Medula Spinalis - Saraf Tepi - Otot - 2

Medulla Spinalis, Medulla Spinalis, Saraf Perifer, OtotSaraf Perifer, Otot

dr. Gea Pandhita S, M.Kes, SpSdr. Gea Pandhita S, M.Kes, SpS Bagian Ilmu Penyakit SarafBagian Ilmu Penyakit Saraf

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Medulla SpinalisMedulla Spinalis












Infeksi =Infeksi =

- Mielitis- Mielitis

- Spondilitis- Spondilitis

- Absces- Absces

Vaskular =Vaskular =

Tumor =Tumor =

- Neoplasma- Neoplasma : : Primer & MetastasisPrimer & Metastasis

LokasiLokasi

- Non-neoplasma :- Non-neoplasma :

Degeneratif =Degeneratif =

- HNP- HNP

- Spondilosis- Spondilosis

Trauma =Trauma =


Saraf Perifer & OtotSaraf Perifer & Otot

Disorders of the Motor UnitDisorders of the Motor Unit

• Motor neuron diseaseMotor neuron disease

• Peripheral nerve disordersPeripheral nerve disorders

• Neuromuscular junction diseaseNeuromuscular junction disease

• Muscle diseaseMuscle disease


Motor Neuron DiseaseMotor Neuron Disease

• Diseases that can involve Betz cells Diseases that can involve Betz cells of the motor cortex, the lower CN of the motor cortex, the lower CN motor nuclei, the CST, and/or the motor nuclei, the CST, and/or the anterior horn cellsanterior horn cells– Amyotrophic Lateral Sclerosis (ALS)Amyotrophic Lateral Sclerosis (ALS)– Progressive bulbar palsyProgressive bulbar palsy– Progressive muscular atrophy, spinal Progressive muscular atrophy, spinal

muscular atrophymuscular atrophy– Primary lateral sclerosisPrimary lateral sclerosis

ALS ALS (Amyotrophic Lateral Sclerosis)(Amyotrophic Lateral Sclerosis)

• Loss of motor neurons in the cortex, Loss of motor neurons in the cortex, brainstem and spinal cordbrainstem and spinal cord

• Mix of upper motor neuron and lower motor Mix of upper motor neuron and lower motor neuron findingsneuron findings– Weakness, atrophy, fasciculationsWeakness, atrophy, fasciculations– Slurred speech, difficulty swallowing, shortness Slurred speech, difficulty swallowing, shortness

of breathof breath

• Can start in any extremity or the bulbar Can start in any extremity or the bulbar musculaturemusculature

• Relentlessly progressiveRelentlessly progressive

ALSALS

• 50 % dead in 3 years, 80% dead in 5 50 % dead in 3 years, 80% dead in 5 years, 5-10% live more than 10 yearsyears, 5-10% live more than 10 years

• Death usually from respiratory failureDeath usually from respiratory failure

• Etiology still only theoreticalEtiology still only theoretical– Excess glutamateExcess glutamate– Oxidative stressOxidative stress– Free radicalsFree radicals– Mitochondrial dysfunctionMitochondrial dysfunction


Peripheral Nerve DisordersPeripheral Nerve Disorders• MononeuropathyMononeuropathy

• Mononeuritis multiplexMononeuritis multiplex

• Polyneuropathy (peripheral Polyneuropathy (peripheral neuropathy)neuropathy)

Peripheral Nerve DisordersPeripheral Nerve Disorders

The spectrum of peripheral nerve disorders The spectrum of peripheral nerve disorders includesincludes– Mononeuropathies (entrapment, trauma, etc)Mononeuropathies (entrapment, trauma, etc)

• CTS, Bell’s palsy (?)CTS, Bell’s palsy (?)

