POLYNEURITIS
IN
CHILDREN
By Niels L. Low, M.D., Julia Schneider, M.D., and Sidney Carter, M.D.
Neurological Institute, Presbyterian Hospital of New York, and Department of Neurology, College of
Physicians and Surgeons, Columbia University
(Accepted June 2, 1958; submitted October 22, 1957.)
Dr. Low was a United Cerebral Palsy Fellow in pediatric neurology.
Dr. Schneider was a United States Public Health Service Trainee in pediatric neurology.
PRESENT ADDRESS: (N.L.L.) Neurological Institute, 710 \Vest 168th Street, New York 32, New York.
PEDIATRICS, November 1958
972
P
OLYNEURITIS is a relatively uncommonbut not rare illness in children.
Casa-major and Alpert1 reported three cases from
the Neurological Institute of New York in
1941. These authors also reviewed the
litera-tune on polyneunitis in childhood up to 1940
and found 19 cases in children less than 12
years of age in the English literature, and
the same number in French publications.
Since then, Roseman and Aring’ and Aning
and Sabin’ together reported five cases in
children less than 12 years whom they had
seen in Cincinnati during a 6-year period.
Hatoff4 reported 17 cases in patients less
than 20 years of age; 12 of these occurred in
children less than i2 years of age. Debre and
Thieffry reported 32 cases occurring in
children during a period of 14 years.
Twenty-three of them had been seen during
the 5 years before the report. The youngest
patient with polyneuritis, in their experience,
was 20 months old. Two of the patients
died. Events reported three cases, one in a
13-month-old child.6 In 1957, Baum7
re-ported an outbreak of “infectious
polyneuni-tis’; three of the patients were children.
Aylett8 reported five pediatric cases from
Britain.
Polyneunitis has been described under a
variety of names during the almost 100
years since Landry’s report of ascending
paralysis.9 When a cytoalbuminous
dissocia-tion coexists with polyneuritis, it is often
called the Guillain-Barr#{233}1#{176} syndrome.
Twenty years later, Guillainhl discussed the
findings in the cerebrospinal fluid necessary
for the diagnosis of “radiculoneuritis” of
the Guillain-Barr#{233} type. To avoid eponyms
in the nomenclature and in order to include
cases of polyneunitis which do not have a
high concentration of protein in the spinal
fluids, other names, like neuronitis,’2’4
Schwannitis,15 and polyneuritis, have been
used in the recent literature.
The definition of this syndrome is
diffi-cult because the clinical picture is variable;
it may be manifested by acute ascending
motor paralysis, by motor and sensory
find-ings or by a combination of signs and
symptoms of peripheral and cranial nerves.
Haymaker and Kernohan’#{176} empimasized that
pathoanatomic terms were inadequate for
good nomenclature, but all other names
that have been proposed appear to us to be
less desirable than the term “polyneunitis.”
The pathologic picture varies with the
severity of the disease and differs according
to the stage at which the patient dies, but
all fatal cases show the same main
charac-tenistic changes. We may assume that the
patients who recover show only mild to
moderate pathologic features. Haymaker
and Kernohan16 imave reported the
patho-logic findings based on 50 necropsies in
adults. In their series, the brain usually
showed only mild to moderate edema with
acute cellular cimanges only in the
fulminat-ing cases. Sparse penivascular collections of
lymphocytes and scattered petechiae were
present only occasionally. The earliest
find-ings in the peripheral nerves were edema
with early disintegration of myelin and
axis cylinders at the end of 8 to 10 days;
phagocytosis and early proliferation of
Schwann cells occurred after the eleventh
8
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12
14+
AGE (years)
Fmc. 1. Age and sex incidence in :30 childremm with polynenritis. mmmacrophages with droplets of myelin were
seemm only in patiemmts with the severest cases
and iii timose \vim() imad survived time longest.
A patient who had survived for 77 days
showed demyelinization with astrocytosis
of time posterior columns. Wimateven the
cimanges in an individual case may be, they
were most prominent where time motor and
sensory roots join. When cranial nerves
were immvolved, time cimanges were similar to)
those in peripheral nerves and at times
pro-gressed to time brain stem.
Because polyneunitis appears to be
be-coming more frequent in children, we
timought it advisable to analyze the histories
of 30 patients, un(ler the age of 17 years,
who were studied at the
Columbia-Presby-teniamm Medical Center. We included all
cases in childnemm timat were diagnosed
poly-neuritis since the report of Casamajor and
.Aipert’ regardless of whether the etiology
vas proven or not.
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ANALYSIS OF CASES
Age, Sex, Race
Of time 30 cases reported in this series, 17
occurred in boys, and 13 immgirls. The ages
ranged from 17 montims to 16 years, the
arbitrary upper limit of this study (Fig. 1).
Time graph suggests an increased
simscepti-bility between the ages of 4 and 9 years, in
timat 16 of the 30 cases reported here fell
within a 6-year period while the other 14
were scattered between the remaining 10
years. The increased incidence during this
period is reflected in both sexes. Time drop
in number of cases between 10 and 12 years
of age is probably not a significant one, as
one of the patients in time 8 to 10 year age
range was 93 years o)ld, and one in time 12 to
14 year age range was 12% years old.
Two patients were Negro; the remainder
Incidence
The number of patients with polymmeunitis
admitted to this hospital each year has
steadily increased (Table I), and has
ac-tually doubled in the past 3 years. There
has been no shift in age incidence. Seasonal
variations in time incidence are not
statis-tically significant in this series.
