PEMICU 8 BLOK SARAF & KEJIWAAN

Vivian Saputra
405140126
 Mind Map
Central (X) (tidak ada sakit kepala & melihat berbayang)

Laki” 50 tahun lemah Primer :

kedua tungkai sejak -Trauma
2 minggu -Infeksi
Perifer Etiologi

Sekunder :
-Metabolik
LMN UMN (x)
- Infeksi

Anatomi

a) Cornu Anterior (GBS, Motor Neuron Disease, Sindrom horner

Myastenia Gravis) Referred pain
b) Radiks (Radikular syndrom, HNP) Reticular syndrome
c) Saraf Tepi (Neuropati, CTS, TTS, Nyeri Neuropati)
d) Neuromuscular Junction
e) Otot (Kelemahan Otot, Miositis)
• Neurofibromatosis
• Cauda Equina
• Peroneal Palsy
 LI
1. Patofisiologi Kelemahan Otot Pernapasan
2. Anatomi (Cornu Anterior, Radix, Saraf Tepi, NMJ,
otot)
3. Histologi
4. Fisiologi
5. Kelainan LMN (Definisi, Etiologi, Tanda & Gejala, PF,
PP, tatalaksana, komplikasi, prognosis)
PATHOPHYSIOLOGY OF
RESPIRATORY DYSFUNCTION
 Pathophysiology of respiratory
dysfunction
• The pathophysiological hallmark of respiratory dysfunction is
the presence of demyelinating lesions in the central nervous
system.
• These lesions may involve one or more locations associated
with production and/or propagation of neural impulses to the
respiratory muscles.
• Depending upon the location and extent of demyelinating
lesions, respiratory dysfunction may manifest with symptoms
due to respiratory muscle weakness and impaired cough,
dysfunction of bulbar muscles, abnormalities in the control of
breathing, or respiratory failure
 Respiratory failure
• Respiratory failure may be acute, typically
secondary to demyelinating lesions in the
cervical cord or the medulla or chronic,
typically found in the terminal stages of the
disease and related to weak respiratory
muscles, and ineffective cough, leading to
aspiration, atelectasis and pneumonia
ANATOMI
 Anatomy of PNS

Mtui E, Gruener G, Dockery P. Fitzgerald’s clinical neuroanatomy and neuroscience. 7th ed. Philadelphia:
Elsevier; 2016.
 Anatomy of PNS
• Neurons contributing to peripheral nerves  are
partly contained wihtin CNS
– Anterior horn of grey matter: multipolar α & γ neuron
(efferent/motor nerves)
– Dorsal root ganglia: unipolar neurons (cell bodies) 
posterior horn of grey matter (afferent/sensory
process)
• Somatic afferent fibers
• Somatic efferent fibers
• Visceral efferent
Mtui E, Gruener G, Dockery P. Fitzgerald’s clinical neuroanatomy and neuroscience. 7th ed. Philadelphia:
Elsevier; 2016.
 Brachial Plexus
• Formed by the
anterior rami of C5 to
C8, and most of the
anterior ramus of T1
• Origin: neck  passes
laterally and inferiorly
over rib I  enters
the axilla
• Part of brachial
plexus:
– Roots
– Trunks
– Divisions
– Cords
Dermatome
Dermatomes & Myotomes
Dermatomes
Intercostal Nerves
Sympathetic
Trunks
Lumbar
Plexus
Lumbar Plexus: Branches
Sacral
Plexus
Dermatomes: Lower
Limb
Lumbosacra
l Plexus
HISTOLOGI
 PERIPHERAL NERVOUS SYSTEM
• The main components of the peripheral nervous system (PNS)
are the nerves, ganglia, and nerve endings.

Nerve Fibers
• Nerve fibers are analogous to tracts in the CNS, containing
axons enclosed within sheaths of glial cells specialized to
facilitate axonal function.
• In peripheral nerve fibers, axons are sheathed by Schwann
cells, or neurolemmocytes
• The sheath may or may not form myelin around the axons,
depending on their diameter.

Junqueira’s basic histology text & atlas. 13th ed.

Junqueira’s basic histology text & atlas. 13th ed.
Junqueira’s basic histology text
& atlas. 13th ed.
Junqueira’s basic histology text & atlas. 13th ed.
Junqueir
a’s basic
histolog
y text &
atlas.
13th ed.
Junqueira’s basic
histology text &
atlas. 13th ed.
FISIOLOGI
• Slow pain = rasa terbakar, tumpul
• Fast pain = tertusuk, kasar
• Kortikospinal track = eferen
NEUROPATI
MYASTHENIA GRAVIS
 Myasthenia Gravis
• Myasthenia gravis (MG), the damage to the postsynaptic
most common of the membrane.
neuromuscular junction
disorders, is an acquired, Epidemiology:
predominantly antibody-
mediated autoimmune disease. • The prevalence of autoimmune
MG is estimated at 1 case in
10,000–20,000 people.
• In this disorder, antibodies are • Women are affected more often
targeted against the nicotinic in the second and third decades
acetylcholine receptor (AChR) at of life, and men more often in
the neuromuscular junction  the fifth and sixth decades.
resulting in an overall reduction
in the number of AChRs and Greenberg DA, Aminoff MJ, Simon RP. Clinical
neurology. 9th ed. New York: McGraw Hill, 2015.
 Pathogenesis

