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of Progressive
BY
An
PAUL
Instructional
The
the
basic
implicates
the
etiology
of muscular
are
years
families
to lead
a more
They
fiber
system
as the
and
various
normal
it
is
life arid
rso reliable
convinicinsg
weakness.
of pharmacologic
muscular
help
maximum
The
treatnssenst
disease.
with
to
by
myopathies
of muscle
of the
possible
attains
and
no form
characterized
as primary
cause
patients
Surgeons
disorders
itself
forms
managing
that
of Orthopaedic
are defined
muscle
of the
indicated
Academy
nervous
in
OHIO
of hereditary
is unknown,
in any
has
American
a group
Dystrophy
CLEVELAND,
is in the
dystrophy
experience
thirteens
M.D.*,
of muscle.
central
effective
However,
The
wasting
involvement
evidence
is considered
and
JR.,
Lecture,
dystrophies
weakness
because
VIGNOS,
Course
muscular
progressive
J.
Muscular
dystrophy
these
patients
function
for
amid
corssistenst
their
with
the
severity
of the disease.
Ambulation,
imsdepersdently
arid in braces
during
the later
stages
of the disease,
can be prolonged
in the childhood
Duchenne
type of dystrophy.
Joint
conitractures
occur
early
in the disease,
especially
ins Ducheninse
dystrophy
They
may
joints.
arid
be minimized
An
the
ercise
important
family
on
treatment
that
common
no single,
Therefore,
of
the
to the
to
use of any
dystrophy
varies,
The
diagnosis
and
stages,
for
the
drug.
the
with
a prognosis,
on
future.
It seems
diagnostic
abnormality
likely
in the
will
various
be
types
vary.
may
be difficult,
particularly
in the
the
patient
consulted
a number
that
of unrelated
cx-
supportive
increase
ins knowledge
of
it is possible
that
specific
be found
proper
will
diagnosis
the
patienst
of diseases
make
foreseeable
biochemical
dystrophy
to
the
of
physical
anid
a group
order
will
in the
treatment
finding
correct
a number
patient
If the
of muscular
before
in the
of
arid
counseling
In
in
abnormality
specific
it is a frequent
physicians
treated
new
proper
stretchinsg
counseling
schooling,
diagnosis.
be available
place
the
to give
counseling
depend.
With
in muscular
dystrophy,
may
ability
living,
is essential
biochemical
passive
is the
daily
accurate
diagnosis
planning
and
abnormalities
pharmacologic
and
of prophylactic
physician
of
ability
early
correct
which
realistic
the biochemical
activities
physicianss
depends
causes,
unknowns
institutions
of the
the
The
management
early
responsibility
regarding
programs.
basic
by
is made.
conditions,
has
The
patient
such
as
foot
often
vill
have
problems
early
of
beers
or slow
de-
velopment.
The
proper
approach
to diagnosis
in patients
with
muscle
weakness
is the
systematic
performance
of a profile
of tests and procedures
done in proper
sequensce.
The commonly
occurring
types
of muscular
dystrophy
are relatively
limited,
but
the
diseases
fails
with
to follow
necessary
early
dystrophy
Muscle
1212
sequence
Associate
Clinsic,
to
be
and
aided
University
at
be confused
of
subsequent
Medicine,
Hospitals,
are
numerous.
of steps in diagnosis
the correct
diagnosis.
considerably
and laboratory
clinical
Professor
may
arrive
diagnosis
can
of simple
they
proper
procedures
The
profile
which
the
or fails
management
The
of patients
by the routine
performance
tests which
have been found
Western
Cleveland,
Reserve
physicians
to perform
University
School
often
all of the
with
muscular
of the following
to be most useof
Medicine,
and
Ohio.
THE
JOURNAL
OF
BONE
AND
JOINT
SURGERY
P.
ful:
niansual
(1)
cneatinie
The
entire
for
battery
At
determinations
sificati(Ins
merst
(If
serial
(luanstitative
PhYsical
activity
A useful
iatiemits
patient
of the
poliomyelitis,
j)erhaj)s
the
first
skeletal
guide
nseuropathic
hereditary
by
rieuropathic
there
diseases
proxinial
or distal
from
tends
exceptions
to
cases
One
reflexes
neurogensic
of
to
must
this
be cautious
The
terminations
seruns
cates
a primary
suit.
of active
both
phenomena.
in
nieurogenic
suspected
diagnosis.
Unfortunately,
myopathic
sisterstly
abnormal
always
Duchennse
Duchenmie
dystrophy
VOL.
49-A,
as
NO.