Prodromal Illness
Nine patients had no pro(lromal illness
before the onset of paralysis. Prodromai
symptoms, when present, occurred in the
2-week period before onset of weakness in all
but one patient. This one exception was a
child with upper respiratory symptoms 1
month before onset of polyneunitis.
The prodnomal symptoms were
nespira-tory in eight children and were referable to
the gastrointestinal tract in nine patients,
wimile both systems were implicated in two
others. Considerable malaise, out of
pro-portion to the mildness of the other
symp-toms, was a frequent complaint. One or
two patients complained of headacime.#{176}
Mode of Onset
The time of onset of neunologic symptoms
was known in 29 cases. Paralysis without
obvious pain or paresthesia was the initial
neurologic symptom in 10. In three patients,
pain in the feet and legs was the earliest
symptom, and in one patient, paresthesiae
in the left hand. In the remaining 16
pa-tients, pain or paresthesiae accompanied
the onset of paralysis, involving the same
parts of the body concurrently in all but one
case. The exception (Case 3) occurred in a
patient who experienced pain in the calves
and thighs of the legs for 2 weeks before the
onset of left facial paresis, followed shortly
by weakness in the left leg.
Muscle Weakness
In 26 patients, paralysis started in the
lower extremities and ascended. In 20 of
these paralysis was symmetrical and in five
a Rosenmami anu:.l Aring niemition headache as a
fry-1mi’nt symptom, present jim 10 of 16 patieimts.
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the weakness was greater on omme side than
time other. In one patiemmt (Case 16) time
earliest and predominant weakness was in
the arms rather timan time legs, witim absent
reflexes in the arms but eak reflexes in
the legs. lim ammotimer Pttimmt (Case 14) time
weakness started iii omme leg, spread to the
otimen leg, timeim involved time arms, hand,
back, an(l abdomeim, imm that order. lim two
patients the onset was so rapid and
general-ized it vas iml)O)sSibie to) determine any
progressioim.
In 16 patiemmts, weakmmess was nmore
nounced in the distal nmuscles; immsix, in the
proximal ummuscles of time extremmmities. In eight
l)atients, weakimess a)peared to) iimvolve all muscle groups equally. imut iii five O)f these
weakness was so extensive amid severe no
voluntary movement of time extremities was
possible.
Twenty-two l)tttiemlts had no respiratory
symptoms although partlysis was widely
distributed and moderately severe imm some
of them. Eight patieimts of this group imad
extensive tetraparesis amxl weakmmess of the
neck and the mmmuscles umimervate(l by time
cranial nerves. Five p1tie1mts had immoderate
weakness in all extrenmities as well as iim
time back 1fl(1 aiXlo)fllelm. Four patieimts had invoivememmt of only the leg, back, and
ab-(lominal ITII1SCICS. Five patients lmad
weak-ness of the extremities vitimotmt involvernemmt
of the trunk, but three o)f these also imad
cranial nerve palsies.
Severe tetnaparesis \ias Pres(’hmt iii time
eight patieimts iim whommm respinator’ paralysis
O)Cdurne(l. Omme l)atielmt had ()fllv slight
re-ductiomm O)f vital capacity because of
inter-costal )arllysis. In the other sevemm 1)atielmtS
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muscles and muscles innervated by the
lower cranial nerves. Two of these seven
patients also had partial or complete
dia-phragmatic paralysis (one with complete
paralysis died in a respirator). Two patients required tracheotomy and use of an artificial
respirator. Another patient received oxygen
and artificial respiration during several
apneic periods. In the five cases in whicim
mechanical aids were not used, respiratory
paralysis occurred late in the course of
ascending paralysis, was less severe, and
cleared more rapidly.
In eight patients, weakness was severe
with loss of voluntary movement. In four,
weakness was mild although widespread,
and patients were able to walk and perform
most activities. The majority (18 patients)
fell between these two extremes. They
were considerably incapacitated and
bed-ridden, but retained some voluntary
move-ment.
Atrophy of the intrinsic muscles of the
hands and feet developed in four patients
and of time leg muscles as well in one
addi-tional patient. The atrophy was not
asso-ciated with prolonged convalescence or a
severe degree of paralysis in every case, and
in at least two cases occurred relatively
early in the course of paralysis. One patient
(Case 30) continued to have a significant
degree of weakness and atrophy of time
hands and feet after 15 months.
Cranial Nerve Palsies
Thirteen patients had one or more cranial
nerves affected at some time during their
illness. In these 13 patients, all the cranial
nerves were represented with the exception
of the first which was not tested in most
cases (Table II). Bilateral papilledema
oc-curred in two patients during the acute
phase of illness, subsiding with clinical
im-provement. In one of these (Case 3), a
12-year-old girl, visual fields and acuity
meas-urements were normal. The pressure of the
cerebrospinal fluid was 140 mm of water and
the concentration of protein was 180 mg/100
ml initially, rising to 318 mg/100 ml during
the next 2 weeks. In time other (Case 6), a
43k-year-old boy with hemorrhages in one eye
ground, in addition to edema of the nerve
head, acuity and visual fields could only he
estimated but appeared to be grossly
nor-mal. The initial pressure of the
cerebro-spinal fluid was 180 mm of water and the
concentrations of protein on two occasions
were 57 and 67 mg/100 ml, respectively. #{176}
One case (Case 22) had incomplete
bi-lateral ophthalmoplegia with restriction of
gaze in all directions except upward. The
facial nerve was affected bilaterally in 10
cases, unilaterally in 2-a total of 40% of
the cases. Symptoms of dysphagia,
dyspho-nia, nasal speech, and regurgitation of fluid
into the nose, and signs of deviation of the
uvula, loss of palatal movement and gag
reflex were thought to indicate loss of ninth
and tenth nerve function. These
manifesta-0 Drew and Magee,” Krohn,” Ford and Walsh,”
and Feldman et al.’#{176}have reported papilledema as
part of polyneuritis. Gardner, Spitler and Whitten” thought that the high concentration of protein jIm time cerebrospinal fluid was the cause of that
find-ing, but in one child observed in the present study
(Case 6) the pressure of the cerebrospinal fluid and
the concentration of protein were normal in the presence of papilledema. One of the three patients with papilledema that Gilpin et al.2’ reported died.
