neuro muscular

Sistem NeuromuskularSistem Neuromuskular

Sistem NeuromuskularSistem Neuromuskular

Tiga komponen utama NeuromuskularTiga komponen utama Neuromuskular

NerveNerve

Neuromuscular junctionNeuromuscular junction

MuscleMuscle

Upper Motor NeuronUpper Motor Neuron

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

Area Motorik

Upper motorneuron

Lower motorneuron

Lower Motor NeuronLower Motor Neuron

Merupakan neuron-neuron yang Merupakan neuron-neuron yang menyelurkan impuls motorik pada bagian menyelurkan impuls motorik pada bagian perjalanan terakhir (kornu anterior medula perjalanan terakhir (kornu anterior medula spinalis) ke sel-sel otot skeletal. spinalis) ke sel-sel otot skeletal.

Motor end PlateMotor end Plate

Pada ujungnya setiap akson akan Pada ujungnya setiap akson akan bercabang-cabang dan setiap cabang bercabang-cabang dan setiap cabang menghubungi membrane serabut otot. menghubungi membrane serabut otot. Serabut-serabut otot setiap unit motorik Serabut-serabut otot setiap unit motorik berkisar antara 10-500 serabut otot. Tiap berkisar antara 10-500 serabut otot. Tiap serabut otot memilki satu “serabut otot memilki satu “motor end motor end plateplate”. ”.

Ujung-ujung terminal dari akson Ujung-ujung terminal dari akson mengandung mitokondria dan ezim “cholin mengandung mitokondria dan ezim “cholin acertyltransferase”, yang diperlukan untuk acertyltransferase”, yang diperlukan untuk sintesis “sintesis “neurotransmitterneurotransmitter” yang ” yang dinamakan “dinamakan “acetylcholineacetylcholine”. ”.

Pelepasan AcetilkolinPelepasan Acetilkolin

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

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

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

Gangguan yang menyebabkan kelemahan Gangguan yang menyebabkan kelemahan gerak (paralysis)gerak (paralysis)

Kelainan pada ototKelainan pada otot Periodik ParalysisPeriodik Paralysis Inflamatory miopathyInflamatory miopathy Miopati karena steroidMiopati karena steroid RabdomyolisisRabdomyolisis

Neuromuscular junctionNeuromuscular junction Miastenia GravisMiastenia Gravis BotulismBotulism Tick paralysisTick paralysis Lambert Eaton Myastenic SyndromeLambert Eaton Myastenic Syndrome


Neuropati akutNeuropati akut ParaneoplastikParaneoplastik Vaskulitis (lupus, poliarteritis)Vaskulitis (lupus, poliarteritis) Neuropati motorik multifokalNeuropati motorik multifokal

Poliradikulopati akutPoliradikulopati akut Guillain-Barre syndromeGuillain-Barre syndrome Lime DiseaseLime Disease Sindrome Cauda EquinaSindrome Cauda Equina

Penyakit Motor neuronPenyakit Motor neuron PoliomyelitisPoliomyelitis Amyotropic Lateral Sclerosis (ALS)Amyotropic Lateral Sclerosis (ALS)


Medula SpinalisMedula Spinalis Inflamasi (mielitis transversus)Inflamasi (mielitis transversus) Mielopati (spondilosis, hematom, infark)Mielopati (spondilosis, hematom, infark)

Otak (Cerebrum, cerebellum)Otak (Cerebrum, cerebellum) Lesi di PonsLesi di Pons Lesi fokal/multifokal (infark, hematom)Lesi fokal/multifokal (infark, hematom)

Jenis Gangguan SarafJenis Gangguan Saraf

Polyneuropathy: motor, sensory,Polyneuropathy: motor, sensory,

sensorimotorsensorimotorRadiculopathyRadiculopathyPolyradiculopathyPolyradiculopathyPlexopathyPlexopathyMononeuropathy: isolatedMononeuropathy: isolated

multiplexmultiplex

Klasifikasi kausaKlasifikasi kausa InheritedInherited

HMSN and HLPPHMSN and HLPP AmyloidAmyloid

MetabolicMetabolic DiabetesDiabetes Vitamins: B12, B1, EVitamins: B12, B1, E Dialysis, Liver failureDialysis, Liver failure