Greenberg DA, Aminoff MJ, Simon RP. Clinical neurology. 9th ed. New York: McGraw Hill, 2015.
 Clinical Findings
Symptoms and Signs
• MG is clinically characterized by • Because of palatal weakness,
fluctuating, fatigable weakness of patients often have nasal speech
commonly used muscles. and can regurgitate liquids through
• Hallmark features include ptosis, the nose.
diplopia, dysarthria, dysphagia, and • A hallmark of myasthenic weakness
respiratory and limb muscle is its fluctuating and fatigable
weakness. nature. It may increase throughout
• Within the first year of disease the day, worsen with sustained
onset, up to 75% of patients activity, and improve with rest.
develop generalized symptoms. • The most serious of the symptoms
• Bulbar symptoms are common and is respiratory compromise caused by
include dysarthria, dysphagia, facial weakness of diaphragmatic and
weakness, and weakness of intercostal muscles.
mastication.
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
 Diagnostic Studies
1. Tensilon (edrophonium) test 2. Laboratory studies
• This test evaluates the response to • Serologic testing should be
a short-acting cholinesterase performed in several steps.
inhibitor. • The first screening antibody :
• The examiner must identify a AChR-binding antibody, because
clinical feature (most often ptosis) it is the most sensitive.
to observe. • If it is negative, then an AChR-
• Edrophonium is given modulating antibody test
intravenously in a dose of 10 mg (1 increases the diagnostic yield.
mL), of which 2 mg is given initially • Testing for AChR-blocking
as a test dose and the remaining 8 antibodies does not increase
mg approximately 30 seconds later sensitivity.
if the test dose is well tolerated.
– In myasthenic patients, there is an
obvious improvement in the strength
of weak muscles that lasts for
approximately 5 minutes.
Alternatively, 1.5 mg of neostigmine
can be given intramuscularly, with a
response that lasts for about 2 hours.
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
 Diagnostic Studies
3. Electrodiagnostic studies 4. Imaging and other studies
• Routine nerve conduction studies and
electromyography usually do not identify • Because of the association
dysfunction of the neuromuscular between MG and thymoma,
junction. all patients should be
• Slow repetitive nerve stimulation is the screened for this tumor
most commonly used test to evaluate for using either a CT or MRI
MG.
scan of the chest.
• In this test, a nerve is stimulated 6–10
times at a rate of 2 or 3 Hz, and the
compound muscle action potential
(CMAP) is measured over the
corresponding muscle.
– In patients with MG, there is a
decrease of more than 10% in CMAP
with the first four to five stimuli.
• Single-fiber electromyography has a
sensitivity of approximately 95% in MG.
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
 Differential Diagnosis
• For generalized MG, the differential diagnosis includes :
– Lambert-Eaton myasthenic syndrome
– Botulism
– Myopathy.
• For ocular myasthenia, alternative diagnoses include :
– progressive external ophthalmoplegia
– thyroid disease
– oculopharyngeal muscular dystrophy.
• Motor neuron disease, brainstem stroke, diphtheria, and botulism
must be considered in patients with bulbar predominant
myasthenia gravis.

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Treatment
a) Symptomatic Treatment

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Treatment
b) Immunosuppressive Treatment
1. Thymectomy
2. Medical therapy
– Corticosteroids
• High-dose glucocorticoids are often started while patients are
hospitalized for plasmapheresis or intravenous immune
globulin (IVIG) therapy.
• An initial high dose of prednisone (60-100 mg/d orally) can be
tapered gradually to a relatively low-maintenance level (5-15
mg/d) as improvement occurs
– Nonsteroidal immunosuppression
– Short-term treatments
Greenberg DA, Aminoff MJ, Simon RP. Clinical neurology. 9th ed. New York: McGraw Hill, 2015.
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
 Treatment of Myasthenic Crisis
• Myasthenic crisis : an
exacerbation of weakness that
leads to respiratory failure
requiring mechanical ventilation. •
• For patients with myasthenic
exacerbation involving
respiratory and bulbar
symptoms, hospitalization should •
be considered to closely monitor
clinical status and pulmonary
function.
• Once a patient is intubated,
anticholinesterase medications
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
should be discontinued because
they can promote excessive
secretions.
Corticosteroids can actually
prolong the duration of a crisis
by exacerbating weakness or
predisposing to infection.
The mainstay of therapy for
myasthenic crisis is therefore
short-term immunotherapy,
either plasmapheresis or IVIG.
 Prognosis
• Patients with disease limited to the ocular muscles,
cholinesterase inhibitors, lowdose corticosteroids, or
nonmedicinal therapy (eg, eyelid crutches) may be sufficient
to control symptoms.
• However, quality of life may be compromised as a result of
both the limited efficacy and the side effects of available
drugs.
• Patients with an underlying thymoma often have a more
aggressive disease course.

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

HERNIA NUCLEUS PULPOSUS
 Acute Intervertebral
Disk Prolapse
• Lumbar disk prolapse
• Cervical dis prolapse

Greenberg DA, Aminoff MJ, Simon RP. Clinical

neurology. 9th ed. New York: McGraw Hill, 2015.
 Lumbar Disk Prolapse: S/S
• Onset: begin suddenly or insidiously; may
follow trauma
• Back & radicular pain (L5 or S1) in the leg
• Numbness & paresthesias (often)
• Weakness (depends on the root affected)
– L5 radiculopathy  weakness of dorsiflexion of
the foot & toes
– S1  weakness planter flexion of the foot &
depressed ankle jerk
Greenberg DA, Aminoff MJ, Simon RP. Clinical neurology. 9th ed. New York: McGraw Hill, 2015.
 Lumbar Disk Prolapse: PE
• Restriction in spine movement
• Local back tenderness
• Palpable spasm of paraspinous muscles
• SLR (N=80-90 degrees): restricted (often: 20-30
degrees of hip flexion) because of reflex spasm of
the hamstring muscles (Lasegue sign)
• Central prolapse  bilateral s/s & sphincter
involvment
• Pelvic and rectal examination and imaging of
spine  exclude causes such as tumor

Greenberg DA, Aminoff MJ, Simon RP. Clinical neurology. 9th ed. New York: McGraw Hill, 2015.
 Lumbar Disk Prolapse: Treatment
• Bed rest of a firm mattress for 2-3 days followed
by gradual mobilization
• Persisting pain, increasing neurologic deficit,
sphincter dysfunction  should lead to CT, MRI,
CT myelography, followed by surgical treatment
• Drug
– aspirin or acetaminophen with 30 mg of codeine, 2
doses, 3 or 4 times daily
– Other nonsteroidal analgesics: ibuprofen or naproxen
– Muscle spasm  cyclobenzaprine 10 mg orally tid or
diazepam 5-10 mg orally tid

Greenberg DA, Aminoff MJ, Simon RP. Clinical neurology. 9th ed. New York: McGraw Hill, 2015.
 Cervical Disk Prolapse
• Can occur at any age
• Often with no preceding trauma
• Leads to neck and radicular arm pain, exacerbated by
head movement.
• Lateral herniation
– motor, sensory, or reflex deficit may occur in a radicular (usually
C6 or C7) distribution on the affected side
• Central herniation  spinal cord involvement
– spastic paraparesis and sensory disturbance in the legs
– sometimes accompanied by impaired sphincter function.
• The diagnosis is confirmed by CT scanning, MRI, or CT
myelography.
• Surgical treatment may be needed.