TABLE II
INVOLVEMENT OF CRANIAL NERVES IN 13 OF 30
CHILDREN WITH POLYNEURITIS
Papille-dema Ii! IV V VI VII VJJI* IX+X XI Xli
Unilateral
Bilateral ‘2
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1 1
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‘2 10 1
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tions were present in seven of the most
seriously ill patients and contributed to
pul-monary problems of aspiration, pneumonia,
and atelectasis in three of them. Six of these
patients had evidence of spinal accessory
paralysis as well. All cranial nerve signs
disappeared in the 29 patients who
re-covered.
Sensation
In 23 patients a reliable sensory
examina-tion could be done. In eight of these, no
evidence of sensory impairment could be
found. Pain, touch, position, and vibration
were impaired in six patients, pain and
touclm in two, position and vibration alone in
four, and only pain or position in one patient
eacim. One patient had impairment of
posi-tion and pain with apparently normal touch
and vibration. Position sense was most
fre-quently involved, followed by vibration,
pain, and touch in descending order of
fre-quency.
Meningeal Signs
Five patients showed some evidence of
nmeningeal irritation. Three of these had
isolated signs, such as stiff on painful neck
on flexion, on a positive Kernig’s and
Brud-zinski’s sign.
Reflexes
The deep tendon reflexes were absent in
25 cases. There was incomplete loss in five
patients, in two of whom reflexes in the legs
were obtainable with reinforcement, and in
tlmree of whom reflexes in the arms were
elicited. These reflex findings paralleled
the pattern of motor loss. Abdominal
re-flexes were lost in 6 of the 25 patients with
absent deep tendon reflexes. In the one
pa-tient vho died within 4 days of onset of
illness, a Babinski sign was elicited.
Bladder and Bowel Dysfunction
Symptoms of incontinence or retention of
urine occurred in six patients ranging in age
from 5 to 16 years; four were girls and two
were boys. These symptoms were transient,
lasting from 1 to 4 days, and occurred
dimr-ing the stage of progression of weakness.
Only one patient had fecal incontinence
al-though several patients had cramping,
ab-dominal pain, and constipation. In one
pa-tient (Case 30) who had fecal incontinence,
both the anal reflex and rectal sensation
were lost.
Other Findings
One patient was intermittently
imyperten-sive for 1 month, with blood pressure
rang-ing as high as 150/110 for 3 days, followed
by 3 days of normal levels, only to have the
elevation recur. Electrocardiograms in two
patients were normal.
Laboratory Findings
One or more lumbar punctures were done
in each case. In the 28 cases in which the
cerebrospinal fluid pressure was measured
it was below 180 mm of water in all but one
case (Case 23) in which an initial level of
220 mm was obtained. Fewer than five cells
were present in 25 cases; between 10 and
20 cells were found in timnee cases. In 2 cases
(Cases 22 and 25), 188 and 265 leukocytes
were counted, of which 85% and 100%,
re-spectively, were lymphocytes. One of these
patients was thought to have pohiomyelitis.
but necropsy was indicative of
polynadiculo-neuritis. The other patient (Case 25) had
severe loss of all sensory modalities in a
glove-stocking distribution aimd made a
corn-plete recovery by the end of time second
month. Both of these patients had clinical
evidence of mild meningeal irritation.
Twenty-three patients had elevated
con-centrations of protein in the cerebrospinal
fluid obtained in from one to four lumbar
punctures (Table III). Five of these patients
had only one examination of time
cerebro-‘FABLE III
TIlE CONCENTRATION OF PROTEIN IN (‘EuEBuoSI’INAL
FLUID OF 30 CIIILruREN WITH POLYNEPIITIS
Protein (mg/100 ml) <45 45-7.5 75-20() >200
ARTICLES
spinal fluid. Time concentration of protein in
timese cases ranged from 68 mg/100 ml in
the first week to 220 mg/100 ml at the end
of the second montim. Four of the thirty
pa-tients had no rise in concentration of protein
immtime cerebrospinal fluid on repeated
lum-i)ar punctures, and three others had normal
values on single determinations, made early
in the course of the disease.
The most consistent finding was a normal
on slightly elevated concentration of
pro-teimm during the first week of illness,
fol-iowed by a rise during the first 4 to 5 weeks.
Time concentration of protein then tended
to) return toward normal on remained
ele-vated during the next 2 to 3 months. There
was no correlation between the height or
rapidity of rise in time content of protein of
time cerebrospinal fluid and either the age
O)f the patient on clinical condition. Serologic test for syphilis and
concentra-tions of sugar and chloride in the
cerebro-spinal fluid were normal.
