Alzheimer CSF biomarkers in routine clinical setting.

Objectives: Our work was aimed to evaluate Alzheimer's disease diagnosis improvement using cerebrospinal fluid biomarkers (CSF) in neurological daily practice.

Materials And Methods: For this purpose, 150 patients clinically and neurochemically classified as having AD or cognitive impairment with or without other dementia type were included in the study. The following CSF peptides were studied, blindly to the clinical diagnosis: beta-amyloid(1-42) peptide (Aβ(1-42)), Tau (T-tau), threonine-181 hyperphosphorylated tau protein (P-tau(181)), and beta-amyloid(1-40) peptide (Aβ(1-40)).

Atypical electrophysiologic findings in chronic inflammatory demyelinating polyneuropathy (CIDP)--diagnosis confirmed by nerve biopsy.

Objectives: Numerous sets of electrophysiological criteria of chronic inflammatory demyelinating polyneuropathy (CIDP) have been proposed, among which the criteria established by an ad hoc subcommittee of the American Academy of Neurology (AAN) in 1991 (Neurology 41 (1991) 617) are the most widely used. As they seemed rather restrictive, the Inflammatory Neuropathy Cause and Treatment (INCAT) group (Ann. Neurol.

[Importance of the nerve biopsy for the diagnosis of atypical forms of chronic inflammatory demyelinating polyradiculoneuritis: 8 cases].

The objective of the study was to define how could be helpful a nerve biopsy for identification of atypical cases of chronic inflammatory demyelinating polyneuropathy (CIDP). An ad hoc committee in 1991 defined the clinical, electrophysiological and pathological criteria for diagnosis of CIDP. In common with other authors, we regard the rather specific electrophysiological criteria as being too restrictive, and we think that a significant number of patients may therefore not benefit from effective treatment or be excluded from therapeutic trials.

Influence of propofol concentrations on multipulse transcranial motor evoked potentials.

Background: Motor evoked potentials can be affected by propofol anaesthesia. We studied how increasing target concentrations of propofol altered transcranial motor evoked potentials (tcMEP) during scoliosis surgery.

Methods: Fifteen patients undergoing surgery for scoliosis were anaesthetized with remifentanil and propofol without nitrous oxide or neuromuscular blocking agents (BIS<60).

[Glycogenesis due to adult phosphorylase kinase deficiency].

A 42-year-old man presented exercise-induced muscle pain without myogloburia since the age of 12 years. Histochemistry and electronmicroscopy of a muscle biopsy revealed subsarcolemmal and inter-myofibrillar accumulation of glycogen. Exercise on a bicycle ergometer produced a normal raise of lactate.

[Sensitive chronic inflammatory demyelinating polyradiculoneuropathy in Schnitzler's syndrome].

Background: Schnitzler's syndrome is a rare etiology of chronic urticaria. The disease is characterized by the association of chronic urticaria, intermittent chronic fever, bone pain, osteosclerotic bone lesions and IgM monoclonal gammapathy. More than fifty patients with this syndrome have been reported since Schnitzler reported the first case in 1974, but neuropathies are seen only in a few cases.

Interferon beta-1a as an investigational treatment for CIDP.

A prospective, multicenter, open-label study was conducted to determine the safety and efficacy of intramuscular (IM) interferon beta-1a (IFNbeta-1a) (Avonex) for treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Eligible patients received IM IFNbeta-1a 30 microg once weekly for 6 months. Safety and tolerability were evaluated by reporting of adverse events, measurement of vital signs, and results of blood chemistry, hematology, and urinalysis.

[Chronic polyradiculoneuritis and its frontiers].

The Chronic Inflammatory Demyelinating Polyradiculoneuropathies (CIDP) constitute a syndrome whose incidence is difficult to evaluate, and is probably underestimated. In the course of this presentation, we deliberately restricted discussion to issues raised in recent years concerning the extent of this syndrome. We discuss diagnostic criteria, especially electrophysiological ones.

[Chronic inflammatory demyelinating neuropathies and their variants].

The Chronic Inflammatory Demyelinating Polyradiculoneuropathies (CIDP) constitute a syndrome whose incidence is difficult to evaluate, and is probably underestimated. In the course of this presentation, we deliberately restricted discussion to issues raised in recent years concerning the extent of this syndrome. We discuss diagnostic criteria, especially electrophysiological ones.

Diagnostic value of nerve biopsy for atypical chronic inflammatory demyelinating polyneuropathy: evaluation of eight cases.

The diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) relies primarily on clinical and electrophysiologic examination, but the nerve biopsy findings may be supportive, especially in atypical cases. In order to define the usefulness of nerve biopsy in this disease, we retrospectively studied 44 consecutive patients whom we classified as having CIDP on pathological grounds. We found that 8 of these 44 patients had pathological findings indicative of CIDP but did not meet any of the usually accepted electrophysiological criteria for its diagnosis.

Is the morning peak of acute myocardial infarction's onset due to sleep-related breathing disorders? A prospective study.

Many studies have shown that the risk of experiencing a myocardial infarction (MI) is increased during the first hours of the morning. Sleep apnea syndrome (SAS) is associated with an enhanced adrenergic activity, prolonged a few hours after awakening. We aimed at assessing whether sleep breathing disorders could be a culprit for the morning excess rate of MI.

[Ventricular tachycardia by branch to branch re-entry. Familial case with Steinert's disease].

Ventricular tachycardia by branch to branch reentry is a rare arrhythmia. It occurs in cardiomyopathies associated with conduction defects. During tachycardia a His potential precedes each QRS complex which usually has a left bundle branch block appearance.

Myelin widenings and MGUS-IgA: an immunoelectron microscopic study.

A few studies have reported a variety of nonspecific histological lesions in patients with IgA monoclonal gammopathies and polyneuropathy. In our case, using electron microscopy, we observed widenings of the myelin lamellae identical to those commonly described in IgM neuropathies with anti-myelin-associated glycoprotein activity. Using immunoelectron microscopy, we demonstrated a direct involvement of IgA in myelin lesions.

Demyelinating X-linked Charcot-Marie-Tooth disease: unusual electrophysiological findings.

X-linked Charcot-Marie-Tooth disease (CMT-X) is caused by mutations of connexin-32 (Cx-32), which encodes a gap-junction protein. Whether the neuropathy is primarily demyelinative or axonal remains to be established. We report findings of prominent demyelination in a 71-year-old woman with late-onset disease.

Ultrastructural protein zero expression in Charcot-Marie-Tooth type 1B disease.

Charcot-Marie-Tooth type 1B (CMT 1B) disease, an inherited demyelinating peripheral neuropathy, results from different point mutations located in the P0 gene on chromosome 1 q21-23. We have quantified, at the ultrastructural level, the immunocytochemical expression of the P0 protein in two unrelated CMT 1B patients with mutations (Ser 78 to Leu and Asn 122 to Ser) located in two different exons in the extracellular domain of the protein. A twofold decrease in P0 expression was observed in compact myelin in each case, compared with age-matched controls.

Electroencephalographic and polygraphic features of 24-hour recordings in sleeping sickness and healthy African subjects.

Electroencephalographic (EEG) and polygraphic features were analysed in six healthy control subjects and eight patients suffering from sleeping sickness meningoencephalitis in order to determine possible functional relationships. One patient was disqualified because of intermittent metabolic disease. Twenty-four h polygraphic recordings-EEG, electrooculography (EOG), electromyography (EMG), nasal and buccal air flow, chest respiratory movements-were performed continuously both on paper and on cassette tapes.

Ultrastructural PMP22 expression in inherited demyelinating neuropathies.

Charcot-Marie-Tooth type 1A (CMT-1A) disease results from a duplication of the PMP22 gene on chromosome 17p11.2. A deletion of the same region causes hereditary neuropathy with liability to pressure palsies (HNPP).

Survival prediction in sporadic amyotrophic lateral sclerosis. Age and clinical form at onset are independent risk factors.

Amyotrophic lateral sclerosis is a progressive neurological disease of unknown etiology and fatal outcome. Patient management can be aided by careful assessment of prognostic factors. A prospective study of 158 patients was carried out to examine the prognostic significance of age and clinical form at onset.

[Early diagnosis of Guillain-Barré syndrome by electric stimulations of the nerve roots].

Ulnar nerve electrical stimulations from motor roots to wrist were used in the early Guillain-Barré syndrome (GBS), to improve the electrophysiological diagnosis yield. 22 patients with a GBS were investigated with this technique between 3 to 17 days after the onset. Conventional electrophysiological examination was sufficient to diagnose an Acute Inflammatory Demyelinating Polyneuropathy in 12 cases.

Non-Hodgkin malignant lymphomas and peripheral neuropathies--13 cases.

Non-Hodgkin's malignant lymphomas (NHML) are malignant lymphoid proliferations which may be of B or T cell type. Thirteen observations of an association between peripheral neuropathy and B type NHML are reported. None of the cases had evidence of meningeal propagation or neurotoxicity from chemotherapy.