KEY POINT:

•

Specific aspects of the examvnation that may be especiafy pertinent include extraocuar muscle involvement or ptosis, presence of fasciculations of the tongoe or limbs, abilty to lift the head off the pillow, ev dence of spec tic muscle wasting, paradoxical respirations, reflex activrty, ann distribution of weakness.

EMERGENCY NEUROLOGIC CONSULTATION IN THE INTENSIVE CARE UNIT: NEUROMUSCULAR DISORDERS Douglas W. Zochodne

ABSTRACT Neurologists are often asked to see patients in the intensive care unit because of diffuse limb weakness. It is important to entertain a broad-ranging differential diagnosis in such patients. The possibility of a previously unrecognized high cervical myelopathy cannot be ignored. Among neuromuscular disorders to be considered are Guillain-Barr~ syndrome, motor neuron disease, myasthenia gravis, and critical illness polyneuropathy and myopathy. Clinical features, electrophysiologic studies, and selective biopsies may help to differentiate these disorders and direct treatment appropriately.

CLINICAL ASSESSMENT V under ( )ther r lr( unmuim es, the e )nsulti nc~ nerir )l( )L~i5t sL nk1 e mpile :is m nel hi St ( ) IicJ I i I~ t( )f1Th1 t i( )~ J5 P~ ~ sihle \\lt(ifl ASke(l t( ) see u V11ieI~t in the intensive cure nuir ( kit~ Ii unexpluinecl sveukness. ixx~liiing rhA \\ith eririeullv ill p~~rienrs :in urlepn:ire h isr( ~rs n~uv he difficult t( ) ( )htji n [lie \\~i~

.

p:itient if u\vjke, n~nsr he tlnesti( med direct lv. mcI e )nsnltjti( )n \vith reLtives intl f:im iv sh in Id he s( )ut~h t A rL mr— )nL~h exiih.iutic in ( if the current uncl

tht.’niu giiisis unci reeenr sirul illncs~ )r succiliut i( in ( svhich might iivil)licatr (; ciilluin—I3urre s\ntlr( )nie ) lfxpc insure Ii ing muscle-lilcicking ugents, episuclo cit sepsis. uncl chunges in ereutine ki liuse ((K) levels, uim ing cither pert nent ink )rimiti(

mu sh )nlcl emerge fume

u h( )r( inm..it~li ree( inrc I re\iesv.

ienrs in un I( A eun he chullenging us ill nenr( 1 Igists reec Igni/e. he slI( mu 1(1 nlsi m he us rL ~rcim.iin~h ~~pcissihlr )us n~eclicul reec ircls is essent jul. Speeific u5pemits if the exuminutior hut ivius he especiullv pertinen: Lxr~ples ( it especiullv in~~( )rrunt teu— Table 15 ) Inc Icitle extruc inctilur musck ci I~(f5 t( I pursue in(l ncle u hisrc mrs ( lIi5 (mis enient I )~ ~ isis, presence of Lo preexisting nilisele sveukness. rnusele t,i1n~pint~. LiscicciLich )ns. musele \sust- ciccii u c P )ns cit the cc )ngcie ( r I imhs uhilitv t( ) lift the heud ott the pilL isv, cmi iim~. uncl clvsphugiu ( suggestive ( n~( it( )r nei..i l~( )ri c I iseuse ) p~ )sis uncl cli— tience I if specific muscle svusting. pM l~lcmI~iu ( ruising the r m.s.sihilitv ( it mvus— ut Ic mxicu I resp i rut i I )~ 5 reti ex uct ivitr .

clistrihuricmn

of sveakness ( ie. more

pr(II1iinent distally (ir pr Iximaiiv) clis— crepune\ hetsveen eranic inhulhur and iiilili insxmlvenient, response t(I ci(IX1( 105 stilVIuiati(IrI, urid presence (If a sensory lesel ,

ELECTROPHYSIOLOGIC EVALUATION Nc mutine eieetrc Iphvsi( mic igie testin(~ in patients admitted t m the 1(11 can he perf inrmed reliuhlv unci eusilv using portahie equipment. Stimulution next t( I central vusetilur uceess lines or vice—

muker leucls sh luidi he uv lided, mci needle eleetrc imyc ingraphv (EM(;) shc Itildi

he deferred if the putient hus u significant cc Iugulopatllv c w is untie(IagdIlutedl. In 5( inme instunees a single suituhie. oc muec mmpartmented. und compressihle mcisele may he exumined \5ith pressure applied ufterssard to present u hematoma. Iumh teslivg 5h(Iuldl evuluute motor c( intl uctic mu in ut least median (wrist, elhcmsv), ulnur (svrist. helosv elhoxv, ahose elhdlsv), peroneal (unkie, fihulur heuci, knee). unci tihiul (ankle. knee) nerves. The lutenev, amplitude, duration. areu. and cc inn— dueticinn velclcities (If the eomp(Iund muscle

action

potentials

KEY POINTS:

•

performed reliably and easily using portable eguipment. Stimulation next to central vascular access lines or

( CMAPs

should he measured. and

Routine eiectrophysiologic testing in patients admitted to the CU can be

pacemaker

F-svave

leads should be avoided, and

needle [MG

TABLE 15

should be deferred if the patient has a significant coagulopathy or is anticoagulated.

Bedside Clues to the Diagnosis of Neurologic Disorders in the Intensive Care Unit

Sign

Diagnosis

Relatively intact eye, facial, and neck movements but severe limb quadriparesis

High cervical myelopathy

~

Unexplained ophthalmoparesis

Thiamine deficiency

~

Facial myokymia

GBS

~

Tongue or limb fasciculations

ALS

~

Ptosis alone

MG, LEMS, myotonic dystrophy

~

Ptosis with ophthalmoparesis

MG, CIM, prolonged blockade

Routine recordings of CMAPs in at least one “resting” territory after 2-Hz or 3-Hz st i mu I at on

~

Bifacial weakness

GBS, CIM, prolonged blockade

should be

~

Loss of deep tendon reflexes

GBS, CIP, CIM, prolonged blockade

~

Facilitation of deep tendon reflexes

LEMS

~

Preserved abdominal reflexes

ALS

~

Early bladder involvement

High cervical myelopathy

~

Locked in

Pontine lesion, GBS, prolonged blockade

~

Grimaces to nailbed pressure but does not withdraw limbs

Severe myopathy, neuropathy with lesser sensory involvement

Intact deep tendon reflexes

Myelopathy, CPM, myopathy, NM junction

•

ALS amyotrophic lateral sclerosis or motor neuron disease; CIM critical illness myopathy; CIP critical illness polyneuropathy; CPM central pontine myelinolysis; CBS Guillain-Barrb syndrome; LEMS Lambert-Eaton myasthenic syndrome; MG myasthenia gravis; NM neuromuscular.

included to evaluate the neuromuscular junction, but admittedly most patients n an CU are unable to exercise for a full protocol.

C ONTTNIJUM

KEY POINTS:

•

•

•

Needle EMG should sample both distal and proximal muscles from the limbs. In some patients, needle electrode recordings from intercostal muscles or the daphragm may be aoded, altnough both procedures carry a slight risk of pneu mothorax. [MG studies should especially seek fibrillations, positive sharp waves, and fasciculations. Dense runs of fibrillations ann positive sharp waves are characteristic of acute denervation of muscle, but it must be borne in mino that fibrillations can also occur in myopathies with prominent fiber necrosis. Absent C MAPs may arise from severe myopathy or neuropathy. To distinguish toe two, subcutaneous recordings of C MAPS from drect muscle stimulation may be of assistance.

EMERGENCY CONSULTATION IN THE ICU

latencies dieterminedi. Limh temperattire measured during the srciclies shcldild he reeclrdledl. Routine reeclrdiings (If CMAPs in at least dine resting’ rerrirc rv after 2—liz or 3—Hz stimcilatic In shc Itildi he ineludledi tc in evaltiate the neurclmdi.setiiar juneticln, Kit admit— redly n inc 1st patients in an ICt are tinahie t I exercise fo w a full prc mrc I—

ings of (MAPS frc mm direct mtisck stimtilatic mu mus he cif assistance (Rid; et al. 1995). Directly evoikeol (MAP’ can he recrtiitecl in netirc ininathies ssitt inexeitahle mc into inr nerves. syherca’ thes are ahsent in sesere m\olpathie’ ssith inexeitahie mtisele menihrunc’ (Rich et ul. 199””).

cc II. Antidlrclmie reecinrclings (If sensc Irs nerve aeticinn pcintentiais (SNAPs) 5hcIuldl ineididle at least median, ulnar. ratlial, and stiral nerves. If the patient has

ADDITIONAL TESTING

pedal edema and surfaee-reec Irdledi sural po itentials are redidleeci in am— plitticle, near-nerve needle eleetrcldle reec Irdling can he cc Insidleredl. Phrenie Pc Itentials reec Irdledl from the clia— phragni assess respiratclrv system mo-

rclr fihers ((Ihen er al. 1995). Needle EME; Shodild sample hclth distal and proximal muscles from the limhs. In some patients, needle eleetro Idle reec mrclings frcinm intercostal mcisdes o mr the diaphragm may he added (Boircmn et al, 1992), aithotigh bcinth prc icedures carry a slight risk (If puennic ithc irax. In patients svith ehrc innie clhstrdietise pul mc Inarv disease. needle eleetrotle stciclies (If the diaphragm

slic incilti he usc inidledi hecause (If an adidledi risk cif pnecimc itlnc wax. EMG srticlies shc lOud1 especialls seek fihriilatic ins, pc Isitise sharp svaves. and fasci-

ddulati(Iris. Dense rtins (If fihrilluticlns and pc isirise sharp svases are eharueteristic c mf aedite clenervaric inn (If muscle. hdit it must he ho inrne in mind that fihrillaric Ins o,an also m 0 iccoir in mvc ip— athies svith prcinminent fiher necrosis. Reerdiitment dinf vcinluntarv mointoIr tinit 1)0 Itentials (In EMG is difficult in patients svhcin are tinahie toin edo Iperate. hut when testahie stich mas he vaInahie in identifying shcinrt—dldiraticinn, icinsyamplitude, p0 Il\l)hasid’ mc Ito inr unit pc inten— tials suggestive (If a primarv mvc Ipathv. Alinsermt (MAPs may arise frcmm sesere mvc inpathv cmr necirc inpathv. Ic in clistin— guish the two. stiheditanec mus reec inrcl-

