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Non-pulmonary Critical Care: Managing Multisystem Critical Illness Care of the critically ill patient is truly multi-system management of highly complex patients who typically have numer

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Non-pulmonary Critical Care: Managing Multisystem Critical Illness

Care of the critically ill patient is truly

multi-system management of highly complex patients who

typically have numerous acute physiological

derange-ments superimposed upon underlying medical ailderange-ments

Historically, the majority of patients admitted to

inten-sive care units (particularly medical ICUs) have

respira-tory failure requiring mechanical ventilation, often along

with other acute and chronic pulmonary problems

Much ICU-related research and education has similarly

focused on pulmonary issues such as the acute respiratory

distress syndrome (ARDS), pneumonia, and mechanical

ventilation Additionally, a great majority of intensivists

have received training in pulmonary and critical care

medicine Thus it is not surprising that the phrase

‘‘non-pulmonary critical care’’ has arisen to address many

general critical care issues However, this vast body of

knowledge might more appropriately be considered

‘‘multisystem critical care’’ as an inclusive term that

encompasses the many important organ system

derange-ments that plague our ICU patients

This issue of Seminars provides scholarly and

clinically relevant reviews of major non–pulmonary

or-gan dysfunction in the ICU setting Clearly, a compre-hensive review of the numerous organ system–based medical conditions seen in our ICUs is well beyond the scope of a single issue of Seminars, more appropri-ately filling several thousand pages and hundreds of chapters typical of current major textbooks on critical care medicine Nevertheless, we have identified a cross-section of key topics and have solicited state-of-the-art reviews by highly qualified experts

Starting at the top, so to speak, Dr Bleck presents

a discussion of neurological complications of critical illness, with particular emphasis on metabolic encepha-lopathies, seizures, and neuromuscular conditions Drs

Miller and Ely share their current understanding of the rapidly evolving areas of delirium and cognitive dysfunc-tion in the ICU Drs Tarditi and Hollenberg present a structured approach to the ICU patient who has cardiac arrhythmias, emphasizing the challenges of managing tachyarrhythmias, whereas Dr Axler discusses the eval-uation and management of shock Drs Rinella and Sanyal provide a comprehensive review of acute hepatic failure as well as acute complications of chronic advanced

1

Division of Pulmonary and Critical Care Medicine, Virginia

Com-monwealth University Health System, Richmond, Virginia.

Address for correspondence and reprint requests: Curtis N Sessler,

M.D., Division of Pulmonary and Critical Care Medicine, Box 980050,

Virginia Commonwealth University Health System, Richmond, VA

23298-0050 E-mail: csessler@hsc.vcu.edu.

Non-pulmonary Critical Care: Managing Multisystem Critical Illness;

Guest Editor, Curtis N Sessler, M.D.

Semin Respir Crit Care Med 2006;27:199–200 Copyright # 2006

by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York,

NY 10001, USA Tel: +1(212) 584-4662.

DOI 10.1055/s-2006-945524 ISSN 1069-3424.

199

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liver disease Drs Weisbord and Palevsky examine acute

renal failure, including sections on epidemiology, causes,

prevention, and management

Drs Raghavan and Marik present an in-depth

review of adrenal insufficiency and the many issues

related to hyperglycemia and glycemic control in the

ICU Drs Mercer, Macik, and Williams expertly

ad-dress hematological disorders in the ICU, with particular

emphasis on thrombocytopenia and bleeding disorders

Drs Afessa and Peters provide a practical framework for

evaluating and managing the many serious infectious

and non-infectious complications related to

hemato-poietic stem cell transplantation Drs Bearman, Munro,

Sessler, and Wenzel conclude with a comprehensive

overview of infection control and prevention of nosoco-mial infections in the ICU, with emphasis on ventilator-associated pneumonia and catheter-related bloodstream infection