ParaneoplasticParaneoplastic sensory (anti-Hu)sensory (anti-Hu)

ToxicToxic Drugs, alcohol, Drugs, alcohol,

organophosphatesorganophosphates

Inflammatory/ImmuneInflammatory/Immune GBS, CIDPGBS, CIDP Vasculitis Vasculitis

InfectiveInfective Leprosy, Lyme, HIV, Leprosy, Lyme, HIV,

DiphtheriaDiphtheria

TraumaticTraumatic

Klasifikasi tipe kerusakanKlasifikasi tipe kerusakan

DemyelinatingDemyelinatingAxonalAxonalSmall fibreSmall fibreLarge fibreLarge fibreAutonomicAutonomic

Physical findingsPhysical 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

The Motor UnitThe Motor Unit

Motor NeuroneDisorders

Myopathies

Myasthenia etcPeripheral Neuropathy

Gangguan pada saraf: Gangguan pada saraf:

Variasi:Variasi: Cell body, axon & myelinCell body, axon & myelin Fiber size: large, smallFiber size: large, small Motor, sensory, autonomicMotor, sensory, autonomic Distribution: focal, multifocal, generalizedDistribution: focal, multifocal, generalized Course: acute, subacute, chronic, lifelongCourse: acute, subacute, chronic, lifelong Etiology: genetic, toxic, metabolic, Etiology: genetic, toxic, metabolic,

autoimmune, traumatic, vascular, infectiousautoimmune, traumatic, vascular, infectious

Gangguan pada Saraf: Gangguan pada Saraf: berdasarkan Lokasiberdasarkan Lokasi

RadixRadix radiculopathyradiculopathy PlexusPlexus plexopathyplexopathy Single nerveSingle nerve mononeuropathymononeuropathy Several nervesSeveral nerves multiple mononeuropathy,multiple mononeuropathy,

mononeuritis multiplexmononeuritis multiplex All nerves, All nerves, polyneuropathypolyneuropathy

length-dependentlength-dependent All nerves, All nerves, polyradiculoneuropathypolyradiculoneuropathy

not length-dependentnot length-dependent

RadixRadix

Segmental loss ofSegmental loss ofmotormotor

atrophyatrophyweaknessweakness

reflexesreflexessensationsensation

Signs usually minimal; symptoms can be Signs usually minimal; symptoms can be severe (pain); severe (pain);

Usually only one limb.Usually only one limb.

PlexusPlexus

PainPainWeakness, atrophy, variable, but Weakness, atrophy, variable, but

usually more severe than radiculopathyusually more severe than radiculopathyUsually restricted to one limbUsually restricted to one limbEtiology: Etiology:

Brachial: trauma, neoplasm, idiopathicBrachial: trauma, neoplasm, idiopathicLumbosacral: diabetes, neoplasmLumbosacral: diabetes, neoplasm

Single nerve (mononeuropathy)Single nerve (mononeuropathy)

Restricted distributionRestricted distributionPain, numbness or tingling, Pain, numbness or tingling,

atrophy, weaknessatrophy, weaknessEtiology:Etiology:

entrapmententrapment traumatrauma

Carpal tunnel syndromeCarpal tunnel syndrome

N.MedianusN.MedianusPain in hand, Pain in hand,

forearm, armforearm, armNumbness in Numbness in

median distributionmedian distributionSymptoms Symptoms

aggravated by wrist aggravated by wrist flexionflexion

Ulnar neuropathyUlnar neuropathy

NumbnessNumbness Atrophy of first dorsal Atrophy of first dorsal

interosseousinterosseous WeaknessWeakness Compression at elbowCompression at elbow Entrapment in cubital Entrapment in cubital

tunneltunnel Distal injuryDistal injury

Radial nerve: Saturday night palsyRadial nerve: Saturday night palsy

Weakness of wrist & Weakness of wrist & finger extensors, finger extensors, brachioradialisbrachioradialis

Pressure palsyPressure palsy Trauma (humerus Trauma (humerus

fracture)fracture)

Peroneal palsyPeroneal palsy

Crossing legsCrossing legsWeight lossWeight lossHospitalizationHospitalizationSurgerySurgery

Several nerves (mononeuritis multiplex)Several nerves (mononeuritis multiplex)

