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Biomedical subjects

B Pautrizel

Publications and source records attributed to B Pautrizel.

5 recordsLinked to original sources

[Wilson's disease: demonstration of lesions of the cortex and white matter by MRI].

Lesions of cortex and white matter are present in Wilson's disease in approximately 10 p. cent of cases. Before Magnetic Resonance Imaging (MRI) only post-mortem examination evidenced them. We report the case of a 15 year-old girl in whom Wilson's disease was diagnosed after partial motor epileptic seizures. She later presented with progressive extrapyramidal and pyramidal signs, severe cognitive weakening and disorder of behaviour. MRI clearly showed cortical and subcortical lesions by an enhanced signal (T2 values). It showed as well the classical picture of lesion in the lenticular nuclei. With T1 values it showed an extensive cortical atrophy and a widening of the ventricules.

Adolescent

[Hypermyelination in a case of peripheral neuropathy with benign IgM monoclonal gammopathy].

A 65 year-old man presented with a 8 months history of ataxia and weakness of the lower limbs. Acroparesthesias had been present for ten years in all four limbs. Serum protein electrophoresis showed a monoclonal IgM kappa spike. Bone marrow showed no increase in plasma cells. Motor conduction velocities were reduced. A biopsy from the right superficial peroneal nerve revealed several enlarged myelin sheaths in each fascicle on semi-thin sections. Under electron microscopy these large myelin sheaths were either regularly arrayed around the axon or formed several large additional loops. The outer lamellae of these abnormal myelin sheaths and of the additional loops were frequently widened. Other fibers showed a widening of some myelin lamellae (W.M.L.) with a myelin sheath of varying thickness, and these widened lamellae were frequently joined together. A direct immunopathological study could not be made. The widening of some myelin lamellae is present in most cases of peripheral neuropathy with IgM monoclonal gammopathy and a fixation of anti IgM serum on some myelin sheaths may be demonstrated by direct immunopathology. Hypermyelination has been reported in a few cases. In this patient hypermyelination was as developed as in typical cases of tomaculous neuropathy. An association in some myelin sheaths of hypermyelination and W.M.L. has been noted in serum induced myelin aberrations in peripheral nervous system cultures.

Aged

Adult dementia due to intraneuronal accumulation of ceroidlipofuscinosis (Kufs' disease): ultrastructural study of two cases.

Two cases, a man and a woman, presented the initial signs of progressive dementia at about age 40 years. Aphasia, facial dyskinesias, and ataxia were associated with behavioral disturbances in the first patient. Aphasia, oculomotor disturbances, and ataxia coexisted with dementia in the second patient. In both cases, ultrastructural examination of a cerebral biopsy showed intraneuronal granular osmiophilic deposits mixed with lamellar structures and fingerprint profiles. Such deposits are characteristic of Kufs' disease. In the first patient, brain autopsy was performed 3 years after cerebral biopsy. Our two cases presenting dementia with motor disturbances correspond to type B Kufs' disease.

Adult

Opiate withdrawal and electro-stimulation. Double blind experiments.

A rapid detoxification technique for heroin addicts is described. The technique uses an impulsional electric current developed by Limoge. The treatment is nonaggressive, very well tolerated by the addicts and leads to a good psychotherapeutic relationship with the medical staff. A successful physical detoxification is achieved in more than 80% of the cases (this figure is based on 400 patients treated by the method). The technique was subjected to two double-blind experiments. The first experiment tested the real efficacity of the electrical stimulation. The difference between the group of subjects stimulated and the unstimulated control group had a "p-value" which was significant at the 0,5% level. This leads us to believe that the electrical stimulation has a real positive effect. The second experiment reconfirmed the efficacity of the stimulation and showed that 24 hour continuous stimulation was not sufficient to produce a lasting effect. After about 50 hours stimulation a Naloxone test produced little or no reaction in the patients.

Adult