I would like to acknowledge the amazing exper-tise, attention to detail, and hard work of the authors of these articles, and convey my thanks for the steady hand

of the Seminars Editor-in-Chief, Joseph P Lynch, III, M.D., and the understanding and support of my wife and children, in creating this issue of Seminars

Curtis N Sessler, M.D

Guest Editor1

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Neurological Disorders in the Intensive Care

Unit

Thomas P Bleck, M.D., F.C.C.M.1,2,3

ABSTRACT

Neurological problems are common among critically ill patients; they often signal that other organs are failing, but are themselves important causes of morbidity and mortality Cognitive function may suffer as a consequence of septic encephalopathy, the pathophysiology of which is poorly understood; however, the affected patients usually return to their baseline when sepsis resolves Seizures and cerebrovascular disorders are also common in the intensive care unit Neuromuscular complications are important causes of failure to wean from mechanical ventilation and lead to substantial long-term morbidity

syndrome, critical illness myopathy, critical illness polyneuropathy, fulminant hepatic failure, seizure, septic encephalopathy

Many conditions encountered in intensive care

affect the nervous system The onset of an abrupt

neuro-logical complication is frequently obscured by the effects

of the primary illness (e.g., metabolic encephalopathy

may delay the recognition of an intracerebral

hemor-rhage) or its treatment (such as sedation to allow greater

synchrony with a ventilator) Other neural problems,

such as critical illness polyneuropathy, may develop

insidiously and become apparent only as the patient

improves At times the neurological problem has been

visible, but its manifestations may be inappropriately

attributed to the presenting illness.1 The intensivist

should be perspicacious about changes in level of

con-sciousness or movement when investigating a fall in

oxygen saturation or a rising white blood cell count.2

EPIDEMIOLOGY

Isensee et al evaluated neurological problems in 100

consecutive medical intensive care unit (ICU) patients

within 72 hours of admission.3 The study included all patients in need of intensive care except those with primarily cardiac disorders Eighteen were admitted for acute neurological disease and five others for encephal-opathy caused by drug overdose Of the remaining 67, 33% had a neurological complication of their medical conditions (11 metabolic encephalopathy, four hypoxic-ischemic encephalopathy [HIE], and seven other neuro-logical problems) Fifty-nine percent of the patients with neurological complications died, compared with 20% of the nonneurological patients

At the same time, we performed a 2-year pro-spective study of neurological complications among medical intensive care unit (MICU) patients to describe the neurological complications encountered and to iden-tify their effects on mortality and length of stay (LOS).4 Patients with a primarily neurological reason for admis-sion to the MICU were excluded from analyses of mortality and LOS; more than half of this group had ischemic stroke or intracranial hemorrhage The others

Departments of1Neurology,2Neurological Surgery,3Internal Medicine,

University of Virginia School of Medicine, Charlottesville, Virginia.

Address for correspondence and reprint requests: Thomas P Bleck,

M.D., University of Virginia School of Medicine, Neurology 800394,

McKim Hall 2025, Charlottesville, VA 22908-0394 E-mail: tbleck@

virginia.edu.

Non-pulmonary Critical Care: Managing Multisystem Critical Ill-ness; Guest Editor, Curtis N Sessler, M.D.

Semin Respir Crit Care Med 2006;27:201–209 Copyright # 2006

by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York,

NY 10001, USA Tel: +1(212) 584-4662.

DOI 10.1055/s-2006-945531 ISSN 1069-3424.

201

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were classified as having a complication of a critical

illness if they developed a neurological problem from a

medical disorder or its treatment We collapsed the

medical diagnoses into four categories: (1) sepsis,

in-cluding bacteremia with shock, the sepsis syndrome,

and the acute respiratory distress syndrome (ARDS);