Often painful at onsetOften painful at onsetOften suddenOften suddenDeficits in the distribution of several Deficits in the distribution of several

peripheral nerves (one at a time)peripheral nerves (one at a time)Etiology: vasculitisEtiology: vasculitis

All nerves: Length-dependent All nerves: Length-dependent (polyneuropathy)(polyneuropathy)

Lower before upper extremityLower before upper extremity Distal first (feet)Distal first (feet) Atrophy of intrinsic foot musclesAtrophy of intrinsic foot muscles Decreased ankle jerksDecreased ankle jerks Stocking, then glove sensory lossStocking, then glove sensory loss Distal motor and sensory findings Distal motor and sensory findings

always much more severe than proximalalways much more severe than proximal

Polyneuropathy (cont’d)Polyneuropathy (cont’d)

Polyneuropathy (cont’d)Polyneuropathy (cont’d)

Most common kind of neuropathyMost common kind of neuropathyEtiologyEtiology

metabolic metabolic (diabetes, renal failure)(diabetes, renal failure)nutritional nutritional (thiamine, B12 deficiency)(thiamine, B12 deficiency) toxic toxic (heavy metals, organic solvents, (heavy metals, organic solvents,

some drugs)some drugs) familial familial (Charcot-Marie-Tooth)(Charcot-Marie-Tooth)

All nerves, not length-dependentAll nerves, not length-dependent(polyradiculoneuropathy)(polyradiculoneuropathy)

Both proximal and distal weaknessBoth proximal and distal weaknessVariable sensory symptomsVariable sensory symptomsAutonomic symptoms (pulse, blood Autonomic symptoms (pulse, blood

pressure, urination...)pressure, urination...)Can affect respiration, swallowingCan affect respiration, swallowingAutoimmuneAutoimmune

Guillain-Barré Syndrome (GBS)Guillain-Barré Syndrome (GBS)

Definisi GBS :Definisi GBS :Penyakit demyelinasi akut, yang terutama Penyakit demyelinasi akut, yang terutama

mengenai susunan saraf tepi. mengenai susunan saraf tepi. Penyakit Penyakit inflamasi pada sistim saraf tepi mempunyai inflamasi pada sistim saraf tepi mempunyai karakteristik adanya infiltrasi limfosit dan karakteristik adanya infiltrasi limfosit dan makrofag dengan destruksi myelinmakrofag dengan destruksi myelin

Derajad dan lokasi kerusakan tergantung Derajad dan lokasi kerusakan tergantung saraf yang bermyelin: Motoriksaraf yang bermyelin: Motorik

Merupakan penyakit Autoimmun

Guillain-Barre syndromeGuillain-Barre syndrome

Progresses over days to <4 weeksProgresses over days to <4 weeksTypically ascending weaknessTypically ascending weaknessReflexes lost earlyReflexes lost earlyMotor symptoms predominate, but can Motor symptoms predominate, but can

affect sensation and autonomic functionaffect sensation and autonomic functionRespiratory failure requires supportRespiratory failure requires support

Guillain-Barre syndrome (cont’d)Guillain-Barre syndrome (cont’d)

Penyebab : autoimmunPenyebab : autoimmun Target Antigen biasanya tidak diketahuiTarget Antigen biasanya tidak diketahui Pada beberapa kasus: Target serangan imun Pada beberapa kasus: Target serangan imun

gangliosida (GM1, GQ1b)gangliosida (GM1, GQ1b)

Faktor presipitasi: Faktor presipitasi: Infeksi virus (HIV, CMV, varicella zoster)Infeksi virus (HIV, CMV, varicella zoster) Infeksi bakteri (campylobacter jenjuni, typhoid, Infeksi bakteri (campylobacter jenjuni, typhoid,

paratyphoid)paratyphoid) ImmunisasiImmunisasi Sistemik (Hodgkins disease, leukemia, hipertiroidisme, Sistemik (Hodgkins disease, leukemia, hipertiroidisme,

sarkoidosis)sarkoidosis) Transplantasi organ, operasi, kehamilanTransplantasi organ, operasi, kehamilan

Latar belakang GBSLatar belakang GBS

Epidemiologi GBSEpidemiologi GBS

1- 4 kasus/100.0001- 4 kasus/100.000 Paling banyak pada priaPaling banyak pada pria Meningkat sesuai usiaMeningkat sesuai usia Insidennya bervariasi sesuai musimInsidennya bervariasi sesuai musim

