PENYAKIT SARAF TEPI

Motor
system
Physical findings

Nerve NMJ Muscle

Reflexes Usually decr. NL or decr. NL or decr.

Atrophy Can be severe Minimal Variable

Fascic. Sometimes None None

Sensory loss Sometimes None None

Disorders of Nerve:
Localization
 Root radiculopathy
 Plexus plexopathy
 Single nerve mononeuropathy
 Several nerves multiple mononeuropathy,
mononeuritis multiplex
 All nerves, polyneuropathy
length-dependent
 All nerves, polyradiculoneuropathy
not length-dependent
Single nerve
(mononeuropathy)
 Restricted distribution
 Pain, numbness or tingling, atrophy,
weakness
 Etiology:
 entrapment
 trauma
Carpal tunnel syndrome

 Pain in hand,
forearm, arm
 Numbness in median
distribution
 Symptoms
aggravated by wrist
flexion
Stevens, Neurology 2001
No causal relationship
Rates ~ general population
Anatomy of the Carpal Tunnel

FCR
FPL
FDS
-----
FDP
 Phalen’s test

 Described in 1951
 Originally: rested elbows on table
 better without elbow flexion
 Median nerve trapped b/n proximal TCL and
underlying flexor tendons & radius
 “reverse” Phalen’s maneuver
 Abnormal = reproduce Sx in 30-60 sec
 Limitations
 decreased wrist motion, severe CTS
 wide variation in reported sensitivity (10%-80%) and
specificity (40%-100%)
 Tinel’s Sign

 Gently tapping along the median nerve at the

wrist
 Abnormal = tingling in median nerve dist.
 Careful to tap “gently”
 Phalen reported 60%-73% of patients with CTS
had a Tinel’s sign present
 Wide range of sensitivity (26%-79%) and
specificity (40%-100%)
 Durkan Compression
Test

 Gentle pressure directly over carpal tunnel

 paresthesias in 30 seconds or less
 Better for wrists with limited motion
 Highest sensitivity/specificity of all physical
exam tests
Ulnar neuropathy

 Numbness
 Atrophy of first dorsal
interosseous
 Weakness
 Compression at elbow
 Entrapment in cubital
tunnel
 Distal injury
Radial nerve:
Saturday night palsy
 Weakness of wrist &
finger extensors,
brachioradialis
 Pressure palsy
 Trauma (humerus
fracture)
Peroneal palsy

 Crossing legs
 Weight loss
 Hospitalization
 Surgery
Several nerves
(mononeuritis multiplex)
 Often painful at onset
 Often sudden
 Deficits in the distribution of several
peripheral nerves (one at a time)
 Etiology: vasculitis
polyneuropathy

 Lower before upper extremity

 Distal first (feet)
 Atrophy of intrinsic foot muscles
 Decreased ankle jerks
 Stocking, then glove sensory loss
 Distal motor and sensory findings always
much more severe than proximal
Polyneuropathy (cont’d)
Polyneuropathy (cont’d)

 Most common kind of neuropathy

 Etiology
 metabolic (diabetes, renal failure)
 nutritional (thiamine, B12 deficiency)
 toxic (heavy metals, organic solvents, some drugs)
 familial (Charcot-Marie-Tooth)
All nerves, not length-dependent
(polyradiculoneuropathy)

 Both proximal and distal weakness

 Variable sensory symptoms
 Autonomic symptoms (pulse, blood pressure,
urination...)
 Can affect respiration, swallowing
 Autoimmune
Summary of nerve disorders

