Iwan Setiawan
Bagian Ilmu Penyakit Saraf
Fakultas Kedokteran UMS
Sistem Neuromuskular
Tiga komponen utama Neuromuskular
Nerve
Neuromuscular junction
Muscle
Area Motorik
Upper motorneuron
Lower motorneuron
neuron-neuron yang
menyelurkan impuls motorik pada bagian
perjalanan terakhir (kornu anterior medula
spinalis) ke sel-sel otot skeletal.
Ujung-ujung
Pelepasan Acetilkolin
Nerves
The
Tanda-tanda
kelumpuhan UMN :
Hiperrefleksia
Terdapat refleks
patologis
Tonus otot meninggi
atau hipertonia
Terdapat Klonus
Tidak terdapat atrofi
otot yang lumpuh
Refleks automatisme
spinal (-)
Tanda-tanda
kelumpuhan LMN :
Arefleksia (hilangnya
refleks tendo)
Tidak ada refleks
patologis
Hilangnya tonus otot
(flacid)
Tidak terdapat klonus
Terdapat atrofi pada
otot yang lumpuh
Periodik Paralysis
Inflamatory miopathy
Miopati karena steroid
Rabdomyolisis
Neuromuscular junction
Miastenia Gravis
Botulism
Tick paralysis
Lambert Eaton Myastenic Syndrome
Paraneoplastik
Vaskulitis (lupus, poliarteritis)
Neuropati motorik multifokal
Poliradikulopati akut
Guillain-Barre syndrome
Lime Disease
Sindrome Cauda Equina
Poliomyelitis
Amyotropic Lateral Sclerosis (ALS)
Lesi di Pons
Lesi fokal/multifokal (infark, hematom)
motor, sensory,
sensorimotor
Radiculopathy
Polyradiculopathy
Plexopathy
Mononeuropathy:
isolated
multiplex
Klasifikasi kausa
Toxic
GBS, CIDP
Vasculitis
Infective
Drugs, alcohol,
organophosphates
Inflammatory/Immune
Traumatic
Inherited
HMSN and HLPP
Amyloid
Metabolic
Diabetes
Vitamins: B12, B1, E
Dialysis, Liver failure
Paraneoplastic
sensory (anti-Hu)
fibre
Large fibre
Autonomic
Physical findings
Nerve
NMJ
Muscle
Reflexes
Usually decr.
NL or decr.
NL or decr.
Atrophy
Can be severe
Minimal
Variable
Fascic.
Sometimes
None
None
None
None
Motor Neurone
Disorders
Peripheral Neuropathy
Myasthenia etc
nerve
Several nerves
All
radiculopathy
plexopathy
mononeuropathy
multiple mononeuropathy,
mononeuritis multiplex
polyneuropathy
nerves,
length-dependent
All nerves,
polyradiculoneuropathy
not length-dependent
Radix
Segmental
loss of
motor
atrophy
weakness
reflexes
sensation
Signs
Plexus
Pain
Weakness,
distribution
Pain, numbness or tingling,
atrophy, weakness
Etiology:
entrapment
trauma
in hand,
forearm, arm
Numbness in
median distribution
Symptoms
aggravated by wrist
flexion
Ulnar neuropathy
Numbness
Atrophy
of first dorsal
interosseous
Weakness
Compression at elbow
Entrapment in cubital
tunnel
Distal injury
of wrist &
finger extensors,
brachioradialis
Pressure palsy
Trauma (humerus
fracture)
Peroneal palsy
Crossing
legs
Weight loss
Hospitalization
Surgery
painful at onset
Often sudden
Deficits in the distribution of several
peripheral nerves (one at a time)
Etiology: vasculitis
Polyneuropathy (contd)
Polyneuropathy (contd)
Most
Definisi GBS :
Penyakit demyelinasi akut, yang terutama
mengenai susunan saraf tepi. Penyakit
inflamasi pada sistim saraf tepi mempunyai
karakteristik adanya infiltrasi limfosit dan
makrofag dengan destruksi myelin
Derajad dan lokasi kerusakan tergantung
saraf yang bermyelin: Motorik
Guillain-Barre syndrome
Progresses
Faktor presipitasi:
GBS
4 kasus/100.000
