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Chronic inflammatory demyelinating polyradiculoneuropathy associated with pregnancy.

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Chronic Inflammatory
Demyelinating
Polyradiculoneuropathy
Associated with Pregnancy
P. A. McCombe, MB, BS, P. G. McManis, MB, RS,
J. A. Frith, MB, BS, J. D. Pollard, PhD,
and J. G. McLeod, DPhil(0xon)
In a series of 61 patients with the relapsing variety of
chronic inflammatory demyelinating polyneuropathy,
there were 16 women of childbearing age, 9 of whom
became pregnant. In 4 of these women, the onset of
neuropathy occurred in pregnancy and in the other 5
relapses occurred during pregnancy. There was a significant increase in the number of relapses during the
year of pregnancy, and a tendency for symptoms to
worsen during the third trimester or immediate postpartum period. It is concluded that there is an increased
risk of relapse of chronic inflammatory demyelinating
polyneuropathy in pregnancy.
McCombe PA, McManis PG, Frith JA, Pollard JD,
McLeod JG: Chronic inflammatory demyelinating
polyradiculoneuropathy associated with pregnancy.
Ann Neurol 21:102-104, 1987
Although the onset and relapses of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)
have been described in pregnancy in isolated case reports 12, 4, 5, 8, 111, there has been no study of the
association in a large series of cases. We have therefore
reviewed 93 patients with the clinical, electrophysiological, and pathological features of CIDP, in 9 of
whom the onset or relapse of polyneuropathy was associated with pregnancy, in order to evaluate the effect
of pregnancy on the course of the illness.
Methods
Patients
Ninety-three patients (57 males, 36 females) with CIDP
were referred to OUT department for investigation; sural
nerve biopsy was performed on 87. All patients fulfilled the
established criteria for the diagnosis of CIDP [6, 12). Sixtyone patients had a relapsing course and 32 had a subacute
progressive course.
Statistical Analysis
For the purposes of statistical analysis, a. relapse of CIDP was
defined as a worsening of symptoms or signs, resulting in an
From the Department of Medicine, University of Sydney, N.S.W.
2006, Australia.
Received Mar 12, 1986, and in revised form June 9. Accepted for
publication June 10, 1986.
Address reprint requests to Dr McLeod.
102
increase in disability by one or more grades on the disability
scale [12) with subsequent improvement. The onset of relapses was not related to change in therapy. The number of
relapses of CIDP for a patient is defined as the total number
of episodes of disease, excluding the initial episode. The
pregnancy year is the 9 months of pregnancy and the 3
months immediately post partum. No patient had more than
one relapse during pregnancy.
The relapse rate was defined as the number of relapses
divided by the number of years of observation. Relapse rates
were compared between groups by using an exact test based
on the binomial distribution. The chi-squared test, with a
correction factor for continuity, was also used but was of
limited value as the expected value in some cases was less
than 5. For the comparison of pregnant and nonpregnant
periods in childbearing women, the variability among women
was allowed for using the Mantel-Haenszel test.
Results
Of the 61 patients with the relapsing form of CIDP, 10
had insufficient information to determine the duration
of their illness and relapse rate. The relapse rate for the
remaining 51 patients of both sexes (31 males, 20 females) was 0.33 per year. Four of the 20 females were
outside the childbearing age (arbitrarily defined as 17
to 50 years) during their illness. Of the remaining 16
premenopausal females, 9 had pregnancies. The course
in one patient (PO) with pregnancy-associated relapses
is shown in the Figure. The obstetric and relapse histories of these 9 women are summarized in Table 1.
Their ages at onset ranged from 18 to 32 years (mean,
23.9 years; SD, 5.8). They all displayed the clinical
features of CIDP during relapse, with paresthesias,
weakness, hyporeflexia or areflexia, and sensory impairment. One patient (SF) had diplopia as an additional symptom. Lumbar puncture was performed o n
all patients. The cerebrospinal fluid protein concentration ranged from 0.15 to 2.12 &liter (mean, 0.93;
SD, 0.57).
The 9 childbearing women had a total of 30 pregnancies. Eleven pregnancies occurred before the onset
of CIDP; the disease onset occurred during 4 pregnancies, and as these initial episodes were not defined as
relapses, they were excluded from analysis; the remaining 15 pregnancies occurred after the disease onset and were analyzed statistically for an association
with relapses. Table 2 shows the relapse rate for the
women of childbearing age who had never had a pregnancy, compared with those women who became pregnant after the disease onset. The relapse rate for the
parous women was further analyzed to compare the
relapse rate during the pregnancy years with those
when they were not pregnant. The difference is
significant (exact test, p = 0.02; x2 = 6.49; p < 0.02).