Serum (1K leseis mus lie mc mclestls cit vateci (2 tom 3 times the tip~mei’ limit ~i: no mrmai I in clenervutic in tro mm po mivuce ro input ii ies cmi’ mc Ito mu’ ii eui’o mu cl iseuse. svhile serv high leseis usso meiatedl ssirt nBc mgic ihinuria mas he o ihsersecl it mtuseie fiher neei’c Isis a550 inciateci svitt mvoinpathies. Patients ssith critical ill ness m~ o inpathy mus have no mrmal CE levels. Magnetic resolnanee imagine MV!) c mf the den tral nervomtis svstc’e (N’S) sho intulci he cc mnsiclei’ecl in ant patient stispeetedi c mf having a PoIn tine c mr high dervicul cc mu’cl iesicmn. While imaging omt I neripho..’rai nerves has no it entered rolcutine clinical pr;ie rice. fo ineul necuro inpathies c mr plexus lesic inns ssith scuspecteol dO Impressicmn might he assessed.

DIFFERENTIAL DIAGNOSIS Central Nervous System Disease Linih sveakno.’ss mus cleselo ip in ICli patients fro mm disease inso using the (NS. Imaging stucli es are cleans ercucial in identifying tinese, svhile keeping in mind that such patients may also m have cc Ineturrent peripheu’ui neu’se. necuom mouscular junetic inn. o ir muscle disease. Hi lateu’ai eeu’ehru I in fa lot 10 mn is cisu ails easily identifiedI hv its presentation; syith ulrei’ecl dx munseic miusness. Central P~ Intine ni’ceiinc Il\5i5 niu5 Pi’eseuint ssitli initialls tiaccici au’etlexie linnhs andi hul har clvsfuneric inn. High eeu’sieui ccmmcl nivelo Ipaths ni cist he cc mnsidleu’edl cli;ig nomstieuiis; ‘‘o mcc’oilt causes c mf sesere

cervical mvelo mpathv ineicude transseu’se mseiit is. C i-u atlauito maxial sdibi dixaticinn in rhecumato mid arrlii’iris, unreec ignized disc p~’~ mtrusioinn and cc mu’cl eoinmpu’essioinn, (‘ersical sdihdlciu’al ahseess. and acute central cc mu’cl ssnolu’o mine, amoinug cinthers (Case 10. I~eprcmmeningeaI eareino inmarc insus, is mphoinmaro insis, cinr, u’arelv. (dic minato isis may present with dliffuse limh sveakness syith a ccinmhiumarioinn mf eu’aniai nerve palsies (eg, hilateral sixt li-nerve palsy) audi sensoiry loinss: sveakness might he fu’eim infultu’atio inn oinf mdiltiple u’omoints (Figure 19) (Case 11.

Motor Neuron Disease Eespuu uto mis tuuicuu e mus Pu e~ ecle the ci iugn (Isis mt mc into mu’ ii e di i’O in ciisease

eg, :inivc mru’c inlihic lateral seieu’o insis AIS 1) (CIueui et al. 1997, “Fheu’e may ‘ills’ base heeui (n’idltu, pro ingu’essise limb sseakness, clvsau’th u’ia, c mr dvsphagia svith u’espiu’atc mu’s fail ci ~.

adtite

u’e pu’eeipi-

rated by aspiu’at ic mn cmi’ pneciuiicinnia. Findings cmii examinatic mn max ineicucle to inngcie and limb faseiccilatic inns in the setting omfsveakness andi mcisele wasting with tipper mc into mr neuu’c mu signs cmi’ inappu’o mpu’iatelv hu’isk u’etlexes Se nso mrs examin atic in is no mu’mui hevo mdi so mine hicinting o mf sibu’arcinu’v sense in the feet.

I)efiuiitise elect u’o mphvsio mic ingic eu’iteu’ia fo mr Al~S u’edltuiu’e clenersatic inn in tsvo in teru’irc inries cif each o mf thu’ee limhs

mr in hulbar nvuseies CMAPs may he u’edlueedl syith pu’esers’ecl SNAPs Needle EMG dliseIoinses faseieulaticinns, fihrillatic inns, pomsirise sharp ssaves. and enlar(~edl vcinlcunrars molt ir tunit pointen— tials (Figure 20). Fibriliatic inns may also in may he dlemolnstratedi in intercoistal mtiseies dr the cliaphu’agm. I nfoinu’tunately, patients svhoin uu’e cliagno inseci ssith A1~S in the ICIT may never he sueeessftuilv sveanedl. Scinme decline dmngoining lcinng—ierm ventilatioinn and u”ecicuesr exrcilinatioinn. A incisele hioinpsv in scuch a patient umuv add a ftuu’ther lesel cinf diagno instie certainty tom help with this kind oinf deeisioinn. ‘1’he hicmpsv shoinuld ecmnfirm sc’atteu’edl c mr gu’ointuped fiber ati’oinphv indicatise of clenersatic inn. (0

).

Polyneuropathies GtuilLuiui—I-3ai’i’c ss’nclu’omme cuscualiv pu’e— cipitates admissicmn tom an ICt after seseu’al class (If WO mi’sening motoinr sseakness and senso irs svmptoinins after. fomr example, an infeetic inn oinr saceinaticmn. Chi’oinnie intlammateirs cleinseli— nating Pcinlvnetiu’c inpathv u’au”eiv c’acuses u’espiu’atoinrs sseakness. Cu’itieal illness -

i~o inlvnecuu’o inpathy ~seebeic inssu cleselo Ips in I~atients ssith pu’o inleingeci ICI stays ssho in cleselo inp sc.’psis and incultipie oinu’gan faiiciu’e. Pu’cinlo inngecl thianiine clefieienc’s nias be asso ineiarecl ssith o inphubaimemplemu fu’o inni \Vernieke’s eneephalo inpathv ‘

Case 10 A 65-year-old-man with rheumatoid arthritis develops vague difficulty walking and urosepsis. He is transferred to the ICU in septic shock with multiple organ failure. He has diffuse limb weakness and remains ventilatordependent despite stabilization of his sepsis and organ failure. Neurologic examination demonstrates an awake, intubated patient with intact cranial nerve function with retained ability to move his eyes, face, and neck to command. The limbs are flaccid, areflexic, and immobile to voluntary command, and he does not grimace to limb nailbed pressure. Electrophysologic studies are normal. MRI of the cervical spine identifies C12 atlantoaxial subluxation with severe high cervical spinal cord compression. Comment. This case is an example of “occult” myelopathy. Spared bulbar function with severe limb weakness suggests this localization.

VEY POINTS:

•

Limb weakness may aevelop in CU patients from disease involving the CNS. Imaging studies are clearly crucial in identifying these, while keeping in mind that such patients may also have concurrent peripheral nerve, neuromuscular junction, or muscle disease.

•

High cervical cord myelopathy must be considered diagnostically; “occult” causes of severe cervical myelopathy include transverse atlantoaxial subluxation in rheumatoid arthritis, unrecognized disc protrusion and cord compression, cervical subdural abscess, and acute central cord syndrome, among others.

CONTTNUJUM

EMERGENCY CONSULTATION IN THE ICU

Case 11 A 20-year-old male is operated on for the third debulking of a glioblastoma multiform. Postoperatively he deteriorates, with progressive weakness of cranial nerves and limb and respiratory muscles. He requires intubation and admission to the ICU. Neurologic examination documents bilateral sixth-nerve palsies, bifacial weakness, diffuse and severe limb weakness, flaccid tone, and areflexia. Reliable sensory testing is not possible. A diagnosis of Guillain-

Barr~ syndrome is suspected. Cerebrospinal fluid (CSF), however, demonstrates not only a very high protein level (196 mg%) but also a reduced glucose (9 mg/dL) and a mild pleocytosis. Nerve conduction studies of motor and sensory fibers in the limbs are normal, but needle EMG shows diffuse fibrillation potentials, positive sharp waves, and absent voluntary motor unit potential recruitment, suggesting diffuse denervation. MRI demonstrates multiple enhancing nodules involving nerve roots all along the spinal cord, establishing the

FIGURE 19

Enhancing nodules in the lumbosacral roots of a patient with diffuse spinal gliomatosis presenting with ascending flaccin areflexic weakness. Renroo.cea o,ith nermission from cooke Li, Morr Sn 55’, Becker ‘Nj 0 coassoma muitiforme ann ascending meakness can Nerni Sc 2’N2; 29:372 —37u.

diagnosis of diffuse meningeal gliomatosis (Figure 19). Comment. This patient developed severe generalized flaccid weakness with sixth-nerve palsies from diffuse tumor infiltration of the spinal fluid spaces.

