[Report from the 58th annual meeting of the American Academy of Neurology].
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Academies and Institutes↗
Biomedical subjects
J M Léger
Publications and source records attributed to J M Léger.
European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society.
Numerous sets of diagnostic criteria have sought to define chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and randomized trials and systematic reviews of treatment have been published. The objective is to prepare consensus guidelines on the definition, investigation and treatment of CIDP. Disease experts and a patient representative considered references retrieved from MEDLINE and Cochrane Systematic Reviews in May 2004 and prepared statements which were agreed in an iterative fashion. The Task Force agreed on good practice points to define clinical and electrophysiological diagnostic criteria for CIDP with or without concomitant diseases and investigations to be considered. The principal treatment recommendations were: (1) intravenous immunoglobulin (IVIg) or corticosteroids should be considered in sensory and motor CIDP (level B recommendation); (2) IVIg should be considered as the initial treatment in pure motor CIDP (Good Practice Point); (3) if IVIg and corticosteroids are ineffective plasma exchange (PE) should be considered (level A recommendation); (4) If the response is inadequate or the maintenance doses of the initial treatment are high, combination treatments or adding an immunosuppressant or immunomodulatory drug should be considered (Good Practice Point); (5) Symptomatic treatment and multidisciplinary management should be considered (Good Practice Point).
Humans↗
European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of paraproteinaemic demyelinating neuropathies: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society.
BACKGROUND: Paraprotein-associated neuropathies have heterogeneous clinical, neurophysiological, neuropathological and haematological features. Objectives. To prepare evidence-based and consensus guidelines on the clinical management of patients with both a demyelinating neuropathy and a paraprotein (paraproteinaemic demyelinating neuropathy, PDN). METHODS: Search of MEDLINE and the Cochrane library, review of evidence and consensus agreement of an expert panel. RECOMMENDATIONS: In the absence of adequate data, evidence based recommendations were not possible but the panel agreed the following good practice points: (1) Patients with PDN should be investigated for a malignant plasma cell dyscrasia. (2) The paraprotein is more likely to be causing the neuropathy if the paraprotein is immunoglobulin (Ig)M, antibodies are present in serum or on biopsy, or the clinical phenotype is chronic distal sensory neuropathy. (3) Patients with IgM PDN usually have predominantly distal and sensory impairment, with prolonged distal motor latencies, and often anti-myelin associated glycoprotein antibodies. (4) IgM PDN sometimes responds to immune therapies. Their potential benefit should be balanced against their possible side-effects and the usually slow disease progression. (5) IgG and IgA PDN may be indistinguishable from chronic inflammatory demyelinating polyradiculoneuropathy, clinically, electrophysiologically, and in response to treatment. (6) For POEMS syndrome, local irradiation or resection of an isolated plasmacytoma, or melphalan with or without corticosteroids, should be considered, with haemato-oncology advice.
Advisory Committees↗
Immunosuppressant and immunomodulatory treatments for multifocal motor neuropathy.