Greenberg DA, Aminoff MJ, Simon RP. Clinical neurology. 9th ed. New York: McGraw Hill, 2015.
NEUROMUSCULAR JUNCTION
 LAMBERT-EATON MYASTHENIC SYNDROME

• Lambert-Eaton myasthenic syndrome (LEMS) is an autoimmune or

paraneoplastic disease.
• characterized by chronic fluctuating weakness of the proximal limb
muscles, especially the legs.
• Approximately 60% of patients with LEMS have an associated small cell
carcinoma of the lung or, less often, another type of malignancy.
• In those who do not have an underlying malignancy, a concurrent
autoimmune disease is common.

Etiology:
• LEMS is caused by antibodies directed at P/Q-type voltage-gated calcium
channels (VGCCs) and reduced neurotransmitter release at the
neuromuscular junction and autonomic nerve terminals.
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
 Clinical Findings
Symptoms and Signs
• The onset of symptoms is usually insidious.
• Generalized fatigable weakness is the major symptom.
• Patients often complain of myalgia, muscle tenderness, and
stiffness.
• Oculobulbar and respiratory symptoms are much less
common than with MG.
• Unlike patients with MG, those with LEMS may complain of a
metallic taste, and often have autonomic dysfunction causing
dry mouth, orthostasis, constipation, and impotence.

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Diagnosis
• The diagnosis is confirmed electrophysiologically by the
incremental response to repetitive nerve stimulation.
• The presence of autoantibodies to the P/Q subtype of voltage-
gated calcium channels, found on the presynaptic membrane
of the neuromuscular junction, is highly sensitive and specific
to the Lambert-Eaton syndrome of any etiology.

Greenberg DA, Aminoff MJ, Simon RP. Clinical neurology. 9th ed. New York: McGraw Hill, 2015.
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
 Differential Diagnosis
• The main alternative diagnosis to consider is MG.
• Electrodiagnostic abnormalities are often more prominent in
LEMS than in MG despite the often more severe weakness in
MG.
• LEMS is often misdiagnosed as a myopathy because of the
predominantly proximal weakness.

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Treatment
• The first step in management should be an evaluation for malignancy,
especially in older patients or those with a history of smoking.
• Immunosuppressive drug therapy (corticosteroids, mycophenolate,
and azathioprine) and plasmapheresis or intravenous
immunoglobulin therapy may lead to symptomatic improvement.
• Guanidine hydrochloride (25 mg/kg/d in 3 or 4 divided doses to a
maximum of 1,000 mg/d)  sometimes helpful by enhancing the
release of acetylcholine in seriously disabled patients.
• 3,4-Diaminopyridine used in doses up to 25 mg orally four times daily,
may improve weakness and autonomic dysfunction.

Greenberg DA, Aminoff MJ, Simon RP. Clinical neurology. 9th ed. New York: McGraw Hill, 2015.
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
 Prognosis
• Prognosis in patients with underlying malignancy is
determined by the prognosis of that malignancy.
• Because LEMS is less responsive to immunosuppressive
therapy than MG, most patients with LEMS have residual
weakness even with optimal immunosuppression.

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 BOTULISM
• Botulism is caused by ingesting the neurotoxin of the bacterium
Clostridium botulinum, an obligate anaerobic, robust, spore-
forming bacillus commonly found in soil.
• After absorption into the bloodstream, botulinum toxin binds
irreversibly to the presynaptic nerve endings of the peripheral
nervous system and cranial nerves.
• Once internalized, the toxin inhibits the release of acetylcholine
through the cleavage of polypeptides.
• Botulism occurs most commonly after ingestion of home-canned
food contaminated with the toxin; it occurs rarely from infected
wounds.

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Symptoms and Signs
• The initial symptoms of food-borne •
botulism may be gastrointestinal—
nausea, vomiting, and diarrhea.
• The earliest neurologic symptoms are
oculobulbar and include dry mouth,
blurred vision, diplopia, dysarthria,
dysphagia, and dysphonia.
• Botulism is characterized by a
descending paralysis. The weakness
progresses from proximal to distal
muscles.
• Botulism also affects autonomic
synaptic transmission, resulting in
constipation, postural hypotension,
and urinary retention.
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
Most infantile cases occur before the
age of 6 months, and the first signs
may be constipation, weak cry, and
poor feeding.
– Weakness then progresses over
days, causing poor suck and head
control, hypotonia, and deceased
movement.
– Autonomic signs and symptoms
include hypotension, tachycardia,
and dry mouth.
 Laboratory and Electrodiagnostic Findings

• Both blood and stool can be sent for detection of the

botulinum toxin.
• C botulinum itself can be detected in stool.
• Electrodiagnostic studies :
– The most consistent finding is a small CMAP in response to
a supramaximal stimulus.
– As with LEMS, repetitive stimulation testing may show a
decrement of the CMAP to low rates of stimulation and
postexercise facilitation of the CMAP amplitude.

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Treatment
• The major treatment is intensive supportive care.
• Patients should be closely monitored for respiratory
decompensation. If the ingestion is recent, removal of unabsorbed
gut toxin can be considered.