– Mononeuritis multiplex (DM, vasculitis)Mononeuritis multiplex (DM, vasculitis)

– Plexopathies (immune, neoplastic)Plexopathies (immune, neoplastic)

– Radiculopathies (discs, immune)Radiculopathies (discs, immune)– Peripheral NeuropathiesPeripheral Neuropathies

Normal peripheral motor nerve anatomy and responses to injuryNormal peripheral motor nerve anatomy and responses to injury ((Quan, 1999)Quan, 1999)

Peripheral Nerve DisordersPeripheral Nerve Disorders• MononeuropathyMononeuropathy

– Pattern of weakness and sensory loss conforms to the Pattern of weakness and sensory loss conforms to the distribution of a single nervedistribution of a single nerve

• Carpal tunnel syndromeCarpal tunnel syndrome• Peroneal palsy at the fibular headPeroneal palsy at the fibular head

• Mononeuritis multiplexMononeuritis multiplex– Multiple nerves affected in a random patternMultiple nerves affected in a random pattern

• Acute onset, frequently painfulAcute onset, frequently painful• Diabetes mellitus, vasculitisDiabetes mellitus, vasculitis

• Polyneuropathy (peripheral neuropathy)Polyneuropathy (peripheral neuropathy)– Distal, symmetricDistal, symmetric

PolyneuropathiesPolyneuropathies

• Can affect different types of fibersCan affect different types of fibers– AutonomicAutonomic– Motor Motor – SensorySensory

• Large well myelinatedLarge well myelinated

• Small poorly myelinated or unmyelinatedSmall poorly myelinated or unmyelinated

Symptoms of a PolyneuropathySymptoms of a Polyneuropathy

• Sensory symptomsSensory symptoms– Start in feet, move proximallyStart in feet, move proximally– Hand sxs appear when LE sxs up to kneesHand sxs appear when LE sxs up to knees– PositivePositive

• Pins and needlesPins and needles

• TinglingTingling

• BurningBurning

– NegativeNegative• NumbnessNumbness

• DeadnessDeadness

• ““Like I’m walking with thick socks on”Like I’m walking with thick socks on”

Polyneuropathy SymptomsPolyneuropathy Symptoms

• MotorMotor– Weakness first in feetWeakness first in feet

• TrippingTripping

• Turn anklesTurn ankles

– Progress to weakness in handsProgress to weakness in hands• Trouble opening jarsTrouble opening jars

• Trouble turning key in lockTrouble turning key in lock

Polyneuropathy: SignsPolyneuropathy: Signs

• Distal sensory lossDistal sensory loss– Large fiberLarge fiber– Small fiberSmall fiber

• Distal weakness and atrophyDistal weakness and atrophy

• Decreased or absent reflexesDecreased or absent reflexes– Ankle jerks lost firstAnkle jerks lost first

Classification of PolyneuropathiesClassification of Polyneuropathies• By types of fibers involvedBy types of fibers involved

– Pure sensoryPure sensory

– Sensory motorSensory motor

– Pure motorPure motor

– AutonomicAutonomic

• By pathologyBy pathology– DemyelinatingDemyelinating

– AxonalAxonal

– MixedMixed

• By tempoBy tempo– AcuteAcute

– SubacuteSubacute

– ChronicChronic

Acute PolyneuropathiesAcute Polyneuropathies

• Guillain Barre SyndromeGuillain Barre Syndrome

• PorphyriaPorphyria– Neuropathy, psychiatric disorder, Neuropathy, psychiatric disorder,

unexplained GI complaintsunexplained GI complaints

• ToxinsToxins– Glue sniffing (n-hexane)Glue sniffing (n-hexane)– ArsenicArsenic

Guillain Barre SyndromeGuillain Barre Syndrome

• Definition of GBS

– acute demyelinating disorder, primarily involving the peripheral nervous system (PNS)

– degree and location of disability depends on the myelinated nerves involved: MOTOR


an inflammatory disease of the peripheral nervous system characterized by lymphocytic and macrophagic infiltration with destruction of myelin.