Gambaran klinis GBSGambaran klinis GBS

- Gangguan Motorik:Gangguan Motorik: paralisis yang progressif, simetris pada extremitas paralisis yang progressif, simetris pada extremitas

bawah dan atas, bersifat asendernbawah dan atas, bersifat asendern

dimulai dari distal ke proksimaldimulai dari distal ke proksimal

- Gangguan sensibilitas: Gangguan sensibilitas: Stocking, dan glove Stocking, dan glove sensory loss (dysesthesia)sensory loss (dysesthesia)

- Gangguan otonom:Gangguan otonom:

penyebab kematianpenyebab kematian

Clinical Picture of Polyneuropahty(Valenstein, 2000)

Gambaran klinis GBSGambaran klinis GBS

Atypical presentationsAtypical presentations

Miller-Fisher SyndromeMiller-Fisher Syndrome AreflexiaAreflexia OphthalmoplegiaOphthalmoplegia AtaxiaAtaxia

diagnosis GBSdiagnosis GBS

Riwayat penyakit sebelumnya atau vaksinasi Riwayat penyakit sebelumnya atau vaksinasi Dari pemeriksaan fisik (Physical Exam)Dari pemeriksaan fisik (Physical Exam) Laboratoratorium:Laboratoratorium:

Peningkatan kadar protein pada pemeriksaan LCS dan Peningkatan kadar protein pada pemeriksaan LCS dan rendahnya jumlah sel di LCS (disosiasi sitoalbumin)rendahnya jumlah sel di LCS (disosiasi sitoalbumin)

Electromyography – adanya blok konduksi sarafElectromyography – adanya blok konduksi saraf

1. Paralisis flasid simetris, difus

2. Gejala sensoris subyektif

3. Penyembuhan sempurna dalam 6 bulan

4. Disosiasi citoalbumin

5. Tanpa atau sedikit demam saat muncul paralysis

6. AL normal atau lymphositosis dengan sedikit atau tanpa kenaikan KED.

Harus memenuhi 5 kriteria dari 6 kriteria

KRITERIA GBS MENURUT GILROY DAN MEYER (1979)

Pengobatan GBSPengobatan GBSFase akutFase akut Supportive care : monitoring fungsi vital Supportive care : monitoring fungsi vital

(perawatn ICU)(perawatn ICU) Pemberian IV imunoglobulin (ivIg) 400 mg/kg Pemberian IV imunoglobulin (ivIg) 400 mg/kg

selama 5 hari, plasmapheresis 40-50 ml/kg selama 5 hari, plasmapheresis 40-50 ml/kg plasma exchange diberikan 4 kali semingguplasma exchange diberikan 4 kali seminggu

Kortikosteroid Kortikosteroid Artificial ventilation (if necessary) Artificial ventilation (if necessary) paralysis paralysis

diafragmadiafragma

Setelah fese akutSetelah fese akut Program rehabilitasi, bladder training, perbaikan Program rehabilitasi, bladder training, perbaikan

ADL (activity daily living)ADL (activity daily living)

Summary of nerve disordersSummary of nerve disorders

RootRoot Disk, Herpes zosterDisk, Herpes zoster PlexusPlexus Autoimmune, trauma, Autoimmune, trauma,

neoplasmneoplasm MononeuropathyMononeuropathy Trauma, entrapmentTrauma, entrapment MultipleMultiple

mononeuropathymononeuropathy Vasculitis...Vasculitis... PolyneuropathyPolyneuropathy Toxic, metabolic, nutritionalToxic, metabolic, nutritional Polyradiculo-Polyradiculo-

neuropathyneuropathy AutoimmuneAutoimmune

Neuromuscular junctionNeuromuscular junction

Disorders of the neuromusuclar Disorders of the neuromusuclar junctionjunction

Release of acetyl choline:Release of acetyl choline:BotulismBotulism (toxin = endopeptidase targeting (toxin = endopeptidase targeting

various proteins mediating exocytosis)various proteins mediating exocytosis)Lambert-Eaton myasthenic syndrome Lambert-Eaton myasthenic syndrome

(antibodies to voltage-gated calcium channel)(antibodies to voltage-gated calcium channel)Acetylcholine receptor blockade:Acetylcholine receptor blockade:

Myasthenia gravisMyasthenia gravis (antibodies to ACh (antibodies to ACh receptor)receptor)

Myasthenia GravisMyasthenia Gravis Kelemahan yang berfluktuasiKelemahan yang berfluktuasi Mata: ptosis, diplopia Mata: ptosis, diplopia Bulbar weakness: dysarthria, Bulbar weakness: dysarthria,

dysphagiadysphagia Kelemahan otot proksimalKelemahan otot proksimal Kelemahan respirasiKelemahan respirasi Normal reflexesNormal reflexes Normal sensationNormal sensation Berkaitan dg thymomaBerkaitan dg thymoma Berkaitan dg penyakit Berkaitan dg penyakit

autoimunautoimun

Penyakit autoimun pada transmisi neuromuskular junction yang diakibatkan oleh antibodi yang menyerang reseptor asetilkolin atau melawan muscle spesific receptor tyrosine kinase

Myasthenia gravisMyasthenia gravis is a neuromuscular disease leading is a neuromuscular disease leading to fluctuating muscle weakness and fatiguability. to fluctuating muscle weakness and fatiguability.

It is an autoimmune disorder, in which weakness is It is an autoimmune disorder, in which weakness is caused by circulating antibodies that caused by circulating antibodies that block acetylcholine block acetylcholine receptorsreceptors at the post-synaptic neuromuscular junction, at the post-synaptic neuromuscular junction, inhibiting the stimulative effect of the neurotransmitter inhibiting the stimulative effect of the neurotransmitter acetylcholine. acetylcholine.

Myasthenia is treated medically with Myasthenia is treated medically with cholinesterase cholinesterase inhibitorsinhibitors or immunosuppressants, and, in selected or immunosuppressants, and, in selected cases, thymectomy. cases, thymectomy.

At 200–400 cases per million it is one of the less At 200–400 cases per million it is one of the less common autoimmune disorders.common autoimmune disorders.

Muscles become progressively weaker Muscles become progressively weaker during periods of activity and improve after during periods of activity and improve after periods of rest. Muscles that control eye periods of rest. Muscles that control eye and eyelid movement, and eyelid movement,

facial expression, chewing, talking, and facial expression, chewing, talking, and swallowing are especially susceptible. The swallowing are especially susceptible. The muscles that control breathing and neck muscles that control breathing and neck and limb movements can also be affected and limb movements can also be affected

Myasthenia GravisMyasthenia GravisTerapi:Terapi:

Acetyl cholinesterase inhibitors : pyridostigmin bromida 3x 60 Acetyl cholinesterase inhibitors : pyridostigmin bromida 3x 60 mgmg

Plasmapharesis : plasma exchangePlasmapharesis : plasma exchange Imunoglobulin IVImunoglobulin IV Immunosupresan (kontroversi)Immunosupresan (kontroversi)

Steroid : mulai 12-50 mgSteroid : mulai 12-50 mg Azathioprine : 50 mg/hariAzathioprine : 50 mg/hari Cyclosporine : awal 3-4 mg/kg/hari dalam dosis terbagiCyclosporine : awal 3-4 mg/kg/hari dalam dosis terbagi Cyclophosphamide : dosis 1-2 mg/kg/ hariCyclophosphamide : dosis 1-2 mg/kg/ hari

Thymectomy , indikasi:Thymectomy , indikasi: TimomaTimoma Generalized myastenia yang tidak terkontrol dengan Generalized myastenia yang tidak terkontrol dengan

antikolinesterase (< 50 th, 6-12 bulan tidak ada remisi spontan)antikolinesterase (< 50 th, 6-12 bulan tidak ada remisi spontan)

Krisis MisteniaKrisis Mistenia

Adalah keadaan eksaserbasi penyakit Adalah keadaan eksaserbasi penyakit Mistenia gravis dimana kelumpuhan Mistenia gravis dimana kelumpuhan menyebabkan episode akut kegagalan menyebabkan episode akut kegagalan pernafasanpernafasan

Terjadi pada 74% setelah 2 tahun Terjadi pada 74% setelah 2 tahun miastenia gravismiastenia gravis