• In wound botulism, antibiotic therapy is often prescribed but is of

unclear benefit; either
– penicillin G (3 million units IV every 4 hours in adults) or
– Metronidazole (500 mg IV every 8 hours in patients allergic to
penicillin)

• In infants, human-derived botulinum immune globulin should be

given intravenously as soon as possible.
Greenberg DA, Aminoff MJ, Simon RP. Clinical neurology. 9th ed. New York: McGraw Hill, 2015.
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
 Prognosis
• Although significantly reduced, mortality from botulism
remains high at 5–10%.
• Type A toxin is associated with a more severe course and
higher mortality than other toxins.
• Clinical recovery is often prolonged over months
• Recovery of autonomic function may take longer than
recovery of muscle strength.
• For those who survive, the recovery is generally complete.

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

MOTOR NEURON DISEASE
 MOTOR NEURON DISEASE (MND)

• Penyakit yg disebabkan oleh degenerasi badan sel neuron

motorik.
• Tipe MND yg paling sering terjadi : amyotrophic lateral
sclerosis (ALS).
• Berdasarkan anatomi & fisiologi terdapat 2 motor neuron:
– Lower motor neuron (LMN)  terletak di kornu anterior
medula spinalis & di batang otak (nukleus motorik saraf
kranialis) & menginervasi otot” secara langsung.
– Upper motor neuron (UMN)  terletak di korteks motorik
& memberikan jaras ke kortikospinal & kortikobulbar.

Buku Ajar Neurologi FKUI

 MOTOR NEURON DISEASE (MND)
Patofisiologi
• MND dapat bersifat didapat
(acquired) atau diturunkan
(herediter).
• Neuron motorik mengalami•
apoptosis  degenerasi akson
nervus motorik  taut saraf-otot
juga ikut mengalami kerusakan.
• Serabut-serabut otot yg dipersarafi
o/ akson yg berdegenerasi akan
mengalami atrofi.
• Secara elektrofisiologi, masing”
serabut otot menunjukkan fibrilasi
& gelombang (+) akibat tidak
stabilnya membran otot.
– Jika serabut” tsb berkontraksi
bersamaan, motor unit yg terkait
akan mengalami fasikulasi.
Pada MND yg didapat (acquired),
akson saraf motorik terkait akan
memberikan reinervasi terhadap
serabut otot  tampak reinervasi
pada gambaran pemeriksaan EMG.
Buku Ajar Neurologi FKUI
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
 Gejala & Tanda Klinis
• Gejala Klinis LMN pada • Gejala Klinis UMN pada
ALS : ALS :
– Kelemahan & atrofi – Atrofi tidak terlalu jelas
otot” ekstremitas bagian terlihat.
distal yg asimetris – Spastis
– Fasikulasi – pe↑an refleks fisiologis
– Flaksid atau tonus otot pada otot yg atrofi.
dapat normal – Terdapat refleks
– pe↓an refleks fisiologis patologis (refleks
Babinski, refleks
Hoffmann Tromner)

Buku Ajar Neurologi FKUI

Drislane FW et al.
Neurology. Blueprints.
Philadelphia; Lippincot
Williams & Wilkins: 2009
 Sindrom Klinis Motor Neuron Diseases
Sindrom Gejala Klinis
ALS klasik Kelemahan dimulai dari ekstremitas; kelemahan bulbar sering
terjadi; tanda UMN & LMN

Progressive bulbar palsy (PBP) Mulai dari disartria diikuti ggn bicara & menelan

Progressive muscular atrophy Selalu dimulai dari kelemahan ekstremitas; >50% menunjukkan
(PMA) tanda UMN; 85% menunjukkan tanda bulbar

Flail arm syndrome; Sindrom dgn gejala predominan kelemahan LMN pada kedua
progressive amyotrophic lengan; tanda UMN terjadi pada 50-70%; progresif lambat
diplegia; Sindrom Bernhard-
Vulpian
Flail leg syndrome Sindrom kelemahan tungkai yg progresif, predominan LMN

Bentuk Monomelik MND Varian MND yg jarang terjadi, fokal progresif lambat

Primary lateral sclerosis (PLS) Sindrom UMN murni yang progresif

Sindrom MND-Demensia Demensia tipe fronto-temporal, terjadi pada 5% pasien MND
Buku Ajar Neurologi FKUI
 Diagnosis
• ALS ditegakkan murni secara lidah.
klinis berdasarkan anamnesa & – Peningkatan refleks
pemeriksaan fisik. fisiologis
• Pemeriksaan penunjang – Perjalanan penyakit berjalan
(pencitraan)  menyingkirkan secara progresif.
penyakit lain yg dapat • Pada pasien yg dicurigai ALS
disebabkan kelainan struktural. sangat penting dilakukan
• Tanda klinis yg khas : pemeriksaan elektrofisiologi
– Ps dgn tanda klinis atrofi (kecepatan hantar saraf (KHS) &
beberapa kelompok otot di elektromiografi (EMG))
beberapa bagian tubuh.
– Fasikulasi di otot” rangka &

Buku Ajar Neurologi FKUI

Brust JCM. Current
Diagnosis &
Treatment
Neurology, 2nd ed
 Diagnosis Banding
• Multifocal motor neuropathy
• Spinal muscular atrophy
• Spinal bulbar muscular atrophy/ penyakit Kennedy
• Poliomielitis
• Wes Nile Virus
• Paraneoplastic motor neuron disease

Buku Ajar Neurologi FKUI

 Tatalaksana
• Tatalaksana hanya bersifat
simtomatik & bukan bersifat
kuratif.
• Penggunaan Riluzole 100
mg/hari dapat memperpanjang
survival sekitar 15 bulan pada
pasien ALS  belum ada di
Indonesia
• Penggunaan antioksidan (vit C &
vit E) banyak digunakan pada
pasien ALS.
• Terapi exercise / latihan 
direkomendasikan untuk dapat
mempertahankan tonus otot.
• Pasien ALS dapat mengalami
nyeri & spastis
– Obat antiinflamasi
nonsteroid & opioid
digunakan untuk mengatasi
nyeri.
– Spastisitas dapat dikurangi
dengan pemberian baklofen,
fisioterapi, terapi neurolisis.
Buku Ajar Neurologi FKUI
CARPAL TUNNEL SYNDROME
 Median Mononeuropathy at the Wrist
(Carpal Tunnel Syndrome)
• The carpal tunnel, located at the • Several medical conditions also
base of the palm, is formed by increase the risk of carpal
carpal bones on the median, tunnel syndrome:
dorsal, and lateral sides and is – diabetes mellitus,
covered ventrally by the flexor – hypothyroidism,
retinaculum.
– pregnancy,
• The median nerve is prone to
compression at this location – rheumatoid arthritis,
with repetitive flexion or – obesity,
extension. – and, less commonly,
amyloidosis and acromegaly.

