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T Maisonobe

Publications and source records attributed to T Maisonobe.

77 records · Page 5Linked to original sources
PubMed1996

Chronic dysimmune demyelinating polyneuropathy: a clinical and electrophysiological study of 93 patients.

OBJECTIVES: To identify clinical, electrophysiological, and immunological characteristics of chronic immune demyelinating polyneuropathy to define for each group the appropriate therapeutic strategies. METHODS: The clinical and electrophysiological data and the response to treatment of 93 patients with an acquired chronic dysimmune demyelinating polyneuropathy (CDDP) studied over a period of 10 years were reviewed. Two groups were identified: group 1, comprising 64 patients with an idiopathic CDDP, of whom 13 had serum monoclonal or polyclonal gammopathy without detectable antibodies directed against the "myelin associated glycoprotein" (MAG), and group 2, comprising 29 patients with an IgM monoclonal gammopathy of undetermined significance (MGUS) with antibodies binding to the MAG. RESULTS: Group 1 patients had either a progressive or relapsing course. The relapsing course had more pronounced distal slowing of motor conduction velocity. In group 1, there were no significant clinical or electrophysiological differences between patients with or without gammopathy. Patients with anti-MAG antibody (group 2) differed significantly from group 1 patients, especially on the basis of electrophysiological results. They had a more pronounced slowing of peroneal motor nerve conduction velocity, a lower frequency of conduction block, and a distal accentuation of conduction slowing, distinguishing them from those with idiopathic CDDP, Charcot-Marie-Tooth polyneuropathy type 1A, and control subjects. CONCLUSION: The idiopathic CDDP group is heterogeneous with probably different subgroups. Patients with IgM MGUS polyneuropathy and anti-MAG antibodies have characteristics which distinguish them significantly from other CDDP and suggest different immune mechanisms and responses to treatment.

Adolescent↗
PubMed1996

[Chronic idiopathic polyradiculoneuritis. Clinical, electrophysiological and histopathological aspects].

Idiopathic chronic inflammatory polyradiculoneuropathies are probably due to dysimmune mechanisms. They form a distinct entity defined by clinical, electrophysiological, histopathological criteria and by their evolution and response to treatment. The homogeneity of that syndrome, the exact nature of the underlying pathological processus, the natural history and the responses to treatment are still debated. Need to more studies, especially of immunologic nature, to precise limits and criteria of such a syndrome. Chronic polyradiculoneuropathy is different from acute form mainly by evolution. Within the chronic form, the progressive and relapsing forms can be distinguished with different electrophysiologic characteristics. These 2 forms share the same electrophysiologic aspect of chronic demyelination which is characterized by slowing of nerve conduction, conduction block and temporal dispersion. They had some specific different features such as the heterogeneity of the conduction failure and the multifocal distribution of demyelinating lesions. The histopathologic lesions are those of demyelination-remyelination with some degree of axonal damage, although inflammatory infiltrates are not frequent. In the majority of cases, the motor deficit is dominant although pure sensory forms have been reported. In few cases, central nervous system involvement is described. Idiopathic polyradiculoneuropathies had different electrophysiologic features than polyneuropathies associated with IgM monoclonal gammopathy and anti-MAG activity, these later presenting more distal and homogeneous abnormalities.

Acute Disease↗
PubMed1995

Clinical, electrophysiologic, and molecular correlations in 13 families with hereditary neuropathy with liability to pressure palsies and a chromosome 17p11.2 deletion.

Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant disease characterized by recurrent episodes of acute nerve palsies. We performed a clinical, electrophysiologic, and molecular study of 13 French families with HNPP associated with a chromosome 17p11.2 deletion in 36 individuals. There were electrophysiologic abnormalities in all symptomatic (n = 28) and asymptomatic (n = 8) deletion carriers, even in childhood. Bilateral delayed distal motor latency of the median nerve at the wrist, reduced sensory velocity in the palm-wrist segment, and delayed distal motor latency or reduced motor velocity in the peroneal nerve was diagnostic in at-risk relatives. This large series confirms the reliability of molecular analysis combined with a simplified electrophysiologic examination for the diagnosis of HNPP associated with 17p11.2 deletion.

Adolescent↗
PubMedDate not supplied

[Pauci-symptomatic sensory polyneuropathy in Refsum's disease].

Refsum's disease is an autosomal recessive disease caused by defective alpha-oxidation of phytanic acid. The usual clinical presentation is the association of retinitis pigmentosa, ataxia and chronic severe sensorimotor polyneuropathy. A case of mild purely sensory neuropathy in a 40-year-old patient associated to high CSF protein level led to the diagnosis of Refsum's disease. The paucity of sensory symptoms and signs of neuropathy contrasted with severe reduction of motor and sensory nerve conduction velocities and markedly signs of sensory neuropathy observed in the nerve biopsy. Typical ring-scotomas, retinitis pigmentosa, anosmia, deafness, and high plasma phytanic acid level were present in extensive examination. There was no other case in the family.

Adult↗