[Meningeal syndrome. Diagnostic orientation and management].
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Biomedical subjects
Publications and source records attributed to C Confavreux.
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Thirty-two consecutive cases of hypoglossal nerve palsy (excluding syringomyelia and amyotrophic lateral sclerosis) collected between 1971 and 1987 were reviewed. The XIIth nerve palsy was clinically isolated in 8 cases, associated with other cranial nerve palsies in 16 cases and with long tracts involvement in 8 cases. Seventeen cases were related to tumours. Malignant tumours were predominant, especially middle and posterior fossa bone metastases. Carcinomatous meningitis and brainstem glioma were also found, as well as lymphoproliferative disorders and benign tumours such as chemodectoma and neurinoma. A vascular origin was established in 6 cases, related to vertebrobasilar infarct, truncular ischaemia and internal carotid dissection. The paralysis was consecutive to head or neck trauma in 4 cases and to various inflammatory processes in 4 other cases. The last case was caused by Chiari's malformation. To our knowledge, this is the first aetiological review of XIIth nerve palsy in the literature.
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We report the case of myasthenia gravis in a 74-year-old woman, 2 weeks after acebutolol had been introduced as the sole treatment for systemic hypertension. The disease developed at a fulminant and fatal course despite drug withdrawal. The possibilities of coincidental association between the two events, triggering of a preexisting latent myasthenia gravis or full induction of the disease by acebutolol, are discussed.
A 33 year-old woman with multiple sclerosis developed bilateral palatal myoclonus. Rotatory pendular nystagmus and gaze-evoked nystagmus were present and analyzed by electro-oculographic recordings. Magnetic resonance imaging demonstrated abnormal signals in the pons. To our knowledge, this is the first report of palatal myoclonus in the course of a definite case of multiple sclerosis.
Three patients with adult-onset neuronal-ceroid lipofuscinosis (Kufs' disease) are presented. Two cases were familial (autosomal recessive inheritance) and 1 case was possibly sporadic. The main clinical feature was progressive deterioration of cognitive functions. In 2 siblings, aged 37 and 41, dementia was associated with personality and behaviour changes, suggesting a psychotic disorder and with dysarthria and tic-like dyskinetic movements. In a third patient, dementia was only associated with an asymptomatic pigmentary retinal degeneration. CT scan revealed diffuse cerebral atrophy in all cases. Diagnosis was established by brain biopsy in 2 cases. Autofluorescence emission and absorption spectra from the abnormal pigment was studied and was not found contributive.
A 67-year old woman presented with isolated and bilateral external ophthalmoplegia of the myasthenic type following a subacute course and resistant to anticholesterinases and corticosteroids. A biological hyperthyroidism with immunological markers was discovered. An intensive care of corticosteroids was partially effective, but fatal bone marrow aplasia attributed to carbimazole developed. Pure ophthalmoplegia without local signs of inflammation may be the sole manifestation of dysthyroid orbitopathy.
Neck pain and torticollis are very common symptoms, but they may reveal an intracranial or cervical neurological disease. Such diseases are easily diagnosed when these two symptoms are associated with others, but in some rare cases, and especially when it is isolated, torticollis may be mistaken for common neck pain. This is what happened in 21 cases and in some patients the neurological disease had been present for a very long time before it was diagnosed. Prior to admission, most patients were treated with massages, cervical collar or manipulations which sometimes resulted in deterioration. A search for atypical features of neck pain and torticollis should avoid delays in diagnosis.
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There is an urgent need for individualized outcome estimates in multiple sclerosis (MS). This is a prerequisite for selecting appropriate therapies in a disease the outcome of which may vary between malignant and benign forms. This question was addressed by using probabilistic mathematical models. The disease course is described by movements of the patient's condition through well-defined disease states. The data used are based upon 278 reports of definite and probable MS cases collected over a 20-year period (1957-1976) at l'Hôpital Neurologique de Lyon. A Markov model was first prepared. However, it only provided an overall presentation of the disease which was not very meaningful for the individual patient, and limited studies of potential prognostic variables to dichotomous variables and univariate analyses. A stochastic survival model was then elaborated. It was complex at the theoretical level but in practice, easy to use. Personalized prognosis of a patient could be ascertained by combining several single potential prognostic variables and using their actual value in the case of quantitative variables. Although these models still need to be refined and have to be validated from the present state of the patients and other patient cohorts, they provide for the first time a global description of disease course likelihood and prognosis. The survival model may be adapted to each patient.