As women vary in their relapse rates, the MantelHaenszel test was also applied to compare pregnant
and nonpregnant periods in these 9 childbearing
women (x2 = 4.60; p < 0.05). There is also a
i
P
1
2
3
5
4
6
7
YEARS
Course of chronic infEammutory demyelinating polyradiculoneuropathy in Patient PO. Onset and two relapses were related
to pregnancy. Filled horizontal bars indicate time of pregnancy.
D3, Dq, and D s indicate time ofaklivery of third,fourth, and
fifth children. Worsening of symptoms occurred post partum following each delivery. Open horizontal bars indicate duration of
corticosteroid therapy. P indicates commencement of a course of
plusmapheresis. Disability scores are those as described by Prineas
and McLeod {12}.
significant difference between the number of relapses
experienced by nonparous women with CIDP and
those experienced by parous women during their pregnancy years (exact test, p = 0.01).
Five of the 8 relapses (62.5%) associated with pregnancy occurred in the third trimester or 3 months post
parturn, and in 1 patient (PO, Figure) there was significant worsening of symptoms post parturn.
Discussion
All patients had a subacute or chronic polyneuropathy
with the electrophysiological and pathological features
of CIDP [6, 12). There was no family history of polyneuropathy and no clinical or laboratory evidence of
metabolic, neoplastic, or other causes; no patient had a
paraproteinemia. The possibilities were considered,
and are difficult to exclude, that pregnant patients
may have been observed more closely or that they
may have reported symptoms more frequently than
did nonpregnant patients. However, these potential
sources of bias seem unlikely, as in our experience
most patients with CIDP will report worsening of
symptoms of one or more grades of disability.
The statistical approach used for this study is similar
to that used by Korn-Lubetzki and colleagues 193 for
multiple sclerosis; these authors demonstrated a statistically significant decrease in the number of relapses in
the third trimester but an increase post partum.
When the relapse rate for the parous women when
pregnant was compared to that when not pregnant, the
difference was significant. The difference was also
significant when the number of relapses of women
with CIDP who had never been pregnant was compared to that of women during their pregnancy years.
There appeared to be a tendency for relapses, or a
worsening of symptoms during relapse, during the
third trimester or post partum.
Table I. Obstetric Details of Patients with CIDP
Patient
Total
Number of
Pregnancies
AD
JH
PO
4
4
3
PC
3
EA
4
PK
SF
AB
2
2
DH
2
30
1
2
0
0
2
2
3
1
15
Total
5
5
Number of
At Risk
Pregnancies
Associated with
Relapse
Number of
Pregnancies
after Onset
of CIDP (at
risk pregnancies)
1
0
2
0
0
1
1
2
1
8
Relapses Not
Associated
with
Pregnancy
1
4
1
0
0
0
6
2
7
21
Number of
Years of
Observation
11
9
6
7
2
32
15
42
12
136
CIDP = chronic inflammatory demyelinating polyradiculoneuropathy.
Table 2. Relapse Rate
Nonparous women
Parous women
During pregnancy years
At other times
Number of
Years of
Observation
Relapse
Rate
(relapsedyr)
Number
of Women
Number
of Relapses
7
10
75
0.13
9
9
8
21
15
12 1
0.53
0.17
Brief Communication: McCombe et al: CIDP and Pregnancy 103
There have been previous isolated case reports of
subacute and relapsing neuropathy occurring in pregnancy, frequently during its later stages. Castaigne and
colleagues [ 3 ] referred briefly to 2 patients who had
relapses during pregnancy at 3 and 6 months, respectively. Calderon-Gonzalez and associates 121 described
a patient in whom relapses of a symmetrical polyneuropathy occurred during the second and third
trimesters in three successive pregnancies, and subsequently while taking oral contraceptives. Novak and
Johnson [ll] reported a patient who had a subacute
onset of peripheral neuropathy in the third trimester
of her first pregnancy and in the second trimester of
her second pregnancy. Jones and Berry IS] descrihed a
patient who had the onset of CIDP in her first pregnancy and relapses in the subsequent two pregnancies.
Dalakas and Engel 141 studied one patient in whom the
onset occurred in the later months of pregnancy.
DAmbrosio and de Angelis 153 described a patient
who had the onset of relapsing polyneuritis during the
third trimester of her first pregnancy, a relapse late in
the third trimester of the second pregnancy, and a
further relapse six months after commencement of an
oral contraceptive.