 Root Disk, Herpes zoster

 Plexus Autoimmune, trauma,
neoplasm
 Mononeuropathy Trauma, entrapment
 Multiple
mononeuropathy Vasculitis...
 Polyneuropathy Toxic, metabolic,
nutritional
 Polyradiculo-
neuropathy Autoimmune
DD Polyneuropathy
DD Polyneuropathy
DD Polyneuropathy
Sel kornu anterior
 Poliomyelitis
 Motor Neuron Disease :
Amyotrophy Lateral Sclerosis (ALS)
Spinal Muscular Atrophy (SMA)
Saraf perifer
 AIDP (Sindrome Guillain-Barre)
 Neuropati metabolik
 Intoksikasi logam berat (Neuropati toksik)
Paut saraf-otot
 Miastenia gravis
 Miastenia karena obat-obatan
 Eaton Lambert (myasthenic) syndrome (ELS)
 Botulism
 Keracunan organofosfat
 Periodik paralisis
Kelemahan akut pada penderita
yang sebelumnya sehat
Otot
 Polimiositis
 Paralisis periodik
 Miopati toksik
 Mioglobinuria/rabdomiolisis
 Neuroleptic malignant syndrome (NMS)
 Poliomyelitis
 Infeksi viral akut
 Fekal-oral, oral-oral
 Inkubasi 6-20 hari
 Gejala muncul 7-10 hari pasca infeksi, & selama virus dalam
saliva & feses.

Faktor risiko :
 Imunodefisiensi
 Malnutrisi
 Tonsilektomi
 Kehamilan
 Outcome

 • Asymptomatic 90–95%
 • Minor illness 4–8%
 • Non-paralytic aseptic meningitis 1–2%
 • Paralytic poliomyelitis 0.1–0.5%
 • Spinal polio 79%
 • Bulbospinal polio19%
 • Bulbar polio 2%
 Patofisiologi

 Mulut faringintestinalreplikasi viremia ke

limfonodi, otot, jaringan lemak, CNS respon inflamasi
 Poliovirus menyebar ke serabut saraf motorneuron
medulla spinalis/korteks motorik., subkorteks &
cerebellum
 Gejala

 Demam, nyeri kepala, kaku punggung,

kelemahan asimetrik pada otot
 Sensitif sentuhan, sulit menelan, nyeri otot,
reflek turun.
 Iritabilitas, konstipasi
 Paralytic polio

 Dewasa, 1 –ada 75 kasus, quadriplegia

 Anak <5 tahun, paralisis satu tungkai
 • In children < 5yrs, paralysis of one leg is
extensive paralysis of the
 Tersering berhubungan poliovirus type 1.
 Polio spinal
(Paralytic poliomyelitis)

Paralisis asimetris 1/lebih

Atrofi
Proximal>distal
Progresif 2-4 hari
Demam, nyeri otot
Refleks turun/absen
 Bulbar polio

 Area white matter

 Jalur antara korteks serebri-brainstem
 Kelemahan saraf kranial, sesak napas,
sulit bicara dan menelan
 Bulbospinal polio
 • Cervical spinal cord(C3- C5),
 Paralisis N. Phrenicus diafragma
 Parese lengan dan tungkai, denyut jantung.
Postpolio syndrome,
with polio in early infancy.
A and B Foot deformity reveas early
onset. C Very often involvement
of the lower limbs is asymmetric
(om this case right calf is more
atrophic than left)
 Komplikasi

 Skeletal Deformities- Equinus Foot,

 Scoliosis
 Osteoporesis ,Increased bone fractures.
 Neuropathy.
 Pulmonary edema, Aspiration pneumonia,
 UTI, Renal stones
 Paralytic ileus
 Myocarditis, Cor pulmonale.
Diagnosis Banding

 Encephalitis caused by echovirus or coxsackie

virus
 Meningitis
 Guillain-Barre syndrome
 Motor polyneuropathies
 Acute transverse myelitis
 Penatalaksanaan

Terapi suportif
 Simptomatis
 Mencegah komplikasi
 Exercise
 Prognosis

 complete recovery
 Meningitis aseptik 2-10 hari
 Membaik 4-6 minggu sampai 6-8 bulan
 >1 tahun permanen
 50% sembuh total, 25% disabilitas ringan, 25%
disabilitas berat
 Pneumonia, gangguan respirasi
 Meninggal 5-10% anak, 15-30% dewasa
 Tergantung imunitas
 Motorneuron Disease

 Amyotrophic Lateral Sclerosis (ALS)