The leukocyte count and erythrocyte
sedi-mentation rate were usually normal unless
the course was complicated by pneumonia.
One patient had a small number of atypical
lymphocytes on differential blood smear,
but a heterophil agglutination test was not
elevated. Two patients had mild
hypo-chromic, normocytic anemia; however, none
had basophilic stippling or other change
suggestive of lead intoxication. Cultures of
nose and throat for diphtheria, done in 10
cases, were negative. Virus studies (of stool,
serum, cerebrospinal fluid, and nasal swab)
were negative in two cases. Roentgenograms
of the long bones for evidence of heavy
metal poisoning were negative in 12 cases.
Roentgenograms of the spinal column and
skull, which were taken in the majority of
time cases, were also normal. Urine
examina-tion for porphobilmnogen was negative in
four cases. Blood and urine tests for lead
and arsenic done in one case (Case 23) were
negative.
0 Merritt23 found the concentration of protein
less than 45 mg/100 ml in 27% of cases, and hIecimt’ found a negative Pandy reaction in two
of seven children.
Because of progressive alopecia in one
pa-tient (Case 20), tests of blood and urine for
thallium were done and a concentration of
1.8 mg/i of urine was obtained (the upper
limit of normal is 0.1 mg/i). This case has
been previously reported.25
Electroencephalograms were normal in
five patients, showed abnormal slow activity
in two, and transient right panietai-temporal
slow wave focus in one. Elevated concen
trations of calcium and phosphorus in serum
and a positive Sulkowitch test of the urine
were found in three patients after about
a month of immobility; roentgenograms of
the abdomen and intravenous pyelograms
failed to reveal nephrolithiasis in these
pa-tients.
Chronaxie studies were done in 19 of the
cases and electromyographic testing in three
additional patients. Only two patients failed
to show evidence of extensive, usually
sym-metrical, denenvation of the extremities. In
one, the examination was done during the
first week of illness and gave only suggestive evidence of denervation in the extremities.
In the other patient (Case 17), paralysis was
widely distributed but very mild and cleared
completely in 2 months.
Course
The length of time during which
paraly-sis progressed is not known in most cases;
however, onset of clinical improvement was
noted in 25 patients. Eleven began to
im-prove by the end of the second week of
illness, an additional eight by the fourth
week, and the remaining six by the eighth
week (Fig. 2).
Twelve patients were followed sufficiently
long to observe complete recovery of
strength, reflexes, and sensation. The penio)d
of time necessary to attain complete
re-covery varied widely from 2 to 18 months,
but about one-third had recovered
com-pletely after 2 months, another third after
6 months, and the last third from 9 to 18
months. No correlation existed between the
rate of the recovery and such factors as the
age of the patient, severity of the illness,
WEEKS
1 _____I__
2
OF
345678
ILLNESS BEFORE IMPROVEMENT
Fic. 2. Duration of illness before improvement in 25 cases of polyneuritis.
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One cimikl (Case 22) died after an acute,
fulminating 4-day illness characterized by
ascending paralysis, incomplete
ophthal-moplegia, facial diplegia, complete lower
cranial nerve paralysis, and terminal
paraly-sis of time intercostal muscles and diaphragm.
Ammother child (Case 30) has incompletely
recovered after 15 months. He still has
con-siderable weakness and atrophy of the
hands and feet, and only partial return of
reflexes, althougim sensation has returned to
normal. Four patients are still in the stage
of convalescence. Three patients had mild
residual sensory impairment after periods
from 1 to 2 years. Two patients had slight
motor residual disability after a similar
period, and three additional patients had
slight weakness of the hands and feet after
10 weeks but were not followed long
enougim to observe complete recovery. Four
patients were lost to follow-up after the
acute phase of their illness had passed.
Two patients, 4 and 5 years old, have had
relapses. One of these (Case 28) had a
re-currence and progression of weakness after
a severe upper respiratory infection that
occurred after five weeks of steady
im-provement. Weakness, which included all
the extremities, back, abdomen, and neck
muscles, with sparing of the cranial nerves
and sensory system, remained stationary for
the next 33 months in spite of physiotherapy
and supportive therapy. Improvement
be-gan 3 days after administration of
predni-sone (20 mg daily) was started and
pro-gressed rapidly during the next 3 months.
One attempt to decrease the dosage O)f
pnednisone 2 weeks after it was started was
followed by a mild recurrence.
The other patient (Case 19), who had
facial diplegia, moderate involvement of the
lower cranial nerves, in addition to
pro-found tetraparesis and distal sensory loss,
suffered two recurrences during
convales-cence. The first, in the seventh month of
illness, occurred after bilateral acute otitis
media; the second, at the end of 1 year,
occurred after a first poliomyelitis vaccina-tion.
This child received a short course of
therapy with cortisone during the initial
acute illness without noticeable effect. This
was followed by adrenocorticotropin
then-apy (25 units of Acthan#{174} Gel daily) for time
second 2-week period, and during this time
rapid improvement in strength began and
reflexes returned. Adrenocorticotropin was
seemried to) abort the attack in that the
re-flexes, muscle strength, and sensation
re-turned very rapidly after 4 to 5 days of
adrenocorticotropin therapy. The second
re-lapse was shorter, and much less severe,
although both paralysis and loss of
sensa-tion recurred. He recovered uneventfully
from this attack, with adrenocorticotropin
therapy. He received prophylactic
sulfadia-zine therapy for a year following the first
relapse to prevent infections, and has
made slow but continuous improvement in
strength.