S

F b •

I m~I

N,,

I

A ,~ p1~ t

~

DELAY: -1

I

oiv

5 ms

I

Enlarged unstable motor unit potentials from the tibialis anterior recorded by electromyogram in a patient with respiratory failure and amyotrophic lateral sclerosis (ALS). Note the variation in spike configuration in successive units. The units are characteristic of chronic denervation with reinnervation that would be expected in progressive ALS.

FIGURE 20

and a stub:uecute and sesc’u’e axo innul p0 miv— necii’o inpurhy; in fact, the o mbseu’saric in int’ ci nexplai ned c ip hr hal mc mpiegia in

lire’. lii is Iius beei a )u u’t i o’uu Lii’ p,m inbiem ufreu’ pro mic mngec I iii fcusic mu> of s ecci u’o in— niciuvm (Segu’eclo m et al, 1992 (Figure 2U \Nh ile clesei’ibec i in the litei’ut ci i’e, pu’epatients in the hit sho iculci aissass lead coin the cc mnsiclei’atic inn 0 If tiiiamil’ie clefi— ssiiuptie iieciu’c ii’ntiscciiai’ cunctic inn Ho mek— cienes. Aciministraric inn cif thiamine acle hu’o mm ;iminc mglvcc insic les seems i’are, under such circcumsrances sho inculci cu’oi- ii keis heca cise cit o:;u i’e fcu I mcmiii tomu’i ng lize a pui’entei’al rather than c inu’ul u’c incite. (If patients u’eeeising inese agents. Patients ssitli inheu’itecl Lmss plusimi

Neuromuscular Junction Disorders

KEY POINT:

•

pseciclo inoho iiinestei’use ;ucuisit\ m;us base 111,0 mic muigeci pui’u1sis fu’c mm len u’o mm iusccu— Lii’ jou nctic mu 1110 ickucle usso iciuteci ssirh the ouse cmf scieeiiislclic mime arid niisuccii’i dim ( Cei’t’ et a I, 2(1)12 (iliei’i ii gtcinn and I,asarer, l~~3 ), Other rai’e cucises

nespiratory failure may precede toe diagnosis of motor neuron disease (eg, ALS IChenetal, lgg7). Toere may have been gradually progressive limb weakness, dysarthria, or dysphagia with acute respiratory failure precipitated by aspiration or poe u 01001 a.

Ms usthenia gr.isis is genei’aliv chugno inseci befo Ire acimissic in tom the IC t As is tamiliau’ tom all clinical neduu’c micigists. bedside feutcui’es stiggestise of the chug- uu’e listed in Table 16. no insisinci tide ptc Isis, c mphthui mc inpuu’e— sis, tiexo mr c mr extenso mr neck sveukness, and limb ssc.’ukness ssith futigabil— it\. ‘l’iie cliagno isis mu\ be establishc.’cl Day 3 After Vecuronium in the 1(11 bx c’cmi‘nbining a ‘l’ensiiomn Discontinued ~ecli’o mpl’ioinnicim ) test svirli lsttu’e’Mnient assessment of pto msis, limb sti’engt in. eve nio insements, sital capacits, niaxi ni tim inspiu’atc mu’s pi’esscui’e’, 20 uv and i’epc.’titisc.’ cc innclcietic inn stcuchies. (iucuSins tic inn sho moulci be u~)pliedl t( in interpi’etiiig lensilo mn tests syith limited o ir ec~ciiso meal encipo mints, ilecucise false-pc isitise u’esours base been clescu’ihecl ssith brain stem tcimc mu’s and o inther lesic inns. the Iumime,’/-I”aio;i /,i’u’dN1/.incal/c’ 817/Day 16 After Vecuronium Discontinued (IJ’OJflc~ has c iceusic innulis hc.’o’n cc mm— plicateci i’c’sl)i i’uto mu’s tail cuu’e ( N icc mile er ul, 199(m). Mc inst. bout no int all, patients 50 u\~ base asso inciureci small cell eau’ei no inma of the icing. Patients mus base cliffcise Sins limb sseakness, pto Isis ssithc incur o iphthaimo inpiegia and appareii t taci litat ic inn o int limb sti’ength and u’etlexes. initialls neuromuscular transmission FIGURE 21 Severe absent i’eflexes may he Iwo incight c inout bs a deficit following a prolonged vecuronium infusion in a patient admitted to bi’iefe inti’actic inn c inr i’epeutecl taps (Ashbv the intensive care unit with asthma and prolonged respiand 1,ee, 197)), the use mf 3,-c—cliuminc m— ratory failure. Responses are recorded over the abductor digiti minimi after 2-Hz stimulation. This patient also had pvi’ichine iii treatment fo mr this cc inuclitic inn evidence of severe critical illness myopathy (note gain; can be usso iciareol ssith cli’amutic irncompound motor action potentials are severely reduced in amplitude). pi’o insement (McLso is et al, 1959). Patients tu’eutecl ssit h no inuiclepo mluu’iz— From BoSon cc Young OS. The neuroioqicai consultation anu neucoiogioai syndromes in toe ntensse care nt Baiileres do Neuroi ing mcuscie-hicinc’king agents mas base by

,

pi’emlo mngecl paralysis hecuouse cif clelaseci c’leui’unce cml’ the agent c r its merabi in—

1096:5:645—671 Roproducen with permission trom tisevier Scence Lto, Fonburgh Scotland.

CONTTN!JUM

KEY POINTS:

•

Definitive electrophysiologic criteria for ALS reguire denervation in two territories of each of three limbs (or in bulbar muscles).

•

Unfortunately, patients who are diagnosed with ALS in the CU may never be successfully weaned. Some decline ongoing longterm ventilation and reguest extubation. iThe LambertEaton myasthenic syndrome has occasionally been complicated by respiratory failure.

•

•

Presynaptic neuromuscular junction blocKade from aminoglycosides appears to be very rare. Patients with in herited low plasma pseudocholinesterase activity may have prolonged paralysis from neuromuscular junction blockade associated with the use of succinylcholine and mivacurium.

EMERGENCY CONSULTATION IN THE ICU

Myopath ies

CTG trin uciec inricle repeat (inn ebro moinsoinme l9dll3.3. shocuici be scepeeted come tom attenricIn sshen patients pre- bs the ehai’aereristic.’ facies, pi’eniatcirc sent ssith i’espiu’atoinrs mcisele sseakness balding, niasseter and tempoinralis atro in the 1(t’. While cuneoinmmon. rssoin pby and inilci proinsis. Mvointoinnia car inherited myomparhies shoincuici be eoinn— be elicited in a variets of syays. lirot siclei’ed: nisointo innie nicuseular clvsru’oinphv xseakness umus be variable depend and acid maltase deficiency. Mycintoinnie ing (inn the stage omf the clisoini’der and nicisecular clvstu’oipbv. an acitoinsoinmal the ncumber oinf trincuelecinticle u’epeuts. do innininant disc mu’cler assoinciateci syith a tiniike other mcusecuiar clvsru’omphies. Pi’eexustung mcusc’ie discini’clers mas first

TABLE 16

Causes of Diffuse Weakness in Patients Admitted to the Intensive Care Unit

Central Nervous System Disorders Bilateral cerebral infarction or disease Central pontine myelinolysis Other basis pontis disorders: infarction, hemorrhage, multiple sclerosis plaque Cervical cord disease: compression or trauma, multiple sclerosis, transverse myelitis, tumor, other ~

Peripheral Nerve Disorders Guillain-Barr~ syndrome Critical illness polyneuropathy Multiple radiculopathy Porphyric neuropathy Thiamine deficiency Chronic inflammatory demyelinating polyneuropathy (rare) Other

~

Neuromuscular Junction Disorders Myasthenia gravis Lambert-Eaton myasthenic syndrome Prolonged action of nondepolarizing muscle-blocking agents Pseudocholinesterase deficiency Botulism Envenomations Organophosphate poisoning Tick paralysis Aminoglycosides (rare) (oi;ti;nicd on ;w~x’t /ia,tp

KEY POINTS:

TABLE 16

•

Preexisting muscle diso, ders may present with respiratory muscle weakness in toe CU.

•

Myotonic muscular dystrophy is associated with

Muscle Disorders Critical illness myopathy Myotonic muscle dystrophy Other muscle dystrophies Acid maltase deficiency Polymyositis or other inflammatory myopathies

a peculiar

Sarcoid myopathy Mitochondrial myopathv Hypophosphatemic myopathy Carnitine palmitoyl transferase deficiency Trichinosis Human immunodeficiency virus—related myopathy

sensitivity to sedatives (in particular, opioids) that can produce acute respiratory failure.

Malignant hyperthermia Periodic paralyses Acquired hypokalemic myopathy Alcoholic rhabdomyolysis Pyomyositis Rhabdomyolysis secondary to toxic shock syndrome, Legionnaire’s disease, other

msc into inn ic ni cusociiau’ cltsru’c inphv is asso in— ciuteci ssith a peeciliar sensitisits tom seclurises, in pau’t ieculuu’, c inpic micis. sshich can pi’oiclciee aecite i’espiu’atcini’s fuiicuu’e

(Case 121. lneu’eusecl eenru’ai u’espiu’ato mu’s sensirisirs tom these agents has been po insrculatecl tom ucec incint fo inr this effect (Alciridige. I 985). ‘the clinical

Case 12 A 19-year-old male undergoes an orthopedic procedure for bilateral footdrop. Postoperative care includes routine use of meperidine every 4 hours. At 3:00 AM the morning after surgery, the patient is unresponsive and not breathing. He is intubated and transferred to the ICU. He remains unresponsive the next day and cannot be weaned. Further history emerges that the patient has myotonic dystrophy, and neurologic examination confirms characteristic features of this diagnosis, including myotonia. Naloxone injection is associated with brief improvement, but overall he improves very slowly over the next several days. He is not successfully extubated until approximately 2 weeks after the event. After discharge from the hospital, the patient resumes his normal lifestyle.