 ALS

AMYOTROPHIC LATERAL SCLEROSIS (ALS)

- First described in 1897.
- Referred as “Lou Gehrig” disease.
- A progressive neuromuscular condition characterized by
combined upper and lower motor neuron signs.
 ALS

EPIDEMIOLOGY
 1-2/ 100,000
 Males > females 2:1
 90-95% sporadic
 5-10% inherited AD, AR
 Onset >40 years
 Increase with age
 ALS

AETIOLOGY
Unknown
Multifactorial Genetic
Viral
Autoimmune
Neurotoxicity hypothesis
RISK FACTORS
Trauma
Long bone fracture
Manual work
Occupational exposure to toxins; lead;
Solvents
Foods
 ALS

PATHOLOGY

1. Glutamate neurotoxicity,
2. Abnormal accumulation of neurofilaments,
3. Altered neurotrophism
4. Toxicity from oxygen radicals or environmental sources
5. Genetik 20%
6. Mutation of protein cytosolic copper-zinc superoxide
dismutase (SOD1),
 ALS

CLINICAL PRESENTATIONS
 UMN signs (weakness, spasticity, hyperreflexia, extensor
planter)
 LMN signs (weakness, wasting, fasciculations)
 Cachexia
 No sphincteric or sexual disturbances
cerebellar signs
sensory changes
cognitive changes
oculomotor dysfunction
autonomic nervous system dysfunction
 ALS
PROGNOSIS
- Survival is 3-5 years after the onset
- Death occur from respiratory failure ,insufficiency
-Bulbar onset  worst prognosis
- Subacute & reversible type was recorded
 ALS
LOWER MOTOR NEURON AND UPPER MOTOR NEURON SIGNS IN
FOUR CNS REGIONS
Brainstem Cervical Thoracic Lumbosacral
Lower motor jaw, face, neck, arm, back, back, abdomen,
neuron signs palate, hand, abdomen leg, foot
weakness, tongue, diaphragm
atrophy, larynx
fasciculations
Upper motor clonic jaw clonic DTR's loss of clonic DTR's -
neuron signs gag reflex Hoffman reflex superficial extensor plantar
pathologic exaggerated pathologic abdominal response
spread of snout reflex DTR's reflexes pathologic
reflexes, pseudobulbar spastic tone pathologic DTR's
clonus, etc. features DTR's spastic tone
forced yawning preserved reflex spastic tone
pathologic in weak wasted preserved reflex
DTR's limb in weak wasted
spastic tone limb
 ALS
POSITIVE FEATURES
• Definite ALS
- LMN and UMN signs in three to four regions
- Evidence of progression
• Probable ALS
- LMN and UMN signs in at least two regions with UMN
above LMN signs and evidence of progression
• Possible ALS
- LMN and UMN in one region
- UMN in two regions
- LMN above UMN signs
- LMN and UMN signs but no evidence of progression
• Suspected ALS
- LMN signs in two to three regions
 ALS

NEGATIVE FEATURES
• Findings inconsistent with diagnosis of ALS
• Neuroimaging, EMG, clinical or other evidence of an
alternative disease explaining signs or symptoms
• Lack of progression to other body regions
• Cognitive decline
• Sphincter abnormalities
• Sensory dysfunction
• Visual decline
 ALS

DIFFERENTIAL DIAGNOSIS
 Multifocal motor neuropathy with conduction block (MMNCB)
 Myasthenia gravis
 Multiple sclerosis
 Pseudobulbar palsy
 Myopathy
 Postpolio syndrome
 Monomelic muscular atrophy
 Reversible MND
 Denny Brown, Foley syndrome
 ALS

DIAGNOSIS
LABOTATERY STUDIES:
- Magnetic stimulation
Absent or prolonged cortical motor evoked potential
- MRI
BRAIN focal atrophy of precentral gyrus
SPINE normal
- PET scan
Reduced glucose consumption in pericentral area
- Central motor conduction times
Prolonged
- Others
Normal CSF; serum CK; MS panel
 ALS