Corticosteroids, used in timree cases, and
adrenocorticotropin in one, during the acute
phase of illness, had little or no effect on the
course of the illness.
Prognosis
Prognosis for life seems to be good in
childreim except in the most fulminating
cases. Pneumonia was a common
complica-tio)n ism the 50 fatal cases reported by
Hay-maker and Kernoiman,bo but it should be
rarely a cause of death with the variety of
potent antibiotics available at this time. The
greatest danger is respiratory paralysis, and
early tracimeotomy and other mechanical
respiratory aids seem to be mandatory in
these instances. Complete or nearly
corn-plete recovery can usually be expected but
may he delayed for many months.
Tabular Summaries and Illustrative
Case Reports
The important clinical and laboratory
data concerning each patient are given in
Table IV. Cases 11, 22, and 28 are presented
in detail:
Case 11
HIsToRY: G.P., a 4-year-old white girl had
mild upper respiratory iimfectiomm without fever
2 weeks before the onset of a rapidly ascending paralysis accompanied b pain in the legs and back. Weakness involved the legs, trunk, arms,
abdomeim, and intercostal and neck muscles
successively over a 2-day period, and was
fol-lowed by’ dysphagia, dysphonia, inability to
raise secretions and facial diplegia developing
durimmg the third and fourth days of illimess. PHYSICAL FINDINGS: Deep tendon reflexes
were absent at the time of admission omm the
second day, and the child complained of pain
on movement of the extremities. Glove-stocking
type sensory loss of all modalities began
dur-ing the second week of illness. Inability to raise or swallow secretions resulted in
pul-monary atelectasis.
COURSE : Tracheotomy was performed under
local anesthesia on the third day. Respirations
became irregular and rapid, interspersed with
periods of apnea the following day, and an
attempt was made to place her in a tank respi-rator. This was ummsuccessful because of her fear and inability to adjust her owim respiratory
rate to that of the machine. Fortunately, muscle
strength began to improve that same day and respiratory movements became more efficient
and less rapid.
LABORATORY FINDINGS : Routine uritmaiyses
amid urine tests for porphyrins were normal; the
immitial leukocyte count was 25,000 and showed
a shift to the left, later slowly returning to nor-mal as the clinical condition improved.
Cul-tures of mmose, throat, and blood were normal.
Roentgenogram of the chest, taken after the
unsuccessful attempt to place her in the
respi-rator, revealed massive atelectasis of the left
lung with a shift of the heart shadow to the
left. Repeated roentgenograms made during
the mmext few days revealed clearing of the atel-ectasis but the presence of pneumonitis
involv-ing the right upper and middle lobes, which also later cleared. Cerebrospinal fluid obtained
at the time of admission contained two cells
and concentration of protein of 27 mg/100 ml but the concentration of protein had risen to
217 ing/100 ml by the second week.
LATER COURSE: Signs and symptoms
pro-gressed during the first week of illness in spite
of therapy with adrenocorticotropin started on
the third day. Muscle strength began to
im-prove dunilmg the second week although the
clinical condition continued to be precarious. Muscle strength returned in the reverse order
of involvement and sensory perception also
improved, although more slowly. Deep tendon
reflexes returned in the arms within 6 months; in the legs at 10 months. A year after the
ill-imess there was only slight weakness of
dorsi-flexion of the feet, some clumsiness in fine
movements of the hands and slight impairment
of position sense in the toes, all of which had
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Case 22
HIsToRY: S.Y., a 93k-year-old Negro girl,
de-veloped a cold and enlarged cervical nodes,
followed 11 days later by abdominal pain,
vomiting, and diplopia. Three days after onset
of diplopia, weakness began in the legs,
in-volved the arms next, and finally the trunk and cranial nerves.
PHYSICAL FINDINGS: The temperature was 38.9#{176}Cat the time of admission (first day of
weakness) and there were findings of acute
follicular tonsillitis and right otitis media. Neu-nologic findings were incomplete
ophthalrno-plegia with limitation of movement in all
di-rections of gaze except upward, normal
pupil-lany response, and normal funduscopic findings.
In addition, there was weakness of the left
masseter muscles, facial diplegia, deviation of the uvula and tongue to the left, and a normal gag reflex. Paresis was greater in the left than
in the right arm but was equal in the legs
where the distal muscles were weaken. The
neck, intercostal, back and abdominal muscles
were all weak, and breathing was largely
dia-phragmatic. There were nuchal rigidity and
positive Kernig and Brudzinski signs. Deep
tendon reflexes were still present in the arms
initially, but then disappeared. A Babinski sign
was present initially. Sensory examination was
normal although the child complained of numb-ness in the hands and feet before onset of
par-.lvsis. She was rational and co-operative
tlmrough her illness.
LABORATORY FiNDINGs: The leukocyte count
was 20,000/mm3 with a shift to the left;
ervthrocyte sedimentation rate was 21 mm in 1
hour, and urinalysis was normal.
Cerebro-spinal fluid was under normal pressure, had
198 cells/mm3, 170 of which were
lympho-cytes; the concentration of protein was 20 mgI
100 ml and that of sugar 58 mg/100 ml.
Cul-tunes of blood and cerebrospinal fluid were
negative, and cultures of nose and throat
showed no predominant flora.