(2) acute coronary artery disease; (3) other cardiac

disorders; and (4) all other patients (e.g., ventilatory

failure, gastrointestinal hemorrhage, hypotension not

assigned to another category) Patients with

neuro-logical complications were further divided into those

with metabolic encephalopathies, seizures,

cerebrovas-cular disorders, hypoxic-ischemic encephalopathy, or

other global brain disorders These latter categories

were not mutually exclusive Patients with clinically

apparent peripheral nervous system disorders were

in-cluded in the ‘‘other’’ group

We studied 1850 consecutively admitted patients;

of these, 92 (4.9%) were admitted for a primary

neuro-logical reason Of the remaining 1758 patients, 217

(12%) experienced neurological complications of their

underlying medical disease (Table 1) Table 2 details

the neurological complication rates by admission

cate-gory The mortality rate for all MICU patients was 32%,

but it was 55% for the 217 patients with neurological

complications, compared with 29% for those without

neurological complications Patients with neurological

complications also had significantly longer MICU and

hospital stays

Metabolic encephalopathy was the complication

most frequently encountered, seen in 62 patients Of

these, septic encephalopathy, without evidence for sig-nificant hepatic or renal dysfunction or hypoxemia, was most common The frequencies of different forms of metabolic encephalopathy are detailed in Table 3 Seiz-ures occurred in 61 patients, most often in patients with vascular lesions Hypoxic-ischemic encephalopathy oc-curred in 51 patients; in 27, the cause was primarily cardiac, with pulmonary disease accounting for the re-maining 24

Forty-eight patients suffered strokes while in the MICU Thirty-two of these were ischemic infarcts, 14 were intracerebral hemorrhages, and two were subar-achnoid hemorrhages Thirteen stroke patients had an identified cause other than arteriosclerosis, including underlying autoimmune diseases and bacterial endocar-ditis Stroke occurred in only 1% of patients with acute myocardial infarction This was less than the usually cited range of 1.7 to 2.4%.5,6

SEPSIS AND SEPTIC ENCEPHALOPATHY During the past 40 years, clinical analyses and investiga-tions of cytokine mechanisms have markedly improved our understanding of the causes and pathogenesis of sepsis.7Although bacteremia was previously considered

to be the sine qua non of systemic disease, occurring as a consequence of local infection, many patients suffer the same vasomotor disturbances and organ dysfunctions without positive blood cultures The foregoing epidemio-logical data indicate that septic encephalopathy is the most frequent neurological disorder encountered in

Table 1 Neurological Complications Encountered in 217

Patients at Risk with Severe Medical Illnesses in the

Medical Intensive Care Unit

Complication N (percent of patients

with diagnosis)*

Metabolic encephalopathy 62 (28.6)

Seizures 61 (28.1)

Hypoxic-ischemic encephalopathy 51 (23.5)

Stroke 48 (22.1)

Other diagnoses 50 (23.0)

*A single patient could have more than one complication; therefore,

the total number in this column exceeds the total number of patients.

Modified from Bleck et al 4

Table 2 Neurological Complication Rates by Primary Medical Intensive Care Unit Admission Category

Percent of Patients with Complications Category Seizure Vascular HIE Metabolic Other Sepsis 11 6 10 21 11 Other medical condition 4 3 4 3 6 Coronary artery disease 1 1 1 1 1 Other cardiac condition 4 3 3 2 4

4

Table 3 Etiologies of Metabolic Encephalopathy in a Medical Intensive Care Unit Population

Hypertensive 7 Hyperosmolar 4 Hypoglycemic 3

Modified from Sprung et al 12

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medical intensive care; it is also one of the more poorly

recognized and understood Septic encephalopathy was

described in 18278but has only recently become a subject

of organized neurological interest Young and coworkers

provided a thorough prospective analysis of this disorder

in a large university ICU.9This group required fever and

a positive blood culture for inclusion in their study, a very

restrictive definition of sepsis, which provided a

homo-geneous group for analysis Patients were excluded for

preexisting brain disease; frequent sedative or opiate

administration; pulmonary, hepatic, or renal failure;

en-docarditis; or long bone fractures that might have

pro-duced fat embolism

These workers identified 69 patients over 31

months; by clinical examination, 20 of them were not

encephalopathic (NE), 17 were mildly encephalopathic

(ME), and 32 were severely encephalopathic (SE)