TREATMENT
DISEASE MODIFYING DRUGS
Riluzole - decrease glutamte release
- 100 mg / day
- decrease need for tracheostomy 56.8%
- after 18 months vs 50.4% for placebo
- adverse effects; asthma, nausea,
- dizziness, granulocytopenia, increase
- transaminase level
Mecaserin
 ALS

TREATMENT
SYMPTOMATIC TREATEMENT
1. SIALOORHEA
Amitriptyline
Benzotropine
Trihexaphenidyl HCL
Transdermal hyoscine (scopalamine)
Propranolol decrease thick mucus production
Physical measures:
Suction machine
Manual assisted coughing techniques
In-Exsufflator cough machine
External beam irradiation to a single parotid gland
 ALS

TREATMENT
SYMPTOMATIC TREATEMENT

2. NUTRITION & DYSPHAGIA

Modification of the food & fluid consistency
Coaching by speech pathologist
PEG
 ALS

TREATMENT
SYMPTOMATIC TREATEMENT

3. RESPIRATORY INSUFFICIENCY
Non invasive vetillatory support
Respiratory therapist consultation
Ventillatory assisted respiration
 ALS

TREATMENT
SYMPTOMATIC TREATEMENT

4. DEPRESSION & ANXIETY

Tricyclic antidepressant
SSRIs
Supportive & family therapy
 ALS

TREATMENT
SYMPTOMATIC TREATEMENT

5. ANTI- SPASTISITY
Baclofen
Tizanidine
Diazepam
Dantrolene
Streching-exercise
 ALS

TREATMENT
SYMPTOMATIC TREATEMENT

6. FASCICULATION
Lorazepam
Decrease caffeine &nicotine intake
 ALS

TREATMENT
SYMPTOMATIC TREATEMENT

7. PAIN
NSAIDs
Anticonvulsant Tegretol, Phenytoin
Tricyclic antidepressant
 ALS

TREATMENT
INEFFECTIVE TREATMENT
- Branched chain amino acids
- Immunosuppressive therapy
IVIG
Cyclophosphamide
fludarabine
- Total lymphoid irradiation
- Free radicle scavenger
- Dextromethorphan
 ALS

PROGNOSIS

1. 5-year survival is 25%.

2. mean duration of disease from onset of symptoms
to death is 27 to 43 months
 Spinal Muscular Atrophy (SMA)

 neurodegenerative disease
 Autosom resesif
 Mutasi gen motorneuron (SMN) pada kromosom
5q13
 SMN1 Koding Survival protein 1 motorneuron
 Mutasi /Deletion gen SMN1 level protein
turun kematian motorneuron dan atrofi otot
progresif (kornu anterior MS)SMA
Cause of SMA - SMN1

 Deletion or a mutation of the SMN1 gene

leads to SMA
 SMN1 encodes Survival of motor neuron
protein 1
 Functions in snRNP biogenesis and mRNA
processing
 Problem is in the anterior horn cells
 Tipe SMA
Tipe Sign & symptom
SMA1 rapid loss of skeletal muscle mass, hypotonia and general muscle
Severe weakness, poor head control, difficulty with suckling, swallowing
and an inability to sit without support.
Onset 6 and 18 months of age.
SMA2 progressive muscle weakness and can never stand or walk on
Interme their own, respiratory insufficiency due to
diate reduced bulbar function, poor weight gain, fine hand tremors and
joint contractures
SMA3 Onset 18 months to 30 years of age. Patients are able to stand
Mild and walk
unaided, While most walk independently, sometimes require
wheelchair assistance, cramps and joint overuse problems; some
develop scoliosis
SMA4 Mildest form of the condition, adult onset with normal mobility,
Adult mild muscle weakness in adulthood with normal longevity
4 types
 SMA type I- infants born
with very little muscle
tone, very weak and will
have feeding and
breathing problems
 SMA type II- symptoms
don’t start to appear until 6
months to 2 years
 SMA type III- mild disease,
starts in
childhood/adolescence
 SMA type IV- even milder,
weakness starts in
adulthood
D
i
a
g
n
o
s
i
s
 Terapi