COURSE : Her condition remained stationary
for 3 days; then paralysis progressed with
in-ability to talk or swallow, increasing
respira-tory difficulty and inability to handle
secre-tions. Gag and pharyngeal reflexes were
abol-ished. In spite of a tracheotomy and use of a
tank respirator vital signs became irregular and
she died 4 days after onset of paralysis. The
clinical diagnosis was bulbar and spinal
an-terior poliomyelitis.
NECROPSY FINDINGS: Necropsv was
per-formed 7 hours after death. Examination of
organs outside the nervous system revealed
tracheitis, pneumonia, and pulmonary edema.
Gross and microscopic examinatiomms of the
central nervous system were normal. Segments
of several cranial nerves in the region of the
fourth ventricle showed evidence of
degenera-tion with swelling of nerve fibers and large
mononuclear phagocytes in moderate numbers.
A Mahon stain showed myelin degeneration
and a Bodian stain revealed that some of the
axons in the affected nerves were swollen and
vacuolated. Similar involvement was found in
the left trochlear (Fig. 3) and hypoglossal
nerves, and in some of the spinal roots at the
cervical and thoracic areas and the
caudo-equina levels. The first, second, third, fifth, left seventh, eighth, left tenth, and right twelfth cranial nerve roots were normal. No pathologic
changes were found in the left phremmic nerve,
and other peripheral sections of nerves and
muscles were not available for study.
Because of the absence of lesions imm the
central nervous system, in particular in the
cranial nerve nuclei and anterior horn cells
of the spinal cord, plus the presence of
de-generative changes in some cranial and spinal
nerve roots, the conclusion reached was that
this patient most likely suffered from
poiy’-radiculoneuritis.
Case 28
HISTORY: M.P., a 43k-year-old white boy, de-veloped weakness first in the right then the left
leg, without prodromal symptoms, 53 months
before admission to this hospital. He was seen
by his pediatrician who noted complete
are-flexia, symmetrical weakness of the legs, and
normal sensation. One week later, strength had
improved slightly and reflexes had returned,
only to disappear subsequently. Blood count,
erythrocyte sedimentation rate, and
roentgeno-grams of the long bones made at that time
were normal. Two lumbar punctures were
made at the beginning of the fourth week and
reportedly showed normal pressure, no cells,
and concentration of protein of 57 and 63
mg/100 ml, respectively. Repeat examinatioim
at that time demonstrated the weakimess previ-ously described, areflexia, and a “positive
Ker-nig’s sign.” Chronaxie ammd electromvographic
studies done at a local hospital were thought to
how-4-..
3..,. - . -j
FIG. 3. Myeiiim degeneration and nmononuclear infiltration in left trochlear nerve of Case 22. ever, the diagnosis of a polyneuritis was made.
Strength improved slowly for five weeks with
immtensive physiotherapy until 3 months before
admissiomm wimeim, after a respirators’ infection, time weakness in the legs suddenly increased
amid he developed severe weakness of the trunk,
arms, aimd neck. He had mmo pain or paresthe-sia, imo cranial nerve or respiratory involve-ment, and muscle weakness persisted
un-changed for the next 3 months.
PHYSICAL FINDINGS: At the time of
admis-sion there was symmetrical weakness of all
extremities, legs more than arms, with equal
involvement of proximal and distal muscle groups. There was severe weakness of back,
abdominal, psoas, and neck muscles. Faint
knee and ankle jerks could be elicited, but
others were absent. There were no pathologic
reflexes. Sensory and cranial nerves were nor-mal.
LABORATORY FINDINGS: Blood count,
urinal-ysis and electroencephalogram were normal. A lumbar pummcture done at the time of
admis-sion revealed normal pressure, no cells, and
commcentrations of proteimm and sugar of 86 and
69 rng/100 ff11. Electromyographic studies
domme initially revealed diffuse lower motor neu-ron disease with indications of a changing
process. Repeat examinatioim 10 days after
ad-ministration of predmmisone was started showed some improvement in the activity o)f time
volun-tars’ motor unit; fibrillatiomm potentials were less
numerous. Chronaxie studies substammtiated the
electromvographic findings.
COURSE: The clinical condition persisted un-changed in spite of physiotherapy for 3 months until administration of prednisone (20 mg/
day) was begun. Noticeable return of strength began .3 days thereafter, startimmg in the arms,
neck, and trunk, then in the proximal muscles
of the legs. Four months later he was able to
walk relatively easily ammd reflexes had re-turned. A mild regression occurred when the
dosage of prednisone was reduced, and the
dosage was again increased.
COMMENT
The data presented suggest a peak in
age incidence of polyneuritis between 4
and 10 years of age. Both sexes are equally
susceptible to the disease. Two of thirty
cases were Negro, a ratio considerably
be-low the usual inpatient ratio in this hospital.
Thirty-three per cent of the Patients had
symp-toms in the remaining 67% were about
equally divided between the respiratory
and gastrointestinal systems. Only 10% had
symptoms suggestive of nervous system
in-volvement before the onset of paralysis.
Headache was not a prominent symptom in
this series.
Twenty-seven per cent of the patients
had purely motor disease. In one of these
the fulminating course of progressive
pa-ralysis with both clinical and cerebrospinal
fluid evidence of meningeal irritation
sug-gested a diagnosis of bulbar poliomyelitis.
Ascending paralysis occurred in 83%,
din-ically apparent respiratory paralysis in 27%.
Muscle atrophy occurred in 17% and had no
prognostic significance.
