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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_186_библиотеки_им_акад_М_И_Перельмана

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Electrophysiologictestingwillnotdetectsmallfiberneuropathy;EMGandNCSwillbenormal.The lackofEMG/NCSabnormalitiesandanormal,ornearnormal,neurologicexaminationinapatientwith symptomaticneuropathyarewhatshouldleadyoutosuspectasmallfiberneuropathy.Skinbiopsy,which willshowabsentordiminishednerveendings,isthediagnostictestofchoice.Thereisnospecific treatment;variousanticonvulsantsandantidepressantscanbeusedforpaincontrol.
Examplesofnervefibersonskinpunchbiopsiesandmicroscopy,fromahealthycontrolandapatientwith smallfiberneuropathy.Notethelowerdensityofnervefibersinthepatientwithsmallfiberneuropathy. (ModifiedfromHaüserW,PerrotS.FibromyalgiaSyndromeandWidespreadPain.WoltersKluwer;
2018.)
MononeuritisMultiplex
Mononeuritismultiplexisunlikethepolyneuropathieswehavejustdiscussed.Thepolyneuropathies evolvesymmetrically,progressively,andcontinuously,whereasmononeuritismultiplexproceedsina kindofscattershotaccumulationofisolated,noncontiguousmononeuropathies.Bydefinition,itmust involve2ormorenoncontiguousnerveseithersimultaneouslyorsequentially.Mononeuritismultiplexis
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farlesscommonthanpolyneuropathy.
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Multiplex.Ifyoulookquickly,theinvolvedareasmayresemblethestockingglovedistributionofa polyneuropathy.Butacloserevaluationwillrevealthatthelesionsarenotsymmetricandinvolveseveral distinctperipheralnerves.Acarefulexaminationwillrevealthedifference.
Vasculitisisacommoncause,mostoftenthedisordersassociatedwithantineutrophilcytoplasmic autoantibodies (ANCA; such as granulomatosis with polyangiitis and microscopic polyangiitis), polyarteritis nodosa, cryoglobulinemia, and vasculitis associated with connective tissue diseases suchasSjogrensyndrome. Othercausesinclude:
ischemicnervedamage(e.g.,duetodiabetesorsicklecellanemia) variousinfections(mostlyviraldiseasessuchashepatitisB,hepatitisC,HIV,andWestNilevirus) neoplasticprocesses(fromtumorinfiltrationofnervesorasaresultofaparaneoplasticsyndrome).
Nervesofthelowerextremitiesaretypicallyaffectedfirst;footdropduetoperonealnervedamageis themostcommonmotorcomplaint.Overtime,however,virtuallyanyperipheralnervecanbeaffected, includingthosesupplyingstrengthandsensationtotheupperextremities,thecranialnervesandautonomic nerves.
Nervebiopsyisoftenhelpfultodiagnosetheunderlyingetiology;itcanconfirmavasculitisandallow treatmenttobeinitiatedwithcorticosteroids,aswellasruleoutinfiltrativedisorderslikelymphoma.
Managementotherwiseinvolvesrecognizingandtreatingtheunderlyingcondition,aswellaspain controlwiththesamemedicationsusedforthemorecommonpolyneuropathies.
Plexopathies
Theplexopathiesinvolvemultiplenervesbutarecausedbysinglelesionsthatinvolveasubstantialpart oforanentireplexus,eitherthebrachialplexusorthelumbosacralplexus.Thesesyndromesarerareand aremostoftencausedbytrauma,ischemia,inflammation,malignancy,diabetes,andradiationtherapy.
BrachialPlexopathies
Thebrachialplexusisoneofthemosttestedanatomicalstructuresinthebody.ThemnemonicReal TexansDrinkColdBeerisoftenreliedupontohelpremembertheanatomy:
Roots(C5T1) Trunks(superior/upper:C56,middle:C7,inferior/lower:C8T1) Divisions(eachtrunksplitsintoanteriorandposteriordivisions) Cords(lateral,posterior,andmedial) Branches(thenervesgivenoffbyeachcord)
Lateralcord→musculocutaneousnerve(C57),lateralrootofthemediannerve(C5T1) Posteriorcord→axillarynerve(C56),radialnerve(C5T1) Medialcord→ulnarnerve(C8T1),medialrootofthemediannerve(C5T1)
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Thebrachialplexus.