 Suportive
 Rehabilitasi
GUILLAIN-BARRÉ SYNDROME
(GBS)
 Guillain-Barré syndrome (GBS)
 Acute inflammatory demyelinating polyneuropathy (AIDP)
 autoimmunInfeksi akut(postinfection), vaksinasi
saraf perifer :
Sensoriknyeri, suhu
Motor ikgerakan
 Ascending paralysis
 0.6–4 per 100.000, Fisher syndrome 1 per 100.000
 Laki-laki vs wanita  1,5:1
 Usia <30 tahun 1 per 100.000
 Usia > 75 tahun 4 per 100.000
 Guillain-Barré syndrome (GBS)
 Faktor presipitasi
infeksi Campylobacter jejuni, cytomegalovirus, Epstein-Barr
virus, Mycoplasma pneumoniae, Haemophilus influenza,
Varicella-zoster
 Teori “Molecular mimicry”

pathogen & host

antigen identik
(susunan asam amino homolog/identik)

menginduksi respon antibodi & sel T

cross reaction.
Patofisiologi
Infeksi (mukosa/usus)

induksi antibodi gangliosid & glikolipid (BM1 & GD1b)

menembus blood brain barrier ke gangliosid neuron
terutama nodus ranvier

reaksi inflamasi

Kerusakan saraf sensorik dan motorik

 Gejala Klinis
• Progresif beberapa hari sampai 4 minggu
• Kelemahan memberat pada minggu pertama, paling
berat pada minggu ke-3.
• Paralisis flacid, refleks tendon hilang
• Ascending paralysis
• Simetris
 Gangguan sensorik, motorik, otonom
 Kelainan saraf kranial (N.VII)
 Gangguan otot respirasi
 Diagnosis
• Lumbal pungsi
Disosiasi sitoalbumin protein meningkat tanpa
peningkatan sel
 Elektrodiagnostik
 Serologiantigangliosid
 Therapy

Suportif
• Ventilatory support
•Terapi disfungsi otonom
infeksi nosokomial
Aritmia
Tekanan darah labil
Tromboemboli
Komplikasi imobilitas
Mencegah nyeri
ïNyeri radikuler, disestesia distal :
ïOpiat, antikonvulsan
 Imunomodulator
2-4 minggu pasca onset
IVIG
Mengikat dan menetralisis autoantibodi, menghambat
produksi autoantibodi, mengurangi NK cells, menekan
toksisitas antibodi, menekan ekspresi sitokin
proinflamasi
0,4g/kg/hari selama 5 hari atau 2g/kg iv dosis tunggal

Plasma exchange
PE dan IVIG efektif, memperpendek waktu
penyembuhan 50%
 Prognosis

 Perbaikan komplit dalam 6-12 bulan.

 20-30% disabilitas permanen
 10% tidak mampu berjalan tanpa alat bantu.
 Kematian mayoritas akibat gangguan
otonom, cardiac arrest, gagal napas, emboli
paru, infeksi
NEUROPATI DIABETIKA
Definisi

 Gangguan saraf perifer, otonom, ranial yang ada

hubungannya dengan DM
 Lesi N.II, mononeuropati multipleks, amiotrofi
diabetika, painful neuropathy, neuropati
otonom, neuropati torakoabdominal
Patofisiologi

 Teori Metabolik
HiperglikemiaGlu intrasel meningkatjenuh (jalur
glikolitik)jalur poliolsorbitol+fruktosamioinositol
neuron , Na/K-ATPase membran rusakkecepatan
hantar saraf 
 Teori Neovaskuler/Vaskuler (iskemik-hipoksik)
Hiperglikemikresistensi endoneural-vaskuler
iskemik distribusi oksigen&nutrisi ke neuron 
transport aksonal & aktivitas Na/K-ATPase 
Patofisiologi