Comment. This patient with myotonic muscular dystrophy had prolonged respiration failure from opioid analgesics but eventually recovered.

r

C ONTTNIJUM

KEY POINTS:

•

•

Aduit acio maltase deficiency is an autosomal recessive deficiency of alpha glucosidase iacid maltasel bat renders proxima limb, trunk, ann respiratory muscle weakness. Criticai ilness myopathy nevelops in patients already admitted to the IOU with other diagnoses, and it may sometmes be difficult to distinguish from critical bess neuropathy.

•

The presence of norma SNAPs in an anestoetic arm or hand suggests that the lesion is proxima~ to dorsal root ganglia.

•

CBS may be assoc ated witO ife-threatening respiratory muscle weakness. In patients wth imminent respiratory failure, predicting toe need for intubatOO ano artifc dl ventilation is essential when toe vital capacity declines beiow 20 mL/kg.

EMERGENCY CONSULTATION IN THE ICU

:ictio inns cif these agents seeivin tom sse:ur ciff sic issis, gising Lulse initial ilinipres— sic ins cif the sesei’its cif the necii’o in0 igic olc’ficit. Patients ssith msc into innic clvsti’o iphv can gt’nei’uiiv be sseunecl l’u’c mm tlie sent i Lit cmi’ sat istucto mciis 0’‘ iseii eno iciglin ri nie and usc mid lance o mf secla— tise’s and o ipic micis. ‘I’lieu’e is no in esicience that specilic treatment oil’ the mvo into innia is cif benefit in this sircuaric mu. ~\4/ i/I uc’id ii Ui/lclNe de/ieiei ic’ 1’. iii ;ucuto inso mniui I’ecc’ssist’ cleficieiics cml uil3h;i gicuec insicl,usc.’ ) acid malt usc.’) is 30 mnsil3it’ to mu’ 131’o lxi maI iinib. t rcu iik. and i’espii’aro mrs mcuscle sseukness. IfMG clenic inst rut es filTh I Lit ic inns. comm piex u’e— pet it ise po itentiuls. utict mvc ito inn ic ci is— chui’ges. Bic ips\ slic msss mciscie fil3eu’s ssitli succic mit’s cc mlituiliulig glvc cingen and hbei’ clegenei’;itic iii. It is iii 30 ii’tu ii t to c’stuhl ish rho..’ cc mu’i’ect c liuguic isis iii t imese patients. si ice so innie uiin:cs later lie soc— eessfcills sseaiiecl fu’o miii the sentii:itc mr. ( )ther disc mi’clei’s that base been i’:cu’e— is usso mdi at ec I ssi t ii u’esp i i’utc in cv Liii ci i’e iiici cucle po iisni\( insit is, sai’cc mid msc mp— at hs. a ii ci mito ic ho muic I u’i:i I nyc input liv. Sesei’e aedicuii’edl hspo ikaieniiu can he usso mc’iutt’c I ssi t ii general izec I iinib amc I i’esl3ii’atc mu’s mciscle sseukiiess. C,’//ic’u/ ///;/e=N ni1’o/)u1i)t’ ((IINi it’st’lo gis iii ~ it’n ts ul u’eucls ad liii it ted I to i tht’ I(It ssitii o mrlit’r cliugno inst’s. and it so iniiit’tiiiies mus be’ clifficcuir tom ciistiii— gcu is ii tic im cu’irica I ill ii t’55 lie to u’o g i:i t ii 5. St’st’u’e and cliffcust’ inso miseivit’iit of limb. bruihur, and I’espiu’urc mu’s moiscies is thai’:ieteristic. ‘I’ht’ dust’ c If c’o mu’uico i— steu’o inicis and no innclepc miau’izing ni cuscit’— hi c icki ng agents is a ‘is k tiicr cmi’ ic mu’ its clt’seic inpmenr. and it mus appear cc mnccuru’t’ntis ssith pi’o inlo inngt’cl 13t’ti ‘0 inn3cisc’ciiar dinctic in 1310 mck:ic it’ t st’t’ :ui 301st’)

Focal Weakness in the Intensive Care Unit Ic inc ul ss tukntss n3as lit’ clifficcuit to in ic len rifs in putit’nts ucln3ittt’ci tom tht’ 1(4. Wl3ei3 lilesent (INS disc ii’clt’u’s slic incuici i3t’ cc muisic let’t’c I fi u’sr \frt’ r a

1130 into mu’ st’iu icit’ uccid it’iit c ir c rI3ei’ fo mm; cml ti’uciil3,i. 131’:ic13i:ui 131t’x0113:itl3} ini’ icinci: i13 it’ ct’l’sic:i I u’c inc int :isdi isicmii i33us casts tiuccici i3u1’uiYsis cml ti3t’ ui’i33. sculic

Lii’ x’ rIg i 13g. ci iuii13 i’ug133 i3~ Li ivsis. :111. 110 mu’uit’u’’s ssnciu’ommt’. 10 meal i mss cint mc Ilt’xt’s :ii3ci sensut ic ini3 also in appears. Sine Ic mss cif (IMAPs uiici the :ippeul’;incc tiiii’iiLitic inns uftt’u’ pei’ipht’i’ui nt’i’st’ ir cii’s do in no it appeal’ ii33n3t’ciiutt’is, it ii33130 ml’t:ii3t to in cit’Lis t’lt’ctl’c m~ii3ssicinioccj; st cuclit’s scm that tim’ Icuil t’xtt’13t cit tIc 13j dii’5 cui3 i3t’ 133u131meci (1 to in 2 sst’eks ‘l’lut’ vu’t’st’nct’ c inf no irmul SN:\l~s ili unt’su ht’t ic arm cinc h;inci sduggt’srs th;s tl3t’ lt’sic c13 is 131’om\i133u1 tc in cioini’s:ii loom ii3tiii ,i ,il3ci i33us :ictciulis I3t’ i’c inO It u\U~ sic in (Figure 22) NiRI c mf t13t’ ceo; c .ui ui3ti’~ispii3~ui sliuce is uheuu 13t’1131cil icleu3til\il3~ 1310 ilili u3t’u3 t u33t’u3 i u3gc mct’ies 2 lit’ si tt’5 0 int t l3t’ ‘in inc it iii ci ‘it’s St’st’i’u Ic intl 3t’i’ L inca I lit’tin ic m~ iut hit’s a: 133130 mitulir eucist’s o f fo incal sseukness iiurit’lits :icii33ittt’ci tom 1(4 s Bi’:ichi; iiit’xc in~3:it 13s i33us to inibo 13t’i3—l )usici uiici Stali I. I 99 ) t i3iiutcc; mc 13i i:itei’:i I 1313 i’t’13ic tit’cuu’c miin:it i3it’s uls i33:is cc mil313uicutt’ c:ui’c ii:ic scii’gt’i’s \lltc u’ic mc i mitt’i’c msst’o incus i33tciuii3 l3t’cuu’c tiis l33us cit’st’io c~i ho i133 i13u13131’o ini3i’hitin’l chii’t’ctt’ci :ii3tt’ccii3itui st’13i13c113ctcii’t’. Pm cii:ui u3t’dcro m13:it 135 at tI3e lest’i cof th siiiu’ul guc mc inst dm3us lie asso iciutt’ci ssiI; 13d1133t’i’ui tu’uctcii’t’ Iii r lit’ Ic msst’r Ii 13313. t’133i3c ml it isci3e13t I ci 133130 ms:icu’:ui 13it’\c mI3~iti3s i33us 0 iccc i13 13:itit’13t5 ssiti3 5t’5t’l’t’ 13t’i’i13i3t’l.i susccii;ir ciist’ust’ ssiioin cii3cIeu’gom fen3cinm,; uu3giomgu’u il3~ :10 inu’tic ;ui3t’duu’ss133 u’t’1xui ‘-~

‘~

cmi’ ii3ti’:i—:ioli’tic i3uiic mcmli ~3tii33~3il3g[i’:it tdui’t’s o ml ti3t’ pt’isis nius lit’ usso icluto., ssi t 13 icc 133130 msuci’u I it’xo m~ inut 13s Put coin

ss13o m :uu’t’ :ii3tico i;orcii:itt’ci oil’ liuSt’ ;i ci :igtiic in13:it 135 133u\ cit’st’io in13 a cc m133131’t’5sit: ilic in~3so inus 13t’133:itc 1133:1 c:idisll3g id1133I3i s:ucu’ul i3it’xc mI3~iti3s Sc tutic 13t’diro inputlr 133as cmccduu’ :ultei’ ti’:idui33:i cmi’ ligi stil’Ud’i’ Plt’ssu3iu t’t :il 1 ~)99I it i33as bt’ u’t’ccic ti izt’ci 13\ :u dm3 iiutt’u’ul h inO ltd ii’c 113 comic 13i13t’di 55iti3 :113 :u135t’13t ii3sii:itt’i’:ui :113k)1

u’eflt’x. Cc 1133 131’e5515e sciatie i3eduu’oinpathy dir Icunibo insaeu’ai 131t’xo inpatl3ies sl3o incilci be deco 1133131’esseci dui’geI3ri\.

