Lack of evidence for a direct involvement of muscle infection by parvovirus B19 in the pathogenesis of inflammatory myopathies: a follow-up study.

OBJECTIVE: To clarify the association between parvovirus B19 and myositis. METHODS: Biopsy samples of muscle from eight patients with inflammatory myopathies were studied for the presence of B19 DNA by polymerase chain reaction. Expression of VP1 and VP2 capsid proteins was evaluated by immunohistochemistry. Interleukin 6 (IL-6) production was measured in the supernatant of myoblasts following incubation with parvovirus B19. RESULTS: In seven samples, detection of B19 DNA was negative. The expression of VP1 and VP2 capsid proteins was not observed by immunohistochemistry. In one patient, detection was transiently positive but became negative despite a flare-up of muscle disease. In vitro, parvovirus B19 was not able to induce IL-6 production by myoblasts. CONCLUSION: Our results do not support the direct implication of parvovirus B19 in the pathogenesis of myositis.

Survival rates of malignant gliomas in Burgundy from 1990 to 1995.

The aim of this study was to provide current data on case-fatality rates of malignant gliomas in the area of Burgundy (1,300,000 inhabitants). The ascertainment was specific according to imaging and histologic criteria, and was exhaustive because of the existence of a single University Hospital allowing both imaging and histologic diagnosis from stereotaxic cerebral biopsy. During six full years we collected 161 cases of malignant gliomas grades II to IV, 93 men (57.76%) and 68 women (42.23%). The mean age was 61 years. In all the age groups, there were a male predominance. Headache, epilepsy and motor deficit were the most frequent symptoms at onset. In most cases, the gliomas were localized within the frontal area. The post-surgical Karnofsky score was up to 70 in 40% of the cases. There were 89 glioblastomas with grade IV, 37 anaplastic astrocytomas, 13 gliomatous tumors with grade II and IV and six anaplastic oligodendrogliomas. This grading explains the very low survival rates, with a negative effect induced by age. Among the most relevant contributions of this study to the clinical features of malignant gliomas is an analysis of case-fatality rates, evaluation of health care services and therapeutic trials.

[Dysembryoplastic neuroepithelial tumors. A benign tumor cause of partial epilepsy in young adults]. / Tumeurs dysembryoplasiques neuroépithéliales. Une cause tumorale bénigne d'épilepsie partielle de l'adulte jeune.

Dysembryoplastic neuroepithelial tumor (DNT) is a newly recognized brain lesion first reported in 1988 by Daumas-Duport et al. The authors described five cases of DNT, that occurred in young people and were characterized by partial seizures. Seizures could become intractable and secondary generalised. Usually, the interictal neurological examination was normal. In most cases, computed tomography showed a supratentorial, "pseudocystic" low density appearance associated in some cases with calcific hyperdensity or focal contrast enhancement. Magnetic resonance imaging demonstrated a predominantly intracortical lesion. Common features included low signal intensity on T1-weighted images and high signal on T2-weighted images. Temporal and frontal lobes were mainly involved. Dysembryoplastic origin explained the clinical and radiological stability and the benign evolution of this tumor. Tumor resection was required only when epilepsy was intractable or when there was intracranial hypertension. Earlier intervention can prevent the physical and psychosocial damage resulting from chronic seizures and can improve the prognosis for these young patient. Histologically, DNT have been incorporated amond the category of neuronal and mixed neuronoglial tumors. Three patterns are described: a simple form with a unique glioneuronal element, a complex form with specific glioneuronal element, nodules being made of multiple variants looking like astrocytomas, oligodendrogliomas or oligo-astrocytomas, foci of dysplastic cortical disorganisation, and a non specific form. When specific glioneuronal composant is absent (50% of cases), the identification of DNT has therapeutic and prognostic implications because aggressive therapy may be avoided, sparing these young patients the long term effects of radio-or chemotherapy.

[Total permanent auricular paralysis. Review of the literature apropos of 109 cases]. / La paralysie auriculaire permanente totale. Revue de la littérature à propos de 109 cas.