6,
serum
types
enzyme
enzymes
of
content
irs only
aldola.se
dystrophy.
progresses,
1967
muscle
in
but
the
the
degree
amount
serum
slightly
the
de-
aldolase
less
reliable.
probably
muscle
mdi-
fiber
as a re-
permeability,
or
creatimse
kinsase
serum
enzyme
conicenitrationi
suggests
always
of
of
ins
primary
normal
an
distinguish
because
types
are
serum
weakness
all
that
enzyme
myopathy.
elevation
of muscle
are
incorrect
is msot a consThe
enzymes
the severe
boys with
decreases
mass
riot
between
there
arid
not
they
serum
are
weakness
will
as
membrane
arid
muscle
by
signs
in distinguishing
reliable
from
An elevated
neurogensic
have
rigidly
tracts.
above
in muscle
aldolase
atrophy.
elevated
The serum
disease
SEPTEMBER
increase
serum
muscular
muscular
the
enzyme
of having
nseurogensic
serum
abnormally
variety.
are
early
amid
of the
too
increased
valuable
escape
experience,
the
also
enzymes
degeneration,
never
disorders,
serum
group
may
rare in myopathy.
Testinig
of
reduction
or absence
of deep
most
of muscle
are
comigensita
is a helpful
is helpful
The
of these
with
Ins our
are
to
dystrophy.
diseases
corticospirsal
enzymes
atrophy.
transamimsases
myopathy
in a patient
the
kinase;
muscle
serum
diagnosis
concentration
categorizing
reflexes
contrast
which
of fa.sciculations
of the
muscle
differential
creatimie
of the
elevated
of the
mseurogensic
in the
; the
indication
involvement.
in
amyotonia
differens-
first
of muscular
poliomyelitis,
imivolvememst
determination
from
about
presence
as the
amid
The
weakness
types
of all
to the
of muscle
distal
such
of dystrophy
strength
is basic
or
weaknerves.
muscular
forms
of the
pattern
various
atrophy
peromieal
weakmsess.
the
show
rule,
spinal
imivolvensent.
weakness
niyopathy
amid
a specified
has muscle
peripheral
various
test
muscle
of the
disease
of the nervous
system,
superficial
or stretch
reflexes
nsay
Elevation
for
patients
amid is of as-
perforns
as
Testing
is often
suggests
The
clas-
measure-
of the
to
such
muscle
nsyopathic
weakness
disease
some
weakness.
tendons
amid (6)
is invaluable
disease
or the
ins the
manual
characteristic
are
amid
disease,
diagnosis.
a complete
of muscle
involvement
proximal
the
out:
but
follow-up
are
carried
The
for
motor
system
category.
involvement
differential
in
disease
cause
Irequenitly
However,
excretions
ability
with mseurogensic
censtral
nervous
and
muscles
of a mieurogenic
proximal
is essential
the
results
be
Classification
Fre
that
capacity.
diagmiosis,
patients
tests.
test
should
creatinse
to
other
is essential
of the
locomotor
little
the
are included
in the second
specific
patterning
of muscle
of
implications
biopsy.
of ann ap-
age.
The
of involvement
because
principal
of
relates
it
aldolase,
muscle
selections
of the
but
progression.
capacity
it
results
urimsary
adds
of disease
disease.
Neuropathy,
tUitiOn
funictionsal
(4)
the
procedures
hour
loconsotor
the
additional
emizynses
amid
because
all the
quantitative
since
seruns
is performed,
1213
n)YsTR0PHY
a nseuropathic
atrophy,
The
on
tests
excretions
diagnosis,
to
depends
two
the
performed
amid that
assessment
fumsctionsal
irs
of
twenty-four
creatinse
sistansce
MUSCULAR
electromyography,
test
these
time,
patients
urinary
(irranstitative
ness
same
of the
of the
last
ofters
of
be used
the
(3)
be the
niore
PROGRESSIVE
determination
both,
biopsy
or
of tests
appreciated.
(2)
or
one
OF
test,
should
muscle
only
(luenitly,
is
muscle
biopsy
l)rupriate
the
1)1AGNOSIS
phosphokinsase,
muscle
(5)
vIGNOS:
J.
available
ins this
for
1214
A.A.O.S.
releasing
enszyme
insto
abnormal
except
ins
dystrophy
ins the
The
aldolase
serum
the
seruns
States
dystrophy.
The
only
amid ultimately
The
the
20
conimons
Duchenne
per
cent
form
aldolase
of the
serunsi
commons
SO per
of
those
of primary
was elevated
patients
with
aldolase
forms
arsd
ins approximately
ins only
other
the
other
facioscapulohumeral
initially
but
the serum
ins one-half
experience,
are
LECTURE
However,
disease.
is elevated
dystrophy
COURSE
is diminished.
end-stage
United
linib-girdle
our
INSTRUCTIONAL
muscular
limb-girdle
cent
with
renssainss
of
types.
of patients
myopathy
is polymyositis.
initially
ins approximately
polymyositis
(Fig.
1).
Limb-Girdle
with
facioscapulolsuniseral
F. S. H.
Its
uric-third
Polymyositis
8.
7-
S.