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

Brust JCM. Current Diagnosis & Treatment
nd
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
 Symptoms and Signs of Carpal Tunnel
Syndrome
• Patients with carpal tunnel involve only part of the
syndrome complain of pain, dermatome (ie, the thumb).
paresthesias, or numbness in • On examination, sensation may
the first three digits. or may not be abnormal in the
• Symptoms are usually worse median territory at rest.
with repetitive or sustained • Tinel sign is elicited by lightly
wrist flexion or extension (eg, percussing the median nerve at
typing, driving) the wrist with a reflex hammer.
• also often exacerbated during
sleep due to unconscious
sustained wrist flexion.
• In some patients, pain may
radiate up the medial forearm.
• Numbness often is difficult for
patients to localize and may

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Diagnostic Studies
• EMG and nerve conduction abnormalities.
studies  critically important in • Loss of motor amplitudes is a
the evaluation of carpal tunnel worrisome sign.
syndrome. • blood studies to assess for
• The evaluation of motor and diabetes mellitus, thyroid
sensory conduction time through dysfunction, rheumatoid
the carpal tunnel is a standard arthritis, and other systemic
technique. diseases may be indicated.
• Patients with mild nerve
compression demonstrate only
sensory slowing, whereas those
with more severe compression
demonstrate motor
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
 Differential Diagnosis
• The differential diagnosis of numbness or weakness of
the hand :
– Stroke
– cervical radiculopathy
– brachial plexopathy
– more proximal median nerve injury
– and ulnar nerve injury.
• Pain alone may be caused by joint or tissue injury or
inflammation such as flexor tendonitis and arthritis of
the wrist.
• Traumatic injuries may also injure the median nerve.

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Treatment
• Conservative therapy should be
attempted first.
• Conservative therapy consists of
wrist splinting in the neutral
position at night and during
activities that encourage wrist
flexion and extension.
• anti-inflammatory agents,
vitamin B6, or diuretics are
sometimes used.
• Short courses of oral prednisone
may also be useful for
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
symptomatic improvement.
• Local corticosteroid injection 
remains controversial
• Carpal tunnel release surgery is
an option for patients who do
not respond to more
conservative management.
• Newer methods include
endoscopic carpal tunnel release
surgery
TARSAL TUNNEL SYNDROME
 Tarsal Tunnel Syndrome
• Tarsal tunnel syndrome is a condition that is
caused by compression of the tibial nerve or
its associated branches as the nerve passes
underneath the flexor retinaculum at the level
of the ankle or distally.
 Tarsal Tunnel Syndrome
• The posterior tibial nerve or its branches can be compressed
between the floor and the ligamentous roof of the tarsal
tunnel, which is located at the ankle immediately below and
behind the medial malleolus.
• S/S
– burning in the foot, especially at night
– sometimes accompanied by weakness of the intrinsic foot
muscles
• The diagnosis can usually be confirmed electrophysiologically.
• If treatment with local injection of corticosteroids is not
helpful, surgical decompression may be necessary.
PERONEAL PALSY
PERONEAL NERVE

Brust JCM. Current

Diagnosis & Treatment
Neurology, 2nd ed
 Peroneal Palsy
• Injury to the common peroneal nerve is the most
common mononeuropathy of the lower extremity.
• Etiology :
– frequently caused by weight loss during prolonged illness
and hospitalization, with extrinsic compression of the
nerve between the fibular head and firm mattresses or
side rails.
– Fracture of the fibular head
– external blunt trauma
– knee surgery
– Suspension of legs in straps
– positioning for lithotomy during gynecologic procedures

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

Clinical Findings in Peroneal Nerve
Compression

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Peroneal Palsy
• Diagnostic Studies
• Electrophysiologic studies enable
differentiation between peroneal
mononeuropathy and anterior horn
cell disease, lower lumbosacral
radiculopathy, lumbosacral
plexopathy, sciatic nerve injury,
asymmetric polyneuropathy, and
distal myopathy. They can also
localize the site of the peroneal
injury.
• Imaging studies of the pelvis, thigh,
knee, or ankle may also be needed
and should be guided by findings on
electrodiagnostic testing.
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
• Differential Diagnosis
• Peroneal nerve injury is often
confused with L5–S1
radiculopathy.
• Other processes that may
present with prominent foot
drop include stroke and motor
neuron disease.
• Injury to the lumbosacral plexus
and sciatic nerve must also be
considered.
• Some distal myopathies and
generalized polyneuropathies
may also produce foot drop.
 Treatment
• Conservative treatment with bracing may be needed in
common peroneal nerve injuries at the knee.
• Patients should refrain from :
– crossing their legs,
– squatting for prolonged periods, or
– sitting with their knees bent over the surface of a hard
chair or bench.
• Surgical treatment with end-to-end anastomosis may be
indicated in acute laceration injury.

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

NEUROFIBROMATOSIS
 Neurofibromatosis
• Neurofibromatosis is a genetic the disease. NF1 and NF2 that isn't
disorder that causes tumors to form inherited results from new gene
on nerve tissue. mutations.
• These tumors can develop• NF1 and NF2 are both autosomal
anywhere in the nervous system, dominant disorders, which means
including the brain, spinal cord and that any child of a parent with the
nerves. disorder has a 50 percent chance of
• Neurofibromatosis is usually inheriting the genetic mutation.
diagnosed in childhood or early
adulthood.

