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Sistem Neuromuskular

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Title: Neuromuscular Localization Subject: for 2nd year students (ICN) Author: Edward Valenstein Last modified by: user Created Date: 1/7/2000 3:28:22 AM – PowerPoint PPT presentation

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Title: Sistem Neuromuskular


1
Sistem Neuromuskular
2
Sistem Neuromuskular
  • Tiga komponen utama Neuromuskular
  • Nerve
  • Neuromuscular junction
  • Muscle

3
Upper Motor Neuron
  • Semua neuron yang menyalurkan impuls motorik
    secara langsung ke LMN atau melalui
    interneuronnya, tergolong dalam kelompok UMN.
    Neuron-neuron tersebut banyak terdapat di girus
    presentralis dinamakan juga korteks motorik.
    Melalui aksonnya neuron korteks motorik
    menghubungi motoneuron di kornu anterior medulla
    spinalis.

4
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5
Area Motorik
6
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7
Upper motorneuron
Lower motorneuron
8
Lower Motor Neuron
  • Merupakan neuron-neuron yang menyelurkan impuls
    motorik pada bagian perjalanan terakhir (kornu
    anterior medula spinalis) ke sel-sel otot
    skeletal.

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10
Motor end Plate
  • Pada ujungnya setiap akson akan bercabang-cabang
    dan setiap cabang menghubungi membrane serabut
    otot. Serabut-serabut otot setiap unit motorik
    berkisar antara 10-500 serabut otot. Tiap serabut
    otot memilki satu motor end plate.

11
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12
  • Ujung-ujung terminal dari akson mengandung
    mitokondria dan ezim cholin acertyltransferase,
    yang diperlukan untuk sintesis
    neurotransmitter yang dinamakan
    acetylcholine.

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14
Pelepasan Acetilkolin
  • Nerves releasing Achetylcholine at the
    neuromuscular junction (end plate) cause the
    contraction of skeletal muscle. The functional
    unit of a muscle organ is the muscle fiber
    (muscle cell).

15
  • The muscle fiber contracts in an "all-or-none"
    fashion when stimulated by an action potential.
    The action potential first causes intracellular
    Ca release from the sarcoplasmic reticulum and
    the Ca activates a cascade of events which
    results in the movement of actin over myosin
    (sliding filament theory).

16
  • 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

17
Gangguan yang menyebabkan kelemahan gerak
(paralysis)
  • Kelainan pada otot
  • Periodik Paralysis
  • Inflamatory miopathy
  • Miopati karena steroid
  • Rabdomyolisis
  • Neuromuscular junction
  • Miastenia Gravis
  • Botulism
  • Tick paralysis
  • Lambert Eaton Myastenic Syndrome

18
Gangguan yang menyebabkan kelemahan gerak
(paralysis)
  • Neuropati akut
  • Paraneoplastik
  • Vaskulitis (lupus, poliarteritis)
  • Neuropati motorik multifokal
  • Poliradikulopati akut
  • Guillain-Barre syndrome
  • Lime Disease
  • Sindrome Cauda Equina
  • Penyakit Motor neuron
  • Poliomyelitis
  • Amyotropic Lateral Sclerosis (ALS)

19
Gangguan yang menyebabkan kelemahan gerak
(paralysis)
  • Medula Spinalis
  • Inflamasi (mielitis transversus)
  • Mielopati (spondilosis, hematom, infark)
  • Otak (Cerebrum, cerebellum)
  • Lesi di Pons
  • Lesi fokal/multifokal (infark, hematom)

20
Jenis Gangguan Saraf
  • Polyneuropathy motor, sensory,
  • sensorimotor
  • Radiculopathy
  • Polyradiculopathy
  • Plexopathy
  • Mononeuropathy isolated
  • multiplex

21
Klasifikasi kausa
  • Toxic
  • Drugs, alcohol, organophosphates
  • Inflammatory/Immune
  • GBS, CIDP
  • Vasculitis
  • Infective
  • Leprosy, Lyme, HIV, Diphtheria
  • Traumatic
  • Inherited
  • HMSN and HLPP
  • Amyloid
  • Metabolic
  • Diabetes
  • Vitamins B12, B1, E
  • Dialysis, Liver failure
  • Paraneoplastic
  • sensory (anti-Hu)

22
Klasifikasi tipe kerusakan
  • Demyelinating
  • Axonal
  • Small fibre
  • Large fibre
  • Autonomic

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24
Physical findings
Nerve NMJ Muscle Reflexes Usually decr. NL or
decr. NL or decr. Atrophy Can be
severe Minimal Variable Fascic. Sometimes None Non
e Sensory loss Sometimes None None
25
The Motor Unit
Myopathies
Motor Neurone Disorders
Myasthenia etc
Peripheral Neuropathy
26
Gangguan pada saraf
  • Variasi
  • Cell body, axon myelin
  • Fiber size large, small
  • Motor, sensory, autonomic
  • Distribution focal, multifocal, generalized
  • Course acute, subacute, chronic, lifelong
  • Etiology genetic, toxic, metabolic, autoimmune,
    traumatic, vascular, infectious

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28
Gangguan pada Saraf berdasarkan Lokasi
  • Radix radiculopathy
  • Plexus plexopathy
  • Single nerve mononeuropathy
  • Several nerves multiple mononeuropathy, mononeuri
    tis multiplex
  • All nerves, polyneuropathylength-dependent
  • All nerves, polyradiculoneuropathynot
    length-dependent

29
Radix
  • Segmental loss of
  • motor
  • atrophy
  • weakness
  • reflexes
  • sensation
  • Signs usually minimal symptoms can be severe
    (pain)
  • Usually only one limb.