.::..
65-
..:
Aldolase
4
Units
:.
#{149}:
#{149}::
3.
i
S
2-
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.:.
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.#{149}:...
#{149}.#{149}.m#{149}s.
5555
a%
::#{149}#{149}
___
FIG.
aldolase
Serum
It is agreed
ins the
creatimie
The
of
arid
creatinie
has
that
skeletal
The
kinase.
laboratory
concentrations
generally
diagnosis
seruns
the
kimiase
the
two
advamitage
enzyme
most
of having
has
serum
reliable
determinsationss
arid
determinations
tested
arid
preferable
reactioni
because
is nsore
specific
greater
concentration
cardiac
muscle,
muscle
or cerebral
cortex
2 Aldoiase
is founsd
ins high conscenitratiors
hut it is found
also in other
nions-muscular
tissues,
such as the liver
of
The
seruns
Iii(I1)iIth\
the
serum
seruns
i1i(li(ited
gives
it
dystrophy.
prol)able
because
kimiase
there
(onscentration
a clear-cut
An
carrier
kimiase
assay
have
l)een
that
the
advarstage
elevation
of dystrophy.
than
absolute
enzyme
for
Simultarseous
made
greater
on
of creatinie
However,
aldolase
nsuscle
deternsiniat
210
patiersts
of the
detections
kinsase
of
of
lesser
kiniase
JOURNAL
arsd
OF
1sornisal ins
with
the
BONE
The
results
deterniinsat
ions
nssuscular
creatimse
ANI)
arid
aldolase
evidence
an elevated
1)100(1
diagnsusis
Duchieninie
is reliable
ins
in skeletal
ins the
disease.
of
the
amounts
()f seruns
(reatirse
carriers
to find
THE
kiniase
because
muscle
ins a mother
failure
creatinse
increase
over
as (onispared
ions
with
seruns
The
sensitive
amid relative
a given
patient
the
assav
with
to be nsore
sensitivity
ins the
sensitivity.
the
in skeletal
is believed
is a greater
of this
concenst.rations.
kinase
clearly
disease
of creatimse
creatinse
aldolase
creatirse
niuscle
seruns
clinical
evaluated.
to
reactions
for
amid
as
the
ins the
thoroughly
advocated
specificity
erszynsse
aldolase
aldoiase
cells.
of greater
dystrophy.
seruns
the
beers
beers
muscular
reliable
are
is
assay
progressive
disease
aldolase
___
in early
muscle
.55.5
55555
#{149}:#{149}.
U.,
JOINT
of a
kinsase
SIIIGE1(Y
P.
(hoes
riot
rule
or known
VIGNOS:
J.
out
the
is preferred
inivolvemenit
kinsase
tt(tiVit
creatinse
(If
seruns
(If
sli(h
with
possibility
the
or other
ins highs
is
preserst
enzymes
with
facioscapulohumeral
situata)nis
kinase
ficity
far
active
advanced
muscular
ins whom
a definite
advantage
lability
this
of
freezinig
enzyme
ns(Ire
is also
f( )rward
pr(I(edure
The
subject
serum
niotor
individual
the niumber
and rate
(lisenise
nitty
produce
activity
pointing
ins the
to
may
title
5I)ecific
creatinse
kinsase
destructions
of muscle.
dystrophy
arsd
mass
arid
creatimse
of the
elevations
Examples
a few
l)atienits
is so reduced
The
must
arid
advantages
be weighed
to
the
the
being
laboratory
than
ins spec-
against
reach
determination,
in the
useful
tool
the
firings
muscle
the
greater
laboratory
for
technically
more
relatively
straight-
ins size
during
may
It
to differentiate
the
provide
should
be
two
general
the
over-all
withini
nsyopathy
Ins myopathies,
the
contractions
amid
voluntary
increase
relatively,
opposite
direct
process.
whether
weakrsess.
whereas
lower
ions. Spontaneous
ins t he
of fibrihlations
entities
ins distinguishing
for
(lecreases
changes
disease
aldolase
determination
abnormal
specimens
is responsible
of unit
form
as
minsinsuni
aldolase.
l)otenltial
a mseurogemsic
help
raphv
blood
disease
unit
onsly
sensitivity,
ins detecting
aldolase.
kimsase
pitfalls
such
but
muscle
serum
requires
is a second
mseurors
nu)tor
the
creatinse
to more
for
electromyogram
or haver
over
serum
that
quickly.