 Teori Auto-imun
Perubahan imunogenik sel endotel
 Perubahan support neurotropik
(Altered neurotrophic support theory)
Produksi Nerve Growth Factor (NGF) 
NGF memperbaiki survival small fiber saraf perifer
 Teori Laminin
Glikoprotein heteromerik.
DM kekurangan ekspresi gen laminin beta 2 neuropati
Kriteria Dx Neuropati

Bila ada 2 tanda atau lebih:

 Ada satu atau lbh gejala

 Tidak ada 2 atau lbh refleks ankle atau lutut
 Nilai ambang persepsi getaran abnormal
 Fungsi otonomik abnormal, misal OH
Small and large fiber neuropati

Small fiber neuropati

 Serabut A delta dan C
 Nyeri, terbakar, menikam, cramp, sakit
dangkal, hiperalgesia, parestesia
 Hilangnya sensasi nyeri dan suhukaki
dingin, foot ulcer
Large fiber neuropati
 Serabut A beta
 Rasa geli tanpa sakit, ggn vibrasi,
propriosepsi, gaya jalan
 Refleks tendo↓
 Aliran darah↑, kaki hangat.
 Charchot foot

back
CIDP (chronic inflamatory demyelinating
polyneuropathy)
Mrpk sekunder dari berbagai penyebab yang
tidak berhubungan dg DM, ditandai:
 Kelemahan simetris yang progresif
 Demielinisasi
 Adanya blokade konduksi pada ENMG
 Biopsi saraf : demielinisasi
 Respon bgs thd tx imunomodulatory spt
plasmaparesis

back
Cardiovaskuler Otonomik Neuropati

 Hipotensi Ortostatik
adalah penurunan tekanan darah sistolik ≥20
mmHg atau diastolik ≥ 10 mmHg dalam
waktu 3 menit pada posisi berdiri, setelah
tidur atau duduk.

back
Diagnosis

 Elektrodiagnostik
Management ND

 Kontrol gula darah, HbA1C, tek darah, lipid 

met syndrome
 Perubahan pola hidup
 Tx farmakologi:
* analgesik ajuvan : duloxetin, pregabalin
* trisiklik antidepresan (first line) : amitriptilin
* antikonvulsan : gabapentin, pregabalin
* lainnya: tramadol
* topikal : capsaicin
back
NEUROPATI TOKSIK
 Neuropati akibat obat dan bahan kimia
 environmental, occupational, recreational
drug and iatrogenic toxins.
 Gejala progresik beberapa minggu-bulan
setelah kontak
 Gejala pada saraf (ensefalopati, neuropati)
dan sistemik (liver, kidney, nail, skin)
 Terapi

 Tidak ada terapi spesifik

 Antioksidan
 chemoprotective agents (acetylcysteine,
amifostine, calcium and magnesium,
diethyldithiocarbamate, glutathione,
oxycarbazepine, or vitamin E)
Myasthenia Gravis
Myasthenia gravis (MG)
Penyakit autoimun yang
merusak sistem
neuromuskuler sehingga
terjadi penurunan fungsi
otot
Kelainan akibat antibodi menyerang
reseptor asetilkolin pada
neuromuscular junction
PATHOPHYSIOLOGY

 Mekanisme belum jelas

 Mayoritas adanya kelainan timus
 Sel T dan B timus mempengaruhi reseptor
Ach
Pathophysiology (cont)

 Moreover, thymus contains “myoid cells”

(resembling striated muscle) that bear
surface ACh receptors
 The thymic myoids cells are the foci of
immunologic stimulation in MG
Pathophysiology (cont)

 A virus with a tropism for thymic cells that have

ACh receptors might injury such cells and induce
antibody formation
 Thymic lymphocytes of MG can synthesize anti-
Ach receptor antibody, both in culture and
spontaneously
 Pathophysiology

MG is characterised by an
autoimmunological
degradation of acetylcholine
receptors in the neuromuscular
junctions of the skeletal
muscles of the body
 Epidemiology