Risk Factor
• The biggest risk factor is a family
history of the disorder. About half of
people with NF1 and NF2 inherited
http://www.mayoclinic.org/diseases-conditions/neurofibromatosis/diagnosis-treatment/treatment/txc-20167907
 Neurofibromatosis
Causes
• Neurofibromatosis is caused by genetic defects
(mutations) that either are passed on by a parent or
occur spontaneously at conception.
• The specific genes involved depend on the type of
neurofibromatosis:
– NF1. The NF1 gene is located on chromosome 17.
– NF2. The NF2 gene is located on chromosome 22.
– Schwannomatosis

http://www.mayoclinic.org/diseases-conditions/neurofibromatosis/diagnosis-treatment/treatment/txc-20167907
 Neurofibromatosis 1
• Neurofibromatosis 1 (NF1) usually appears in childhood. Signs are
often evident at birth or shortly afterward, and almost always by age
10.
• Signs and symptoms include:
– Flat, light brown spots on the skin (cafe au lait spots)
– Freckling in the armpits or groin area
– Tiny bumps on the iris of your eye (Lisch nodules)
– Soft bumps on or under the skin (neurofibromas)
– Bone deformities
– Tumor on the optic nerve (optic glioma)
– Learning disabilities
– Larger than average head size
– Short stature
http://www.mayoclinic.org/diseases-conditions/neurofibromatosis/diagnosis-treatment/treatment/txc-20167907
 Neurofibromatosis 2
• Neurofibromatosis 2 (NF2) is much less common than
NF1.
• Signs and symptoms of NF2 usually result from the
development of benign, slow-growing tumors (acoustic
neuromas) in both ears.
• Signs and symptoms generally appear in the late teen and
early adult years, and can vary in severity. Signs and
symptoms can include:
– Gradual hearing loss
– Ringing in the ears
– Poor balance
– Headaches

http://www.mayoclinic.org/diseases-conditions/neurofibromatosis/diagnosis-treatment/treatment/txc-20167907
 Schwannomatosis
• This rare type of neurofibromatosis usually affects people
after the age of 20.
• Schwannomatosis causes tumors to develop on skull
(cranial), spinal and peripheral nerves — but not on the
nerve that carries sound and balance information from
the inner ear to the brain.
• Schwannomatosis causes chronic pain, which can occur
anywhere in your body. Other symptoms include:
– Numbness or weakness in various parts of your body
– Loss of muscle

http://www.mayoclinic.org/diseases-conditions/neurofibromatosis/diagnosis-treatment/treatment/txc-20167907
 Diagnosis
• Eye exam  to detect Lisch nodules and cataracts.
• Ear exam.
– Tests such as audiometry, electronystagmography and
brainstem auditory evoked response can help assess
hearing and balance problems in people with NF2.
• Imaging tests
– X-rays, CT scans or MRIs can help identify bone
abnormalities, tumors in the brain or spinal cord, and very
small tumors.
– An MRI might be used to help identify optic gliomas.
Imaging tests are also often used to monitor NF2 and
schwannomatosis.

http://www.mayoclinic.org/diseases-conditions/neurofibromatosis/diagnosis-treatment/treatment/txc-20167907
 Treatment
• Neurofibromatosis can't be cured, – Managing pain is an important
but treatments are available for part of treatment for
your signs and symptoms. schwannomatosis.
• Surgery and other procedures to • Gabapentin (Neurotin) or
treat severe symptoms or pregabalin (Lyrica) for nerve
complications of neurofibromatosis. pain
– Surgery to remove tumors. • Tricyclic antidepressants such
– Stereotactic radiosurgery as amitriptyline
– Auditory brainstem implants • Serotonin and
and cochlear implants norepinephrine reuptake
• Cancer treatment : inhibitors such as duloxetine
(Cymbalta)
– Malignant tumors and other • Epilepsy medications such as
cancers associated with topiramate (Topamax) or
neurofibromatosis are treated carbamazepine (Carbatrol,
with standard cancer therapies, Tegretol)
such as surgery, chemotherapy
and radiation therapy.
• Pain medications
http://www.mayoclinic.org/diseases-conditions/neurofibromatosis/diagnosis-treatment/treatment/txc-20167907
CAUDA EQUINA AND CONUS
LESIONS
 CAUDA EQUINA AND CONUS LESIONS

• Cauda equina lesions are those affecting the spinal nerve

roots within the spinal canal in the lumbar and sacral regions,
without affecting the spinal cord itself.
• Cauda equina lesions produce wasting, weakness, and
fasciculations in the appropriately innervated muscles, often
with substantial pain.
• Etiology :
– narrowing or obstruction of the spinal canal in the
lumbosacral region
– Infections
– central disks below the level of the spinal cord.

Drislane FW et al. Neurology. Blueprints. Philadelphia; Lippincot Williams & Wilkins: 2009
 CAUDA EQUINA AND CONUS LESIONS

• At the tip of the spinal cord, there are lesions of the conus
medullaris, which includes centers controlling bowel, bladder,
and sexual function.
• Causes include compressive lesions and intramedullary
abnormalities such as tumors.
• Lesions here can produce a mixture of upper motor neuron
findings such as hyperreflexia, Babinski signs, and particularly
prominent sphincter dysfunction, as well as radicular pain.
• If they remain below the lumbar cord, conus lesions may
leave leg strength and reflexes intact while severely affecting
bowel, bladder, and sexual function.