SOME SPECIFIC NEUROMUSCULAR DISORDERS Guillain Barre Syndrome Familiar tom all practicing l3ecuu’o ho ingists. Gcuiilain l3aru’oi ss’nclu’0in133e ((;BS) is au~ aecite acuto miniui3 cune i13f1a133133at0mrs 1301Iy— neti i’0 I~inat 13s ti3ar 133a5 i1350 mive 1330 into mc, sec3scinu’s. or adutoIi3oinl33it’ fibeu’s. Classic G135 is assomt’iarecl ssirh aecure al3ci ssiciespi’eaci diei33selil3atic inn o int peu’i I3hel’ai nei’ses, bcur vau’iai3ts 55itl3 seiet’tise inso iisel33eI3r of ri3e axoins base 1330 mu’e u’et’eu3tls bet’n cleseribeci (aecire 1330 intoIr axo 113a1 13t’dil’0 inl3ati3\; at’cute 1330 into ini’ al3dl senso mu’s, :rxo inn l3edui’cII3atl3y) (Feasbs et al, 198(3: Md’Kl3ai3n er al. 1993g G135 mas be asso mc’iarecl 55iti3 iife-rhreareniI3g u’espiu’ato Irs’ n3cuseie sseakness (Case 13).

A

sval’I’aI3redi svi3eul patients ai’e fii’sr transferi’ecl dincut omf rl3e cunir after extcubatic inn. N\’hiie ilidIst pariel3ts 55itl3 GBS 13:1st’ :113 at’dure dleI3us’t’ii l3atiI3g poIiyi3ediu’dinpatby. 13:ttiel3ts ssirl3 seu’s’ ses’ei’e cliseast’ 133a5’ 13:ise a t’oImbil3atio inrin oinf ssiciesl3I’e:tdi dlei33seiir3atioi13 al3dl :txc 113:11 dlegei3t’ra— tioinl3. Pariel3rs 55iti3 aecure axoIl3al foirms

oinf(71135

133:15’

sdlI3l3oinrt.

113

sil3lilal’l\ i’ed]cuii’e vel3tiIato Ii’ gel3ei’ai. sesere axdinl3aI

rxmi~

R

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finger

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3 8 0 0380 011 ~ 1 1 ?IC 02?Ci 013 1 100110 ~

lP~8 Axi II sro~rJ” FOMVr.si

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Wruit’Be~ow e~b~w

ThT~’Y1rrrrl~F.

230

Below e1bow.Pboj~e elhoi ~]~al £ I irt~~r Nri~t Vri~t~&e1au el~ow 13et~u •lbo.~lbovc ~Ibow

Above elbow r~ ciIa FIGURE 22

P1~t* V1~•

~3 ~

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B

_

In? Belo,

A

6

a — •i “UJ’J ?5 6 ScP 65

Rec~rding Site ~l lOW R~c~rdun~ site .m ~tI S[IE

B

Patients with acute axonal forms of GBS may rego ire ventilator support. In general, severe axonal involvement, either alone or combined with demyelination, is associated with much more prolonged disability during recovery.

tO inI’ii3(~ is

-------~I-.-• ——ii rest.

5 41

•

sisicIn is l’eeoini33mendledl. Similar 1330113i-

B

a

K~Y POINT:

113 ptrieilts ssith i133133il3ei3t l’esl3iI’aromu’s failcuu’e, pl’eciieriI3g the need fomr intdul3a— rio 113 and artificial vei3tiiatic inn is esseI3— tial sshel3 the sital eapat’irs’ diet’IiI3es beioinsv 2() ml. kg (see pu~ e’ ~ Roinpper al3cl Kehne, 1 98~). 113 t15, cuse oinf u33oini3ito mu’eci l3edis (ssith cc mhitii3cu incus n3eascui’es O mf t’arcliae rl3sthn3, iieuu’t i’ate. ai3ci omxsgel3 satduraticinn ) svith fredlduenr n3eascii’en3ents (eg. esei’s -4 rein (i 130 mcli’s) cmf vital eapat’irv and eloise ndursing scll3el’-

13~ 2

q

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41 45

Multiple cervical root avulsions following trauma and resulting in focal limb weakness A, Magnetic resonance images of the cervical spine identify a large meningocele on T2-weighted images. B, Electrophysiologic studies identify absent compound motor action potentials in the ulnar territory (top three tracings) with normal sensory nerve action potentials in the same territories (bottom three tracings) The lesion is proximal to the dorsal root ganglion.

CONTTN2LIJUM

KEY POINTS:

•

•

It is possible that following IVIg with plasma excoange might simply negate the impact of IVIg by removing toe agent. In addition to acute respiratory failure, CBS patents are at risk for aspiration pneumonia, deep venous thrombosis and pulmonary embolism, and for acute autonomic instability.

EMERGENCY CONSULTATION IN THE ICU

insoinise’133e13t. :tio ini3e

dr do mu33biu3edl

55iti3

ssirb 133d1t’i3 disability cldurii3g i’e— Weli-esrabiisbt’cl eieeri’c inpl3vs-

d1e1335eiil3atic 113, 15 asso inc’iareci 1330 ire

i31’c mic mu3geci

cc inst’i’s.

ic mic ingic fearcii’es (If dleu33seiinatic 313 ill—

c’lcucit’ p~’~ inlo Il3geci ciist;ii 1330 into mc lareu3eies, do Ii3didid’tio 113 1310 mt’k, c’oinl3dicit’tioIil sic inss— ii3(~ ‘113(1 t~l33~ ini’ai ciisiit’i’sio 113. 113 tO 113— trust, axo 113:01 dlegenerat ic inn is asso inciuteci ssirl3 Ic inss 0 If CMAPs al3dl SNAPs au3ci diei3se fibu’iiiario in135 ~l3 FMG 113e1’apt’tu— ticails, iritl’a5t’130 incus gau33nia gioinbtuiii3 omr 131as133a exehangt’ 133av be adiI33ii3istei’edl in tl3e 1GW, bcut it is inappi’cmpriate tom expect o inbvio incus ciil3iea! i133p1’oIvei3lei3ts after rl3t’ ti’eatil3el3ts. ‘t’bei’aps iu3 G135 has been :tsso meiatt’ci ssiti3 a statistical i13313r0 Iseil3t’13t i13 eiii3id’ai trials, bcit a so in—called i~azarcus t’ffet’r ( fo mc tl3t’ laz:urcis, sshom :tu’o inse fu’o 1133 rl3t’ dead is dul3t’( in133 1330 113 Is c milsersedi ssith ri’eat133t’13t I 113t’ GBS Srcuclv Gi’c mu p 1985: Van dec Mt’el3e :tl3dl Sc’i3i33irz, 1992e 113e1’t’ is no in esiciel3ce tom date t ilat fcurti3er t’o mcuu’ses of intraseno incus i133 113 di130 inglo ilicilill ( IVtg I cli’ cc 111311i13i13(X IN/V ssitil 131:05133:0 exellange is beuit’fitiai ( Plasma Fxehaumge Sauldic ingiclbtiliul G135 ‘l’i’ial (iiu’c indup. I 99”’). lnclet’ci, it is possible that fomiicmssii3g IVIg 55itil pLisil3a exeilal3gt’ n3igi3r 5i133131v ilegart’ the

iillpaet of IVig by ren3cIsing rile agent. (‘~lcuc’cint’c inctiecinicis do in 130 It beilefir CBS (licigiles er ul, 198. 113 acic lit 10113 tom Ao.’dit t’ i’es 13i1’atdmli failcui’c GBS patients ai’e at i’isk fcinr uspii’aticinn 1313t’ti1330 Illia. deep s’eIldinns 13 I’O 1133130 Isis ai3dl Pcuil33o 113:01’s eillbo inlisni l~araiszeci GBS imarit’nrs ill tile cui3il silo intulci lint’ 131’0 insicit’cl 55itil ii3terl33ittent l3i3ecuIllarid’ d’0 1133f11’t’55i0 113 srcmekiu3gs tmi

ti3ese risks, patit’13t5 55iti3 (ABS :tdlrllitted toin tilt’ hit’ ai’e also in at risk fo mc aecote acuro 1130 IIl3ic’ iilstabiiits (/omWo indille. 199-u) ‘Fl3e 133(1st seu’io incus acuto 1130 Il33it’ clistcuu’hun c’es ai’e scudidiell tilailges in heuu’t i’;itc

and u’13sthill or l3icIomdi 131’t’ssduu’e. Vac~ai spells au’e ei~iso inc It’s cmf sducidleul bi’aclvc:ir clia dr assstoit’ that eal3 l3t’ 131’o insx inked 13i13g. iulsel’tio Ill cint 13:050 in(~’t5ti’iC

13s stictidin :tss mc’i:iteci 55itil

i3t’1330ldisIlai3lit’

t’o inilt

131’0 1i33i5e ssai’u’allr 131:tt’t’133e13t o mf a tern 130 inI’a17 puet’i33akt’r. Pariei3ts ssirh (;Bs silo incuici eucitic cusiv 13t’ giseil saso in:tctis’t :tgt’13t5 fo inc il\peu’tt’ilsioinn.

Critical Illness Polyneuropathy (113a1’ies I3cliroIll oIi’igiilaiiv dieseril3edi 2 cilliclcue aI3cl sest’i’e :txoIil:ti 130 inlvnecucclii’ at ils 0 m135e1’se’di ill 13:itieilrs adllllittedi tom

Case 13 A 65-year old woman is admitted to the ICU with herpes simplex encephalitis (HSE). The patient is comatose and on a ventilator. HSE has evolved over approximately 2 to 3 weeks with characteristic clinical, imaging, CSF, and electroencephalogram abnormalities. She then develops diffuse limb weakness and areflexia suggesting irreversible, bihemispheric CNS damage or superimposed critical illness polyneuropathy. Neurologic examination discloses an intubated but unresponsive patient with normal pupillary reactions, corneal reflexes, and oculovestibular reflexes. The limbs are flaccid, unresponsive to deep nailbed pressure (no grimacing or withdrawal), and areflexic. Serum CK is normal. Electrophysiologic studies identify widespread features of primary demyelination with prolonged distal motor latencies, slowing of conduction velocity, conduction block, temporal dispersion, and only occasional fibrillations. The diagnosis is acute GBS following HSE viral infection. Comment. In this case, the patient’s flaccid limbs were presumed to be secondary to CNS HSE. Electrophysiologic studies established the diagnosis.