Guillain-Bar& syndrome may occur in pregnancy
but it does not appear to be more commcm in pregnant
women than in the general population, and pregnancy
does not seem to influence the course or severity of
the disease [I, 5 , 10, 131.
The mechanism of relapses of CIDP in pregnancy is
uncertain. Studies of the immune system in pregnancy
have been directed mainly toward the mechanism of
protection of the fetus from rejection by the mother
[71. It is not known how immunological responses in
pregnancy could cause or exacerbate CIDP. PossibIe
mechanisms may be the existence of a cross-reactivity
between fetal and maternal neural antigens or an immune response in pregnancy that provokes an anamnestic response to neural antigen.
P. A. McCombe was a National Health and Medical Research Council Postgraduate Medical Scholar and P. G. McManis was a Bushell
Research Fellow. The statistical advice of Professor G. Berry, School
of Public Health and Tropical Medicine, and Dr J . Simes, Royal
Prince Alfred Hospital, and the skilled technical assistance of Miss
P. Martin are gratefully acknowledged.
-
neuropathy: pathogenesis and treatment. Ann Neurol
~ ( s u P P ~134) : 135, 1981
5. D’Ambrosio G , de Angelis G: Syndrome de Guillain-Barre au
cours de la grossesse. Rev Neurol (Paris) 141:33-36, 1985
6. Dyck PJ, Lais AC, Ohta M, et al: Chronic inflammatory polyradiculoneuropathy. Mayo Clin Proc 50:62 1-637, 1975
7. Jacoby DR, Olding LB, Oldstone MBA: Immunological regulation of fetal-maternal balance. Adv Immunol35:157-208, 1984
8. Jones MW, Berry K Chronic relapsing polyneuritis associated
with pregnancy. Ann Neucol9:413, 1981
9. Korn-Lubetzki I, Kahana E, Cooper G, Abramsky 0: Activity
of multiple sclerosis during pregnancy and puerperium. Ann
Neurol 16229-231, 1984; (reply to letter) 18:101, 1985
10. McFarland HR, Heller G L Guillain-Barr6 disease complex: a
statement of diagnostic criteria and analysis of 100 cases. Arch
Neurol 14:196-201, 1966
11 Novak DJ, Johnson KF’: Relapsing idiopathic polyneuritis during pregnancy. Arch Neurol 28:219-223, 1977
12 Prineas JW, McLeod JG: Chronic relapsing polyneuritis. J
Neurol Sci 27:427-458, 1976
13 Ravn H: The Landry-Guillain-Barre syndrome. Acta Neurol
Stand 43:l-64, 1967
Whipple’s Disease
Confined to the Central
Nervous System
Melanie Adams, MD,’ Patricia A. Rhyner, MD,t
John Day, MD,S Stephen DeArmond, MD, PhD,+
and Edward A. Smuckler, MD, PhD’
Progressive hypersomnia, memory disturbance, and
vertical ophthalmoplegia developed in a 63-year-old
woman. The diagnosis of Whipple’s disease of the central nervous system was suggested by her presentation
and results of studies using magnetic resonance imaging. Despite a one-month course of antibiotics, active
Whipple’s disease, localized to the central nervous system, was found at autopsy.
Adams M, Rhyner PA, Day J, DeArmond S,
Smuckler EA: Whipple’s disease confined
to the central nervous system.
Ann Neurol 21:104-108, 1987
Whipple’s disease is a rare multisystem disorder presenting most often with symptoms of gastrointestinal
malabsorption, fevers, arthralgias, and lymphadenopa-
References
1. Ahlberg G, Ahlmark G. The Landry-Guillain-Barre syndrome
and pregnancy. Acta Obstet Gynecol Scand 57:377-380, 19.78
2. Calderon-Gonzalez R, Gonzalez-Cantu N, Rizzi-Hernandez H:
Recurrent polyneuropathy with pregnancy and oral contraceptives. N Engl J Med 282:1307-1308, 1970
3. Castaigne P, Brunet P, Nouailhat F: Enquete cilinique sur les
polyradiculonCvrites inflammatoires en France. Rev Neurol
(Paris) 115:849-872, 1966
4. Dalakas MC, Engel W K Chronic relapsing (dysimmune) poly-
104
From the Departments of ’Pathology, t Radiology, and $Neurology,
School of Medicine, University of California, San Francisco, San
Francisco, CA 94143.
Received Feb 18, 1986, and in revised form June 9, 1986. Accepted
for publication June 9, 1986.
Address reprint requests to Dr Adams, Department of Pathology,
HSW-501, School of Medicine, University of California, San Francisco, CA 94143.
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pregnancy, associates, polyradiculoneuropathy, inflammatory, demyelination, chronic
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