(lifficUlt,
The
of the
of probable
serum
creatinie
diseases
with
is so small
that
only the more
sensitive
an abmiormalit
v. Relatively
few cli mucal
of muscle
disease
ins which
the creatinie
enizvnse
has
the
1215
two-thirds
emszynies,
ins liver,
minsimum
dystrophy
Uhid sensitivity
pnithic
Glycolytic
leaking
into the blood
ki nsase determiniat
ions will reveal
arise
ins the differential
diagnosis
of
i ne
(Feat
organs.
concentration
with
ins patients
those
for only
Because
of increased
to be advantageous
appears
are
is a carrier,
DYSTROPHY
liver
kimiase
she
MUSCULAR
elevations
of creatine
kimsase
The
greater
specificity
ins multiple-system
found
i)atienits
ansounit
that
are
(If
transaminiases,
serum
OF PROGRESSIVE
have abnormal
also for its
carriers
kinsase
DIAGNOSIS
an additional
helpful
stressed
that
although
types
of disease,
categories
motor
diagniostic
clue
electromyog-
it will
of
neurons
electrical
nsot differen-
myopathic
or
nseuro-
(li5es5e.
A third
1irst,
tans.
important
it usually
niotor
lOIVCl
muscle
fibrils
the
sh(Iwing
neuron,
as
evidenced
caused
by
motor
tyj)ictLl
fibers
flillscle
makes
it
test is the
determines
j)ossihle
b
unit
changes
amid
of
biopsy,
the
migratu)ni
to
an
which
disease
a histological
picture
degeneration
inspressiomi
nuclei.
the
of
the
severity
(If
groups
of
of
is myopathic
characterize(l
sarcolemmel
(If
atrophy
disease
(If
or whether
involvement,
muscle
central
gains
muscle
svhether
l)y
necrosis
of
Second,
muscle
biopsy
(lamage
to
muscle
the
tissue.
The
pains
(livi(lUntl
hi st
j)rimary
(Table
types
The
of
muscle
disease
is the
fresh
using
These
onsly
heniatuxhini
sj)e(ial stairss,
Biopsy
iill
(Ic(asiomsallv
frozen
disease
tissue
VOL.
perfornse(1
without
(hOseli
49-A,
NO.
manual
prior
6,
sarcoid
SEPTEMBER
that
in this
are
1967
nius(le
cans
the
he
the
between
of myopathy
nemalimie
my(Ipathy
types
sufficienstly
reliably
arid
(If
by the
the
specialized
must
muscular
dystrophy.
chara(teristic
to
other
handling
microscope
(If
ins-
central
Sarcoid
diagmsosis
nit
spe(ific
niitochonidria
stairs,
electrons
the
have
be distinguished
trichronse
muscular
for
forms
wi t 1s
weakness
reliably
a histological
commons
biopsy
niuscie
fornial
For
or
nsuscle
abniornnal
that
group
Gonsori
from
rare
myopathy,
stairs.
three
by
distinguish
hints giant
required
the
usually
riot
a few of the
arid eosini
511(15 as the
riot differentiate
will the muscle
zed
(Iloes
i uclude
)lvflsvoSit
i5 unequivocally
An important
Prere(lnnisite
(nsrefnnhl\
characteri
Only
nsivopathv
stani(lar(l
diseases
are
bioj)sy
mv(Ii)athv.
(ore
hies
nisyopat
I).
he
used.
Onsly
differentiate
dystrophy.
selections
test.
testing.
All
of the
too
I)rl)er
frequently
\Iuscles
muscle
the
often
are
muscle
selected
for biopsy
for
is a
l)iopsy
for
biopsy
is
1216
A.A.O.S.
INSTRUCTIONAL
COURSE
TABLE
PRIMARY
MYOPATHY:
LECTURE
MUSCLE
WEAKNESS
WITHOUT
PAIN
Proximal
Distal
Frequent
Muscular
dystrophy
Ducheisne
Myotonsic
dystrophy
Limb-girdle
Facioscapulohumeral
PolynsyositiS
Steroid
myopathy
Rare
Thyrotoxic
Sarcoid
myopathy
Distal
muscular
dystrophy
myopathy
Ophthalmoplegic
muscular
Nemaline
myopathy
t
Central
core disease
t
Myopathy
with
giant
because
of
results
oftens
acuteness
onset
indicates
are
and
miegative.
type
a positive
a very
weak
end-stage
importance
of
of patients
with
with
the
selecting
the muscles
biopsied
positive
results
were
muscles
were chosen
as ins some
accessible
Ins chroisic
criteria
muscle
for
are
If the
patienits
muscle
disease,
with
correlation
in muscular
diagnosis
is a poor
muscle
used
for
the
depenids
disease
biopsy
on
polymyositis,
fat
the
is of recent
of suitable
size
such as muscular
Such
a weak
muscle
of muscle
fibers
by
of the
it is
ins order
dystrophy,
to
often
shows
onily
amid connective
positive
pathological
dystrophy
is decreased.
choice
biopsy
In
may
be
often
has
the
the
strength
chance
additions,
for biopsy.
diagniosis
poor choice
for biopsy,
because
in early muscular
dystrophy
and
this
used
biopsy
histological
leg
considerations.
reasonis,
the
under
be chosen.
replacement
by
positive
involving
these
proper
muscle
biopsied
Irs this
the muscle
biopsies
were
of the biopsies.