KEY POINTS:

Case 14 A 56-year-old previously healthy man is admitted to the hospital for emergency repair of a ruptured abdominal aortic aneurysm. Adult respiratory distress syndrome, encephalopathy, and abdominal sepsis complicate postoperative recovery. He requires intubation, antibiotics, and inutropic support over the next 3 weeks, then gradually improves. By 4 weeks after admission, he is awake and responsive but appears to have diffuse limb weakness and cannot be weaned from the ventilator. On neurologic examination, he is awake and has intact cranial nerves but cannot lift his head from the bed. The limbs have prominent wasting without fasciculations and are immobile and areflexic. He does not withdraw or grimace to deep nailbed pressure. Serum CK is normal. Electrophysiologic studies identify very low amplitude to absent CMAPs and SNAPs, normal conduction velocities, and trains of fibrillations without voluntary motor unit potentials in multiple sampled muscles (including intercostals and the diaphragm). Quadriceps muscle biopsy identifies scattered and grouped muscle fiber atrophy indicative of denervation. The diagnosis is severe critical illness polyneuropathy (CIP). Comment. This patient has the typical scenario in which CIP arises after prolonged ICU admission for a nonneurologic problem.

hit’ toi’ c inthei’, no 11313d7c11’0 micIgic’, ili’o Ill— 55itil 135110 mtei3sic i13 ai3di i33cliti1lie 0 inl’gai3 1e1335 (Case 14). ( BoIitoIi3 er al, 1 98—c; dissfdui3t’tio 113 ssu3dli’o 1133e. i3atreu’t’133ia ai3ci /oIelloIdlI3e er al, 198”’) Despite svidiell- sepsis are 130 int svumo inilvdllo incus: tl3t’ fo inri33t’r sililIlis i’efeu’s to in a ~ insitive ilicindldi cciiiilg attellti 0113, tIle eacuse of CIiI, o mc Bomitomul’s neduroinilari3y, i’eil3aiIls oinbsecure, rdui’e, ssllit’il is 130 It :113 abso inicutt’ i’t’c~cuii’t’hcut it iS 133(1st t’iO 15t’i5 iii3kt’di to in tile i33e13t fo inc sepsis. Putit’13r5 ullet’tii3g ti3t’ s\Ilclu’o 1133t’ o if 5e135i5 and 133 diltillit’ 011’- ti’ireI’iu fo inc SIRS are st’u’s ill :ui3di i3:ist’ a 1, 55iti3ii3 gai3 failcure o inrberssise clt’fineci as rl3e 1330 ini’taiits I’: ire a~1pi’diuei3il3g (ad ai3 hit. 113 a ili’0 insilt’d’tist’ sei’ies o mf 132 ssstell3it’ ii3tlafl3i33atc ml’s i’espo incise s513dirdinn3e (SIRS) (Bt’iljan’uiil er al, 1992: patiei3ts i’eferi’ecl fo Ic eieeti’o Ipllvsio IIc ingit’ srcuclies (133ea13 iilrel’sai cinf -() ci:uss 13o inllt’, 199 1): (1) tei33ilt’u’atcui’e lligi3t’r ufrt’r adil33issio Ill), /it’kc in al3dl cc Iieac~cies than 38 (3 oinr 10 msser thaul 36 C: (2 ) heart (19981 cliagilo insecl critical iiii3t’55 Pc ivcart’ gi’eater tilall 91) bears per lllil3dIte: 3 u’t’sllii’aro inrs rate gi’t’atei’ tilan 21) n3i13 ilt’dll’dIilarils i13 (a2, 0inc up~li’c inxii33:itt’is or PaC() less tl3all 32 133133 Hg: Ilaif, oinf ti3t’ u’eferu’t’ci eoinl3cmrr. l~atieu3ts 133a5 ilt’ reft’ru’ecl becacuse o inf ssbite 131010 incl t’eiis il3 bic inc mci gu’eateu’ cliffcuse sseakness :hulci clifficcuirs i13 ileilig tllan 12{WI() t’t’lis 1331. or ie’ss tllail sseai3edl fi’o 1133 tilt’ st’iltiluto mc. Ssi3311t0 1133s —,l)()() t’t’l is 1331, ou’ gi’earei’ t 13a13 ~( )~k int 110 ilvIlecil’oIll:ttils :ui’e cuscu:tiis 110 It il33l33atdii’e (band ) foIrI33s. SIRS e:in cieret’recl, sillet’ il;itit’llrs are iiltciilatt’ci cieseic Ill after seseral types oinf iI3sduit, aildi seciart’cl alldl Illas ilase t’uldeilllaii3t’ididliilg infeeric inn (seilsis )‘ pailt’u’eIc inilarils. Ill 50 Il33~ illstaild’es. 110 inssest’r, antis, isehenlia, IlldIltiille ti’acui33a 55itll tissdit’ iIlj di l’s, 13e1330 Irrilagie silo inc’k, ilear siloIt’k, irulnlcullt’-ll3ecliateci cinI’guI3 illjdu— rilesiae didll’iilg rlleir u’t’co mst’i’s. :lildi IllaIls llariellrs 130 Itedi illciseit’ sse:uki’s, aI3ci ex 0 ingt’u3o incus :tcil33il3istl’;itio 113 omf iless clcui’iimg tilt’il’ l’t’ilaililitatio Ill. (Iu’aili:ui illtlai33illaro ii’s ll3ediiatc mi’s. SIRS assoici— ilel’ses art’ gellt’u’:liis silal’eci, all iillpo ii’— ureci ssirb illfet’tioini3 is terlllt’cl sepsis. tallt d’iillit’al fiildiirug clisriilgcuisilillg ssllereas st’prie silo mc’k i’efei’s to in selIsis ,

—~

•

The cause of CIP remains c’ score, but it Es most closeiy ,mnKed to the syndrome of sepsis and multiple organ failure and defined as SIRS.

•

Pat ents meeting the criteria for SIRS have a mortality rate approaching 600o wthin an CU.

•

Cranial nerves are generally spared, an important clinical finding dstinguishing SIRS from GRS.

CONTTNUUM

KEY POINTS:

•

•

At present, CIP is thought most likely to represent an end-organ complication of SIRS involving a cascade of widespread tissue ischemia, mitochondrial dysfunction, oxidative stress, o itrative stress, and damage from inflammatory mediator molecules. Fulminant mya sth en i a gravis may occasionally produce severe acute limb, bulbar, and respiratory weakness.

EMERGENCY CONSULTATION IN THE ICU

rllis ciisomu’cier fu’oinun (;135 aildi scinille 13300 inlIarilies 0 inc ilecui’c inllldust’dulal’ jtiilt’— tic Ill disc Ii’ciel’s, lile linlims exilibit flat’cici sseaklless aildI arl’oinlllls, aitIldindIgh distal atu’o hIlls’ Illas’ be o mbsecuu’ecl bs’ liullIl edleulla, auldI tllel’e Illas be iomss

u’eercuirecl crinas’ ilase su~iall a133131i trudes aildi poinlspllasia. Scieb ‘‘naseen: rib 15t’ cuilits tic inn,

cleselomp cidurillg earls’ airIlo IdugIl tiles’ illaS’

reiIllleu’s2 l’eseillhk

rIb 150’ u’eercuited ill a 13150 inilarils. t i3iik~ (;135, CSF ilu’cmteiul leseis au’e IloinrIllal em mf lll’es’ioIdusi} 110 inrIllal deep teildidinIl Illildils elesatecl, Patilcinlomint’ stcuclin’ 0’ u’eflexes. Senso mu’s’ exanlillaricinll Illas’ be if IlerillIleral Ilei’s’es ill CIP iclentit diill’eiiable ditlriulg tile linarieilts at’dlre aecure dxc Illal cie~ellel’aric Ill aIldi 2rxcmr ililless, bcir distal p~ill330incial sromt’kiulg- lomss ill distal aulci lll’c Ixiullal 1130 into mu’ ant aildI—olo inse loiss tom all seulsomu’s’ Illoindlal— sensomi’s’ fibel’s (/~omeboindille et al, 198 ities call become es’idleulr later. Patiellrs (Figure 23). Mcuseie iinioinpsies 5130)6 ssirll CIP Illas’ be asvake set fail tom aecure seartereci Illdust’ie filler atroinpim gi’iillat’e or svitildlrass rlleir iilllbs tom cii’ t’ilroinllit’ gi’oincuped filler atl’cinllils’ ss’itt scattered llet’rointie llldusele fibeu’s, Scuct Eieeru’dinpilssicinlo ingit’ srcuciies (130mlro Ill eilal3~e5 Illas’ i115’0 mlse lll’o lxi illal ailci clis er al, 198(i) idieultifs dleelilles of CMAP ral illdlseles as sseii ~5 tile diia~llli’agn: ailci SNAP aIllplirdudles ssirll noinrullal t’0 Ill- aildi iIltel’edIstai nldlsc’ies. BoinrIl retro cicut’tio Ill selomeiries, distal 1110 into mu’ latell— simet’rise aulcI ill’dlsllet’tis’e stcuciies n: cues, aIldi F-ssas’e lateilcies. Sinmilaris’, CIP ilas’e failed tom link irs dies’eloIplllcmn: tile diiaplli’agnl CMAP Illas ilase i’e- tom spet’ific’ clrcugs. scid’il as aillillos clcut’eci alllpiitcudie (/itkcm er al, 1998). gisco insides, 110 lildielmo inlarizillg i3ldisd’lt’ ifMG idlelltifies sviciespi’eacl fibrillatic inn bic mc’kiIlg ageults. cmi’ gi cut’o mec mu’tit’omicis 110 IreIltials aildI 110 insitise silarlI ssas’es, Siullilaris’, it eallIbo It be hulked tom specific