Brachialplexopathiesaretypicallyclassifiedintothosethataretraumatic(usuallyaresultofsports injuriesormotorvehicleaccidents)andthosethatarenot.Afewspecificsyndromesinvolvingthe brachialplexusinclude:
Erbpalsyiscausedbyatearofthesuperiororuppertrunkofthebrachialplexus(involvingC5and C6).Thismostoftenoccursininfantsduringdelivery,aresultoflateraltractionontheneckofthe baby due to shoulder dystocia. It presents with impaired shoulder abduction (due to deltoid and supraspinatus involvement; the arm hangs limply by the side), lateral rotation (infraspinatus involvement; the arm is medially rotated) and arm flexion and supination (biceps brachii involvement; the arm is extended and pronated). The majority of cases resolve with time and physicaltherapy.Surgicalrepairissecondlinetreatmentifneeded.
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AsummaryofthecommonneurologiccomplicationsseenininfantswithErbpalsy.
Klumpkepalsyiscausedbyatearoftheinferiororlowertrunkofthebrachialplexus(involving C8T1).ItisalsotypicallycausedbytraumaduringdeliverybutislesscommonthanErbpalsy.This palsy presents with weakness in the intrinsic hand muscles innervated by the ulnar and median nerves(resultingin“clawhand,”characterizedbyanextendedwrist,extendedmetacarpophalangeal joints,andflexeddistal interphalangealjoints),C8T1dermatomalnumbnessand,not uncommonly, Hornersyndrome,duetothecloseapproximationoftheT1nerveroottothesympatheticchain.
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The“clawhand”ofKlumpkepalsy.
ParsonageTurner syndrome (also known as neuralgic amyotrophy) is an acute inflammatory brachialplexitisthatcaninvolveanyportionofthebrachialplexus.Mostoftenitisidiopathic,but
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approximately 50% of patients report some sort of antecedent event, such as surgery or a viral illness.Itclassicallypresentswiththeacuteonsetofseverearmandshoulderpainfollowedwithin days to weeks by patchy upper extremity weakness. Most cases are unilateral, but bilateral involvement can occur. Recovery is gradual, often over months to years. There is no specific treatment.
Box11.7ThoracicOutletSyndrome
Thoracicoutletsyndromereferstoagroupofdisorderscausedbycompressionofthe nerves,arteries,andveinswithinthethoracicoutlet(thespacebetweentheclavicleandfirst rib).Themostcommoncauseisthepresenceofanomalousribsoraninjury.Theclinical pictureisusuallydominatedbycompressionofthebloodvessels,causingupperextremity edema.However,whenthenervesofthebrachialplexusarealsoinvolved(thisis exceedinglyrare!),thepatientwillcomplainofpain,numbness,anddysesthesiasofthe upperextremityprovokedbyelevatingthearmorturningtheneck.Iftheconditionis untreated,muscularatrophycandevelop.
LumbosacralPlexopathies
Theseareuncommon,sowewon’tspendtoomuchtimehere(and,happily,unlikewiththebrachial plexus,thereisnoneedtolearnthepreciseanatomy).Thereareastrikinglywiderangeofpotential etiologies,including:
Diabetes. Diabetic amyotrophy (also known as diabetic radiculoplexus neuropathy) typically presents acutely withasymmetric focal legpain followed by proximal legweakness. Associated autonomicsymptomsarecommon.Partialrecoveryoverweekstomonthsisstandard.  Idiopathic. Idiopathic lumbosacral plexopathy presents similarly to diabetic amyotrophy, but in patientswithoutdiabetes. Neoplasm.Neoplasticinvasionofthelumbosacralplexusismostoftenduetodirectextensionofa tumor(nearlyanyformofcarcinoma,melanoma,orlymphomacanbeimplicated),butitcanalsobe caused by leptomeningeal involvement or hematogenous or lymphatic spread. Neoplastic lumbosacralplexopathyis nearlyalwayspainful, characterizedbyshocklikepainsinthe involved lowerextremity. Radiation.Radiationlumbosacralplexopathytendstooccurmonthstoyearsafterpelvicradiation. Itis oftenbilateralandrarelypainful,presentinginsteadwithweaknessand,occasionally,sensory loss.  Retroperitoneal hematoma. These typically develop within the psoas muscle and can occur spontaneously or following femoral arterial or venous catheterizations (most often in patients on anticoagulation).Severebackandlegpainandfemoralneuropathyarecommon.