The muscle
biopsied
This is a relatively
is well preserved
When
of the
polymyositis
thirds
riot
of approximately
biopsy
findings.
as acute,
with
disease
a positive
disease
should
muscle
muscle
choice
the weakest
specimens.
select
biopsy
A muscle
tissue.
signs.
or tradition.
The
classed
to
ndria
histological
of muscle
therefore,
advantageous
obtains
and
specific
accessibility
surgical
and,
mitocho
symptoms
t Underlining
give
abnormal
Extramu.scular
dystrophy
for
beems the
physical
Psychologically,
attributed
by
and
any
the
gastrocniemius.
of the gastrocmsensius
of obtainsimsg
a definite
child
psychologic
future
to the
weakness
surgical
pro-
cedure.
Furthermore,
discomfort
after
the biopsy
may cause
muscle
spasm,
tensporarily
impair
walking,
and increase
contracture
of the heel cord.
The
rectus
abdominis
muscle,
which
is involved
early,
has givers
umsifornily
positive
variety.
physical
histological
This
muscle
reaction.
Performance
raphy,
arid
placed
irs one
results
in progressive
muscular
dystrophy
can be biopsied
without
apparent
adverse
of three
muscle
of the
procedures-serum
biopsy-usually
two
major
will
disease
enzyme
allow
the
categories,
THE
patient
determination,
with
neurogenic
JOURNAL
of the Duchemsne
psychological
or
electromyog-
muscle
weakness
or myopathic.
OF
BONE
AND
JOINT
to be
Ability
SURGERY
P.
to
J.
VIGNOS:
the
categorize
illness
The
of these
of
types
the
these
features
In our
literature
that
they
are
The
weakness
(Table
IiiUscles
muscles
the
(If
leg
5411(1 masseter
tinictlv
primary
commons
irs which
amid
different
\Iyotonia,
patterning
to
relax
t(I
gi rdle,
inichinded
that
the
of the
ins the
after
on
seruns
muscle
biopsy,
manual
tests
of
of this
Idenstificationi
an
aid
The
muscle
most
of the
accurate
difficult
muscle
is,
dependently
aid
the
in Class
early
univ
the
irs Class
because
children.
while
the
the
The
analyzed
phase
disease.
or
it
implied
reproducible
6,
the
frequently
ins
testicular
classified
essentially
Duchemsmse,
twenty-six
Evaluations
of these
serial
follow-up
tests of physical
electrodiagniostic
accordlimbpatients
clinical
cxperformance,
studies,
Computer
analysis
of muscle
weakness
to each of the three
ins the
irsvolvement.
early
the
(If
conimons
to
major
types.
ins the
patient
with
A patient
as having
categonnes
five
early
disease
has
of
or six years
The
scale
the
ins Class
1 cars
ins
higher
the
walk
in-
than
twenty-five
seconds
withto negotiate
stairs,
he is placed
early
disease.
dystrophy
are
of age
disease.
classifications
or limb-girdle
dystrophy
are
1 or Class
2. Ins facioscapulohumeral
regarded
manual
whether
A muscle
below.
grade
NO.
most
dis-
diagrsosis.
to confirm
the diagnosis.
have
beers performed
oni 121
standard
steps
in less
If a raihinsg is required
former
children
data
from
to determine
of the
49-A,
This
the
often
use
regarded
This
more
as havinsg
dystrophy,
differentiation
a railing
is
ins small
common
for
assistance
stairs.
strength)
because
1 are
two
Normal
climbing
cemst
severe
arid climb
eight
of a stair railing.
patients
temporahis
occurs.
examinations,
involvement
is diagnosis
2. Patients
with Duchennse
disease
if they
are in Class
made
peroneal
diagnosis.
problem
more
muscles
arid
suggests
strength.
patterns
peculiar
proximal
of the
also
early (lisease
cars be defined
by use of a standard
functional
patients
are graded
on a scale of 1 through
10. On this
number
VOL.
patterning
irs early
Atrophy
is seers
patient
sample,
severity-eight
dystrophies.
a complete
history
amid physical
examinations,
at three-month
intervals,
serial functional
aldolase
arid
urinianv
creatimie
determinations,
serial
tibial
critical
a commons
ins the
slit-lamp
ins males-help
evaluations
on
of distal
generally
amisinsationis
arsd
weak-
a more
is found
facies,
contraction,
data
has demonstrated
a characteristic
all dvstrophiies
and a patterns
of weakness
of
find-
muscle
weakness
anterior
hand.
skull-like
weakness
a muscle
speed
clinsical
However,
early
of the
lesiomss-cataracts
usual
variations
usually
nsuscles
finn!amid
arid
individual
produce
with
a typical
may
clinical
The impressions
conveyed
irs the
of the major
types
of dystrophy
is weakness
with muscular
dystrophy.
