Journal olthe ,,\:euroiogtca/M
i~'~J~. [*~4 i i 99 i ) ~;x 'ii
,c 1991 Elsevier Science P u b l i s h e r s B.V 0!)22-510X91 $1 ~-,t~
Quantitative analysis of monoclonal immunoglobulins in serum of patients
with amyotrophic lateral sclerosis
Fran~oise Duarte 1, St6phane Binet 2, Lucette Lacomblez
2, Pierre Bouche 2,
Jean-Louis Preud'homme I and Vincent Meininger 2
t Laboratoire d Tmmunologie et d'Immunopathologie, UA 1172 CNRS, H6pital de la Mil$trie, 86021 Poitiers (France), and 2Centre SLA, H6tel-Dieu de
Paris, Centre de Diagnostic, 1, rue de la Cit~, 75004 Paris (France)
(Received 25 January, 1991)
(Accepted 18 February, 1991)
Key words."
Amyotrophic lateral sclerosis; Monoclonal immunoglobulins; IgG; IgM
Summary
In a prospective study, we analysed the presence of monoclonal immunoglobulin (molg) in the serum from 30 patients with
amyotrophic lateral sclerosis (ALS) and 30 matched controls using a sensitive Western blot technique. The incidence of serum molg
was 60~o in the ALS group and 13.3~o in the control group. Most ALS sera contained 2 or 3 monoclonal components. They were IgG
(72.7~g) and IgM (27.3 %). These results corroborate the concept of a probable association between ALS and serum molg.
Introduction
An increasing body of data favors the hypothesis of an
unusually high frequency of serum monoclonal immunoglobulins
(moIg) in motor neuron disease (MND) and particularly
in amyotrophic lateral sclerosis (ALS) (Latov
1982; Shy etal. 1986; Rowland 1987). Altogether, Shy
et al. (1986) collected reports of 43 MND patients with
moIg. More recently, Younger et al. (1990) detected paraproteins
in 11 of 120 patients (93) using immunofixation,
and in only 4 of the 120 patients (3 ~o) by cellulose acetate.
They observed that 10 of the 11 patients with paraproteins
had either ALS (5 patients) or ALS with probable upper
motor neuron signs (5 patients), suggesting a high frequency
of paraproteins in ALS and the need for highly sensitive
techniques to detect the paraproteins in this disease.
The availability of a sensitive method to detect moIg and
to determine their isotypes with a Western blot technique
(Aucouturier and Preud'homme 1987; Briault et al. 1988)
prompted us to undertake a prospective analysis of sera
from ALS patients. In the present paper, we report on the
high incidence of moIg in this condition.
Correspondence to."
V. Meininger, Centre SLA, H6tel-Dieu de Paris,
Centre de Diagnostic, 1, rue de la Cit6, 75004 Paris, France.
Patients and methods
Patients
Serum was collected in 30 patients with ALS, 17 men
and 13 women, mean age was 52 + 11 years, mean duration
of symptoms at time of examination was
22 + 10 months. 28 of the 30 patients had typical ALS, as
assessed by the association of both upper motor neuron and
lower motor neuron signs and a more or less constant
progression of the disease (Mulder 1982; Rowland 1983;
Tandan and Bradley 1985). Upper motor neuron signs
included hyperreflexia and Babinski sign. Lower motor
neuron signs included persistent muscular weakness with
atrophy. In 2 of the 30 patients, upper motor neuron signs
were not obvious, since the Babinski sign was lacking. They
were included as the tendon reflexes were present and
hyperactive in limbs with weak and wasted muscles. 14 of
the 30 patients had bulbar signs with the association of
muscular weakness and atrophy of the muscles of the
tongue and pseudobulbar features. In all patients, electromyographic
and laboratory tests, including radiotogic investigations,
were supportive and excluded other pathological
conditions.
Control sera were obtained from 30 age- and sexmatched
healthy subjects, 17 men and 13 women, either
spouses of patients or volunteers. Their mean age was
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