Mononeuropathies
Mononeuropathy,theinvolvementofasinglenerve,canresultfromalmostanydiseaseprocess.Any nervecanbeaffected,andwecouldlistasmanymononeuropathiesastherearenervesinthebody,buta fewsyndromesarecommonanddeservetobesingledout:
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Medianneuropathyatthewrist(carpaltunnelsyndrome) Ulnarneuropathyattheelbow Peronealandtibialneuropathies Bellpalsy Meralgiaparesthetica
CarpalTunnelSyndrome
Carpaltunnelsyndrome(CTS)isaformofmedianneuropathyduetoentrapmentofthemediannerve (whichisderivedfromspinalnervesC5T1)withinthenarrowcarpaltunnelinthewrist.Thisisusually causedbyrepetitivetraumafromoccupationsthatrequirefrequentflexionandextensionofthewrist (although,surprisingly,spendingthedayhammeringawayatacomputerkeyboardhasnotbeen convincinglyfoundtocauseCTS!).Otherimportantriskfactorstokeepinmindincludehypothyroidism (duetoaccumulationofmucinousmaterialwithinthecarpaltunnel),pregnancy(duetofluidaccumulation andedema),andamyloidosis(due,nosurprise,toamyloiddeposition).
Patientswillcomplainofnumbnessandparesthesiasinthehand,oftendescribedastinglingandoften worseatnight.Examinationwillrevealthatthesensorysymptomsarelimitedtothefirstthreedigitsof thehandandthemedialsideoftheringfinger.Accompanyingpain,however,neednotbelimitedtothe mediannervedistributionandcanradiatethroughouttheentirehandandevenupthearm.Bilateral symptomsaresurprisinglycommon,affectingmorethanhalfofpatientswithCTS.
Compressionofthemediannervewithinthecarpaltunnelleadstosensorycomplaintsinthefirstthree digitsandthemedialsideoftheringfinger.
Variousdiagnosticmaneuversmayhelpyoumakethediagnosis.ThePhalentestinvolveshaving
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patientsflextheirwristfor1minuteandseeingifthatelicitsorexacerbatestheirsymptoms.TheTinel testinvolvestappingoverthecarpaltunnelandseeingifthatproducessymptomsinthefingers.If
positive,bothtestsaresuggestiveofthediagnosis,buttheirsensitivityandspecificityarelimited(cited asaround50%to80%).Wristxraysmaybeindicatedtoexcludeotherdiagnoses,andelectrophysiologic testingcanbehelpfulwhenthediagnosisremainsunclear.
Phalentestforcarpaltunnelsyndrome.
Conservativetreatmentinvolvesavoidanceoftheactivitythatiscausingtheproblem(unfortunatelynot alwayspossible)andtheuseofawristsplintatnight.Physicaltherapyandtargetedexercisesdon’t appeartoaddmuch.Somepatientsmaybenefitfromlocalinjectionofacorticosteroid.Forpatientswith persistentsymptoms,surgicalreleaseofthecarpaltunnelisarelativelysimpleprocedurethatcanleadto completeresolutionofsymptoms.
UlnarNeuropathy
Ulnarneuropathyislesscommonthanmedianneuropathy.Itcanoccurwhentheulnarnerve(derivedfrom spinalnervesC8T1)iscompressedattheelbow(inthecubitaltunnel;thisisthemostcommonsite)orthe wrist(inGuyoncanal).Sensorysymptomsintheulnardistributiontendtopredominateintheformer,
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