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Chronic relapsing polyneuritis associated with pregnancy.

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Chronic Relapsing
Polyneuritis Associated
with Pregnancy
M. W. Jones, M D , FRCP(C),*
and I(. Berry, MD, FRCP(C)I.
The occurrence of the Guillain-Barrk syndrome in pregnancy is an infrequent but well known and extensively
described phenomenon, whereas “recurrent bouts of polyneuritis” during subsequent pregnancies are far less common. W e describe a patient who had recurrent bouts of
polyneuritis with her first two pregnancies and then had a
third episode not associated with pregnancy.
At age 24 years, with her first pregnancy, our patient developed distal paresthesias from the feet up to the waist
with some leg weakness and calf pain. Examination revealed absent deep tendon reflexes and diminished pinprick and light touch sensation distally. Cerebrospinal fluid
(CSF) protein was 242 m d d l with 6 white blood cells. H e r
deficits remained static for the last six months of her pregnancy. She then delivered a healthy child and made a full
A t age 26, with her second pregnancy, she had an identical recurrence of her distal paresthesias and leg weakness.
The clinical impression was again that of an acute idiopathic
polyneuritis. The pregnancy evolved normally with a
healthy child.
At age 33 she presented with a five-month history of an
annoying numbness in her hands and feet with loss of dexterity in her fingers and weakness in her legs and ankles.
Examination revealed absent reflexes and sensory loss to
pinprick in a stocking-and-glove pattern. Investigations
showed normal complete blood count, protein electrophoresis, urine porphyrins, and blood sugars. The erythrocyte sedimentation rate was 12. Pregnancy test, lupus erythematosus cells, antinuclear factor, and anti-DNA were all
negative. The CSF contained 8 lymphocytes and a protein
of 103 mg/dl with 20 mg/dl IgG and 13% gammaglobulin.
Sural nerve biopsy and teased fiber preparation revealed
occasional instances of segmental demyelination. With the
electron microscope a rare example of active demyelination
by macrophages was observed. The maximum motor conduction velocity for the median nerve was 13 m/sec, ulnar
nerve 14 m/sec, and peroneal nerve 22 m/sec. Median,
ulnar, and sural nerve sensory action potentials were absent. She responded to high-dose alternate-day prednisone
with complete remission of her subjective symptoms over
the ensuing weeks. One year later, while she remained
asymptomatic, nerve conduction velocities had not changed.
This patient fulfills the requirements for a diagnosis of
chronic neuropathy [2]. Novak and Johnson [3] reported a
case, as did Calderon-Gonzalez et a1 [ 11, implying that each
recurrent bout of polyneuritis was intrinsically related to
pregnancy, possibly due to some hormonal influence playing a role in the genesis of this disorder.
W e contend that it is simply the “nonspecific stress” of
pregnancy that served to bring out our patient’s symptoms
and made the occult manifest. O n e need not necessarily
postulate a direct causal relationship. The mechanism of
this association has yet to be explained, and the solution to
the problem undoubtedly will provide further insight into
the mechanism of the chronic demyelinating neuropathies.
*Division of Neurology
+Division of Neuropathology
University of British Columbia
Faculty of Medicine
Vancouver, BC, Canada V 6 T 1 WS
Calderon-Gonzalez R, Gonzalez-Canter N, Rizzo-Hernandez
H: Recurrent polyneuropathy with pregnancy and oral contraceptives. N Engl J Med 282:1307-1308, 1970
Dyck PJ, Laid AC, Ohta M, Bastron JA, Okezaki H, Groover
R: Chronic inflammatory polyneuropathy. Mayo Clin Proc
50:621-637, 1975
Novak DJ, Johnson K P Relapsing idiopathic polyneuritis
during pregnancy. Arch Neurol 28:219-223, 1977
ComDressive MveloDathv
in MLcopolysaccharidosis
Clarence E. Ballenger, MD, Thomas R. Swift, MD,
and Robert T. Leshner, MD
We wish to comment on the recent report by D r Young
and his associates [2] concerning a case of compressive
myelopathy in Maroteaux-Lamy syndrome with description
of the light and electron microscopic findings in the cervical
meninges. This was believed to be the first such report insofar as the light and electron microscopy findings were
concerned. W e recently reported a case of Hunter syndrome with compressive myelopathy due to impingement
of thickened meninges upon the cervical spinal cord [I].
Light and electron microscopy revealed clear inclusions
believed to contain mucopolysaccharide as well as lamellated inclusions thought to contain ganglioside. I t appears
that two related mucopolysaccharidoses with cervical myelopathy have been studied in this manner.
Department of Neurology
Medical College of Georgia
Augusta, G A 30912
1. Ballenger CE, Swift TR, Leshner RT, El Gammal TA,
McDonald TF: Myelopathy in mucopolysaccharidosistype I1
(Hunter syndrome). Ann Neurol 7:382-385, 1980
2. Young R, Kleinman G, Ojemann RG, Kolodny E, Davis K,
Halperin J, Zalneraitis E, DeLong GR: Compressive myelopathy in Maroteaux-Lamy syndrome: clinical and pathological findings. Ann Neurol 8:336-340, 1980
Editor’s Note
Any failure of association here is the fault of the Editor, not
D r Young, whose manuscript went to the publisher before
Dr Ballenger’s appeared.
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pregnancy, polyneuritis, associates, relapsing, chronic
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