Analysis of 109 well documented cases of permanent total atrial paralysis reported in the literature illustrated the features of this arrhythmia which is a well defined entity consisting of suppression of all electrical and mechanical activity of both atria lasting for more than 6 months. Standard electrocardiogram reveals junctional bradycardia of about 40 bpm without any visible P waves and narrow supraventricular QRS complexes in 80% of cases. This diagnosis can only be confirmed by meticulous bipolar endocavitary recordings exploring all atrial walls without recording an auriculogram and by right intra-atrial and coronary sinus stimulation which proves to be ineffective. This disease has a male predominance in two-thirds of cases and a familial nature in 18% of cases. Seventy one per cent of affected subjects are under the age of 50 years. In 33% of cases, it is associated with Emery-Dreifuss muscular dystrophy, in which it constitutes a specific sign allowing this dystrophy to be differentiated from all other forms, especially facio-scapulo-humeral myopathy, and in 30% of cases, it is associated with a degenerative disease such as diabetes, amyloidosis or primary cardiomyopathy. Idiopathic dilatation of the right atrium is revealed in 15% of cases. The arrhythmia is responsible for syncope or faintness in 31% of cases, cerebral vascular accidents in 21% of cases and heart failure in 35% of cases. Cardiac activation is dependent on a junctional escape rhythm. The mechanism of the lesion responsible is atrial fibrosis which may extend to the sinoatrial node. The treatment of choice consists of implantation of a VVI or VVIR mode cardiac pacemaker in combination with anticoagulant therapy.

[Multiple cerebral embolism in acute alcoholic intoxication. A pathological case]. / Embolies cérébrales multiples au cours d'une intoxication alcoolique aiguë. Un cas anatomo-pathologique.

A 37 year-old man sustained 2 ischemic strokes during acute alcohol intoxication. One month after the onset of strokes, a spontaneous atrial flutter occurred. Autopsy revealed an embolic rostral occlusion of the basilar artery. Cardiac verification did not show myocardiopathy or coronary disease. The relations between acute alcoholic intoxication and paroxystic cardiac rhythm disorders are discussed.

[Mental development in Duchenne muscular dystrophy. Correlation of data of the brain scanner]. / Le développement mental dans la dystrophie musculaire de Duchenne. Corrélation avec les données du scanner cérébral.

In an investigation of 15 patients with Duchenne muscular dystrophy, the authors found mental retardation in all cases. In addition, CT scan showed a cortical atrophy after the age of 10 years. This finding suggests that a cerebral degenerative process is present in cases of Duchenne muscular dystrophy.

[Epilepsy and bilateral occipital calcifications: 3 cases]. / Epilepsie et calcifications occipitales bilatérales: 3 cas.

We report 3 cases of epilepsy with bilateral occipital calcifications followed up for several years. These cases were compared with 21 published cases and were found to differ from the classical Sturge-Weber syndrome on several points: 1) the disease appeared around the age of 5 years and consisted of focal epilepsy without neurological or mental disorders; 2) the epilepsy was easy to control during 2 to 5 years. This was followed by a diffuse encephalopathy with severe, treatment-resistant epilepsy, Gerstmann's syndrome, optic ataxia, cerebellar syndrome and slow activity at EEG. It appears from these 3 cases that: 1) occipital calcifications may be unilateral at the onset of the disease; 2) visual evoked potentials are affected at a late stage, and 3) CT scans are of considerable value in the prognosis of benign epilepsy in childhood.

[Multiple sclerosis associated with biological symptoms of systemic lupus erythematosus. A case with anatomical study]. / Sclérose en plaques associée à une symptomatologie biologique évocatrice d'un lupus érythémateux disséminé. Une observation avec étude anatomique.