BACKGROUND: Multifocal motor neuropathy is a distinct clinical entity characterised by progressive, predominantly distal, asymmetrical limb weakness and minimal sensory abnormality. The diagnostic feature of this condition is the presence of multiple partial motor nerve conduction blocks. Controlled trials have demonstrated the efficacy of regular intravenous immunoglobulin infusions. Immunosuppressive agents have been used as primary, second-line or adjunctive agents for its treatment. This review was undertaken to identify and review systematically randomised controlled trials of immunosuppressive agents. The use of intravenous immunoglobulin will be the subject of a separate review. OBJECTIVES: To provide the best available evidence from randomised controlled trials on the role of immunosuppressive agents for the treatment of multifocal motor neuropathy. SEARCH STRATEGY: We searched the Cochrane Neuromuscular Disease Group trials register for all trials of multifocal motor neuropathy published, using 'multifocal motor neuropathy' OR 'chronic inflammatory demyelinating polyradiculoneuropathy' OR ' conduction block' OR ' motor neuropathy' AND 'immunosuppressive agents', 'immunosuppressants', 'corticosteroids', 'plasma exchange', 'azathioprine', 'cyclophosphamide', 'cyclosporin', 'ciclosporin', 'methotrexate', and 'mycophenolate', 'immunomodulatory agents', 'interferon', 'total lymphoid irradiation' or 'bone marrow transplantation' as search terms. In addition we searched MEDLINE, EMBASE for 2000 and 2001 and CINAHL, LILACS for all years. We updated the register search in February 2004 and searched MEDLINE (January 1966 to end May 2004) and EMBASE (January 1980 to end May 2004). SELECTION CRITERIA: All randomised controlled trials and quasi-randomised clinical trials in which allocation was not random but was intended to be unbiased (e.g. alternate allocation) were to have been selected. Since no such trials were discovered, all prospective and retrospective case series were included in the 'background' or 'discussion' sections of the review. DATA COLLECTION AND ANALYSIS: All studies on multifocal motor neuropathy or lower motor neuron weakness with conduction block and no sensory abnormality were scrutinised for data on patients treated with any form of immunosuppressive agents besides intravenous immunoglobulin. The information on the outcome of treatment was then collated and summarised. MAIN RESULTS: We found no randomised controlled trials of any immunosuppressive agents for multifocal motor neuropathy. We summarised the results of retrospective and prospective case series in the discussion of the review. AUTHORS' CONCLUSIONS: There are no randomised controlled trials to indicate whether immunosuppressive agents are beneficial in multifocal motor neuropathy.
Humans↗
[Report from the 57th Annual Meeting of the American Academy of Neurology].
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Arteriosclerosis↗
[Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic strategy. Guidelines of the French CIDP study group].
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) comprises a group of dysimmune neuropathies easily diagnosed in more than half of the patients. Diagnosis is based on clinical, electrophysiological and biological clues. In some patients, diagnosis is unclear because of the debated value of the available clues. In such circumstances, dysimmune neuropathies may not be diagnosed, leading to insufficient treatment. This is an important category of patients because immunomodulatory drugs have proven efficacy. The CIDP spectrum includes a relatively wide range of diseases. Besides the easily recognized classic forms, there are many clinical variants, sometimes with a paucisymptomatic presentation leading to uncertain diagnosis. The French CIDP study group has established guidelines for diagnostic strategy in CIDP patients. The first part of this paper is devoted to the clinical aspects of the disease, classical forms and variants. In the second part, the results of electrophysiological studies are reported. In a third chapter, complementary examinations useful for diagnosis are discussed. The fourth chapter deals with the diagnostic strategy, discussed in relation to the different situations which may be encountered in clinical practice. details the technical modalities of appropriate electrophysiological studies and presents normal results together with those indicating demyelinating neuropathy. Nerve biopsy technique and results are given in appendix II.
Humans↗
[Epidemiologic, clinical, and etiologic features of acute delirious episodes (ADE) in Togo: findings of a hospital survey].
This transverse descriptive epidemiologic study carried out in a hospital setting in Togo over a period of 80 days included a total of 63 patients presenting acute delirious episodes. There were 25 women and 38 men ranging in age from 18 to 45 were included. Most patients were young (20 to 34 years) and presented symptoms comparable to those described elsewhere in France and Africa. However several particularites were noted in this fairly protected traditional rural setting in which delirious episodes are uncommon as compared to urban settings in which such cases are more common due to sociocultural stress, drug addiction, and HIV infection.
Acute Disease↗
Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndrome.