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Twenty nine cases of acute inflammatory brachial neuropathy were collected from 1969 to 1985. Only five cases could be considered as definite Parsonage Turner's "shoulder girdle" syndrome. The twenty four other cases were atypical with respect to this entity and were classified as symptomatic, evolutive, biological and/or associated forms. Symptomatic variants were present in twenty cases, consisting in distal or global motor deficit, or in painlessness. Evolution was atypical in four cases, with no recovery of motor deficit or relapsing course. Thirteen cases had abnormal CSF, usually with increase of protein content. Association with cutaneous manifestations or systemic diseases was found in nine cases. Beyond their individual differences, these twenty nine cases shared a common general profile of clinical presentation and evolution. This led to recognize the nosological relationships of these atypical cases with Parsonage-Turner's syndrome and to emphasize the similarities with Guillain-Barré syndrome.
Association between narcolepsy and HLA-DR2 antigen is the strongest so far described between an HLA antigen and a disease. Among 28 narcoleptic patients, we found two HLA-DR2 negative cases: a caucasoid woman also suffering from dystrophia myotonica and a negroid. All of our patients were HLA-DQW1 positive. An hypothetical narcolepsy susceptibility gene could be located in the HLA region, closer to the DQ than to the DR gene. It could be a pathologic allele of a sleep controlling gene in linkage disequilibrium with DQW1. Presence of DQW1 is a quasi-requisite for the expression of narcolepsy. It is not sufficient as it is observed in 70 p. 100 of controls.
A 31 year-old patient suffered from a subacute and major dementia, sixty months after whole brain irradiation with 54 grays for a pinealoma. Clinical features and biological investigations led to a diagnosis of hypothalamic insufficiency. A dramatic clinical recovery followed therapy with hydrocortisone and thyroxine. An hypothalamic radionecrosis and a vascular mechanism are presumed.
A questionnaire-based prevalence study was conducted in the Chalon-sur-Saône and Avignon areas, in the Rhône-Saône valley, France, to determine the frequency of multiple sclerosis. These areas are 300 km apart and lie on the 47 degrees and 44 degrees North parallels respectively. Age-adjusted prevalence rates on March 20, 1984 were 58.5 and 48.6 per 100,000 inhabitants respectively. There was no significant difference between the two areas. These preliminary data suggest that south-eastern France, as represented by Avignon, may fall within the high risk area for multiple sclerosis.
Follow-up data from a prognostic study done at L'Hôpital Neurologique de Lyon have been reanalyzed using a new methodologic approach. A stochastic survival model which allows the course of the disease to be described through the movement of patients from one disease state to another is presented. This approach extends previous modelling work by enabling the risk of transition between pairs of states to be written as a function of any number of discrete or continuous prognostic variables. Length of the first remission, sex, and mode of onset (mono- vs. polysymptomatic) were found to be significant prognostic indicators.
A case of a 33 year-old woman presenting an intrapetrous facial nerve neurinoma is reported. The neurological finding was a quickly progressive facial nerve palsy without hearing loss which the initial diagnosis was a Bell's palsy. The radiological findings showing an petrous bone erosion located at the third portion of the facial nerve gave the diagnosis. It was confirmed by CT scan and arteriography that allows a pre-operative embolisation. A one stage operation performed by a triple retromastoid, transpetrous and retroparotid approach, allowed a complete removal. The post-operative course was uneventful and a reconstructive facial surgery was performed to restore a facial symmetry six months later. The authors review the literature about this tumor which remains rare and point out two facts: --A non regressive or recurrent or atypical facial nerve palsy had to be checked up by neuroradiology and even by surgical exploration. --The triple retromastoid, transpetrous and retroparotid approach is very interesting, allowing a one stage complete removal of the tumor and a check up of the facial nerve repair possibilities.