Drislane FW et al. Neurology. Blueprints. Philadelphia; Lippincot Williams & Wilkins: 2009
KELEMAHAN OTOT
 MYOPATHY
• Disorders in which there is a primary structural or functional
impairment of muscle (myopathy) can result from a variety of
inherited and acquired disorders

Brust JCM. Current Diagnosis &

Treatment Neurology, 2nd ed
Symptoms and Signs of Myopathy
• Muscle weakness and fatigability are the most frequent
symptoms.
• although fatigability is a common complaint in those with
muscle diseases, excessive fatigability out of proportion to the
degree of weakness should raise suspicion of a NMJ disorder.
• Patients should be asked about the color of their urine, which,
when dark red, suggests myoglobinuria.
• Double vision, difficulty swallowing, and shortness of breath
may be present.
• Muscle tone is usually reduced and in infants may result in a
“floppy infant.”

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

Brust JCM.
Current
Diagnosis &
Treatment
Neurology, 2nd
ed
Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed
MIOSITIS
 Polymyositis
• Polymyositis may occur alone but is frequently associated
with systemic autoimmune diseases
• It may be the first clinical sign of HIV infection.
• T cells are thought to govern the series of inflammatory
events in polymyositis, unlike B cells, which are implicated in
dermatomyositis.

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Clinical Findings
A. Symptoms and Signs
• a progressive limb-girdle pattern
of symmetric weakness
develops usually over weeks to
months (rarely days).
• Additional symptoms and signs
occur when polymyositis is
associated with systemic
autoimmune diseases.
• Shortness of breath may be the •
consequence of cardiac or
pulmonary muscle involvement
or interstitial lung disease.
• Patients with the antisynthetase
syndrome, which is associated
with antibodies to
aminoacyltRNA synthetases
present with fevers, interstitial
lung disease, Raynaud
phenomenon, mechanic hand,
arthralgias, and pulmonary
involvement.
Weight loss,fatigue, and
generalized malaise are
common.

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Laboratory Findings
• The serum CK level may be normal even in patients with
active disease, but is usually 50 times greater than the upper
limit of normal.
• Levels of other muscle enzymes (aldolase, aminotransferases,
and lactate dehydrogenase) may also be elevated.
• When the CK level is high, it may be used to assess disease
activity and response to treatment.
• The presence of antinuclear antibodies (positive ANA titers in
the blood) suggests associated systemic autoimmune disease.

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Treatment
• There is no cure for polymyositis, • If the clinical response is poor :
although its symptoms can often be – the patient may be switched to
effectively treated. azathioprine, 2–3 mg/kg/day
• First-line treatment : divided 2 or 3 a day, beginning
– Prednisone given initially in high with an initial dose of 50 mg/day
doses intravenously, or orally and slowly titrating upward.
depending on clinical severity – Alternately, methotrexate can be
– A maintenance dose of 1 given at a dose of 0.5–0.8
mg/kg/day should be mg/kg/week IM or 15–25
administered for at least 3 mg/week orally.
months. – Mycophenolate mofetil, 1 g
• If a good response is seen, the dose twice a day orally, can also be
should be tapered slowly and used.
maintained at the lowest possible
effective dose. nd
Brust JCM. Current Diagnosis & Treatment Neurology, 2 ed
 Dermatomyositis
• Dermatomyositis usually occurs alone but may be
associated with systemic sclerosis, mixed connective
tissue diseases, and malignancies as a paraneoplastic
manifestation.
• B cells are thought to govern the series of
inflammatory events in dermatomyositis

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Symptoms and Signs
• In general, the clinical manifestations of dermatomyositis are the
same as those of polymyositis with the exception of the following
characteristic skin lesions:
1. Heliotrope rash with eyelid edema and a facial rash
2. Gottron sign (erythema of knuckles accompanied by a raised
violaceous scaly eruption)
3. Erythematous rash over the knees, elbows, malleoli, at the
base of the neck and upper chest (“V” sign), or over upper back
and shoulders (shawl sign) that worsens with sun exposure
4. Dilated capillary loops at the base of the fingernails

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Laboratory Findings
• Laboratory testing is the same as for polymyositis, and EMG
findings are identical in the two disorders.
• Patients with antibodies directed against the Mi-2 antigen
usually have a “V” sign or “shawl” sign skin rash and are highly
steroid sensitive.
• Muscle biopsy
 shows perivascular or interfascicular inflammatory
infiltrates, or both, with perifascicular atrophy

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Treatment
• Treatment and prognosis are generally similar to those of
polymyositis.
• However, the major differences in treatments when compared
to polymyositis are related to skin involvement, which may
require topical corticosteroids and practical steps such as
high-protection sunscreen and protective clothing.

Brust JCM. Current Diagnosis & Treatment Neurology, 2nd ed

 Inclusion-Body Myositis
• This disorder is more common in men than women and has an insidious
onset, usually after 50 years of age.
• The etiology of the myositis is unknown, but accumulating evidence
suggests a T-cell-mediated myocytotoxicity and probably a
multifactorial genetic susceptibility to the disease.
• Clinical Findings
– Weakness and atrophy of the quadriceps and of the forearm flexor
and extensor muscles are characteristic.
– The disease is progressive and is associated with early depression of
the knee reflexes.
– Muscle pain occurs in some patients.
– Dysphagia

Greenberg DA, Aminoff MJ, Simon RP. Clinical neurology. 9th ed. New York: McGraw Hill, 2015.
 Diagnosis
• Serum CK levels may be
normal or mildly increased.
• The diagnosis is confirmed by
histologic examination of
biopsied muscle, showing
– endomysial inflammation,
– vacuolated muscle fibers,
– muscle fiber inclusions of
beta-amyloid,
– and intranuclear and
intracytoplasmic filaments
by electron microscopy or
with immunohistologic
staining.
Greenberg DA, Aminoff MJ, Simon RP. Clinical neurology. 9th ed. New York: McGraw Hill, 2015.
Treatment
• The disorder is unresponsive
to immunosuppressive or
immunomodulatory therapies.
• Intravenous globulin therapy
 its role unclear.
• Patients may eventually
become chair-bound and
require help with the activities
of daily life.
Greenberg DA, Aminoff MJ, Simon RP. Clinical neurology. 9th ed. New York: McGraw Hill, 2015.
GUILLAIN–BARRÉ SYNDROME
 Guillain–Barré Syndrome
• Described as a collection of clinical
syndromes that manifests as an acute
inflammatory polyradiculoneuropathy with
resultant weakness and diminished reflexes.
• Plasma exchange
• Double yg plasma exchange sama
imunoglobulin  kurang efektif

http://emedicine.medscape.com/article/315632-overview
 Guillain–Barré Syndrome
• The main clinical features of GBS:
– Motor weakness,
– Areflexia,
– Paresthesias with minor sensory loss, and
– Increased protein in CSF without pleocytosis

Bradley’s neurology in clinical practice. 7th ed.