The condition is often designated as the Landry-Guillain-Barré-Strohl syndrome in recognition of the descriptions provided by these authors



• Most common cause of rapidly Most common cause of rapidly progressive weaknessprogressive weakness

• Demyelinating neuropathyDemyelinating neuropathy

• Ascending weakness which may Ascending weakness which may include cranial neuropathiesinclude cranial neuropathies

• Exam reveals symmetric weakness Exam reveals symmetric weakness with areflexia and large fiber sensory with areflexia and large fiber sensory lossloss

• Bowel and bladder usually preservedBowel and bladder usually preserved


• Respiratory failure can be precipitousRespiratory failure can be precipitous• Other causes of morbidity and mortalityOther causes of morbidity and mortality

– Autonomic instabilityAutonomic instability

– DVTDVT

– InfectionInfection

• Immune mediated, may be post infectiousImmune mediated, may be post infectious• TreatmentTreatment

– Plasma exchangePlasma exchange

– Intravenous immunoglobulinIntravenous immunoglobulin


Subacute PolyneuropathiesSubacute Polyneuropathies• VasculitisVasculitis

– Can be isolated to peripheral nerves or part of a Can be isolated to peripheral nerves or part of a more systemic processmore systemic process

• ParaneoplasticParaneoplastic– May be presenting symptom of the cancerMay be presenting symptom of the cancer

• Chronic inflammatory demyelinating Chronic inflammatory demyelinating polyneuropathypolyneuropathy– With or without a gammopathyWith or without a gammopathy

• ToxinsToxins• DrugDrug

Chronic PolyneuropathiesChronic Polyneuropathies• MetabolicMetabolic

– Diabetes mellitusDiabetes mellitus

– Chronic renal failureChronic renal failure

– Chronic liver failureChronic liver failure

– Thyroid diseaseThyroid disease

• NutritionalNutritional– B12 deficiencyB12 deficiency

• InfectionsInfections– HIVHIV

– LeprosyLeprosy

• InheritedInherited

Evaluation of a PolyneuropathyEvaluation of a Polyneuropathy

• Lab workLab work

• Nerve conduction Nerve conduction study/electromyographystudy/electromyography– Distinguishes between axonal and Distinguishes between axonal and

demyelinatingdemyelinating

– Helps ascertain severityHelps ascertain severity

• Nerve biopsyNerve biopsy– Frequently non-diagnosticFrequently non-diagnostic

– Can establish the dx in certain disorders, such Can establish the dx in certain disorders, such as vasculitis and amyloidosisas vasculitis and amyloidosis


Disorders of the Disorders of the Neuromuscular Junction Neuromuscular Junction

NMJNMJ

• Pre-synapticPre-synaptic– Lambert Eaton myasthenic syndromeLambert Eaton myasthenic syndrome– BotulismBotulism

• Post-synapticPost-synaptic– Myasthenia GravisMyasthenia Gravis

Myasthenia GravisMyasthenia Gravis

• Antibody that alters the acetylcholine Antibody that alters the acetylcholine receptorreceptor– BindingBinding– BlockingBlocking– ModulatingModulating

• Antibody detected inAntibody detected in– 50% of pts with pure ocular MG50% of pts with pure ocular MG– 90-95% of pts with generalized MG90-95% of pts with generalized MG

Clinical Manifestation of MGClinical Manifestation of MG• Sxs worsen with exercise, end of day Sxs worsen with exercise, end of day

(Fatigue)(Fatigue)• OcularOcular

– Droopy eyelids (ptosis)Droopy eyelids (ptosis)– Double vision (diplopia)Double vision (diplopia)

• Extremity weaknessExtremity weakness– Arms > legsArms > legs

• BulbarBulbar– DysarthriaDysarthria– DysphagiaDysphagia

• RespiratoryRespiratory– Shortness of breathShortness of breath

Approach to treating MG

• Remove any exacerbating factors– Infections, medication, endocrine disease

• Acetylcholinesterase inhibitors• Plasma exchange/ intravenous

immunoglobulin• Thymectomy• Immunosuppressants

– Prednisone

– Imuran (azathioprin)


