PERIPHERAL NERVE DISORDERDr. RoslainiDepartement of NeurologyAl ISLAM HospitalBandung

Spinal nerve anterior Horn med spinal

Distal peripheral nerveCranial nerveOtonomic nerveThe distribution of peripheral nerve disorder includes :

Peripheral nerve spread out in all of bodyCharacteristic lower motor neuronParalysisdependlocationdegreefiber nerveOnsetdisorder

Pathology reaction of peripheral nerve

NeuropathyIt is condition where perifer nerve gets dysfunction and distruction.Clinical manifestation of lower motor neuron paralysis included :SensoryNegative phenomenon :Hypestesia, the loss of sense, pain, temperature and deep sensePositive phenomenon :paresthesiahiperalgesiaincomportable, burning sensationClinical symptom :Distal gloves stocking hypesthesiaDermatomel

MotoricWeakness from light to severe especially distalLoss of tendon reflexAutonomicExample :Cool on limbPostural syncopeOrthostatic hypotensionHyperhidrosisNeuropathy ( contd )

Principal Neuropathic SyndromeSyndrome of acute motor paralysis with variable disturbances of sensory and autonomic functionGuillain Barre Syndrome ( GBS )Syndrome of sub acute sensorimotor paralysisSymmetrical polyneuropathiesVitamin deficiencyUnusual sensory neuropathiesDiabetic neuropathiesPolyradiculopathy

Principal Neuropathic SyndromeSyndrome of chronic sensorimotor polyneuropathyLess chronic, acquired formsCIDPSyndrome of more chronic polyneuropathyInherited polyneuropathyInherited polyneuropathy of mixed sensorimotor typesNeuropathy associated with mitochondrial diseasesSyndrome of recurrent or relapsing polyneuropathySyndrome of mononeuropathy or plexopathyContd

Peripheral nerve disorderLMNPolyneuropathiesMononeuropathiesApproachAcuteSubacutechronicGuillain Barre SyndromeVitamin deficiencyamyloidosismetabolicTraumaPressure: CTSLeprosy

Acute idiopathic polyneuropathies ( Guillain Barre Syndrome )Etiology : - unknown - post viral infection - self immunity deseasePathology : Demyelinitation segmentalSevere axonal degeneration

Clinical symptoms

Non spesific viral infectionAcute paralysis progresif bilateral symetricAutonomic disorderCranial nerve disorderSensoric abnormality : gloves and stocking hypesthesia

Diagnostic test :Lumbal puncture : elevated cerebrospinal fluid proteinNCV - EMGCriteria to make a diagnosis of GBS :Rapid onsetlead to frank paralysis : Leg and armsSometimes respiratory muscleAccompanied by sensoric abnormalitiesLoss of deep tendon reflex / decreaseElevated cerebrospinal fluid protein, and normal cellAbsence of other causes of peripheral neuropathy

Prognosis :75 85 % spontaneus recovery10 17 % recover with disability8 % recurrent5 % diedTherapy :Self limitingCorticosteroidImmunoglobulinPlasmapharesis

Diabetic NeuropathyComplications of diabetes mellitusInsidens 30 % - 70 % of DM patientDefinition :A disorder somatic or autonomic perifer nerve. Cause only by diabetes mellitus can happen clinically or sub clinic without other causes

PathogenesaVascularMetabolikOtherClinical featuresDiabetic opthalmoplegiaAcute mononeuropathyRapidly evening painfull, asimetric, predominantly motoricMultiple neuropathyAsimetric proximal motor weaknessDistal symetric, primarily sensory polineuropathyAutonomic neuropathyPainfull thoraco abdoment radiculopathy

Diagnostic testBlood glucose level testNerve conduction study : NCV TherapyMaintenance of the blood glucose levelMicroangiopathy antiplatelet agregasi ASASymptomatic theraphy for neuropathic painPsikotherapy, physiotherapy

Nutritional polineuropathyEtiology :Deficiency vitamin in daily foodTight dietMalabsorptionPost operatif GITAlcoholic Patology anatomy feature : axonal degeneration

Clinical featuresSensoric :Paresthesia, hyperalgeria, hyperaesthesiaMotoric : ParalysisFisiologi reflex decreaseSistemic disorder : delirium anemi, etc

Diagnostic testSerum level of vitamin B1, B6, B12, Folic acidElectroneuromyography : NCS Nerve Conduction Study NCV TherapyVitamin suplement administration vitamin B1, B6, B12

MononeuropathyRadial nerveArises from the C. 5 8Motor nerve innervated : the chief extensor of the fore arm, wirst, and fingerCause by external pressure in axilla and fracture humerusPressure against some hard surface especially in sleep : saturday night palsy

Radial Nerve

Median nerveArises from the C. 5 6 7 8 th IIts fairly common as a result of compression of the nerve by transverse carpal segment ( Carpal tunnel syndrome / CTS ) The symptoms : severe pain often weakly patient from sleep, usually in the thumb and index fingerAthrophy M. ThenarTrauma axilla area ape hand

Median Nerve

Ulnar nerveArises from the C7 8 th IIt is frequently injured by gunshot, wound in olecranon or head radius bone, most compressed at elbow, leprosy, diabetic neuropathyClawn hand its on the ring finger

Ulnar Nerve

Sciatic nerveDerived from the L 4 5 S1Its commonly by fracture of pelvis or femur, wound of the buttock and thigh.

In fossa poplitea the nerves devides : Common perineal nerve Tibial nerve

Sciatic nerve

Common perineal nerveMost frequent cause of injury, pressure, obstetrical, prolonged lying the ill patient.