Faktor pencetus :Faktor pencetus : Infeksi, terutama infeksi saluran nafasInfeksi, terutama infeksi saluran nafas Pemakaian obat2an: aminoglikosid, Pemakaian obat2an: aminoglikosid,

ciprofloksasin, klindamisin, propanolol, fenitoinciprofloksasin, klindamisin, propanolol, fenitoin Tidak diketahui (30-40%)Tidak diketahui (30-40%)


Terapi :Terapi : Kontrol airways, dan perbaiki ventilasi (jika perlu Kontrol airways, dan perbaiki ventilasi (jika perlu

menggunakan ventilator)menggunakan ventilator) Terapi antikolinesteraseTerapi antikolinesterase KortikosteroidKortikosteroid Plasma axchange atau IV IgPlasma axchange atau IV Ig

Penyakit otot (myopathy)Penyakit otot (myopathy)

Symmetrical proximal weaknessSymmetrical proximal weaknessReflexes normal (sometimes depressed)Reflexes normal (sometimes depressed)No sensory lossNo sensory loss

Myopathy (cont’d)Myopathy (cont’d)

InheritedInheritedDystrophies Dystrophies Congenital myopathiesCongenital myopathiesChannelopathies Channelopathies

AcquiredAcquiredendocrineendocrine inflammatory, including autoimmuneinflammatory, including autoimmune toxic (drugs...)toxic (drugs...)

Inflammatory myopathiesInflammatory myopathiesPolymyositisPolymyositis

isolatedisolatedwith collagen vascular with collagen vascular

diseasediseaseDermatomyositisDermatomyositis

childhoodchildhoodadult: association with adult: association with

cancercancerothersothers

Dystrophy MusculorumDystrophy Musculorum

Muscular dystrophy is a genetic condition causing muscle weakness

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)

Dermatomyositis - PolymyositisDermatomyositis - Polymyositis

PolymyositisPolymyositis

Polymyositis is a disease of muscle Polymyositis is a disease of muscle featuring inflammation of the muscle fibers featuring inflammation of the muscle fibers

The cause of the disease is not knownThe cause of the disease is not knownPolymyositis is slightly more common in Polymyositis is slightly more common in

females. It affects all age groups, although females. It affects all age groups, although its onset is most common in middle its onset is most common in middle childhood and in the 20childhood and in the 20ss

Weakness of muscles is the most common Weakness of muscles is the most common symptom of polymyositis symptom of polymyositis

Amyotrophic lateral sclerosisAmyotrophic lateral sclerosis

Lou Gehrig's disease Lou Gehrig's disease Amyotrophic lateral sclerosis (ALS) is a Amyotrophic lateral sclerosis (ALS) is a

nervous system disease that attacks nerve nervous system disease that attacks nerve cells called neurons in your brain and cells called neurons in your brain and spinal cord spinal cord

The cause of ALS is not known The cause of ALS is not known

Amyotrophic lateral sclerosisAmyotrophic lateral sclerosis The disease belongs to a group of disorders The disease belongs to a group of disorders

known as known as motor neuron diseases,motor neuron diseases, which are which are characterized by the gradual degeneration and characterized by the gradual degeneration and death of motor neurons. death of motor neurons.

In ALS, both the upper motor neurons and the In ALS, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing lower motor neurons degenerate or die, ceasing to send messages to muscles to send messages to muscles

At first, this causes mild muscle problems. Some At first, this causes mild muscle problems. Some people noticepeople notice Trouble walking or running Trouble walking or running Trouble writing Trouble writing Speech problemsSpeech problems

Multiple sclerosisMultiple sclerosis

Multiple sclerosis (MS) is a nervous system disease that Multiple sclerosis (MS) is a nervous system disease that affects your brain and spinal cord. It damages the myelin affects your brain and spinal cord. It damages the myelin sheathsheath

No one knows what causes MS. However, viral and No one knows what causes MS. However, viral and autoimmune etiologies have been hypothesized. It may autoimmune etiologies have been hypothesized. It may be an autoimmune disease be an autoimmune disease

The symptom can include :The symptom can include : Visual disturbancesVisual disturbances Muscle weaknessMuscle weakness Trouble with coordination and balanceTrouble with coordination and balance Sensations such as numbness, prickling, or "pins and needles" Sensations such as numbness, prickling, or "pins and needles" Thinking and memory problemsThinking 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

Key clinical features used to localize a neuromuscular disorder

neuro muscular

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