30
Plexus
  • Pain
  • Weakness, atrophy, variable, but usually more
    severe than radiculopathy
  • Usually restricted to one limb
  • Etiology
  • Brachial trauma, neoplasm, idiopathic
  • Lumbosacral diabetes, neoplasm

31
Single nerve (mononeuropathy)
  • Restricted distribution
  • Pain, numbness or tingling, atrophy, weakness
  • Etiology
  • entrapment
  • trauma

32
Carpal tunnel syndrome
  • N.Medianus
  • Pain in hand, forearm, arm
  • Numbness in median distribution
  • Symptoms aggravated by wrist flexion

33
Ulnar neuropathy
  • Numbness
  • Atrophy of first dorsal interosseous
  • Weakness
  • Compression at elbow
  • Entrapment in cubital tunnel
  • Distal injury

34
Radial nerve Saturday night palsy
  • Weakness of wrist finger extensors,
    brachioradialis
  • Pressure palsy
  • Trauma (humerus fracture)

35
Peroneal palsy
  • Crossing legs
  • Weight loss
  • Hospitalization
  • Surgery

36
Several nerves (mononeuritis multiplex)
  • Often painful at onset
  • Often sudden
  • Deficits in the distribution of several
    peripheral nerves (one at a time)
  • Etiology vasculitis

37
All nerves Length-dependent (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

38
Polyneuropathy (contd)
39
Polyneuropathy (contd)
  • 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)

40
All nerves, not length-dependent(polyradiculoneur
opathy)
  • Both proximal and distal weakness
  • Variable sensory symptoms
  • Autonomic symptoms (pulse, blood pressure,
    urination...)
  • Can affect respiration, swallowing
  • Autoimmune

41
Guillain-Barré Syndrome (GBS)
Merupakan penyakit Autoimmun
  • 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

42
Guillain-Barre syndrome
  • Progresses over days to lt4 weeks
  • Typically ascending weakness
  • Reflexes lost early
  • Motor symptoms predominate, but can affect
    sensation and autonomic function
  • Respiratory failure requires support

43
Guillain-Barre syndrome (contd)
  • Penyebab autoimmun
  • Target Antigen biasanya tidak diketahui
  • Pada beberapa kasus Target serangan imun
    gangliosida (GM1, GQ1b)
  • Faktor presipitasi
  • Infeksi virus (HIV, CMV, varicella zoster)
  • Infeksi bakteri (campylobacter jenjuni, typhoid,
    paratyphoid)
  • Immunisasi
  • Sistemik (Hodgkins disease, leukemia,
    hipertiroidisme, sarkoidosis)
  • Transplantasi organ, operasi, kehamilan

44
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45
Latar belakang GBS
  • Epidemiologi GBS
  • 1- 4 kasus/100.000
  • Paling banyak pada pria
  • Meningkat sesuai usia
  • Insidennya bervariasi sesuai musim

46
Gambaran klinis GBS
  • Gangguan Motorik
  • paralisis yang progressif, simetris pada
    extremitas bawah dan atas, bersifat asendern
  • dimulai dari distal ke proksimal
  • Gangguan sensibilitas Stocking, dan glove
    sensory loss (dysesthesia)
  • Gangguan otonom
  • penyebab kematian

47
Clinical Picture of Polyneuropahty (Valenstein,
2000)
48
Gambaran klinis GBS
  • Atypical presentations
  • Miller-Fisher Syndrome
  • Areflexia
  • Ophthalmoplegia
  • Ataxia

49
diagnosis GBS
  • Riwayat penyakit sebelumnya atau vaksinasi
  • Dari pemeriksaan fisik (Physical Exam)
  • Laboratoratorium
  • Peningkatan kadar protein pada pemeriksaan LCS
    dan rendahnya jumlah sel di LCS (disosiasi
    sitoalbumin)
  • Electromyography adanya blok konduksi saraf

50
KRITERIA GBS MENURUT GILROY DAN MEYER (1979)
  • Paralisis flasid simetris, difus
  • Gejala sensoris subyektif
  • Penyembuhan sempurna dalam 6 bulan
  • Disosiasi citoalbumin
  • Tanpa atau sedikit demam saat muncul paralysis
  • AL normal atau lymphositosis dengan sedikit atau
    tanpa kenaikan KED.
  • Harus memenuhi 5 kriteria dari 6 kriteria

51
Pengobatan GBS
  • Fase akut
  • Supportive care monitoring fungsi vital
    (perawatn 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
  • Setelah fese akut
  • Program rehabilitasi, bladder training, perbaikan
    ADL (activity daily living)