The
the scheme
of Walton,
included
arid evemsteers
facioscapulohumeral
l)atiensts
which
the
limb-girdle,
much
myopathies
of muscle
Noni-nsuscular
the
so
diseases
atrophy,
arid j)remature
frontal
baldnsess
Over a period
of thirteens
years
serial
term
narrows
dystrophy
on
of onset,
characteristic.
the
distal
based
age
patterns
myopathy
producing
inisibihit
hands.
the
there
ins the
muscles,
of muscular
heredity,
for diagnosis.
of weakness
completely
only
dystrophy
is nsivotomiic
overlap
that
irs the
I). The
type
is usually
to
experience,
out
greatly
dystrophy-Duchennse,
respect
most reliable
basis
is that
the patterns
so distinctive
been
1217
DYSTROPHY
of disease
specific
dystrophies
muscular
with
helpful.
beers
medical
big
MUSCULAR
two
of the
of the
forms
However,
progression.
the
PROGRESSIVE
diagnosis
classifications
inigs. The
principal
facioscapulohumeral-differ
are
as one
differential
However,
(lifficlilt.
msess
OF
possibilities.
diagnostic
he
DIAGNOSIS
SEPTEMBER
This
muscle
specific
was
grade
significant
in the
standard
1967
defined
was
functional
nsuscle
as involved
selected
impairment
testing
as
with early
or spared
if it was
indicative
and
examinations.
disease
at this
graded
fair
of definite
was
the
most
Muscles
were
early
(50
per
weakness
reliably
described
1218
A.A.O.S.
INSTRUCTIONAL
COURSE
TABLE
PATTERNS
iIUSCLE
OF
WEAKNESS
Neck
AND
Commons
ins all
Lower
Middle
groups
II
SPARmNG
mN EARLY
Involved
LECTURE
MUSCULAR
Spared
trapezius
trapezius
Neck
1)YsYRonHY
extenisors
Extremity
Spared
Gastrocniemius
Tibialis
posterior
Toe
Common
in
Ducheninse
Rhomboids
Latissimu.s
arid
limb-girdle
Upper
Biceps
dorsi
Inward
rotators
Anterior
neck
trapezius
Gluteus
Hip
flexors
maximus
adductors
Triceps
Unsique pattern.s
l)uchersnse
Anterior
(SCM)
fiexors
Limb-girdle
Facioscapulo-
Upper
humeral
Ilanist
rings
Back
extenisors
abdominsals
pectoralis
Brachioradialis
Gluteus
Tensor
Iliopsoas
Lower
Tibialis
major
pectoralis
medius
fasciae
latae
anterior
major
Iliopsoas
(iluteus me(lius
Tensor
fasciae
latae
Quadriceps
Early:
functional
classes
the facioscapulohumeral
muscle
t
as
grade
wrist
involved
were
plus
group
All
found
cent
or
limb-girdle
fair
or below
more
whereas
ins 90 per
muscular
of
types;
functional
ins 66 per
the
muscles,
cent
has
the
cent
Class
of tests.
examinsationis
described
ins
Spared:
of
patients
as spared
or more
of examinations
defined
by
(graded
ins this
A commons
middle
were
of muscle
II),
early
nsamely
of the
neck
criteria
same
extensors
of the
muscles.
foumsd irs all
arid
certain
early
proximal
the impressions
types
of early
muscle
tests,
however,
a commons
patterns
of
involvement
trapezius
was also
mansifest
in differemst
analysis
of manual
of dystrophy
have
of sparing
the
weakness
Computer
three
types
part
patterns
spared
these
is proximal
in distributions.
This
been
reported
in the past,
but
patterns
(Table
amid the
dystrophy
that
quite
distinct.
actually
the
iisvolvememst
muscles
found
is that
are
that
muscle
per
disease,
weakness
in dystrophy
conveyed
trapezius
ins 66
of early
of muscle
weakness
dystrophy
revealed
and
muscles.
early
were
grade
of tests.
patients.
types
pattern
muscle
muscle
hand
as having
or above),
of
oftemi
and
2 in Duchensne
Involved:
Excluding
characterized
fair
1 arid
type.
types
muscles
lower
part
of
of dystrophy.
of the
the
The
posterior
com-
of the
leg, specifically,
the gastrocnemius,
posterior
tibial,
arid toe flexors.
