We present electrodiagnostic data on 30 patients with inclusion body myo-

sitis (IBM) in order to better delineate its electrophysiological features.

Comprehensive electromyography (EMG) and nerve conduction studies
(NCS) were performed in all cases. Twelve patients had single fiber electro-
myography (SFEMG). EMG showed abundant short-small motor unit poten-
tials (MUP) with fibrillations and positive sharp waves in 56.6% of patients,
and a mixed pattern of large and small MUP in 36.7%. In 6.7%, only "neu-
rogenic" features were seen. NCS were slow in 33.3%. SFEMG revealed a
mildly abnormal jitter and a slightly increased fiber density. IBM demon-
strates a heterogeneous EMG profile. A pattern of large and small MUP is
highly suggestive of IBM but is seen in only about one third of cases.
Key words: inclusion body myositis electrodiagnosis electromyography
single fiber electromyography nerve conduction studies
MUSCLE & NERVE 13:949-951 1990

ELECTROPHYSIOLOGICAL SPECTRUM
OF INCLUSION BODY MYOSlTlS
JUAN L. JOY, MD, SHIN J. OH, MD, and ALI 1. B A S A L , MD

Inclusion body myositis (IBM) is a recently
described inflammatory myopathy characterized
by rimmed vacuoles, filamentous inclusions,
slowly progressive proximal and/or distal weak-
ness, prominent muscle atrophy, high inci-
dence of hyporeflexia, male predominance, and
poor response to corticosteroids, which is being
reported with increasing fre uency in the last
several years.2,3,5-7,9- 11.13,20-%25 Previous re-
ports have focused on its clinicopathological
aspects.2-6~9~1 1,17,20,22
We present electrophysiological data on 30
IBM patients in order to better delineate the elec-
trodiagnostic features of this rare disorder.
PATIENTS AND METHODS
We studied 30 biopsy-proven cases of IBM: 23
males and 7 females (mean age = 59.7 years). All
had compatible clinical features as outlined above.
Studies were performed using a DISA 1500 elec-
tromyograph (Dantec Corporation, Santa Clara,
CA). Motor, sensory, and mixed nerve conduction
From the Department of Neurology, University of Alabama at Birming-
ham, Birmingham, Alabama.
Presented in part at the Annual Meeting of the American Association of
Electromyography and Electrodiagnosis, San Diego, California, October,
1988.
Address correspondence to Dr. Oh, Department of Neurology, University
of Alabama at Birmingham, UAB Station, Birmingham, AL 35294.
Accepted for publication November 5, 1989.
CCC 0148-639W90/0100949-03 $04.00
8 1990 John Wiley B Sons, Inc.
studies (NCS) were done following conventional
methods." Nerves tested included the median, ul-
nar, peroneal, posterior tibial, and sural. Skin
temperature was controlled at 2 31°C. Needle
electromyography (EMG) was performed using
monopolar electrodes. Weak muscles in at least
two limbs were examined. A minimum of 4 quad-
rants were explored in each muscle and at least 10
motor unit potentials (MUP) were measured on
the screen. MUP were considered to be of short-
duration/small-amplitude (SS) if their duration
was <6 msec and their amplitude <500 p.V. Long-
duratiodhigh-amplitude (LH) MUP were defined
as being >5 mV in amplitude and >18 msec in
duration.
Single fiber electromyography (SFEMG) of the
extensor digitorum communis (EDC) was ob-
tained in 1 1 patients, and of the rectus femoris in
one, following previously published techniques. l9
Jitter and blocking were electronically measured
using a DISA 15G22 single fiber unit. SFEMG
was considered abnormal if one of the following
was present: jitter >40.4 ksec (>60 psesc for the
rectus femoris), >lo% of potentials pairs show
blocking or a mean consecutive difference (MCD)
>54 ~ s e c . ' Fiber
~ density was considered in-
creased if >Z."
RESULTS
EMG was abnormal in all 30 cases. Three distinct
patterns (PTN) were observed:
PTN 1-fibrillation potentials/positive sharp
waves (F/PSW) and SS MUP in 17 cases (56.6%).