Pa-tient age, blood pressure on entry into the study, and

temperature did not vary significantly among the groups

The lowest systolic and diastolic blood pressures were

statistically significantly lower (but probably not

biolog-ically significantly lower) in the ME and SE groups when

compared with the NE group Mortality depended on the

category of encephalopathy: none of the NE patients

died, whereas 35% of the ME and 53% of the SE patients

died Several laboratory values showed a linear

relation-ship with the severity of encephalopathy, including white

blood cell count, PaO2, blood urea nitrogen (BUN),

creatinine, bilirubin, alkaline phosphatase, and

potas-sium The serum albumin concentration was inversely

related to encephalopathy cerebrospinal fluid (CSF)

protein content was mildly elevated (60 to 85 mg/dL)

Electroencephalographic abnormalities are more sensitive

indicators of central nervous system (CNS) dysfunction

than the clinical examination, and also a powerful

pre-dictor of survival.10Evoked potential studies suggest that

brain dysfunction is even more prevalent in sepsis, being

abnormal in 84%.11 A Veterans Administration (VA)

cooperative sepsis study also showed that ‘‘alterations in

mental status are common in septic patients, and are

associated with significantly higher mortality.’’12

Eidelman and colleagues studied 50 patients with

severe sepsis and showed that encephalopathy was

asso-ciated with bacteremia and hepatic dysfunction.13 The

severity of encephalopathy correlated with mortality

PATHOLOGY AND PATHOPHYSIOLOGY

The pathological basis of septic encephalopathy remains

obscure Jackson et al autopsied 12 patients dying after

severe, prolonged sepsis.14 They found cerebral

micro-abscesses in eight patients and proliferation of astrocytes

and microglia in three others; these findings suggested

metastatic infection Three of these patients also had

central pontine myelinolysis, and three had ischemic

strokes The remaining patient demonstrated only

pur-puric lesions Eight of the patients had electroencepha-lograms (EEGs), three of which showed multifocal epileptiform activity Pendlebury and associates identi-fied 35 patients with multiple CNS microabscesses among 2107 consecutive autopsies.15All these patients had chronic, usually immunocompromising, diseases, and were frequently septic before death The most common organisms implicated were Staphylococcus aureus and Candida albicans In contrast, we did not find microabscesses in four patients autopsied of our 14 fatal septic encephalopathy cases; this may represent sampling error or other differences in the populations studied.4 The pathophysiology of septic encephalopathy is

of great interest The systemic mediators of inflamma-tion are capable of damaging the blood–brain barrier.16 Such disruption has been documented in an animal model early in sepsis.17 The behavioral effects of cyto-kines vary with the neuroanatomical structures affected but include thermogenic behaviors in the hypothala-mus18 and somnolence in the locus caeruleus.19 Inter-ferons also alter individual cortical and hippocampal neuronal functions, suggesting a myriad of effects on memory and emotion.20 Brain catecholamine concen-trations are decreased in experimental sepsis.21 Sepsis leads to the release of S-100B, a protein predominantly expressed in CNS glial cells, into the systemic circula-tion; this leakage was not affected by steroids in a human trial.22 Increased serum concentrations of S-100B are usually viewed as evidence of cellular damage, which may not be reparable

Cerebral blood flow (CBF) and cerebral oxygen extraction decrease in septic encephalopathy,23 along with the development of cerebral edema and disruption

of the blood–brain barrier.24Preliminary human studies suggest that these problems occur in several gray matter structures of the brain.25,26 Failure of cerebrovascular autoregulation is likely to compound these disorders,27 producing cerebral ischemia Both cerebral edema and blood–brain barrier disruption appear to correlate with damage to astrocytic foot-processes.28,29 Aquaporin-4 expression increases in septic encephalopathy, but the cellular events that trigger this change in sepsis require further research.30