Common Peroneal Nerve

Tibial nerveLession in tibial nerve are uncommonCaused by trauma, and compression in tarsal tunnel

The brachial plexusFormation of brachial plexus :Upper : from C5 6Middle : from C7 8Lower : from C8 Th. 1Causes the lession brachial plexus :CutGunshotWoundDirect traumaTumor or aneurysmTraction in delivery at birth

The brachial plexus

Site of the lessionUpper brachial syndrome ( Erb Duchene Syndrome )Middle brachial syndromeLower brachial syndrome ( Klumpke deyerine syndrome

Common root syndromes of invertebral disc disease

Disc spaceL3 4L4 5L5 S1Root affectedL4L5S1Muscles affectedQuadricepsPeroneals; anterior tibial; extensor hallucis longusGluteus maximus; gastrocnemius; plantar flexors of toesArea of pain and sensory lossAnterior thigh, medial shinGreat toe, dorsum of footLateral foot, small toeReflex affectedKnee jerkPosterior tibialAnkle jerkStraight leg raisingMay not increase painAggravates root painAggravates root pain

Common root syndromes of invertebral disc disease

Disc spaceC4 5C5 6C6 7C7 T1Root affectedC5C6C7C8Muscles affectedDeltoid; bicepsTriceps; wrist extensorsIntrinsic hand musclesArea of pain and sensory lossShoulder, anterior arm, radial forearmThumb, middle fingersIndex, fourth, fifth fingersReflex affectedBicepsTricepsTriceps

MyopathyDiseases concerning with sceletal musclesSymptoms of skeletal muscle disease :FaliqueWeaknessAtrophyMuscle ticCrampsPainfull

Classification muscle diseases

The inflammatory myopathiesPolymyositisThe muscular dystrophiesDuchene muscular dystrophyThe metabolic and toxic myopathiesThe congenital neuromuscular disordersThe hereditary myotonias and periodic paralysisDisorders of muscle characterized by cramp, spasm, pain and localized masses

Diagnosis of muscle disease

Clinical symptomsHereditaryLaboratory examinationElectromyographyNerve conduction studyMuscle biopsy

Duchene muscular dystrophyInherited muscle disease, degeneratif and progresifSimilar x linked diseaseAtrophy shortageMuscle distrophy

Clinical manifestationMostly find on children under 3 years ageUsually boyThe story late to walk often fallen, cant run difficult to stand up from the squad position ( Gowers sign ) finally paralysis

Physical diagnosticAtrophy on proximal muscle calf musclePseudo hypertrophyProgresifity varietyFatal cases respiratory muscle failure and secondary infection

Diagnostic testIncreases the CPK levelEMG, classical myogen featureMuscle biopsy

TherapyNo medication for diseasePhysiotherapyGenetic conseling

Disorders of sceletal muscleOnset acute / subacutePA infiltration of muscle by lymphocytPolymyositisEtiologyUnknown idiopathic polymyositis / autoimmunCollagen vascular diseaseInfectionDrugSystemic diseaseEndocrin diseaseMetabolic diseaseToxic

Clinical manifestation

No family historyProgressition is measured in week / monthSymptom may improve spontaniouslyDysphagia, weakness neck / flexor muscleArthralgia, myalgia

Diagnostic testEMG ; myopathy featureTherapyCorticosteroid and immuno supresif drugImmunoglobulin intravenous

Autoimmun diseaseCauses by transmission disorders on neuro muscular junctionAcetyl cholin receptor ist attack by antibodyRelated with thymus glandMyasthenia Gravis

Clinical featuresFluctuated weakness of certain voluntary muscleViral, other infection, emotional, can worst the symptomThe symptom response to anticholin esterase drugSporadically to all agesTendon reflex normalNo sensory deficit

ClassificationOcular myasthenia : 20 % casesA. Mild generalized myasthenia - Slow progression No crisis drug responsB. Moderate generalized myasthenia - Severe secletal and bulbar involment - No crisis drug respon less than statisfactoryAcute fulminating myasthenia- Rapid progression of severe symptom- Respiratory crisis high mortalityLate severe mysthenia

Diagnostic testTensilon test or prostigmin testMeasurement of receptor antibody for acetylcholineRepetitive stimulating test : progressif decrementSingle fiber electromyographyX ray, CT Scan, MRI, for thymoma glandDifferential diagnostic :Eaton lambert syndromeFollow lung carcinomaMyasthenia gravis related with drug, toxinTherapy :Anticholine esterase drug prostigminCortico steroidThymectomyPlasma pharesis and immunosupressanImmunoglobulin

BIOPSYDr RoslainiDEPARTEMENT OF NEUROLOGYAL ISLAM HOSPITALBandung

BiopsyBiopsy done if no other diagnostic testClinical test is not suffition for diseaseSupporting the diagnostic when biochemistry test is positiveNeurology diagnostic biopsy done on the primary tissue of skin, sceletal muscle, nerve, bone marrow, and brain.

Skin biopsyStorage disease Neuroaxonal dystrophy Mythochondrial cytopathies

Sceletal muscle biopsyOn the normal and abnormal muscle areaPrimary muscle diseaseDenervation diseaseSystemic diseaseMitochondria diseaseNerve biopsyDiagnostic chronic neuropathyInherrited neuropathySural nerve is common

Bone marrow biopsyNieman pick diseaseBrain biopsyAn infasive test / high risk procedureNo other diagnostic testScar biopsy can be epileptic focusIts done for Alzheimer disease, levy body disease, brain tumor, infectionRectum biopsyAutonomic nerve diseaseAmyloid disease