11350 miseulleilt (If illrel’t’oinstai Ilatilo ingeils cmr dlefieieuleies o mf sitalllins. aildi tile diia~lili’agil3. Pariellrs ellrcinnlicunl, iillOinit’llit’ acid, oinr lillomic/iC Illas 110 It be able to in u’t’t’rclir ally 50 ini- at’ici. At ill’eseIlt, (.11~ is rIb indlgilt 1330)51 illeididiillg

lllcust’ies duIltal’s

1130 intO II’

dullit

P~ intelltials,

aIldi

likeis’ to in i’ellu’t’sellt

~ll eildl—o inl’gall eo3ic

lliic’atioinil omf SIES i115’c mls’iIlg a cascade oinf svicit’spu’eacl tissdle iselleIllia, IllitO c’ilo inIlcIrial dis’sfcullcriomll. cixiciatise stu’ess, llitl’atis’e

stu’ess.

auldi

dlaIliage

fu’cinn’

illtlalllillatoinl’s’ illedliaroinr nicinleccules.

Myasthenia Gravis Fdliullillallt ll3s’astllellia gl’as’is Illas’ circa

siomIlalls’ proindlcut’e sevel’e ac’dute liullb, bcuibar, aIldI l’esllil’ar( mi’s sseaklless. A t’ai’efculls’ IllaulIledl leulsilo Ill tesr is ileip. frul dliagilo instieaiis see ‘‘NeclroIIlldlseciial JtuIlctiomil Disc mrdeu’s’’ 4. This rest aisom is ill te u33s:is rlleulic’ c’i’isis fu’o 1133 c’ilo mliuleu’gic erisis Repetitive d’Oinlldldid’tiOinil srcuclies ulias’ he peu’fomrrnecl ill a llu’oinxilllai ullduscle (07g. ,-,

FIGURE 23

Transverse section of a deep peroneal

tile accesso mi’s

nerve in a patient with critical illness

cinser ti’apezidus) cmi’ 55itil stiullcllarioIil cm)

polyneuropathy. Severe loss of myeli-

nated fibers and profiles of myelinated fibers undergoing

acute axonal degeneration are present

ileu’s’e ssirll

u’eco inl’diillt

tile ilill’eulid’ ileu’s’e aildi u’ecoini’diiulgs fu’ul3r tile chiapllu’agnl (Mailer

Viral

capacirs’ ;ur omc beicmsv

al, 19981 2(4 1111. kg cmr

et

KEY POINT:

Case 15

•

A 35-year-old female with a history of severe asthma requires admission to the hospital and intubation for severe bronchospasm. Despite the use of inhaled and intravenous bronchodilators, she has very high airway

resistance; she is therefore paralyzed with a bolus, then infusion of vecuronium is started to facilitate pulmonary oxygen exchange. High doses of intravenous corticosteroids are given. Adult respiratory distress syndrome complicates the pulmonary management over the next 2 weeks, and paralysis is continued for a total of 10 days. After vecuronium is discontinued and presumably eliminated, the patient remains flaccid and immobile for

the next 24 hours. On neurologic examination, the patient is found to be intubated. She makes no spontaneous facial, eye, or limb movements. Pupillary responses are intact, but corneal and oculovestibular reflexes

are absent. The limbs are flaccid and areflexic, and no limb movement or grimacing is apparent on nailbed pressure. Serum CK is over 10,000, and there is myoglobinuria. Electrophysiologic tests identify a severe neuromuscular transmission deficit when stimulating in the ulnar motor territory at 2 Hz. One week after discontinuing vecuronium, the patient appears responsive, having fully regained eye and facial movements but not limb movements. The patient grimaces but does not withdraw to nailbed pressure, CK level has gradually declined to 2,000. The neuromuscular junction

transmission deficit has disappeared, but CMAPs are very reduced in amplitude and appear prolonged in duration. Conduction velocities and SNAPs are normal. EMG discloses only occasional fibrillations. A few low-amplitude, polyphasic, voluntary motor unit potentials are recruited. Quadriceps muscle biopsy identifies a large number of muscle fibers undergoing acute necrosis, vacuolated muscle fibers, and intact muscle fibers

with loss of myosin-thick filaments. Regenerating muscle fibers are prominent. The patient eventually recovers completely. The diagnosis is CIM. Comment. This case illustrates the risk factors of nondepolarizing muscle-blocking agents and corticosteroids in developing CIM. Paralysis may be severe.

ii’s p~’t’s~tict’ imelo 1W

(\t’r133:i aIldi ( )gt’r. 1992 ) . Pcuiso.’cl iiltu’u-

p~’t’c iictt’ci I :ui’o.’ sigils

5t’nc icis i33t’t i3Yiili’t’d illisO inic lilt’ lll~5 c mffo.’r 1330 il’o.’ i’;iilici :illci ill to.’ilsiso.’ tilt’l’ulls fo inc

il3:ixi ill ci ill t’xili Vito

—il)

dill

II

_

( ) (31)’

i’t’c1tiii’t’ci at silo ini’t 130

intict’. ic ii’

ti’euti~~ei~t

t I 3t’ l( t , 35i’ic Ic inst gill I lit’ cull ilt’ gist’l3 ims lt’t’diiilg t cui~t’, sduilpo insito mt’s. o mc ill

~0,~

‘

~k”

1 15th coin 1 31 Iril o int tilt’ oini’ui do iso.’. Pulients ssit ii ‘eceilt— Ilui’o.’llto.’i5’li do inst is inilset

,

5t’5t’l’t’, (‘t’ilt’i’ulizt’di ilisust ilt’i3 iu

o.~u’usis st :u u’tt’c10113 13igi cioinst’s o int cc il’t icc in—

stt’u’o micis

:uu’o.’ at

i’isk

to ini’

purucic ixic:ui

cit’tt’u’io il’:itic ill didil’iilg tilt tii’st +8 tom 90 130 incurs 0 int tl’t’uull3t’llt . :il3di ti3ti’ui35 jli’c inil:uili 5 si3cm cold I iit’st I 3.’ cc inillili ltd I 55itil a cc indii’se cmi l3Li5l33’i o.’xc’llailgo.’ cml’ 110 mssiimis iiltl’ust’llc incos g:il3ll33u glo inildilill

FIGURE 24

Low-amplitude polyphasic voluntary motor unit potentials in a patient with critical illness myopathy. (Gain is 100 iV, 10 ms/div.I rrom Boiton cc, Young 08 Toe neurniog cal consciitaton ann neurorogical synorornes in the intensive care unit. Ba bores ci n Neuro t 996, 5645—67? Bepronuced ~sth nero 5500 0mm Eise. or Science Lto, bonourgir, Scot anm

A canacity at or beKsv 20 mLJkg or a maximLim expiratory pressure below 40 cm H 20 is a sign that intubation ann ventilation may be reguired at short ootce.

CONTTh3JUM

EMERGENCY CONSULTATION IN THE ICU

KEY POINTS:

•

•

•

Patients with CIM have most often lalthough not always) received either nondepolarizing muscle blocking agents, high doses of co rt i costero iris, or both. CIM patients have greater cranial nerve involvement than those wth CIP. In transplant dots CIM may be particularly inKed to the use of bingo doses of cortcosteroids administered to prevent rejection. This variant of CIM has been called acute steroid myopathy.

•

On muscle biopsy a selective loss of thicK myosmo f laments is coa racte ristic of CIM

•

Important pathologic features of CIM inclune type II fiber atrophy, muscle fiber necrosis, vacuolated muscle fibers, and regenerating muscle fibers.

.~

il:isc.’ gu’t’utt’i’ c’i’:iuliui i3o.’i’5t’ iilsx in15eilld’m’

er ul, I 985 ) sicieu’eci ill

iilsi33t’c’to inulls cull lb.’ do Ill—

innst’t geileu’aiizt’ci i33sust ilell Li gi’usis ci ci u’i ilg tile aecoto.’ stugo.’. ‘I’lb.’ tiilliilg (If cc milcdurilutit’llts

55itil

ilt’55-0

l’t’ilt to Il’tio.’c instt’i’o mid dust’ ill stidil put it’13t5

is ssc inl’til

o.’0 inllsiciei’iilg, ilecucist’

c mf

tilt’

(lIP: 011113til:li 1130 in13io.’Pi. ime o.’iic’o Idiilto.’i’eci ill 50 mill.’ iilst;iuldo Sitsso.’ii o.’t :ui , 199 1). (i’si put it’ilts ;uis i3:l\ o.’ 513:uu’t’dl 5t’135:itio Ill ( t’g, d~u’iu33:ic’ul3, lintut 130 It 55itilcil’:lss:li tom l3:uilil.’di ili’d” 11:113 t130 in5o.’ ssitil

113:15

dil’t’).