The cause of the changes in CBF and oxygen extraction are less well understood Focal elevations in intracellular free calcium may cause neuronal dysfunc-tion and also contribute to apoptotic31 or necrotic cell loss.32 Activation of adenosine A1 receptors may be important in the development of a local CNS inflam-matory response.33Although cytokines have been sug-gested as mediators, a study of the effects of tumor necrosis factor was unable to confirm its role in this regard.34 However, a decline in CSF ascorbic acid concentration may reflect difficulty in safely handling the oxygen-derived free radicals potentially resulting from both cytokine and nitric oxide excess.35 These

NEUROLOGICAL DISORDERSIN THEICU/BLECK 203

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radicals may interfere with the mitochondrial electron

transport chain, leading to cellular energy deprivation.36

Magnesium administration may be able to attenuate

some of these problems,37 although this remains to be

demonstrated in humans

Abnormal systemic metabolism in sepsis may also

contribute to CNS dysfunction Mizock et al

demon-strated altered phenylalanine metabolism (elevated blood

and CSF levels, and elevated phenylalanine metabolites)

in 11 patients with septic encephalopathy; in contrast,

patients with hepatic encephalopathy had elevated CSF

concentrations of many other aromatic amino acids.38

Sprung and coworkers found elevated serum levels of

phenylalanine, ammonia, and tryptophan in

encephalo-pathic patients compared with infected patients with

normal sensoria, along with lower concentrations of

isoleucine.39 Other studies have confirmed this, and

find it linked to concentrations of calcitonin precursors

and interleukin-6.40The significance of these correlative

studies for the pathogenesis of encephalopathy is

un-certain Several authors have tried to implicate abnormal

hepatic and muscular metabolism of aromatic amino

acids in both hepatic and septic encephalopathies, but

this hypothesis has been challenged (see further

discus-sion in the next section) Antibiotic treatment of septic

patients may actually enhance these problems.41

Septic patients are prey to a wide variety of other

metabolic disorders and intoxications that cause

ence-phalopathy apart from the direct effects of sepsis on the

brain and on cerebral blood flow.42Nonconvulsive status

epilepticus is an underrecognized problem in the

pop-ulation at risk.43 There is neither a diagnostic test to

discriminate sepsis from other causes of encephalopathy

nor a specific treatment for the CNS disturbance From a

clinical standpoint, the diagnosis of septic

encephalop-athy remains one of exclusion.44

Hepatic Failure and the Central Nervous System

The current debate over the pathogenesis of hepatic

encephalopathy is of great importance to clinical

neuro-scientists In fulminant hepatic failure (FHF), increased

intracranial pressure (ICP) has become a major cause of

death in patients awaiting transplantation.45 Patients

whose ICP has been elevated may survive a transplant

but be left with CNS deficits.46

The mechanism of the cerebral edema that

devel-ops in FHF appears to be related to mitochondrial

dysfunction47; it requires aggressive treatment.48

Ste-roids are not effective, but mannitol has been useful.49

As in many other causes of brain edema, abnormal

function of matrix metalloproteinases probably

contrib-utes to the problem.50As with most other causes of brain

edema, aquaporin-4 expression is involved; it may be

possible to decrease this by the administration of

calci-neurin antagonists.51

Hyperventilation had previously been thought to increase mortality, but a controlled trial demonstrated its utility.52High-dose barbiturates may be used if mannitol and hyperventilation fail to control ICP.53The effect of posture is unpredictable,54 and computed tomographic (CT) scanning is debated as an indicator of the severity

of intracranial hypertension.55,56Attempts to lower ICP

by reducing extracellular volume through hemofiltration have not been effective.57Dialysis against albumin may hold more promise.58There is no current substitute for invasive ICP monitoring in patients with FHF who are

in grade 3 (stuporous) or grade 4 (comatose) The need for monitoring extends through the liver transplant operation into at least the first postoperative day.59 Recombinant factor VII administration immediately before ICP monitor placement appears to reduce the risk of intracerebral hemorrhage.60