Sensory examination could not be done
in 23% of these patients because of age or
mental retardation. Position sense was most
frequently impaired, followed in order by
sensibility to vibration and pain. Position
sense, however, is more easily tested in
children who frequently resent pin prick.
It is impossible to evaluate position sense
accurately in a very young child.
All the cranial nerves, with the exception
of the first, were involved in one or more
patients. The two patients with swelling of
the optic discs had normal visual acuity and
fields, and hence it was concluded that the
swelling was not due to optic neuritis. The
papilledema could not be explained on the
basis of either a consistently elevated
pres-sure or concentration of protein of the
cerebrospinal fluid. Incomplete
ophthal-moplegia, a relatively infrequent finding
in peripheral neuritis, occurred in one
pa-tient.
Five patients ( 17%) had clinical evidence
of meningeal irritation and in two of these
cells were found in the cerebrospinal fluid.
All the patients showed clinical
improve-ment within the first 2 molmths, the majority
within the first mommtim. The duration of
weakness, aneflexia, ammd seimsony inmpainimient
varied frommm 4 weeks to 2 years and did not
correlate vith the extensiveness of the
pa-ralysis and, only roughly, with its severity.
Cranial nerve signs appeared late in the
course of ascending paralysis in most cases,
and remitted early, thus aiding considerably
in management of respiratory complications. Two patients have suffered clinical relapses
and their course has been prolonged. One
patient died (mortality rate 3.3%),
substan-tiating the good prognosis reported by otimer
atmthors. Only one patient after prolonged
follow-up has been left with residual
weak-ness and atrophy in the hands and feet.
The management of the patients was
largely supportive. Adrenal steroids21
were used in six patients, and, although the
number is small, time material suggests that
their greatest usefulness is in patients who
suffer relapses or have a prolonged course
without much clinical improvement.
Di-mercaprol “ 32, 33 was not used irk
these cases.
The etiology of the neuritis was
undeter-mined in all but one of our cases in which
a toxic level of thallium was found in the
urine. Studies for toxic substances, such as
lead and arsenic, for infections, such as
diphtheria and infectious mononucleosis,
and for metabolic disorders, such as acute
porphyria, proved unrevealing although
ad-mittedly these were not done in every case.
From the review of the literature and
from the experience at time
Columbia-Pres-bytenian Medical Center, the incidence of
polyneuritis in children seems to be
increas-ing. One case was seen between 1937 and
1940, 2 between 1941 and 1944, 6 from
1945 to 1948, 7 between 1949 aimd 1952,
and 14 from 1953 through 1956. It is
un-likely that this increase is solely due to an
increased diagnostic acumen of the staff.
Because the etiology of the syndrome
was unknown in all but one case it is
im-possible even to speculate on the higher
incidence in recent years. Definite toxic
agents (alcohol, arsenic, lead, porpimynins),
preceding or coexisting diseases (
chicken-pox, rubella, gonorrhea, leukemia, malaria,
nmumps, I)eniartenitis imodosa, diphtheria, and
others), and recent immunizations were not
found. Injections have been immmplicated as
possible etiologic agents in polyneuritis.
serum are more commmmon now than they umsed
to be but the onset of the paralysis was not
preceded by such an injection in any of the
cases, except in time one case in which
re-lapse occurred after poliomyelitis
vaccina-tion. Antibiotics by injection are used more
now than previously but they have only
been observed to give a localized
neu-34
The mortality rate was low (3.3%). Death
in these patients is usually due to
respira-tory complications. Early and judicious use
of respirators together with tracheotomy
and the administration of antibiotics should
continue to keep the mortality rate low.
SUMMARY
Polyneunitis in children is becoming more
common. Time clinical picture is variable; it
may be manifested by motor and sensory
findings, or by a combination of peripheral
and cranial nerve signs and symptoms. The
etiology is usually obscure. The pathologic
picture varies with the severity of the
dis-ease and differs according to the stage at
wimich time patient dies, but all fatal cases
simow the same main characteristic changes.
Clinical records of 30 cases, 17 boys and 13
girls from 17 months to 16 years of age,
are reviewed. The clinical manifestations, coimrse and treatment are described.
REFERENCES
1. Casamajor, L., and Alpert, G. R. : Guillain-Barr#{233}syndrome in children. Am.
J.
Dis.Child., 61:99, 1941.
2. Roseman, E., and Aring, C. D. : Infectious polmmeuritis. Medicine, 20:463, 1941.
3. Aring, C. D., and Sabin, A. B. : Fatal in-fectious polyneuritis in childhood. Arch. Neurol. & Psychiat., 47:938, 1942. 4. Hatoff, A. : Polyneunitis of unknown
eti-ology immchildhood.
J.
Pediat., 24:393, 1944.5. Debre, R., ammd Thieffry, S. : Remarques sur le syndrome de Guillain-Barr#{233} chez l’eimfammt. Arch. franc. p#{233}diat., 8:357,
1951.
6. Everts, W. H. : The
Landrv-Guillain-Barr#{233}-Strohl syndrome. Dis. Nerv. System, 17: 1, 1956.
7. Baum, R. N. : Infectious polvneumritis, a
dis-ease to be distinguished from
poliomve-litis. California Med., 87:317, 1957.
8. Aylett, P. : Five cases of acute immfective polyneuritis in children. Arch. Dis.
Child-hood, 29:531, 1954.
9. Landry,
J.
B. : Note sur paralysieascen-dante aigue. Gaz. hebd. med., 6:472,
1859.