Lewis-Sumner syndrome (LSS) is a dysimmune peripheral nerve disorder, characterized by a predominantly distal, asymmetric weakness mostly affecting the upper limbs with sensory impairment, and by the presence of multifocal persistent conduction blocks. The nosological position of this neuropathy in relation to multifocal motor neuropathy (MMN) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is still debated. We report the clinical, biological and electrophysiological features, the course and the response to treatment in 23 LSS patients. The initial symptoms started in the distal part of an upper limb in 70% of patients. They were sensorimotor in 65% and purely sensory in 35% of patients. A cranial nerve involvement was observed in 26% of patients and a distal limb amyotrophy in 52%. The CSF protein level was normal in 67% of patients and mildly elevated in the remainder. None had serum anti-GM1 antibodies. There were multiple motor conduction blocks (average of 2.87/patient), predominantly located in the forearm, whereas demyelinating features outside the blocked nerves were rare. Abnormal distal sensory potentials were found in 87% of patients. The electrophysiological pattern suggests a very focal motor fibre demyelination sparing the nerve endings, whereas sensory fibre involvement was widespread. The course was chronic progressive in 71% of patients and relapsing-remitting in the others. During the follow-up study (median duration of 4 years), half of the patients progressed with a multifocal pattern and the distribution of the motor deficit remained similar to the initial presentation. The other patients showed a progression to the other limbs, suggesting a more diffuse process. Fifty-four percent of the patients treated with intravenous immunoglobulin showed an improvement, compared with 33% of the patients treated with oral steroids. Overall, 73% of patients had a positive response to immune-mediated therapy. LSS may be distinguished from MMN by the presence of sensory involvement, the absence of serum anti-GM1 antibodies and, in some cases, a positive response to steroids. In some of the patients in our study, LSS evolved into a more diffuse neuropathy sharing similarities with CIDP. Others had a clinical course characterized by a striking multifocal neuropathy, which suggests underlying mechanisms different from CIDP. Overall, whatever the clinical course, LSS responded to immune-mediated treatment in a manner similar to CIDP.
Action Potentials↗
[Report from the 56th annual meeting of the American Academy of Neurology].
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Ataxia↗
[Value of electrophysiology in the follow-up of dysimmune polyneuropathies].
Electrophysiological follow-up of dysimmune neuropathies leads to consider various situations depending on the type of neuropathy. In Guillain-Barré syndrome, studies have highlighted the usefulness of early measurements of distal motor latencies and late responses at onset of paralysis, and of motor conduction velocities (MCVs), conduction blocks, sensory nerve action potentials and possible signs of axonal degeneration at the plateau of symptoms. However, none of these signs are predictive of clinical recovery, except early appearance of axonal degeneration which often indicates poor prognosis. In chronic inflammatory idiopathic demyelinating polyneuropathies, the follow-up mostly relies on motor conduction studies: increase or slowing of MCVs, shortening or prolongation of distal motor latencies, improvement or lengthening/disappearance of late responses and, more ancillary, sensory nerve action potential changes. Signs of axonal degeneration can worsen the prognosis. In multiple motor neuropathies with persistent conduction blocks, the most relevant electrophysiological criterion is increase or reduction of conduction blocks, as well as occurrence of new blocks and signs of axonal degeneration. In neuropathies associated with IgM monoclonal gammopathy of undetermined significance (MGUS) with anti-MAG antibodies, the electrophysiological follow-up mostly relies on the comparison between MCVs and distal motor latencies, as well as improvement or worsening of sensory nerve action potential amplitudes, and possible signs of axonal degeneration. In all those neuropathies however, therapeutic choices remain largely dependent on clinical scores, which have become increasingly standardized in recent years, although scales used by different groups are not universally accepted.
Chronic Disease↗
Collapsing cristobalitelike structures in silica analogues at high pressure.
The cristobalitelike forms of the ternary silica analogues BPO4 and BAsO4 were investigated at high pressure by x-ray diffraction and theoretical methods. The behavior of these compounds represents an extreme case in which the tilt angle of the constituent tetrahedra increases in a spectacular way at high pressure resulting in a major change in topology from a cristobalitelike framework towards a "collapsed cristobalite" structure. These compounds provide the first examples of the collapse of a framework structure to a close-packed form in a continuous manner without an intervening phase transition.
Journal Article↗
[Report of the 55th meeting of the American Academy of Neurology. Honolulu, March 29-April 6, 2003].
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Adult↗
Immunosuppressive treatment for multifocal motor neuropathy.