Patients with FHF may also have elevated levels

of apparently endogenous 1,4-benzodiazepines,(3) which may explain stupor or coma in patients who do not have ICP elevations An interaction between gamma-amino butyric acid (GABA) and elevated con-centrations of ammonia may be important in both FHF and more chronic forms of hepatic encephalopathy.61 More recent work has concentrated on the putative role

of neurosteroid agonists of the benzodiazepine recep-tor62because researchers have been unable to identify a compound that structurally resembles the pharmaceut-ical benzodiazepine nucleus

Chronic hepatic encephalopathy does not appear

to cause ICP elevation unless intracranial bleeding supervenes As in FHF, endogenous benzodiazepine-like compounds (GABAA agonists) likely play a major role,(4) and the use of GABA antagonists is being actively investigated but has not yet become standard practice About 70% of chronic hepatic encephalopathy patients awaken rapidly when given flumazenil (for the duration of the drug’s effect).63,64 These patients have numerous other metabolic abnormalities that may con-tribute to their encephalopathy, including abnormalities

in the Krebs cycle65and of methionine metabolism.66

OTHER CAUSES OF ENCEPHALOPATHY IN THE INTENSIVE CARE UNIT SETTING Renal and hypoxic encephalopathies are quite common

in the ICU environment Details of their diagnosis and management are beyond the scope of this article, and have been reviewed.67

Iatrogenic causes of encephalopathy are also com-mon in ICUs and should be actively excluded Hypno-sedative drugs and narcotic analgesics are the most common agents in this category Flumazenil and nalox-one will reverse these drug-induced encephalopathies The need to reverse the effects of these drugs in an individual ICU patient should be carefully assessed

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because the emergence of agitation or severe pain may be

detrimental to the patient If these drugs are used to

determine whether encephalopathy is due to

medica-tions, doses much smaller than those to treat respiratory

depression should be used initially Flumazenil (0.1 to

0.2 mg intravenously over 15 seconds), may be given

every 60 seconds to a maximum of 1.0 mg The risk of

seizures is always present when this drug is

adminis-tered.68 Naloxone, 0.04 to 0.08 mg intravenously, may

be given every 60 seconds to a total dose of 0.8 mg

Barbiturates are not antagonized by available agents It

should be apparent from the preceding section on

hepatic encephalopathy that a response to flumazenil is

not specific for a benzodiazepine overdose

Other encephalopathic conditions may arise

dur-ing the course of ICU treatment One of our poorly

nourished patients developed Wernicke’s

encephalop-athy from the dextrose administered as the vehicle for a

lidocaine infusion to treat ventricular arrhythmias after

an acute myocardial infarction The clinician must

al-ways be aware that admission to the ICU does not

prevent the development of intercurrent illnesses

Seizures in the Intensive Care Unit

In our epidemiological study, 34 patients had simple

partial seizures (with or without secondary

generaliza-tion), and six had complex partial seizures (with or

without secondary generalization).4 Twenty patients

had seizures that appeared to be generalized at onset,

and six patients developed status epilepticus in the

MICU; all required at least two agents to terminate

their status (usually a benzodiazepine and phenytoin)

Two of these patients developed refractory status

epi-lepticus and were treated with pentobarbital coma

Three patients were admitted to the MICU for

refrac-tory hypotension after receiving phenytoin infusions at

rates between 25 and 50 mg/min The blood pressures of

these patients did not improve with fluid resuscitation

alone; all required dopamine infusions for several hours

to maintain blood pressure and systemic perfusion

In contrast to our experience in other hospitalized

patients, the focal onset of partial seizures with

secon-dary generalization was usually noted and adequately

described This was a useful guide to management

because patients with partial seizures generally

experi-enced seizure recurrence and thus probably benefited

from anticonvulsant treatment.69

The diagnosis and management of seizures

should be pursued differently in ICU patients than in

other patients We had hoped to develop rules to predict

which patients having seizures in the ICU might be

evaluated without imaging procedures, but were unable

to do so because most patients had vascular or infectious

causes for their seizures All patients experiencing a

single seizure in the ICU were treated with some form

of anticonvulsant therapy; this was often justified by the argument that their underlying medical condition might

be adversely affected by subsequent seizures We believe that this constitutes excessively aggressive management and that it postpones an appropriate search for etiology

