Muscle Nerve. 2005 Nov 30;33(3):356-36
A study of three patients with amyotrophic lateral
sclerosis and a polyneuropathy resembling CIDP.
Echaniz-Laguna A, Degos B, Mohr M, Kessler R,
Urban-Kraemer E, Tranchant C.
Departement de Neurologie, Hopital Civil de Strasbourg,
1 Place de l'Hopital, BP426, 67091 Strasbourg, France.
We report three patients with a syndrome that fulfilled
clinical and laboratory criteria for definite chronic
inflammatory demyelinating polyradiculoneuropathy (CIDP)
who failed immunosuppressive treatment and eventually
developed progressive amyotrophic lateral sclerosis
(ALS). Mean disease duration was 23 months (13-38)
before death. Two patients had a family history of ALS
without mutations of the SOD1 gene. Postmortem
examination in one patient showed an endoneurial
infiltration of mononuclear cells in lumbar roots and
distal and proximal peripheral nerves, mainly around
myelinated fibers, with demyelination and axonal loss,
consistent with CIDP. The spinal cord revealed severe
neuronal loss in the anterior horn, axonal loss in the
corticospinal tract, and large numbers of phagocytes in
the anterior and lateral tracts, indicative of ALS.
Whether demyelinating polyneuropathy was coincident with
ALS or was a cause or consequence of motor neuron
degeneration in these patients remains to be elucidated.
This unusual combination may provide an important clue
in elucidating the pathogenesis of ALS in some patients.
Muscle Nerve, 2006.
PMID: 16320313 [PubMed - as supplied by publisher]
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J Neuroimmunol. 2010 Aug 25;225(1-2):149-52. Epub
2010 May 26.
Epstein-Barr virus antibodies in serum and cerebrospinal
fluid from multiple sclerosis, chronic inflammatory
demyelinating polyradiculoneuropathy and amyotrophic
lateral sclerosis.
Nociti V, Frisullo G, Marti A, Luigetti M, Iorio R,
Patanella AK, Bianco A, Tonali PA, Grillo RL, Sabatelli
M, Batocchi AP.
Source
Institute of Neurology, Department of Neurosciences,
Catholic University, Rome, Italy.
Abstract
Elevated anti-Epstein-Barr virus (EBV) antibody levels
are present in serum of Multiple sclerosis (MS) patients
but literature lacks of studies comparing anti-EBV
antibody levels between MS and other neurological
diseases. We evaluate anti-VCA IgG and IgM, anti-EBNA1
IgG, anti-Cytomegalovirus IgG and IgM titres in serum
and cerebrospinal fluid (CSF) of 267 MS, 50 Chronic
Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)
and 88 Amyotrophic Lateral Sclerosis (ALS) patients. We
found increased titres of anti-EBV-IgG in serum and CSF
of MS subjects as compared to CIDP and ALS patients thus
providing additional evidence for a possible involvement
of EBV in MS
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Muscle Nerve. 2008 Jul;38(1):855-60.
Chronic inflammatory demyelinating polyneuropathy-like
disorder associated with amyotrophic lateral sclerosis.
Rajabally YA, Jacob S.
Source
Neuromuscular Clinic, Department of Neurology,
University Hospitals of Leicester, Leicester General
Hospital, Leicester, UK. yusuf.rajabally@uhl-tr.nhs.uk
Abstract
The association between demyelinating neuropathy and
amyotrophic lateral sclerosis (ALS) has been reported
rarely. We report four patients who presented with
clinical features and investigations suggestive of a
neuropathy but who later progressed and received a final
diagnosis of ALS according to the original El Escorial
criteria. Three patients met the European Federation of
Neurological Societies/Peripheral Nerve Society (EFNS/PNS)
2006 definition for "definite" chronic inflammatory
demyelinating polyneuropathy (CIDP), as well as the
American Academy of Neurology (AAN) 1991 and Nicolas et
al. [2002] electrodiagnostic criteria for CIDP. Two of
them showed segmental demyelination on teased-fiber
preparations and one had a raised cerebrospinal fluid
protein level. Another patient met the Nicolas et al.
CIDP criteria and presented with brachial plexus
hyperintensity on T2-weighted magnetic resonance
imaging, indicative of an inflammatory process. No
significant objective response to immunomodulatory
treatment was observed in any of these patients who all
subsequently progressed unfavorably.
(c) 2008 Wiley Periodicals, Inc.
PMID:
18508343
[PubMed - indexed for MEDLINE]