MyopathiesMyopathies

Clinical Manifestations of MyopathiesClinical Manifestations of Myopathies

• Proximal muscle weaknessProximal muscle weakness– Waddling gaitWaddling gait

– Difficulty climbing stairsDifficulty climbing stairs

– Trouble lifting arms over headTrouble lifting arms over head

• Cramps with the metabolic myopathiesCramps with the metabolic myopathies• Myalgias with the inflammatory Myalgias with the inflammatory

myopathiesmyopathies• Swallowing and breathing difficulties, Swallowing and breathing difficulties,

when present, are usually latewhen present, are usually late

Classification of Muscle DiseaseClassification of Muscle Disease

• DystrophiesDystrophies– Duchenne’s Muscular DystrophyDuchenne’s Muscular Dystrophy

– Myotonic DystrophyMyotonic Dystrophy

• Congenital MyopathiesCongenital Myopathies– GlycogenosesGlycogenoses

– MitochondrialMitochondrial

• Acquired MyopathiesAcquired Myopathies– PolymyositisPolymyositis

– DermatomyositisDermatomyositis

– Inclusion body myositisInclusion body myositis

– Drug relatedDrug related

Duchenne’s Muscular DystrophyDuchenne’s Muscular Dystrophy

• X-linked recessive X-linked recessive • Absence of dystrophin proteinAbsence of dystrophin protein• Slow to reach motor milestones, sxs by age 5Slow to reach motor milestones, sxs by age 5

– All walk, may never runAll walk, may never run

– End up in wheelchair by age 10-12End up in wheelchair by age 10-12• Steroids may delay time until wheelchair boundSteroids may delay time until wheelchair bound

• Muscles replaced by fat may appear Muscles replaced by fat may appear hypertrophichypertrophic

• Frequently mildly mentally retardedFrequently mildly mentally retarded• Life expectancy < 20 years with death related Life expectancy < 20 years with death related

to respiratory failure or cardiomyopathyto respiratory failure or cardiomyopathy

PolymyositisPolymyositis

• Presents with proximal muscle weakness in Presents with proximal muscle weakness in 92%92%

• Myalgias in 25%Myalgias in 25%• Associated symptoms may include fever, Associated symptoms may include fever,

weight lossweight loss• Slightly increased risk of cancerSlightly increased risk of cancer

– Bladder, lung, lymphomaBladder, lung, lymphoma

• Biopsy of muscle confirms diagnosisBiopsy of muscle confirms diagnosis• Treatment with immunosuppressionTreatment with immunosuppression

– PrednisonePrednisone

– MethotrexateMethotrexate

Evaluation of the Patient with Evaluation of the Patient with Suspected Muscle DiseaseSuspected Muscle Disease

• LabLab– Muscle enzymes (CPK, aldolase)Muscle enzymes (CPK, aldolase)

– Erythrocyte sedimentation rate (ESR or sed Erythrocyte sedimentation rate (ESR or sed rate) if suspect inflammatory diseaserate) if suspect inflammatory disease

– Genetic testGenetic test• Duchenne’sDuchenne’s

• Myotonic dystrophyMyotonic dystrophy

• EMG/NCSEMG/NCS• Muscle biopsyMuscle biopsy

• May provide a definitive diagnosisMay provide a definitive diagnosis

Extremity CN Reflexes Sensation

Weakness

ALS Random yes Increased Normal

Polyneuro- Distal> rare Decreased Lost distally >

pathy Proximal distally proximally

LEMS LE > UE rare Decreased or Normal

Prox>distal absent

MG UE>LE yes Normal or dec Normal

+/-prox>distal

Myopathy Prox>distal occ Normal or dec Normal

Terima Kasih

Medula Spinalis - Saraf Tepi - Otot - 2

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