For example, all three patients with recurrent seizures caused by nonketotic hyperglycemia were treated with at least two anticonvulsants; these drugs are known to be ineffective in this condition.70

When ICU patients do require treatment, phe-nytoin remains a reasonable first choice However, a second agent, usually phenobarbital, was required in most patients Lorazepam was useful for the suppression

of breakthrough seizures, but the use of this drug was often continued without adequate attention to optimal use of phenytoin or phenobarbital

Pentobarbital coma is often considered the treat-ment of choice for refractory status epilepticus in ICU patients We have adopted the use of high-dose mid-azolam in this circumstance.71This method appears to

be more rapidly effective and to have substantially fewer and less severe adverse effects than pentobarbital, thio-pental, or propofol.72

NEUROMUSCULAR COMPLICATIONS

OF SEPSIS Although recognized by earlier authors such as Osler, the modern era of interest in this problem began with the independent reports of three groups reported by Rivner

et al (four patients),73Bolton et al (17 patients),74and Roelofs (four patients).75Bolton’s group followed their initial description with a series of papers that character-ized the clinical,76electrophysiological,77and patholog-ical aspects of critpatholog-ical illness polyneuropathy.78Although many other groups have made significant contributions

to this area (e.g., Op de Coul et al),75the work of Bolton and his colleagues is in great measure responsible for the recognition of this problem among intensivists More recent work continues to show a high incidence of this condition.79

These neuromuscular complications are presently categorized anatomically Critical illness polyneuropathy

is an axonal disorder affecting both sensory and motor nerves.78Electrophysiological evidence of this condition,

as already noted, is present in 70% of septic patients, but the percentage with weakness sufficient to impede ventilator weaning or ambulation is less The phrenic nerves are typically the most severely involved Neuro-muscular junction (NMJ) dysfunction in the setting of critical illness is typically a consequence of a prolonged effect of NMJ blocking agents, usually because of im-paired clearance.80Myopathy in critically ill patients is most commonly seen in those who have received NMJ blocking agents and corticosteroids in the treatment of severe asthma.81 Although this has been reported most

NEUROLOGICAL DISORDERSIN THEICU/BLECK 205

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commonly after vecuronium use, it also occurs after NMJ