A clinical history typical of multisclerosis began in a 20-year old man with transient, then permanent manifestations involving the optic tract and the pyramidal, extrapyramidal and cerebellar systems. The patient died at the age of 62, at the end-stage of a complex clinical situation which included paraplegia, bilateral cerebellar syndrome, optic nerve atrophy, epileptic seizures and dementia. When the patient was 54 years old, laboratory findings suggestive of systemic lupus erythematosus (SLE) were discovered, namely: antinuclear, native anti-DNA, anti-Sm antibodies, circulating anticoagulant, cryoglobulinaemia and low complement level. These abnormalities persisted up to the patient's death, 8 years later, without any non-neurological sign of SLE. Post-mortem examination showed lesions of focal demyelination characteristic of multiple sclerosis, but no evidence of cerebral or extracerebral SLE. This case raises the problem of borderlines or associations between systemic lupus erythematosus and multiple sclerosis. In our case, as in other cases of "lupoid sclerosis" reported in the literature, there was a frank and isolated elevation of serum IgM levels.

[Adrenoleukodystrophy in the child and adrenomyeloneuropathy. Study of 2 families]. / Adrénoleucodystrophie de l'enfant et adrénomyéloneuropathie. Etude de deux familles.

Ten subjects from 2 families with adrenoleukodystrophy (ALD) and adrenomyeloneuropathy (AMN), hereditary X-linked diseases, were systematically explored. We performed endocrinological, biochemical assays and neurophysiological tests; the latter consisted of nerve conductions (CNV), Hoffmann's reflex and multimodal evoked potentials: visual (flash and pattern, VEP), brainstem auditory (BAEP) and somesthetic (SEP) using median nerve stimulation at the wrist. We only considered values above 2 SD. The purpose of our study was to determine the correlation between neurophysiological and endocrinological perturbations and the presence of pathological traits. Our results suggest that the correlation is high in diseased male patients, lower for the ALD carriers (BAEP, SEP and CNV were more frequently abnormal) and very low for the AMN carriers. Only the biochemical assays appeared to have any value for the characterization of female carriers of ALD and AMN.

Altered peripheral nerve conduction in HIV-patients.

An electrophysiological study on peripheral nerves conduction was performed on HIV-seropositive patients without neurological signs on clinical examination. Eight of the 28 patients (28%) had an infraclinical neuropathy, which was myelinic or axonal and rather distal than proximal. The mechanism of these involvements is not known, but their early existence could justify an early treatment even for asymptomatic patients.

Somatostatin 28 and neuropeptide Y innervation in the septal area and related cortical and subcortical structures of the human brain. Distribution, relationships and evidence for differential coexistence.

Somatostatin 28- and neuropeptide Y-containing innervations were mapped in the human medial forebrain (eight control brains) with immunohistochemistry, using the sensitive avidin-biotin-peroxidase method. Peptidergic perikarya and fibers had an extensive distribution: they were densest in the ventral striatum (nucleus accumbens, olfactory tubercle and bed nucleus of the stria terminalis) and infralimbic cortex, of intermediate density in the medial septal area and of lowest density in the dorsal and caudal lateral septal nucleus. Somatostatin-like immunoreactive perikarya and fibers were generally more numerous than the neuropeptide Y-like immunoreactive ones, but more faintly labeled. Their pattern of distribution was strikingly similar in some of the limbic structures studied but clearly distinct in others. Excellent overlap of neuropeptide Y and somatostatin-like immunoreactivity was detected in: (1) the medial septal area, where innervation occasionally formed perivascular clusters; (2) the nucleus accumbens and olfactory tubercle, characterized by dense patchy innervation; and (3) the laterodorsal septal nucleus, scarcely innervated. In the latter structures, most peptidergic neurons were double-labeled. On the other hand, both peptidergic innervations clearly differed in the lateroventral septal nucleus and the bed nucleus of the stria terminalis which contained distinct clusters of somatostatin-like immunoreactive neurons devoid of neuropeptide Y-like immunoreactivity. Also, the perineuronal and peridendritic axonal plexuses ('woolly fibers') present in these structures were only labeled with somatostatin. In the infralimbic cortex, the relation between the peptides varied according to the cortical laminae. Coexistence of somatostatin and neuropeptide Y frequently occurred in layer VI and in the subcortical white matter, whereas layer V and particularly layers II and III contained a contingent of neurons labeled only with somatostatin. Dense horizontal terminal networks in layers I and VI however were similar for both peptides. These findings support the existence of two different types of somatostatin-like immunoreactive perikarya as regards colocalization with neuropeptide Y. Their particular topographical segregation within the cortical and subcortical structures analysed suggest that they could have different connections and functional properties.(ABSTRACT TRUNCATED AT 400 WORDS)

[Peripheral neuropathies during treatment with cisplatin: clinical and electrophysiologic study of 11 cases]. / Neuropathies périphériques au cours des traitements au cisplatine: étude clinique et électrophysiologique de 11 cas.

