Visual evoked potentials study in chronic idiopathic inflammatory demyelinating polyneuropathy.

BACKGROUND: The frequency of the association between chronic demyelinating inflammatory polyneuropathy (CIDP) and central nervous system (CNS) demyelinating lesions is probably underestimated. OBJECTIVE: To investigate the occurrence of combined central and peripheral demyelination in CIDP patients and to correlate visual evoked potential (VEP) abnormalities with CNS demyelinating lesions, observed on brain magnetic resonance imaging, and antibodies against glycolipids. METHODS: Nerve conduction studies, brain MRI and antibodies against glycolipids were prospectively studied in 17 patients who fulfilled the diagnostic criteria proposed for CIDP (Cornblath DR, Asbury AK, Albers JW, Feasby TE, Hahn AF, McLeod JG, Mendell JR, Parry GJ, Pollard JD, Thomas PK. Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force. Research criteria for diagnosis of chronic inflammatory demyelinating polyneuropathy. Neurology, 1991;41:617-618). VEPs were performed in each case before and after 6 months treatment with either intravenous immunoglobulins (IVIG) or steroids. RESULTS: Eight patients (47%) had increased latencies in at least one eye or showed increased interocular latency difference. Four patients (23%) presented a significant high signal intensity on T2-weighted brain MRI images. Of these 4 patients, 3 had prolonged VEP latency. Two patients with delayed VEP latency had antibodies against GM1, and SGLPG and anti-sulfatides, respectively. One patient with normal VEPs also had antibodies to GM1. VEP results were not significantly modified after treatment, either with steroids or IVIG. CONCLUSION: This study confirmed the high frequency of abnormal VEPs in CIDP patients, and found that they are poorly correlated with CNS demyelinating lesions and antibodies against glycolipids. The VEP abnormalities of these patients may be explained by the susceptibility to immune-mediated damage of both the peripheral nervous system and the optic nerve.

[Acute demyelinating motor neuropathy: an atypical form of the Guillain-Barre syndrome?]. / Neuropathie motrice aiguë démyélinisante secondaire à une gastro-entérite: une forme atypique de syndrome de Guillain-Barré?

A 33-year-old man presented an acute motor demyelinating neuropathy following Campylobacter jejuni enteritis. The patient was improved with an IgIV treatment. Clinical features and course time were compatible with the diagnosis of a Guillain-Barré syndrome. The electrophysiologic studies were in favor of multifocal motor neuropathy with conduction blocks. We discuss the nosologic group of this neuropathy.

[Ataxic neuropathy associated with disialosylated antibodies: description of new clinical and biochemical forms]. / Neuropathie ataxiante associée à des anticorps anti-gangliosides disialylés: description de nouvelles formes cliniques et biologiques.

INTRODUCTION: Polyneuropathies associated with IgM monoclonal gammopathy were recently recognized. Antibodies can react with glycoproteins such as myelin associated glycoprotein (MAG), or gangliosides containing one sialosyl epitope such as GM1 or several sialosyl epitopes (polysialyted gangliosides) including GD2, GD3, GT1b, GT1a, GQ1b. METHODS: We report on three patients presenting oculomotor dysfunction, chronic sensitive ataxic polyneuropathy, high sedimentation rate, IgM monoclonal paraprotein of unknown signification and antidisialosyl IgM antibodies and for two of them cold agglutinins. Such features have been previously described under the acronym "CANOMAD" (chronic ataxic neuropathy with ophthalmoplegia, M protein, agglutination and disialosyl antibodies). RESULTS: One of the patients presents extramembranous glomerulopathy and severe motor disability associated with this syndrome. The pathophysiology of the glomerulopathy seems to be linked with the polyneuropathy. Patients were treated either by intravenous immunoglobulin, corticosteroids or cyclophosphamid. Response to treatment differs in the three cases and there is currently no consensus. CONCLUSION: Our study demonstrates that spectrum of polyneuropathy associated with monoclonal polyneuropathy may be larger than originally described.

[Prevalence of pneumocystosis in HIV infected patients in a pneumology unit. Autopsy study performed in Abidjan (Côte d'Ivoire)]. / Prévalence de la pneumocystose chez les sujets infectés par le VIH dans un service de pneumophtisiologie. Etude autopsique réalisée à Abidjan (Côte d'Ivoire).

Autopsies were performed in the Pathology Department of the Treichville University Hospital, Abidjan, Ivory Coast in 70 HIV infected subjects who had died in the Department of Pneumophtisiology. The prevalence of Pneumocystis carinii pneumonia was determined. None of the patients had received prophylaxis against P. carinii and none had bee treated for pneumocystosis. Autopsies were performed within 6 to 48 hours after death and the diagnosis of pneumocystosis was confirmed with the Gomori-Grocott staining technique on lung specimens. Among the 70 autopsies Pneumocystis carinii pneumonia was observed in 6. Thus the prevalence of P. carinii pneumonia in these patients infected with HIV was 8.57%.

High incidence of serum monoclonal Igs detected by a sensitive immunoblotting technique in B-cell chronic lymphocytic leukemia.

In a prospective study in 65 untreated patients with early-stage B-cell chronic lymphocytic leukemia (B-CLL), serum monoclonal Igs (moIg) were evidenced in 80% of cases by a sensitive immunoblotting procedure. These low-abundance moIg were generally undetectable by immunoelectrophoresis and individual sera often contained several of them. Their kappa/lambda ratio was close to 1 instead of 2.8 for the lymphocyte surface Igs. A monoclonal IgM of the same light-chain type as the lymphocyte surface IgM was found in 26 sera only. The distribution of the heavy-chain classes and subclasses and light-chain types of the serum moIg was similar to those observed in aging (with a higher incidence and no correlation with age in B-CLL) and conditions with defective T-cell functions. Using a specific filter affinity-transfer assay, rheumatoid factors were detected in 58.5% of sera. However, homogeneous anti-IgG antibodies corresponding to a monoclonal IgM of the same light-chain type as the surface IgM were found in 10 patients only. These data suggest that the majority of discrete serum moIg in B-CLL are not secretion products of the leukemic clones and likely result from the immunodeficiency state inherent in the disease.