SOIillt’

purit’13t5

il3:1\

t’xi3uii;

cc mllo.’t’rlls tilar it ullus sic insy stt’rilc into mIlls 550 intuildi ileaiillg. lilt’ cc ut’ o If illsc’c inililo.’—

i’o.’:urcuu’o.’s cmf 110 intil (Ii”~l :ui3di (Ai~. In ri’uc’

IC t is 130 It set c’ie;uu’, bcit it las i’t’c’t’iltis ilt’o.’ul cuso.’ci ill

iil3kt’ci to in tilt’ dust’ omf iligil do inst’s cc ini’tic’0 instt’l’0 inicis :idiilliilistt’u’t’cl to in 1310 5t’13t l’t’jt’c’tlc Ill ( (u13311t’i Ic inilt’ o.’t 2. 190)8 ) , Ill is s:iri:uult o mf (AM il:i5 h~cm

130 miart’ 1310 inferil ill tilt’ seseu’o.’

i’t’fi’uc’rc ii’s

1335ustilo.’ilia

gl’usis

likeis acts to inc in sic issis to in be c If saicit’ ill uccite ullsastilo.’u3iu gi’usis.

11i011355 is iill1bo ini’t:uilt i13 tilis 5t’ttii30.~ t t’xc’l cud.’ c mtho.’r fo ini’1335 c mf lllso Ilbatils tiia c’:uil 0 ic’c’cur ill Sticil 11:itit’13t5, eg, tcIxm~

Critical Illness Myopathy illus ao.c’o Ituilt fo

putit’ilts ill 1(4

‘5

mc tluec’ici sseuki3t’ss cif

(Case 15 ). Scuch 133so in-

ilarilit’s illas c’iiilic’aiis aildi elt’d’ti’o 11inils— sic mic ingica I is l’t’5ei33i1io.’ (A P ( luico inillis er ul, 2h1P0: i~uc’c inullis t’t ul, 199Cr Ruillsas o.’r al , 1993: /0 intilo indille er al , 1 99~: ) . 1’omc tilis do mlldiitio 113, t13t’ diesigilatic Ill c’i’itic’ui iiillt’55 13150 inil:itils ( (A\I ) 5t’o.’1335 fll’efo.’u’able’, airilo incigil sest’i’ui o intilo.’r tt’rills ilu5t’ ilt’t’ll ciso.’ci, stidil :15 fib inilP} po.’u’so Ill 55lldi ‘0 inillt’, llt’d’i’c intizi ilg illso inilarils of

1(4 tilick fiiull3enr 11350 miluri3s, sto.’u’o inidi—illdidio.’t’ci reru’upit’gia. awl uccito.’ ciciuciu’ii’mit’gic’ 133sc input ils. Parit’llrs ssit 11 (AM i3:15t’ 1330 inst o mfrt’u3 ( aitilo indigil 130 int ulsyass ) (1 Ic inke o.’r al, I 999) l’t’c’t’iso.’d I 130 inildit’po Iiai’izillg illtisc’lt’—ilio mc’kiulg ugt’13r5, i3igil do inst’s c mf cc mu’tio.’c mstt’u’c micis, oinr lic intil if 1110 mc’kiulg agt’llts ilust’ ilt’o.’i3 gist’13. tilt’

,

a cc Ii3o.’ti ru’ei3r 111,0 mic inngt’ci i~io incLude o if tile

i3ecui’c mlllcusc’diiur

j dillo.’t 10113

illas

131:i5113:l go inlldiii 13350 ISitiS Oil’ gu’:itr—st’u’stis 30 inst 133S0 isit 15. Ill (A,’\I o.’io.’c’ti’c iIlil55i( micIgic’ hl3diil3t’ :uu’o.’ Ic in55—:iilliliitdicio.’ :uildi i11’oinloinllgcs (IMAPs, u’ei:itiseiv 111’esei’sedi SNAP (if t’dit’i3l:i is :ui15o.’ult ) 50 mine ( btit io.’s’ ,

,

po Isitise 511:1rpm ss:iso.’s, :uildi slll:iii :u133J11m truclo.’ 110 ini51113:15io.’ so Iidiilt:ul’5 1330 into Ii’ 0.1130

(Figure 24)

013

ill cuscie

bic mpss

so.’io.’c t us o.’ Ic mm cif dl ic’k 113So insill fii:uullcu’e’ is c’il:uu’:ic’to.’u’ist ic c mf (liNt. \Nhs’ scict

Seit’c’tisits 0 mc’c’cc u’s is 130 it c’io.’:ui’, :ui3dl nlas be t11:ur tilick tii:uul3eult iomss 15 13cm: t’iltil’o.’i5 51b.’c’itic’ Lii’ t ilis discini’cicinr Other ill3ibcmi’t:uilt ~l:iti3cinioingic’ f.’:itcii’c” iilc’i tic it’ tsilt’ II fiimt’r :lti’c 111135, illdlSdlc fiimo.’r i3t’c’l’0 insis, s:uc’dco mi:ito.’ci il3tu5dlc fiht’i’s, :iuldi l’o.’geilo.’u’:ltiulg 133 rusc’io.’ fihci’s ‘I’ll.’ c’il:iulgo.’s 0 mimso.’i’so.’ci ill :i L~i\o.’r

lb.’

ib:itio.’ult 131:15 dit’ilo.’11d1 0113 tilt’ tiillii3g 00 tilt’ ilic 11155. 113 11:itio.’13t5 55itil 5o.’5o.’tc after tilo.’ir clisc’c ini3tii3ciatio 113 ( set’ ‘‘Nt’cu- sst’:1k13t’ss, iligil (IlK io.’st’is, :iildi ril:uildicm i’0 inillcisc’ti Ia I’ I ci 13c’t i (Ill I)i 50 mu’c it’u’s ) . lii is il3Sc missis, fibt’r l3o.’c’V Isis 133:is ilu’edicm3i’ p~’o mllit’n3 :ulllleau’s ill ilatit’13t5 gist’u3 p~’c in— i13:itt’ :113d1 o mbsc’ci u’o.’ ti3ic’k fii:unl.’ulr icmss Ic inilgedi iilfdisiO 1135 o inf 5t’c’tui’o 113 in 133 0 ml’ ‘l’ilese eil;lulgo.’s l33:iv :iiso I ilt’ 1330 ini’t’ i3i’01133 ili’t’5eilt :11301 133:is Lust fo inc seseu’ui class

illt’ilt

cif i’eul:ii fail cuu’e (Figure 21 ) (INI ull:is

ill putit’llts

11o.’gi1313 iilg :idiul3ittedi tom i(4s. (~I~I il:itieults :uiso in

i’o.’co.’isillg fl~’0 inlo inilgoic

ii3fdisio IllS cif 5o.’c’tii’c inilituill, Ill lbutiellt’ tom

u’ec’o inse’u’,

i~ic I~1Ss

133:i\

idio.’iltifS ili’0 ini3liilo.’ilt fiimo.’c i’t’gt’l3t’i’:itic ill

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appi’cinach to

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(:1 M

~ Maher i, Grand’maison F, Nicolle MW, et al. Diagnostic difficulties in myasthenia gravis. Muscle Nerve 1 998;21 :577-583. Rcpctitixc ccinnclucticn stuclics cif thc phrcnic ncr~c.

~ McEvoy KM, Windebank Ai, Daube iR, et al. 3,4-Diaminopyridine in t~ treatment of Lambert-Eaton myasthenic syndrome. N EngI i Med 1989;321 1567—1571. ~ McKhann GM, Cornblath DR, Griffin iW, et al. Acute motor axonal neuropathy: a frequent cause of acute flaccid paralysis in China. Ann Neuri 1993;33:333—342. ~ Nicolle MW, Stewart Di, Remtulla H, et al. Lambert-Eaton myasthenic syndrome presenting with severe respiratory failure. Muscle Nerve 1996;19:1328—1333. ~ Plasma Exchange/Sandoglobulin Guillain-Barre Syndrome Trial Group. Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barre syndrome. Lancet 1997;349:225-231 lntraxcncini.us inuinuncglcinhulin ,1ildl plasiuu~u cxch.ungc cini siinilar cfficac~ in (~3S.

~ Plewnia C, Wallace C, Zochodne D. Traumatic sciatic neuropathy: a novi cause, local experience, and a review of the literature. i Trauma 1999;47: 986—991. ~ Ramsay DA, Zochodne DW, Robertson DM, et al. A syndrome of acute sever muscle necrosis in intensive care unit patients. i Neuropathol Exp Neurol 1 993;52:387 —398. Pathoinlogic snudics cinf CI M p~uticnts with prclmincnt nc’crcinsis.

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~ Ropper AH, Kehne SM. Guillain-Barre syndrome: management of respiratory failure. Neurology 1985;35:1662—1665. ~ Segredo V. CaIdwell iE, Matthay MA, et al. Persistent paralysis in critically ill patients after long-term administration of vecuronium. N EngI i Med I992;327:524— 528. Des-aco.’tvl metaholite cf ~‘ecuroinniumis asscciatcd with prclcngeol neurcinmuscular hlockaclc

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Verma P. Oger i. Treatment of acquired autoimmune myasthenia gravis: a topic review. Can i Neurol Sci 1992;19:360-375.

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Zochodne DW. Autonomic involvement in Guillain-Barre: a review. Muscle Nerve 1994;17(1O):1145—1155.

~ Zochodne DW, Bolton CF. Neuromuscular disorders of critical illness. Baillieres Clin Neurol 1996;5:645-671. ~ Zochodne DW, Bolton CF, Wells GA, et al. Critical illness polyneuropathy a complication of sepsis and multiple organ failure. Brain 1987;11O:819-842. (:Iiiuic~il, pathologic, and electrophvsiologic dlescriptioln of CAP.

~ Zochodne DW, Ramsay DA, Saly V. Acute necrotizing myopathy of intensive care: electrophysiological studies. Muscle Nerve 1994;17:285—292. I+leccrophvsiologic features of CIM with prcmiiuewut necrosis.