1:20.000
Wanita >>
 Signs and
symptoms

Patients with MG typically

present to the practice
with symptoms of variable
ocular fatigue and
weakness
 Myasthenia Gravis
 Fluctuating weakness
 Eyes: ptosis, diplopia (spares pupils)
 Bulbar weakness: dysarthria, dysphagia
 Proximal muscle weakness
 Respiratory weakness
 Normal reflexes
 Normal sensation
 Assoc w/ thymoma
 Assoc w/
other autoimmune d/o
Systemic symptoms include
intermittent fatigue of the libs,
weakness of the facial muscles
and difficulty breating, chewing,
talking and swallowing (dysphasia)
Ocular Examination for Suspected MG

Limitation of adduction

Eyelid drooping (ptosis)

Normal pupil size and reaction

Thymus
Sign and symptoms may be initiated,
exacerbated or mimicked by medications
such as D-penicillamine, antibiotics
(polymyxn B, neomycin, gentamicin,
streptomycin), beta blockers and
anticonvulsants
1.Edrophonium (Tensilon) Testing
IInitially
 Dosing: 2 mg of edrophonium is
administered intravenously as a test
dose
 Monitoring heart rate: Bradycardia or
ventricular fibrillation may develop

Follow-up
 After observing for about 2 minutes, if no
clear response develops
 Up to 8 additional mg of edrophonium is
injected
Positive test
► Most myasthenic muscles respond in 30 to 45
seconds after injection
► Improvement in strength that may persist for
up to 5 minutes
► Requires objective improvement in muscle
strength.
► Subjective or minor responses, such as
reduction of a sense of fatigue, should not be
over interpreted
Patofisiologi Myasthenia Gravis (Drachman, 1994)
 Bilateral weakness of the upper and lower limbs

Myopathy Neuropathy Neuromuscular junction

Distribution of weakness Proximal Distal (initially) General

Ocular and bulbar muscle weakness Rare Infrequent Common

Muscle pain and/or tenderness +/- - -

Muscle atrophy Late Early -

Reflexes Preserved - early Absent - early Present

Absent- late

Sensory deficit Absent May be present Absent

Pascuzzi R M. Myasthenia Gravis
and Lambert-Eaton Syndrome.Ther
Apher, Vol. 6, No. 1, 2002
 Plasmapheresis

1. Removes antibodies from the circulation and

produces short-term clinical improvement in patients
with myasthenia gravis

2. It is used primarily to stabilize the condition of

patients in myasthenic crisis or for the short-term
treatment of patients undergoing thymectomy

3. Five exchange treatments of 3 to 4 liters each are

carried out over a two-week period
Disorders of the neuromusuclar
junction
 Release of acetyl choline:
 Botulism (toxin = endopeptidase targeting various
proteins mediating exocytosis)
 Lambert-Eaton myasthenic syndrome (antibodies to
voltage-gated calcium channel)
 Acetylcholine receptor blockade:
 Myasthenia gravis (antibodies to ACh receptor)
LAMBERT-EATON SYNDROME
Pascuzzi R M. Myasthenia Gravis and Lambert-Eaton
Syndrome.Ther Apher, Vol. 6, No. 1, 2002
Pascuzzi R M. Myasthenia Gravis and Lambert-Eaton
Syndrome.Ther Apher, Vol. 6, No. 1, 2002
Pascuzzi R M. Myasthenia Gravis and Lambert-Eaton
Syndrome.Ther Apher, Vol. 6, No. 1, 2002
PARALISIS HIPOKALEMIA
 Manifestasi klinis :
Kelemahan/paralisis episodik , intermiten pada tungkai
ke lengan.
Serangan muncul setelah tidur/istirahat Dicetuskan oleh
latihan fisik.
 Diagnosis :
Kelemahan otot dan kadar kalium plasma rendah (<3,0
mEq/L) dan kelemahan otot membaik setelah pemberian
kalium.
 Hipokalemi periodik paralisis