BACKGROUND: Multifocal motor neuropathy is a distinct clinical entity characterised by progressive, predominantly distal, asymmetrical limb weakness and minimal sensory abnormality. The pathognomonic feature of this condition is the presence of multiple partial motor nerve conduction blocks. Controlled trials have demonstrated the efficacy of regular intravenous immunoglobulin infusions. Immunosuppressive agents have been used as primary, second-line or adjunctive agents for its treatment. This review was undertaken to identify and review systematically randomised controlled trials of immunosuppressive agents. The use of intravenous immunoglobulin will be the subject of a separate review. OBJECTIVES: To provide the best available evidence from randomised controlled trials on the role of immunosuppressive agents for the treatment of multifocal motor neuropathy. SEARCH STRATEGY: The Cochrane Neuromuscular Disease Group register was searched for all trials of multifocal motor neuropathy published, using 'multifocal motor neuropathy' OR 'chronic inflammatory demyelinating polyradiculoneuropathy' OR ' conduction block' OR ' motor neuropathy' AND 'immunosuppressive agents', 'immunosuppressants', 'corticosteroids', 'plasma exchange', 'azathioprine', 'cyclophosphamide', 'cyclosporin', 'methotrexate', and 'mycophenolate', 'immunomodulatory agents', 'interferon', 'total lymphoid irradiation' or 'bone marrow transplantation' as search terms. In addition we searched MEDLINE, EMBASE for 2000 and 2001 and CINAHL, LILACS for all years. SELECTION CRITERIA: All randomised controlled trials and quasi-randomised clinical trials in which allocation was not random but was intended to be unbiased (e.g. alternate allocation) were to have been selected. Since no such trials were discovered, all prospective and retrospective case series were included in 'background' or 'discussion' sections of the review. DATA COLLECTION AND ANALYSIS: All studies on multifocal motor neuropathy or lower motor neuron weakness with conduction block and no sensory abnormality were scrutinized for data on patients treated with any form of immunosuppressive agents besides intravenous immunoglobulin. The information on the outcome of treatment was then collated and summarised. MAIN RESULTS: We found no randomised controlled trials of any immunosuppressive agents for multifocal motor neuropathy. We summarised the results of retrospective and prospective case series in the discussion of the review. REVIEWER'S CONCLUSIONS: There are no randomised controlled trials to indicate whether immunosuppressive agents are beneficial in multifocal motor neuropathy.
Humans↗
[Neurological manifestations in cryoglobulinemia].
Cryoglobulins are immunoglobulins that persist in the serum, precipitate at cold temperature and resolubilize when rewarmed. Type I is often associated with hematological disorders. Type II and III are mixed cryoglobulins, composed of different immunoglobulins with a monoclonal component in type II and only polyclonal immunoglobulins in type III. Mixed cryoglobulins are associated with connective tissues or infectious diseases. Hepatitis C virus is involved in most of previously called "essential" mixed cryoglobulinemia. Dermatological, rhumatological, and nephrological manifestations are the most frequent, and neurological complications are found in 20 percent of cases. However, in mixed cryoglobulinemia with low cryoglobulins level, neurological signs may reveal the disease. Ischemic central nervous system complications are rare, but sensory, axonal, peripheral neuropathies or sensory and motor multiple mononeuropathies are more frequent. Prognostic and treatment depend on association with hepatitis C, and presence of vasculitis lesions in the nerve biopsy.
Central Nervous System Diseases↗
Crystalline post-quartz phase in silica at high pressure.
alpha-quartz, which has been reported to undergo pressure-induced amorphization, was found to transform to a monoclinic, crystalline phase when compressed to 45 GPa at room temperature in a close to hydrostatic, helium pressure medium. The x-ray powder diffraction data obtained could be indexed based on a monoclinic cell, and the intensities are in agreement with a P2(1)/c model structure built up of 3x2 zigzag chains of SiO6 octahedra. This new polymorph of silica, which is metastable under ambient conditions, has been isolated for the first time and is one of several possible competing dense forms containing octahedrally coordinated silicon.
Journal Article↗
Family, Alzheimer's disease and negative symptoms.
