved.
● Neostigmine 7.5 mg- 45 mg q 2-6 h PO
● Pyridostigmine (Mestinon) 60-180 mg BID-QID
● Ambenonium (Mytelase) 5 - 25 mg q 3-4 h
● Expect day-to-day variations
● S/E - cholinergic crisis, TX with atropine
 TERIMAKASIH
Jazakumulloh Khoiron