EMG in Inclusion Body Myositis MUSCLE & NERVE October 1990 949
 PTN 2-F/PSW and a mixed pattern of SS and
LH MUP in 11 (36.7%).
PTN 3-F/PSW, normal and LH MUP in 2
(6.7%).
In PTN 2 patients, SS and LH MUP were re-
corded from the same muscle in some, but in oth-
ers, certain muscles showed SS MUP while others
evidenced LH MUP. The interference pattern
(IP) at maximal voluntary effort was full in 58.8%
of PTN 1 patients and 36.4% of PTN 2 cases. A
reduced IP was seen in 41.2% of PTN 1 individu-
als, 63.6% of PTN 2, and in both PTN 3 subjects.
All 30 cases showed an excess of polyphasic MUP.
NCS showed mild slowing in 33.3% of cases.
When PTN 1 patients are excluded, the incidence
of NCS abnormalities rises to 43.3%. Only 2/17
PTN 1 patients showed slowing of conduction ve-
locity.
SFEMG (12 cases) demonstrated a mildly ab-
normal jitter in 58.3% (mean = 46.5 psec). Fiber
density (FD) was modestly increased (mean = 2.6).
Blocking was not prominent (2/12 2 10%). Over-
all, SFEMG was abnormal and FD increased in
83% of patients.
Table 1 summarizes the distribution of muscle
weakness according to EMG pattern. Involvement
of distal musculature was more frequent in PTN 2
and PTN 3 patients.
DISCUSSION
IBM reveals a heterogeneous electrodiagnos-
tic profile. Previous reports have described
a “myopathic” pattern with FIPSW, a mixed
pattern of “myopathic” and “neurogenic” fea-
tUreS,2-7,Y-11,13,20-22,25 or, rarely, patients with
only “neurogenic” changes.7217In our experience,
a mixed pattern of LH and SS MUP accompanied
by abnormal spontaneous activity (PTN 2) is
highly suggestive of IBM. Unfortunately, this pat-
tern is seen in only one third of cases, the most
common pattern being one of FIPSW and SS
MUP, similar to polymyositis.l 2 Some authors
have described LH MUP intermixed with SS MUP
(PTN 2) in chronic polymyositi~.’~~’~ Lozt and as-
sociates found a PTN 2 EMG to be nonspecific for
IBM because they saw this pattern with similar
frequency in patients with chronic polymy~sitis.’~
In our experience, this is extremely rare. So far,
almost every patient we have seen with a PTN 2
EMG has had IBM proven by biopsy. Other enti-
ties reported to show mixed “myopathic” and
“neurogenic” EMG features include X-linked hu-
meroperoneal neuromuscular disease,24 familial
“rimmed vacuole myopathy” sparing the
quadriceps,’ and sporadic distal myopathy.z3 In-
terestingly, there are strong similarities between
IBM, familial “rimmed vacuole m o athy”,’ and
several types of distal myopathy.R J 1 8 2 3 Some of
these cases might be, in fact, variants of IBM. Two
of our cases (PTN 3) showed pure “neurogenic”
features. Patients with this EMG pattern and nor-
mal NCS might erroneously be thought to have
motor neuron disease.
In our series, NCS abnormalities were not in-
frequent. In the cases with “neurogenic” features
(PTN 2 and PTN 3) these were more prevalent.
Thus, the presencse of unexplained peripheral
neuropathy in a patient with electrodiagnostic evi-
dence of myopathy should suggest the possibility
of IBM. Initial reports im lied that NCS abnor-
malities were rare in IBM~”5,6~”.1’.13.”0.22 but re-
cent series have reported up to a 50% incidence.“
Eisen and coworkers provided the only previ-
ous report of SFEMG changes in IBM.7 They re-
ported 7 cases with strikingly high fiber density
(mean = 6.3) and a moderately abnormal jitter
(mean = 83 psec). This led them to believe that
IBM had a substantial neurogenic c ~ m p o n e n t . ~
We found less striking SFEMG abnormalities. Our
values were in the range seen with chronic myo-
pathic proces~es.’~ All of Eisen and colleagues’ pa-
tients had prominent distal ~ e a k n e s sIn
. ~contrast,
most of our SFEMG patients had proximal in-
volvement. Since the SFEMG is usually performed
in the EDC (a distal muscle), it will be expected to

Table 1. Distribution of muscle weakness by EMG pattern

Distribution of weakness (no. of cases)

EMG pattern Mainly proximal Mainly distal Scapuloperoneal Proximal = distal Total patients

1 12 0 3 0 15
2 8 3 1 1 13
3 0 1 0 1 2
Totals 20 4 4 2 30
Percentage 66.7% 13.3% 13.3% 6 7%

950 EMG in Inclusion Body Myositis MUSCLE & NERVE October 1990
be more abnormal in patients with distal involve-
ment.
IBM possesses a rich electrophysiological spec-
trum. A mixed pattern of SS/LH MUP is very sug-
gestive, but not specific, of IBM. NCS abnormali-
ties seem to be more frequent than previously
thought. Many of the electrodiagnostic features in
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