Therefore,
ins a givers patient
suspected
of having
muscular
dystrophy,
if the eonsmoms muscle
pattern
of involvement
is not seems, or if the muscles
usually
spared
are
involved,
one should
consider
it a strong
possibility
that the patienst
has some other
form
of muscle
disease.
partmemst
The
early
volvememit
lower
extremity
tensor
fasciae
exclusively,
Early
dorsi,
disease
arid
flexors
of the
The
the
of muscle
neck
weakness
latae.
was spared
Irs limb-girdle
ins the
patterns
anterior
were
latissimus
muscle
unique
of the
anterior
muscles
lower
and
inward
in this
shoulder
middle
parts
An
of dystrophy
early
was
also
ins Ducheninse
abdomimsals
of the
rotators.
type
dystrophy,
there
weakness
(sterniocleidomastoids)
unique
and
that
of
the
pattern
THE
JOURNAL
gluteus
was
trunsk
early,
trapezius,
the
was
ins-
arid
medius
involved
finding
the
involvement
a commons
of the
were
important
except
dystrophy
amid ins the
ansd
but
riot
rhomboids,
that
rio
sinsgle
hamstrings.
of the
of muscle
OF
BONE
iliopsoas
was
involvement,
AND
JOINT
founsd.
but
SURGERY
P.
vIGNOS:
J.
it was
rsot. unique
(bided
the
iniward
rotators
1)nAGNOSIS
since
lower
arid
lower
extrensities.
as
the
brachioradialis.
it was
middle
ins the
the
shoulder
The
shows
the
stage
(If
thse disease,
major
Patients
restricted
amid the
arid
early
gluteus
is perhaps
involvement
shoulder
areas
of
the
best
found
ansd
ins the
facioscapulohumeral
this
upper
hip
adductors
more
ins
of dystrophy
type
of dystrophy
In
the
of the
tibial
was
insarid
form
part
anterior
had
dorsi
insvolvemerst.
girdle
disease
patterns
defined
ins the
shoulder
This
latissimus
arid
by
of muscle
was
the
maximus
spared
patternsinsg
inivolvensenit
early
rhomboids,
uniquely
1219
DYSTROPHY
dystrophy.
trapezius,
muscle
earls
unique
neck
The
with
girdle,
omily
MUSCULAR
dystrophy
most
ins the
trensitv.
PROGRESSIVE
also
parts
Facioscapulohunseral
arid
OF
ins the
oftems
early
l)ectoralis
lower
cx-
asymmetrical.
muscles
uniquely
spared
the
l)atienit
with
either
of the other
two types
of dystrophy.
The upper
cxtreniitv
sh(Iwed
sparing
of the lower
part
of the pectorahs
major,
the inswar(i
rotators
arid deltoid.
The nsuscles
of the trunk
ansd l)elvic
girdle
which
were uniquely
spared
than
included
thie
back
shnnld
cxtcnssors,
iliopsoas,
giuteus
niake
Evens when
the patterns
of peripheral
diagnostic
problems;
but these
nitty
he
The
niusculature.
diagnosis
l)eciahlV
by the Iresemice
nsisi- l)e asvnsmetri(al.
rsess
(Iris.
Later,
lids were
Duchenimie
this
closed
type
tended
the
of
iniv(Ilvensenit
fn
Ifli
was
The
the
the
striking
be
subtle
arid
mouth
muscles
the
type.
facial
muscles
there
insvolvensemit
the
The
symmetric
mouth
nsost
during
difficult
Duchennie
dystrophy
panti(ularly
difficult
age of ten
before
the
tern
these
diseases.
also,
The
the
for
There
was
dystrophy.
seruns
overlap
A much
uniquely
gn(Iup
of
diagnostic
frons
the
fli(Irc
of the
aidolase
tended
a high
subtle
were
well
preserved
zygomaticus
problemis
is muscular
form
since,
serunsi
PreviouslY
height
ins Ducheminse
thirty-eight
The
arid
average
1 15, arid
lectual
the
retardation
patterns
1)assivity,
mansifested
arid lack
Von
NO.
49-A,
cles(ribed
abnormal
the
but
by inivolvensenit
sniile.
Absence
the
arid
Duchemsnse
only
which
rarely
raises
the
enszyme
differential
intelligence
was
ins Ducheninse
Point
highly
ins the
significant
dystrophy
was
This
favored
enszymes
diagnosis
of Duchemsnse
intellectual
intelligence
dystrophy
found
ins a variety
and children
with
groups
statistically
froni
Children
level,
seers
quotient
of a
was eighty-
of control
mseurogensic
varied
associated
the
helpful
dystrophy.
decreased
often
was
ins Ducheninse
the
control
is
may
occur
The
pat-
primary
as high
diagnosis
dystrophy
be elevated.
of serum
twice
is the
by dependency,
withdrawal
of anshitioms
arid sponstanseity.
n967
of
the
may
dystrophy.