Paling banyak pada pria
Meningkat sesuai usia
Insidennya bervariasi sesuai musim
Gangguan Motorik:
paralisis yang progressif, simetris pada extremitas
bawah dan atas, bersifat asendern
dimulai dari distal ke proksimal
Gangguan otonom:
penyebab kematian
presentations
Miller-Fisher
Syndrome
Areflexia
Ophthalmoplegia
Ataxia
diagnosis GBS
Riwayat
Electromyography
Pengobatan GBS
Fase akut
Supportive
ICU)
Pemberian IV imunoglobulin (ivIg) 400 mg/kg
selama 5 hari, plasmapheresis 40-50 ml/kg plasma
exchange diberikan 4 kali seminggu
Kortikosteroid
Artificial ventilation (if necessary) paralysis
diafragma
Neuromuscular junction
of acetyl choline:
Botulism
receptor)
receptor blockade:
Myasthenia Gravis
Muscles
Myasthenia Gravis
Terapi:
Krisis Mistenia
Adalah
Terjadi
Krisis Mistenia
Faktor pencetus :
Infeksi,
Krisis Mistenia
Terapi :
Kontrol
proximal weakness
Reflexes normal (sometimes depressed)
No sensory loss
Myopathy (contd)
Inherited
Dystrophies
Congenital
myopathies
Channelopathies
Acquired
endocrine
inflammatory, including
toxic
(drugs...)
autoimmune
Inflammatory myopathies
Polymyositis
isolated
with
collagen vascular
disease
Dermatomyositis
childhood
adult:
others
Dystrophy Musculorum
Muscular
Dermatomyositis - Polymyositis
KRITERIA DIAGNOSIS
Kelemahan otot-otot proksimal simetris
Rash tipikal pada dermatomyositis
Peningkatan enzim otot / plasma muscle enzymes (CK, aldolase, AST),
khususnya creatine kinase
Terdapat korelasi antara beratnya kelemahan dengan peningkatan enzim
Gambaran myopati pada pemeriksaan needle EMG
Gambaran abnormalitas yang khas pada biopsi otot (nekrosis serabut otot
dan degenerasi, dengan infiltrasi sel-sel inflamasi)
Polymyositis
Polymyositis
is a disease of muscle
featuring inflammation of the muscle fibers
The cause of the disease is not known
Polymyositis is slightly more common in
females. It affects all age groups, although
its onset is most common in middle
childhood and in the 20s
Weakness of muscles is the most
common symptom of polymyositis
Gehrig's disease
Amyotrophic lateral sclerosis (ALS) is a
nervous system disease that attacks
nerve cells called neurons in your brain
and spinal cord
The cause of ALS is not known
Multiple sclerosis
Visual disturbances
Muscle weakness
Trouble with coordination and balance
Sensations such as numbness, prickling, or "pins and needles"
Thinking and memory problems
Myopathy
predilection for neck, limb girdle and proximal muscles
occasional respiratory muscle involvement
possible risk of myoglobulinuria
no sensory loss
normal tendon reflexes (early stage)
Neuromuscular junction
cranial, limb girdle and proximal muscles
may affect respiratory muscles
no sensory loss
autonomic symptoms present if pre-synaptic
fatigueability when post-synaptic, post-exercise increase in strength when pre-synaptic
Neuropathy
weakness and sensory signs
may have associated autonomic signs
may involve cranial nerves
tendon reflexes decreased or absent
Motor neuron
predominantly motor signs
occasional sensory symptoms
often asymmetric
tendon reflexes may be increased if amyotrophic lateral sclerosis