BACKGROUND: The aim of this study is to look at the correlation between the presence of apathy measured by Marin's scale and family complaints related to withdrawal and the loss of motivation, or depression. The multicentre study was performed on 58 non-demented elderly people, 132 outpatients with Alzheimer's-type dementia, as well as their main caregiver. METHODS: After agreement of the patients and the family, the patients were assessed using different scales: Cornell's for depression, Marin's for apathy, MMS for cognitive disorders, and IRG for dependence. At the same time, two self-administered questionnaires were given to the patients' families: one concerning a list of complaints scored from 1 to 4 relating to various disorders and the other addressing the boundary ambiguities translated from Boss' questionnaire. The 58 non-demented people were 81.20 years old+/-13.75. One hundred and thirty-two demented patients were included: 39 men and 93 women. The mean age was 79.47 years+/-9.03. RESULTS: The first family complaint relates to the loss of motivation (65%). Apathy and depression occur more frequently in dementia, in particular when the MMS is degraded. Depression and apathy attracted a high complaint score. In our study the score of boundary ambiguity is higher among patients with a weak cognitive status. A high level of ambiguity is accompanied by a high score of family complaints. When the family complaint concerning the loss of motivation is present, apathy is significantly more common. Family complaints about withdrawal and loss of motivation are frequently present, and are congruent with the actual presence of apathy in the patient. It bears witness to the distress felt by families faced with the loss of ability noted in the demented person. The family's difficulties are increased by the patient's depression.
Affective Symptoms↗
Neurological complications of primary Sjögren's syndrome.
OBJECTIVE: To better delineate the spectrum of neurological complications of primary Sjögren's syndrome (PSS). METHODS: A detailed neurological investigation was prospectively performed in a group of 25 consecutive patients with PSS followed in an internal medicine department between June 1996 and December 1997 (Internal Medicine group). In addition, eleven patients with neurological complications of PSS were identified in the Neurological Department of the same institution during the same period (Neurological group). RESULTS: In the Internal Medicine group, neurological complications were discovered in 10/25 (40%) patients. Peripheral nervous system involvement was present in 4/25 patients from the Internal Medicine group and in 10/11 patients from the Neurological group and consisted mainly of axonal sensorimotor/sensory polyneuropathy. A motor neuron syndrome was identified in two patients. CNS involvement occurred in 7/25 patients from the Internal Medicine group and in 4/11 patients from the Neurological group. Three patients had spinal cord involvement. Cognitive dysfunction was the most frequent finding (5/25 in the Internal Medicine group, 3/11 in the Neurological group) characterized either by subcortical or corticosubcortical dysfunction. Cognitive impairment was not attributed to mood disturbance and was not associated with specific laboratory or radiological abnormalities. CONCLUSION: Neurological complications of PSS are frequent since they were present in 40% (10/25) of patients in a consecutive series of patients from a department of Internal Medicine. Although PNS involvement predominates, complications of PSS affecting the brain or spinal cord are not rare, with subcortical dysfunction as the main finding.
Adult↗
[Peripheral neuropathy and hepatitis C virus infection: more than cryoglobulinemia].
PURPOSE: To study characteristics of peripheral neuropathies associated with hepatitis C virus infection. METHODS: A study of two groups of four patients with peripheral neuropathy and infected with hepatitis C virus, and a literature review. RESULTS: We observed four cases of peripheral neuropathy in patients with hepatitis C virus infection with persistent negativity of mixed cryoglobulinemia, and with no purpura, rheumatoid factor, or low C4 level. Neuropathy improved with treatment in three patients between 8 and 28 months after treatment was begun. We report four other observations of peripheral neuropathy with mixed cryoglobulinemia. Worsening of peripheral neuropathy was observed 2 to 13 weeks after initiation of interferon alpha despite improvement of hepatic parameters. After stopping interferon, peripheral neuropathy worsened (one case), stabilized (one case), or improved (two cases). DISCUSSION: In patients infected with hepatitis C virus, peripheral neuropathy can be due to mixed cryoglobulinemia, periarteritis nodosa, and perhaps hepatitis C virus itself as suggested by our first observations. Low doses of interferon alpha may worsen peripheral neuropathy in patients with mixed cryoglobulinemia, as suggested in our later observations.
Adult↗