The
means
with
Ducheninse
muscular
quotients
was
fornss,
levels
level
is to distinguish
dystrophy
elevations
to be over
serum
muscular
children
difference
6, SEPTEMBER
eye-
niuscie
ins children.
ins both
emizynse
three
which
was strikingly
different
front that
ins(ludinsg
nsale siblinsgs,
children
with diabetes,
disease.
when
manifest
differentiate
limb-girdle
ins ynnnig
boys
years
amsd the
but
the
patients
with
facial
muscles,
transverse
to
ap-
ohicularis
sniilirsg.
inivolvensenit
nisuscle
(If
being
helped
of the
asymmetric
that
The
the
of
esweak-
of the
result
a peculiar
eye
the
of weakness
the
there
nsay
the facial
Initially,
finsdimsg was
with
caused
arounsd
the
of
insvolved
amid
insstarsces
is supported
expression.
because
svnsmetrical
that
t lie facioscapulohunieral
Ins hinish-girdle
disease,
slight
early
whistling
nsight
latae,
ins most
dystrophy
of facial
a rim of white
sclera
was visible.
also showed
early
insvolvensenst
more
around
of
niost
oculi
lightly,
(If disease
mssuscles
corners
fasciae
muscle
insvolvemenst
is kniowni,
be solved
by exansinsations
of
of nsuscles
on attempted
to be nsuch
(If
tensor
facioscapulohumeral
(If
of weakness
of the mouth
the (Ihi(ularis
pearansce
medius,
These
pattermss
of nsusclc
involvement
amid sparing
it. possible
to diagnsose
the type of dystrophy.
(lt11dmi(el)5.
groups
muscle
between
107
This
relative
with
a PersonalitY
outside
world,
with limb-girdle
intel-
inscreased
dystrophy
1220
A.A.O.S.
tended
to
typed
have
niore
Duchenne
difficulty
ings,
laboratory
closely
the
series
The
very
course
with
disease
of muscle
seen
ins any
types
irs polymyositis.
patients
with
to high
dose
high
doses
sufficiently
the
was
with
to
rsever
if the
sixty
there
of
seen
drug
was
importaist
of the
cases
also
inivolved
from
the
was
any
of
early
The
pattern
nseven
one-third
the
except
a definite
givems early
in the
equivalent).
of Contractures
in
seers
of the
dystrophies
of prednisone
ins the
patterns
standard
almost
showed
ins
to be insvolved
muscles
polymyositis.
usually
milligrams
tended
in
simulate
certaims
cent
in approximately
in
may
were
weakness
seers
pains
finid-
difficulties
are
of these
to vary
was
appear,
muscles
muscle
polymyositis
corticosteroids
(fifty
tended
is cxniansifesta-
weakmsess
extenisors
diagnosis
Facial
but
not
neck
These
also
stereo-
dystrophy
cutaneous
in 70 per
Weakness
in swallowing
patients
the
patients
of dystrophy.
the
do
but
and
toward
into
patients,
arid muscle
If these
ancillary
of muscle
involved
of dystrophy.
polymyositis,
Finally,
response
were
point
Difficulty
terminally.
dystrophy,
ins polymyositis
other
features
extensors
the
fall
muscular
of the
patients
patterns
muscular
of
form
should
weakness
irs the
both
polymyositis,
one-third
late
of
of
riot
amid other
of the
over-all
did
of
rashes
one-third
one-half
or
back
of patients
approximately
only
in only
The
forms
points.
diagnosis
of skin
in only
be marked.
various
differential
appears
abnormalities,
may
and
pattern.
differential
presence
appear
LECTURE
quotients
of personality
the
The
may
diagnosis
in
in
edema,
including
or tenderness
COURSE
intelligence
type
of polymyositis.
tions,
our
rsormal
childhood
Another
elusion
INSTRUCTIONAL
clinsical
disease
amid ins
References
K. C., and
1. ARcHIBALD,
Arch.
Phys.
Med.
2. CoLoraBo,
J. P.;
und diagnostische
3.
and
VIGN0S,
Rehab.,
RICHTERICH,
Bedeutung.
P.
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JR.:
A Study
40 : 150-157,
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Wochenschr.,
Muscular
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1959.
E.:
Serum-Kreatin-Phosphokinase:
40 : 37-44,
1962.
Bestimmursg
J. M. S.; PENNINGTON,
R. J. T.; and WALTON,
J. N.: Serum Enzyme
Studies
ins
Muscle Disease. III. Serum
Creatine
Kinase
Activity
in Relatives
of Patients
with the Duchensne
Type Muscular
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J. Neurol.,
Neurosurg.
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27 : 181-185,
1964.
4. SHY, G. M., and MAGEE,
K. R. : A New Congenital
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610-621,
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W. K.; SOMERS, J. E.; and WANKO,
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1964.
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P. J., JR., and LEFKOWITZ,
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1959.
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K. C. : Maintenance
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1\Iuscular
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P. J., JR. ; BOWLING,
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J. N.: Muscular
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SHY,
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1 1.
Res.
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Nerve.
WORDEN,
D.
K.,
Muscular
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29 : 968-977,
1962.
THE
Function
JOURNAL
in Childhood
OF
BONE
AND
Progressive
JOINT
SURGERY