Eleven patients with bronchial epidermoid carcinoma and undergoing treatment with cis-D.D.P. (II) were kept under electrophysiological and clinical surveillance. No other neurotoxic medication was added. The total dose of cis-D.D.P. was 300 mg/m2 over a period of three months: namely, three courses of 100 mg/m2 distributed over 5 days. Following the pretreatment check-up, the patients were divided into two groups: those without any electrophysiological abnormality (group A), and those without clinical abnormality but with a delayed latency H of the Hoffmann Reflex (group B). Patients in group A showed a slowing down of the motor nerve conduction velocity of the Median and Peroneal Nerves after a course of 100 mg, without accompanying worsening of the conduction velocity after 300 mg/m2, and prolongation of the distal latency of the sensory Median Nerve after 300 mg/m2; in group B, no significant change of electrophysiological clinical features were noted. In the two groups a non-significant reduction in amplitude of evoked responses were noted. These findings are more consistent with an axonal injury than with functional myelin injury. The authors review the existing literature and discuss the physiopathologic mechanisms of cis-D.D.P. peripheral neuropathies.

[Electro-clinical monitoring of peripheral neuropathies in Waldenström disease treated with plasmapheresis]. / Surveillance électro-clinique des neuropathies périphériques de la maladie de Waldenström traitées par plasmaphérèse.

The aim of this study was to determine the effects of plasma exchange (PE) associated with Chlorambucil in the treatment of peripheral neuropathies in four patients suffering from Waldenström's disease (three cases with IgM kappa paraproteinemia and one case with IgM lambda). Before PE therapy, there was a severe slowing down of the motor and sensory conduction velocities (MCV and SCV) in three cases (IgM kappa) and a less severe slowing in one case (IgM lambda). In three cases, PE rapidly (as early as the third or the fourth administration) brought about a moderate improvement in the MCV and SCV, especially in the upper limbs, whereas in one case, no effect was noted. Clinically, the paraesthesias and dysaesthesias diminished, but the deep tendon reflexes remained absent or markedly depressed. Three months after the PE, nerve conduction velocities were again very reduced. The current literature on the physiopathological mechanisms of the peripheral neuropathies in the Waldenström's disease and the effects of the treatment by immunosuppressive agents and PE are reviewed.

[Value of visual and somatosensory evoked potentials and the electroencephalogram in chronic respiratory insufficiency]. / Intérêt des potentiels évoqués visuels et somesthésiques et de l'électroencéphalogramme chez les insuffisants respiratoires chroniques.

The visual evoked potentials (VEPs), somatosensory evoked potentials (SEPs) and the electroencephalogram (EEG) have been studied in 16 subjects presenting chronic respiratory insufficiency (CRI) with normal consciousness. The SEPs latencies were increased but the VEPs latencies were not. The EEG was little disturbed and did not seem to provide useful information in CRI without encephalopathy.

[Multiple cerebral tuberculomas. X-ray computed tomographic diagnosis and follow-up: a case]. / Tuberculomes multiples intra-cérébraux. Diagnostic et contrôles tomodensitométriques de l'évolution: un cas.

A 31-year-old man who had never left France, rapidly developed right cerebellar signs accompanied by an alteration of his general condition. CT brain scan showed 7 intracerebral tuberculomas, six of which were asymptomatic. The patient was treated with three anti-tuberculous drugs and steroids, and made a good recovery. The CT changes disappeared. CT brain scan should be performed in patients with extra-cerebral tuberculosis and no CSF abnormalities.