10. Guillain, C., Barr#{233},J.-A., and Stroimi, A.:
Sun un svimdrome de radiculo-mm#{233}vrite
avec hyperalbuminose du hiquide
c#{233}phalo-rachidien sans reaction cellti-laire. Bull. et m#{233}m.Soc. med. hop. Paris,
40: 1462, 1916.
1 1. Guillaiim, C. : Radiculoneuritis with
acellu-lan hyperalbuminosis of the cerebrospinal fluid. Arch. Neurol. & Psvchiat., 36:975, 1936.
12. Mills, C. K. : The reclassification of some organic nervous diseases on the basis of
the neuromm. J.A.M.A., 31:11, 1898.
13. Kennedy, F. : Infective neuronitis. Arch. Neurol. & Psychiat., 2:621, 1919.
14. Nielsen,
J.
M. : Treatrneimt of mmeuronitis with BAL. Bull. Los Ammgeles Neumroi.Soc., 15:61, 1950.
15. Barker, L. F. : Acute diffuse
polyradiculo-neuritis following oral sepsis. Arch.
Neurol. & Psychiat., 31:837, 1934.
16. Havmaker, W., and Kernohan,
J.
W. : TheLandrv-Guillain-Barr#{233} syndrome.
Medi-cine, 28:59, 1949.
17. Drew, A. L., and Magee, K. R. :
Papill-edema in the Guillain-Barr#{233} syndrome.
Arch. Neurol. & Psvchiat., 66:744, 1951.
18. Krohn, W. : A case of polvradiculoneuritis
with papilledema. Acta psychiat. et
neurol. scandinav., 27:303, 1952.
19. Ford, F. R., and Walsh, F. B.:
Guiliaimm-Barr#{233}syndrome with immcreased
intra-cranial pressure and papilledenma: report of 2 cases. Bull. Johns Hopkins Hosp.,
73:391, 1943.
20. Feldman, S., Landau,
J.
, ammd Halpern, L.:Papilledema in the Cuillain-Barr#{233}
syn-drome. Arch. Neurol. & Psvchiat., 73:
678, 1955.
21. Gardner, W.
J.,
Spitler, D. K., and Whitten, C. : Increased intracranial pres-sure caused by increased proteincon-tent in the cerebrospinai fluid. New Eng-land
J.
Med., 250:932, 1954.22. Cilpin, S. F., Moersch, F. P., ammd Kerno-han,
J.
W. : Polyneuritis. Arch. Neurol.& Psychiat., 35:937, 1936.
23. Merritt, H. H. : Textbook of Neurology.
Philadelphia, Lea, 1955, p. 580.
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S., and Ganong, W. F. : The Guillain-Barr#{233} syndrome: report of a case treated with ACTH and corti-sone. New England 1. Med., 246:293,1952.
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A., and Lubin, R. I.:Corti-cotropin (ACTH) therapy in
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and Avol, M. : Two cases of Cuillain-Barr#{233} syndrome treated with cortisomme. Neurology, 3 :935, 1953.30. Esselier, A. F., and Kopp, E. : Uben die Behammdlummg der Polyradiculitis
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32. Furmanski, A. R. : BAL therapy of severe peripheral neuropathies. Arch. Neurol.
& Psychiat., 60:270, 1948.
33. Salzer, H. M. : BAL in the treatment of
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Sys-tern, 15:67, 1954.
34. Scheinberg, L., and Allensworth, M.:
Sciatic neuropathy in infants related to antibiotic injections. PEDIATRICS, 19:261, 1957.
SUMMARIO IN INTERLINGUA
Polyneuritis In Juveniles
Polyneuntis in juveniles deveni plus
corn-mun. Le tableau clinic es vaniabile. Jib pote
consister de manifestationes motoni e sensoni o
de un combination de symptomas e signos de
nervos penipheric e cranial. Le aspectos
pa-thologic vania con le severitate del morbo e
differe con le stadio in que le patiente mori, sed
omne casos mortal exhibi le mesme major
al-terationes characteristic. Es presentate umm
revista del dossiers clinic de 30 casos. Le serie
consiste de 17 pueros e 13 pueras, de etates
de inter 17 menses e 16 annos. Le manifesta-tiones clinic, le curso del monbo, e he tracta-mento usate es descnibite.
L0NG-miu.f Srury OF 105 PATIENTS WITH CYSTIC FIBRoSIs, H. Shwachnmamm et al.
(A.M.A.
J.
Dis. Child., 96:6, July 1958.)The extent to which the clinical recognition of the condition termed cystic fibrosis of time pancreas has progressed, as well as the limitations of this term, are revealed in a study of 105 patients from a single clinic, followed from 5 to 14 years. Time authors
have devised a sonmewhat complicated scheme for evaluation of the clinical condition of patients with this disease and the effects of therapy. When a truly satisfactory treatimment is available we shall probably not need a complicated scheme to appraise
time results. Here once again it may be seen that some patients with this disease may
survive for long periods without the benefit of medical care or regardless of the
therapy they may have received. Ten patients in the series had reached the age of 15 years or over at the time of the report; in 7 of these the diagnosis was not estab-lished umntil the age of 8 years or over. However, the authors point out that the
dura-tion of life prior to diagnosis may not be a measumre of the severity of the disease. That the prognosis has improved is indicated by the finding that prior to 1948 the average age at death was 12 nmonths, and during the period from 1951 through 1956 the average age at death was 59 months. The authors realize that the prognosis is