52
Summary of nerve disorders
  • Root Disk, Herpes zoster
  • Plexus Autoimmune, trauma, neoplasm
  • Mononeuropathy Trauma, entrapment
  • Multiplemononeuropathy Vasculitis...
  • Polyneuropathy Toxic, metabolic, nutritional
  • Polyradiculo-neuropathy Autoimmune

53
Neuromuscular junction
54
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)

55
Myasthenia Gravis
  • Kelemahan yang berfluktuasi
  • Mata ptosis, diplopia
  • Bulbar weakness dysarthria, dysphagia
  • Kelemahan otot proksimal
  • Kelemahan respirasi
  • Normal reflexes
  • Normal sensation
  • Berkaitan dg thymoma
  • Berkaitan dg penyakit autoimun

Penyakit autoimun pada transmisi neuromuskular
junction yang diakibatkan oleh antibodi yang
menyerang reseptor asetilkolin atau melawan
muscle spesific receptor tyrosine kinase
56
  • Myasthenia gravis is a neuromuscular disease
    leading to fluctuating muscle weakness and
    fatiguability.
  • It is an autoimmune disorder, in which weakness
    is caused by circulating antibodies that block
    acetylcholine receptors at the post-synaptic
    neuromuscular junction, inhibiting the
    stimulative effect of the neurotransmitter
    acetylcholine.
  • Myasthenia is treated medically with
    cholinesterase inhibitors or immunosuppressants,
    and, in selected cases, thymectomy.
  • At 200400 cases per million it is one of the
    less common autoimmune disorders.

57
  • Muscles become progressively weaker during
    periods of activity and improve after periods of
    rest. Muscles that control eye and eyelid
    movement,
  • facial expression, chewing, talking, and
    swallowing are especially susceptible. The
    muscles that control breathing and neck and limb
    movements can also be affected

58
Myasthenia Gravis
  • Terapi
  • Acetyl cholinesterase inhibitors pyridostigmin
    bromida 3x 60 mg
  • Plasmapharesis plasma exchange
  • Imunoglobulin IV
  • Immunosupresan (kontroversi)
  • Steroid mulai 12-50 mg
  • Azathioprine 50 mg/hari
  • Cyclosporine awal 3-4 mg/kg/hari dalam dosis
    terbagi
  • Cyclophosphamide dosis 1-2 mg/kg/ hari
  • Thymectomy , indikasi
  • Timoma
  • Generalized myastenia yang tidak terkontrol
    dengan antikolinesterase (lt 50 th, 6-12 bulan
    tidak ada remisi spontan)

59
Krisis Mistenia
  • Adalah keadaan eksaserbasi penyakit Mistenia
    gravis dimana kelumpuhan menyebabkan episode akut
    kegagalan pernafasan
  • Terjadi pada 74 setelah 2 tahun miastenia gravis

60
Krisis Mistenia
  • Faktor pencetus
  • Infeksi, terutama infeksi saluran nafas
  • Pemakaian obat2an aminoglikosid, ciprofloksasin,
    klindamisin, propanolol, fenitoin
  • Tidak diketahui (30-40)

61
Krisis Mistenia
  • Terapi
  • Kontrol airways, dan perbaiki ventilasi (jika
    perlu menggunakan ventilator)
  • Terapi antikolinesterase
  • Kortikosteroid
  • Plasma axchange atau IV Ig

62
Penyakit otot (myopathy)
  • Symmetrical proximal weakness
  • Reflexes normal (sometimes depressed)
  • No sensory loss

63
Myopathy (contd)
  • Inherited
  • Dystrophies
  • Congenital myopathies
  • Channelopathies
  • Acquired
  • endocrine
  • inflammatory, including autoimmune
  • toxic (drugs...)

64
Inflammatory myopathies
  • Polymyositis
  • isolated
  • with collagen vascular disease
  • Dermatomyositis
  • childhood
  • adult association with cancer
  • others

65
Dystrophy Musculorum
  • Muscular dystrophy is a genetic condition causing
    muscle weakness

66
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)
67
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

68
Amyotrophic lateral sclerosis
  • Lou 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

69
Amyotrophic lateral sclerosis
  • The disease belongs to a group of disorders known
    as motor neuron diseases, which are characterized
    by the gradual degeneration and death of motor
    neurons.
  • In ALS, both the upper motor neurons and the
    lower motor neurons degenerate or die, ceasing to
    send messages to muscles
  • At first, this causes mild muscle problems. Some
    people notice
  • Trouble walking or running
  • Trouble writing
  • Speech problems

70
Multiple sclerosis
  • Multiple sclerosis (MS) is a nervous system
    disease that affects your brain and spinal cord.
    It damages the myelin sheath
  • No one knows what causes MS. However, viral and
    autoimmune etiologies have been hypothesized. It
    may be an autoimmune disease
  • The symptom can include
  • Visual disturbances
  • Muscle weakness
  • Trouble with coordination and balance
  • Sensations such as numbness, prickling, or "pins
    and needles"
  • Thinking and memory problems

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