blocking agents that do not depend on renal or hepatic

excretion (e.g., atracurium).82Myopathy may also occur

in the setting of some viral infections, such as influenza

A syndrome of disseminated pyogenic myopathy has also

been described,83 presumably as a consequence of

bac-teremic seeding of muscles

Critical illness polyneuropathy has also been

noted after organ transplantation without sepsis.84

Although most of the patients reported to have critical

illness polyneuropathy have been adults, this condition

has been recognized with increasing frequency in

chil-dren as well.85

In contrast to the body of experimental work on

septic encephalopathy, very little is understood about the

pathogenesis of the neuromuscular complications of

sepsis.86Hyperglycemia correlates with the development

of critical illness polyneuropathy, but these may be

independent markers of disease severity However, the

dramatic reduction in critical illness polyneuropathy

with intensive control of blood glucose in both medical87

and surgical88ICU patients points to an important role

of hyperglycemia in the genesis of this condition, as well

as in many other neurological problems seen in critical

care units.89The neuronal microenvironment is similar

to that of the extracellular space of the brain; the same

alterations that produce septic encephalopathy may be

responsible for critical illness polyneuropathy, but

fur-ther investigation is clearly needed Critical illness

myo-pathy may result from the functional denervation

induced by NMJ blockade; it does not appear to be

simply a severe form of steroid myopathy

Diagnosis, Differential Diagnosis, and Prognosis

Recognition of a neuromuscular complication of sepsis

generally occurs as the patient begins to recover from the

critical illness that first required ventilatory support

Critical illness polyneuropathy is usually suspected

when the patient’s pulmonary mechanics (e.g., static

compliance) and gas exchange suggest that weaning

should be possible, but the patient is too weak to tolerate

it.90There is commonly some evidence of distal

weak-ness on examination Tendon reflexes are often absent or

diminished, but critical illness polyneuropathy may be

present without alteration in reflexes.91Gorson provides

an excellent framework for the evaluation of patients

with these problems.92

The diagnosis depends on electrophysiological

studies, which demonstrate an axonal disorder

Electro-myographic studies of the diaphragm confirm the

pres-ence of denervation.93 The differential diagnosis is

usually limited to consideration of the axonal form of

the Guillain-Barre´ syndrome; this condition usually

causes much more severe generalized weakness than

critical illness polyneuropathy, and typically causes an

increased cerebrospinal fluid protein concentration An-tecedent infection with Campylobacter jejeuni is fre-quently in patients with the axonal form of Guillain-Barre´.94Other differential diagnostic concerns are bot-ulism, which impairs presynaptic acetylcholine release, and myasthenia gravis, in which the motor end plate is damaged These conditions have characteristic nerve conduction and electromyographic characteristics No specific treatment for critical illness polyneuropathy is available Anecdotal evidence does not favor the use of plasma exchange or intravenous immunoglobulin, but there have been no definitive trials Almost all patients recover eventually, but this may require 6 months or more of ventilatory support

The prolonged effect of NMJ blocking agents can

be suspected by lack of tendon reflexes and inability to produce muscle contraction with a bedside neuromus-cular stimulator If the diagnosis is in question, it can be confirmed by formal nerve conduction studies and elec-tromyography There is no specific treatment, but the condition will resolve when the agent in question has cleared

Critical illness myopathy is often accompanied by substantial elevation of the serum creatine kinase (CK) concentration, which serves to distinguish this condition from steroid myopathy, in which the CK is usually normal Electromyography is usually an adequate diag-nostic study, and muscle biopsy is only rarely required.95 Again, no specific treatment is available, but the con-dition will resolve; whether it will resolve faster if systemic steroids are reduced or discontinued is uncer-tain A more recently described syndrome of acute quadriplegic myopathy is also probably associated with steroids; in this condition, muscle is electrically inexcit-able even with direct stimulation.96Nerves are histolog-ically normal, but muscles show thick filament loss.97 Although no treatment is known, the prognosis for this condition is for more rapid recovery than that for critical illness polyneuropathy

There are many other neurological problems that may arise during the course of an ICU stay which may impede weaning from mechanical ventilation Kelly and Matthay prospectively studied 66 consecutive adult pa-tients requiring mechanical ventilation for more than

48 hours to determine the reasons for their ventilatory problems.95 Neurological problems, primarily encepha-lopathies, were held responsible for the continuing need for ventilatory support in 32% of the patients, and contributed to this problem in another 41% Although this study was not directed at patients who failed to wean after resolution of their presenting disease, it does high-light the role of neurological problems early in critical illness Spitzer and colleagues studied 21 patients who failed to wean after their presenting disease had im-proved to the point that their intensivists believed that mechanical ventilation should no longer have been

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necessary.98 Thirteen (62%) of these patients had a

neuromuscular disorder that was either the major cause

of or contributed substantially to their ventilatory

prob-lems Only seven of these 13 patients had critical illness

polyneuropathy; other neuropathic conditions and

un-suspected motor neuron disease were also uncovered

Most intensivists will also be familiar with the less

common acute myopathies which may complicate

crit-ical illnesses, especially in patients who have received

neuromuscular junction blockade.99

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