Beruflich Dokumente
Kultur Dokumente
19 (2008) 97–110
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98 GOODMAN & BOON
Fig. 1. Demonstration of prolonged CMAP duration in critical illness myopathy. Tibial motor
nerve conduction study in a patient with critical illness myopathy (1A) compared with normal
control (1B). Sweep speed 5 ms/division in both studies. The compound muscle action potential
duration in 1A is over twice the duration in 1 B, but there is no decomposition of the wave form
or dispersion between proximal and distal sites of stimulation.
Electrodiagnostic features
Thoughtful electrodiagnostic testing is important in the evaluation of sus-
pected critical illness polyneuropathy. Upper and lower limb motor and sen-
sory NCS are performed. In patients who have suspected respiratory muscle
failure, phrenic NCS are also performed. RNS is normal in critical illness
polyneuropathy, and is helpful to exclude a pre-existing neuromuscular
junction disorder or weakness caused by transient neuromuscular blockade
following administration of a neuromuscular junction blocking agent. Be-
cause the majority of patients are too weak to exercise, brief, high-frequency
repetitive stimulation (at 20 or 50 Hz x 1–2 seconds) should be performed to
evaluate for significant facilitation of the compound muscle action potential
amplitude. This is particularly important if low-amplitude compound mus-
cle action potentials (CMAP) are present with routine NCS, typically seen in
the Lambert-Eaton myasthenic syndrome. Motor CMAP amplitudes and
sensory nerve action potential (SNAP) amplitudes are typically reduced,
with normal or near normal conduction velocities and distal latencies. De-
cline in the CMAP amplitudes occurs early in the course of the illness,
and may be followed by subsequent decline in the SNAP amplitudes later
[9,18]. Significant conduction velocity slowing or distal latency prolongation
and features of temporal dispersion or conduction block should suggest an
alternative diagnosis, such as Guillain-Barre syndrome.
Needle EMG typically shows abnormal spontaneous activity in distal
muscles, and may show reduced recruitment of motor unit potentials. The
presence of small motor unit potentials on needle EMG should alert the
electromyographer to the possibility of a concomitant myopathy, particu-
larly if the SNAP amplitudes are normal or near-normal. Single-fiber
EMG studies in nine patients who had critical illness polyneuropathy
showed increased jitter, suggesting a disorder of nerve terminals in these pa-
tients [19]; however, assessment of motor unit potentials can be difficult in
the encephalopathic patient, who may be unable to activate motor unit po-
tentials. In a patient who has low-amplitude CMAPs and preserved SNAPs,
and who is unable to activate motor unit potentials, it may not be possible
with routine electrodiagnostic studies to determine whether the patient has
a myopathy, polyneuropathy, or both. Prolonged CMAP duration and
102 GOODMAN & BOON
Electrodiagnostic features
NCS in patients who have critical illness myopathy typically show low
amplitude CMAPs, with preserved conduction velocities and distal laten-
cies. The authors and others have observed prolongation of CMAP duration
to be a specific feature of critical illness myopathy (see Fig. 1) [2,23,24].
CRITICAL ILLNESS NEUROMYOPATHY 103
From Lacomis D, Zochodne DW, Bird SJ. Critical illness myopathy. Muscle
Nerve 2000;23(12):1787; with permission. Copyright Ó 2000, Wiley Periodicals,
Inc., A Wiley Company.
CMAP duration may be normal or only mildly prolonged if NCS are per-
formed early in the patient’s illness, but appear to become more prolonged
later in the patient’s course, and subsequently shorten with recovery. SNAP
amplitudes are typically preserved; however, low SNAP amplitudes can be
seen in patients who have concomitant critical illness polyneuropathy or
preexisting disease such as diabetic neuropathy, or can be decreased because
of technical factors such as limb edema. RNS studies are normal in critical
illness myopathy.
Needle EMG in critical illness myopathy frequently shows abnormal spon-
taneous activity, including fibrillation potentials and positive sharp waves.
With voluntary activation, rapid recruitment of small motor unit potentials
is typical. As discussed previously, some patients, because of encephalopathy,
sedation, or profound muscle weakness, are unable to voluntarily activate
motor unit potentials. In these patients, determining whether a patient has
critical illness myopathy or neuropathy can be difficult. In such patients,
the presence of reduced CMAP amplitudes, prolonged CMAP durations,
and preserved SNAPs are most suggestive of critical illness myopathy.
The technique of direct muscle stimulation can be of benefit in distin-
guishing critical illness polyneuropathy from critical illness myopathy.
104 GOODMAN & BOON
Using this technique, it has been demonstrated that muscle in critical illness
myopathy is electrically inexcitable, whereas in critical illness polyneurop-
athy a large potential can be elicited with direct muscle stimulation
[25,26]. This is hypothesized to result from abnormal sodium channel inac-
tivation in critical illness myopathy [27]. Similar pathophysiology likely ex-
plains the finding of CMAP duration in critical illness myopathy.
Fig. 2. (A) Trichrome stained section showing many small fibers among few of normal size. The
small fibers have different staining properties and contain fine granular material. (B) Myosin AT-
Pase (pH4.3) reacted section in which type 1 fibers should stain dark and the other fibers more
faintly. Several reactive fibers show irregularly circumscribed decreases of reactivity and all
remaining fibers fail to react. (C) Electron micrograph showing disarrayed myofibrils devoid
of thick filaments that consist only of thin filaments emanating from Z disks (Z). Dislocated
sarcotubular profiles and mitochondria are scattered in the field. (Courtesy of AG Engel, MD,
Rochester, MN.)
CRITICAL ILLNESS NEUROMYOPATHY 105
Summary
Critical illness polyneuropathy and myopathy are frequent complications
in critically ill patients who have sepsis or SIRS. Patients are typically diag-
nosed with these disorders when patients fail to wean off of ventilatory sup-
port (despite adequate cardiopulmonary status), or when severe limb
weakness is noted during or following recovery from critical illness.
108 GOODMAN & BOON
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