 Etiologi
• Specific infectious agents linked to GBS include:
– CMV,
– Epstein-Barr virus,
– varicella-zoster virus (VZV),
– hepatitis A and B,
– HIV,
– Mycoplasma pneumoniae, and
– Haemophilus influenzae.
– Campylobacter jejuni  Gram-negative rod
• A preceding infection may trigger an autoimmune response
through “molecular mimicry,” in which the host generates an
immune response against an infectious organism that shares
epitopes with the host’s peripheral nerves

Bradley’s neurology in clinical practice. 7th ed.

 Subtipe GBS

Bradley’s neurology in clinical practice. 7th ed.

Bradley’s neurology in clinical practice. 7th ed.
Bradley’s neurology in clinical practice. 7th ed.
Bradley’s neurology in clinical
practice. 7th ed.
 Clinical manifestation
• males are more often affected than females (1.5 : 1)
• Patients with classic GBS
– initially present with weakness with or without paresthetic sensory
symptoms, often worse in the hands and fingers.
– The fairly symmetrical weakness of the lower limbs ascends
proximally over hours to several days and may subsequently involve
arms, facial, and oropharyngeal muscles, and in severe cases,
respiratory muscles.
• Less often, weakness may be descending and begin in the upper limbs or
cranial innervated muscles.
• Its severity varies from mild, in which patients are still capable of walking
unassisted, to a nearly total quadriplegia.
• Hyporeflexia or areflexia are the invariable features of GBS but may be absent
early in the course of the disease
– Moderate to severe pain in the extremities, interscapular area, or
back occurs in about 70% of patients during the acute phase of illness,
and this may persist for a year in a third of those affected

Bradley’s neurology in clinical practice. 7th ed.

 DD

Bradley’s neurology in clinical practice. 7th ed.

RADICULAR SYNDROME
 Cervical Radiculopathy
• Cervical radiculopathy is a disease process
marked by nerve compression from herniated
disk material or arthritic bone spurs.
– This impingement typically produces neck and
radiating arm pain or numbness, sensory deficits,
or motor dysfunction in the neck and upper
extremities

http://www.aafp.org/afp/2010/0101/p33.html
 CLINICAL PRESENTATION
• Chronic neck pain associated with spondylosis is
typically bilateral, whereas neck pain associated
with radiculopathy is more often unilateral
• Pain radiation varies depending on the involved
nerve root, although some distributional overlap
may exist.
• sensory or motor dysfunction may be present
without significant pain
• Symptoms are often exacerbated by extension
and rotation of the neck (Spurling sign) which
decreases the size of the neural foramen
http://www.aafp.org/afp/2010/0101/p33.html
http://www.aafp.org/afp/2010/0101/p33.html
NA = not applicable.

http://www.aafp.org/afp/2010/0101/p33.html
http://www.aafp.org/afp/2010/0101/p33.html
NEUROPATI
 Neuropati
• Neuropati perifer  kondisi yang terjadi ketika
saraf yang membawa pesan ke dan dari otak
dan sumsum tulang belakang dari dan ke
seluruh tubuh yang rusak atau sakit.
• Saraf perifer keluar dari sumsum tulang
belakang dan disusun sepanjang garis dalam
tubuh yang disebut dermatom .Biasanya,
kerusakan saraf akan mempengaruhi satu/lebih
dermatom, yang dapat dilacak ke daerah-daerah
tertentu dari tubuh.Kerusakan saraf ini menyela
komunikasi antara otak dan bagian lain dari
tubuh dan dapat mengganggu gerakan otot,
mencegah sensasi normal pada lengan dan kaki,
dan menyebabkan rasa sakit .

http://www.webmd.com/brain/understanding-peripheral-neuropathy-basics?page=3
 Mononeuropathy
• Kerusakan saraf perifer tunggal
• Etiologi : cedera atau trauma fisik seperti dari
kecelakaan
– tekanan pada saraf yg berkepanjangan seperti di kursi roda
atau tempat tidur,atau gerakan tekanan berulang
• Contoh : Carpal tunnel syndrome Orang-orang yang
bekerja memerlukan gerakan pergelangan tangan
berulang (buruh fisik, menggunakan keyboard
komputer untuk waktu lama)
• Palsy N. ulnaris terjadi ketika saraf yang lewat dekat
permukaan kulit pada siku rusak.
• Palsy N .Radial disebabkan oleh cedera pada saraf yang
berjalan sepanjang lengan bawah.
 Polyneuropathy
• ggn bersifat simetris kedua sisi
• tungkai lebih dulu dibanding lengan
• Gejala sensorik: parestesia, disestesia, baal ujung
kaki menyebar ke proksimal
• Kadang parestesi berupa: rasa tidak
menyenangkan, rasa seperti terbakar.
• atrofi otot,hipotoni, refleks tendon turun.
• Saraf otonom terkena: ggn trofik kulit, hilangnya
keringat, ggn vaskuler nyebabkan hipotensi
Tanda dan Gejala Diagnosis
• Gangguan sensorik: • EMG
• Tes konduksi saraf
– Nyeri • Biopsi saraf
– Kehilangan sensorik • Tes tambahan:
– ANA
• Defisit motorik
– Tes darah
• Kehilangan refleks tendon – CRP
– Imaging scan
• Gangguan otonom
– Rheumatoid factor
• Pembesaran saraf – Tes tiroid
– X-ray