Biomedical subjects
Publications and source records attributed to C Confavreux.
A 67-year old patient with meningoradiculitis due to Borrelia Burgdorferi presented with unilateral trunk dysesthesias and severe asymmetrical abdominal distension. The causes of abdominal wall paralysis are reviewed.
We reviewed retrospectively 47 patients with a Chiari malformation (CM) who had been admitted to the Clinique de Neurologie of Lyon from 1971 to 1991. The purpose of this report is to assess the possible influence on the frequency and the clinical pattern at presentation of CM since 1985 when Magnetic Resonance Imaging (MRI) became available to us. A diagnosis of CM was made for 21 patients before 1985 (group 1) and for 26 patients thereafter (group 2), i.e., a 2.5 increase of incidence of CM frequency (1.5 patients/year before 1985 vs 3.7 patients/year thereafter). This increase was even more striking for cases without syringomyelic symptoms and signs (SSS) (42.9% in group 1, vs 76.9% in group 2). The accuracy of clinical diagnosis was 68.1% for the total surveyed period (90.5% before 1985 and 50% thereafter). For cases with SS at presentation, a syringomyelic cavity was radiologically demonstrated in 80% of the cases in the pre-MRI period and 90.9% of the cases since MRI was available. On the other hand, when a syringomyelic cavity was radiologically demonstrated, there was a related clinical SS in 70% of group 1 patients and 50% of group 2 patients.
A twenty-five year old woman developed a progressive right hemiparesis which remitted within three months, without treatment. The diagnosis was a first relapse of multiple sclerosis. After a 10 year fully asymptomatic period, the patient developed weakness of the legs with falls and swallowing difficulties. Fasciculations and amyotrophy were present in the limbs and the tongue. There were no sensory abnormalities. The electromyogram confirmed the peripheral neurogenic degeneration with signs of anterior horn involvement. Motor and sensory nerve conductions were normal. Muscle weakness and atrophy increased in the limbs and the bulbar territory and the patient died nine months later. The autopsy showed characteristic "old" plaques of multiple sclerosis in the cerebrum with anterior horn cell and pyramidal tracts degeneration, typical of amyotrophic lateral sclerosis, in the spinal cord. Although exceptional, this association of amyotrophic lateral sclerosis and multiple sclerosis leads to the discussion of an etiological immunological dysregulation common to these two diseases.
Zinc treatment of Wilson's disease was introduced by Schouwink en 1961 and is still uncommon in France. We evaluated the effectiveness and safety of zinc in 5 patients with Wilson's disease aged from 19 to 40 years. There were three neurological, one hepatic and one asymptomatic cases. Zinc was administered in doses of 120 to 272 mg/day, alone in 3 cases and combined with D-penicillamine in 2 cases. After 1 to 7 years of zinc therapy, our experience is consistent with data from recent literature and provides further evidence of zinc effectiveness. Zinc may be prescribed as first treatment in most patients, including asymptomatic cases. The only exception concerns patients with severe symptoms in whom it is recommended to combine zinc with D-penicillamine during the early phase of treatment for more rapid effectiveness. Because of its safety, zinc is particularly indicated in cases of intolerance to D-penicillamine and trien.
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Twenty-seven out of 45 patients who had Wallenberg's syndrome were re-examined as out-patients. Nineteen were complaining of pain on the side opposite to that with loss or temperature and pain sensations or trigeminal deficit. The follow-up showed that pain was both intense and disabling and its treatment was disappointing. The most striking finding was the high frequency of painful sequelae to Wallenberg's syndrome, since they affected almost one out of two patients. The occurrence of these painful symptoms, usually after lesion of the spinothalamic tract, is difficult to explain.
We report a case of slowly progressive amusia and aprosody in association with orofacial and eyelid apraxias. The patient was independent in daily living activities. Insight, judgment, and behavior were intact. Her language was normal, and she demonstrated no limb, dressing, or constructional apraxia. She had no prosopagnosia, no visuospatial disturbances, and no memory impairment. Imaging studies (computed tomography, magnetic resonance imaging, single photon emission computed tomography) indicated a selective disorder of the right frontal and temporal regions. Review of the literature shows an increasing number of reports of this degenerative syndrome affecting the left dominant hemisphere and language areas, whereas cases of the syndrome affecting the right hemisphere are rare. To our knowledge, this is the first case in which aprosody and amusia were associated with a focal cortical degeneration.
EDMUS is a minimal descriptive record developed for research purposes to document clinical and laboratory data in patients with multiple sclerosis (MS). It has been designed by a committee of the European Concerted Action for MS, organised under the auspices of the Commission of the European Communities. The software is user-friendly and fast, with a minimal set of obligatory data. Priority has been given to analytical data and the system is capable of automatically generating data, such as diagnosis classification, using appropriate algorithms. This procedure saves time, ensures a uniform approach to individual cases and allows automatic updating of the classification whenever additional information becomes available. It is also compatible with future developments and requirements since new algorithms can be entered in the programme when necessary. This system is flexible and may be adapted to the users needs. It is run on Apple and IBM-PC personal microcomputers. Great care has been taken to preserve confidentiality of the data. It is anticipated that this "common" language will enable the collection of appropriate cases for specific purposes, including population-based studies of MS and will be particularly useful in projects where the collaboration of several centres is needed to recruit a critical number of patients.