 Serangan akut, tiba2 potasium masuk kedalam sel, sehingga

kadarnya rendah diplasma, bisa mencapai kurang dari 1,5
meq/L.
 Hipokalemia sering disertai hipophosphatemia dan
hipomagnesemia
 Pencetus :
Asupan tinggi karbohidrat, insulin, stres emosional,
pemakaian obat tertentu (seperti amfoterisin-B, adrenalin,
relaksan otot, beta-bloker, tranquilizer, analgesik,
antihistamin, antiasma puff aerosol, dan obat anestesi lokal.
 Dikenal 3 jenis yaitu
 Periodik paralisis hipokalemik familial
(autosom dominan)
 Periodik paralisis hiperkalemik
 Periodik paralisis normokalemik
 Algoritma pendekatan diagnosis PPH

TPP : thyrotoxic periodic paralysis

RTA : renal tubular acidosis
FPP : familial periodic paralysis
BS : Bartter syndrome
SPP : sporadic periodic paralysis
GS : Gitelman syndrome
Ada 2 bentuk HypoKPP :
1. Bentuk paralitik
2. Bentuk miopatik
Bentuk Paralitik
- Lebih sering
- Serangan secara episodik, bervariasi (fattique
hingga flaksid).
- Serangan dicetuskan oleh turunnya kadar K di
serum.
- Faktor pencetus utama : berkeringat, makanan
tinggi CHO dan natrium, tidur dan istirahat setelah
exercise
- Sekitar 25% jatuh ke tipe miopatik atau permanent
muscle weakness (PMW)
Bentuk Miopatik
 Serangan tidak bervariasi
 Kelemahan dirasakan setelah aktivitas berlebihan
(pada masa anak) dan setelah usia pertengahan jadi
permanent muscle weakness (PMW).
 Pasien tidak pernah mengalami serangan lumpuh
yang episodik
 Penyakit ini diturunkan secara autosomal dominant
 1/3 kasus sporadik dan tidak ada riwayat keluarga
 Mutasi tersering ditemukan pada channel calsium
kromosom 1
 Sering ditemukan juga pada hipertiroid
Hipertiroid
 Bagaimana mekanisme hipertiroid menyebabkan
hipkalmia periodic paralysi, belum sepenuhnya
diketahui.
 Hormon tiroid meningkatkan aktivitas Na-K-ATP ase (yg
cenderung memindahkan kalium kedalam sel).
 Kelebihan hormon tiroid dapat menjadi predisposisi
tejadinya paralisis secara episodik, akibat pengaruh
epinefrin dan insulin.
Diagnosis

 Riwayat kelemahan yang periodik

 Kadar potasium serum yang rendah
 Kadar Creatine phosphokinase (CPK) meningkat saat
serangan.
 ECG menunjukkan sinus bradikardi dan tanda2
hpokalemi (gel T datar, gel U tinggi di lead II, V2, V3, and
V4, dan depresi segmen ST)
Komplikasi Kardiak

 Sinus bradikardi
 Tanda hipokalemi pada EKG (gel Udi lead II, V-2,
V-3, dan V-4, gel T mendatar dan depresi segmen
ST).
 PR dan QT interval memanjang, gel T mendatar
yang berhubungan dengan menonjolnya gel U
Elektrofisiologis

 Amplitudo aksi potensial otot menurun disaat

serangan, sering ditemukan pada hypokalemic
PP.
 Konduksi saraf sensorik normal.
 Bila ditemukan gangguan konduksi saraf , perlu
dipikirkan, apakah ada penyakit penyerta seperti
neuropati yg berhubungan dengan tirotoxicosis.
 Pencegahan dan Pengobatan
Pengobatan
 Bila kadar K lasma sangat rendah, bisa langsung di koreksi
secara intravena dengan kecepatan pemberian 10 meq/ jam atau
0,5 mEq/kg selama 1 jam, infus kontinu
 Dikoreksi dengan rumus : [K normal – Kpasien] x 1/3 BB
 Asetazolamid (inhibitor anhidrase karbonat) 125-250 mg 2-3
x/hari
 Spironolakton 100-200 mg/hari
Pencegahan :
Pemberian preparat KCl oral 60 – 120 meq
Makanan yang mengandung K seperti Pisang,
semangka, korma dll