A series of 43 cases with multiple cranial nerve deficits was collected between 1972 and 1990. No diagnosis was established in 15 cases. The facial and the trigeminal nerves were most frequently affected. In 10 cases, a monophasic course was observed usually with recovery. Recurrence was present in 5 cases. An inflammatory mechanism was likely in 10 cases. Nosological relations between these cases and either Bell's palsy (idiopathic facial paralysis) or Tolosa-Hunt's syndrome are discussed.
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Three cases of bilateral deafness with cytologically-demonstrated meningeal carcinomatosis are reported. The first patient, a 64-year old man, presented with bilateral deafness, gait disturbances, and bilateral facial paresis. The second patient, a 78-year-old man, had bilateral deafness, unsteady gait and fluctuations in consciousness. The last patient, a 69-year-old man, complained of bilateral deafness and severe headache, and presented with right facial paresis and left laterodeviation while walking. All three patients had abnormal cochleo-vestibular findings and brainstem auditory evoked responses (BAER) that suggested peripheral lesions with absent or very delayed I waves. The brain CT scans with an without contrast enhancement were entirely normal, and the diagnosis was established by lumbar puncture. From our own cases and a review of the literature, deafness in meningeal carcinomatosis may start unilaterally but becomes bilateral in less than a week. Vestibular disturbances may not be apparent, but they can be demonstrated in almost all cases. Facial paresis or plegia is also a very frequent finding. The destruction of the eighth and seventh cranial nerves is probably due to direct infiltration by neoplastic cells as well as to ischemia through compression of the nerve supplying vessels. The 3 cases presented here emphasize once again the important fact that meningeal carcinomatosis remains a cytological diagnosis, several lumbar punctures being sometimes necessary, and that cerebrospinal fluid studies cannot yet be supplanted by other diagnostic techniques like contrast-enhanced CT or MRI with gadolinium.
The risk of developing Multiple Sclerosis is under the combined influence of environmental and endogenous factors, as shown by epidemiological and genetic studies. Immunological abnormalities are a salient feature of the disease but their myelinotoxic effect is still to be demonstrated. The pathogenesis of the disease remains unknown. The auto-immune theory as well as the infectious theory are arguable but they may, also, be both valid. Multiple sclerosis could be a post-infectious auto-immune disease, "final common way" of common childhood infections. Anyway, no infectious agent is demonstrable inside the central nervous system at the overt disease stage.
Typical cauda equina syndrome secondary to long-standing ankylosing spondylitis is reported in a 63-year-old man. Radionuclide cisternography demonstrated a resorption defect of cerebrospinal fluid in the enlarged lumbosacral dural sac. After transient symptomatic improvement with acetazolamide, a lumboperitoneal shunt was placed. The rate of cerebrospinal fluid, isotope resorption became normal. In the 5 years of follow-up, partial remission has been observed.
A 37-year-old man presented with an acute amnestic syndrome of Korsakoff's type and an upper brain-stem oculomotor syndrome. After a moderate improvement with steroid therapy, he developed progressive behavioural changes due to a frontal lobe syndrome, in addition to motor and visual impairment. Memory performance was investigated on several occasions during an 11-year follow-up. Diagnosis of laboratory-supported definite multiple sclerosis was established and magnetic resonance imaging showed disseminated white matter lesions, especially in both medial temporal lobes. No other cause than multiple sclerosis was found for the amnestic syndrome. Among the rare cases of Korsakoff's syndrome in the course of multiple sclerosis, this is to our knowledge the first case in which a memory deficit was the initial manifestation of the disease.
A case of subdural arachnoid cyst of the thoracic spine was studied by magnetic resonance imaging (MRI), myelography and CT myelography. Myelography and especially CT myelography suggested the diagnosis; MRI established it, showing the communication between the cyst and the subarachnoid space. Final characterization was based on surgical findings and pathological examination.
The P.O.E.M.S. syndrome is a rare clinical entity that has been described mainly in Japan. It is characterized by a progressive polyneuropathy with raised CSF protein content, organomegaly, endocrinopathy, skin changes and plasma cell dyscrasia. We report a new documented case associated with a solitary plasmocytoma of the 12th thoracic vertebra. Immunopathological and ultrastructural studies of the peripheral nerve did not disclose any immune-specific changes. Surgery and radiation therapy of the plasmocytoma allowed a complete recovery, with a 5-year follow-up. This case report illustrates the need for serial full skeletal survey, including scintigraphy, in middle-aged patients with progressive polyneuropathy of obscure origin.
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