Detection of Epstein-Barr virus sequences in primary brain lymphoma without immunodeficiency.

We searched for Epstein-Barr virus (EBV) sequences by enzymatic DNA amplification in nine primary brain lymphomas from patients without immunodeficiency. We used seven nonlymphoma brain tumors as negative controls, and the Raji cell line as a positive control. We detected EBV DNA, using ethidium bromide-stained-agarose minigel electrophoresis and dot blot hybridization, in the positive control and in only one brain lymphoma tumor; we did not detect EBV DNA in the other tumors. The EBV-positive patient had a second B-cell monoclonal population in the peripheral blood without detectable EBV DNA, suggesting a direct role for EBV in the development of the brain lymphoma.

Narcolepsy associated with arteriovenous malformation of the diencephalon.

Narcolepsy associated with localized brain lesions is rare, and few reports of well-documented cases have been published. We describe the case of a 20-year-old male (HLA DQw1 negative) who fulfilled clinical and polygraphic criteria of symptomatic narcolepsy. Narcolepsy in this patient was associated with an arteriovenous malformation involving the structures around the third ventricle. Clinical symptoms improved after embolization and radiosurgery. These findings support the hypothesis that lesions in the vicinity of the third ventricle can cause symptomatic narcolepsy.

Apomorphine and diphasic dyskinesia.

We report on three observations of parkinsonian patients with levo-dopa-induced diphasic dyskinesias, who received subcutaneous apomorphine to reduce the duration of abnormal movements. Apomorphine was effective in reducing the duration of diphasic dyskinesias at doses higher than the threshold doses necessary to induce an "on" phase (mean increase: 43%). However, after a few months of treatment, apomorphine was ineffective in stopping abnormal movements, even when doses were increased. In two patients, apomorphine remained effective in the morning, but increased the intensity of the dyskinesias in the afternoon. Acute diurnal variations of the pharmacodynamic striatal response are suggested explanation for these clinical observations.

Relation between clinical efficacy and pharmacokinetic parameters after sublingual apomorphine in Parkinson's disease.

Apomorphine was administered sublingually in two single doses (0.3 and 0.6 mg/kg) to seven patients with idiopathic Parkinson's disease (PD) to assess the relation between clinical efficacy, dosage, and pharmacokinetic parameters of apomorphine. On day 1 and day 3, patients were given 0.3 mg/kg and 0.6 mg/kg of apomorphine, respectively (3 mg tablets). Before apomorphine administration and during the following 4 h, motor score was assessed by measuring tremor, akinesia scores, rising from a chair, and walking speed. The delay to turn on was not different between the two doses but after the 0.3 mg/kg dose, only three patients turned on, whereas all the patients treated with 0.6 mg/kg turned on. Apomorphine (0.3 mg/kg) induced a shorter duration of the "on" period than 0.6 mg/kg (0.3 mg/kg: 24.2 +/- 14.6 min; 0.6 mg/kg: 86.7 +/- 14.9 min). The time to obtain the peak plasma concentration (tmax) obtained with the two doses were not different (0.3 mg/kg: 31.5 +/- 3.4 min; 0.6 mg/kg: 38.3 +/- 2.8 min). Peak plasma concentrations (Cmax) and areas under the curve (AUC) were significantly higher after 0.6 mg/kg than 0.3 mg/kg (Cmax: 0.3 mg/kg: 7.5 +/- 3.2 ng/ml; 0.6 mg/kg: 22.7 +/- 3.6 ng/ml; p < 0.01; AUC: 0.3 mg/kg: 929 +/- 109 ng/ml/min; 0.6 mg/kg; 2,277 +/- 209 ng/ml/min; p < 0.01). There was a significant linear correlation between the duration of therapeutic effect, AUC, and Cmax (r = 0.86, p < 0.01 for AUC; r = 0.63, p < 0.05 for Cmax). These results show that sublingual apomorphine could be of interest in the treatment of "off" phases in parkinsonian patients with motor fluctuations.

[Autoimmune familial myasthenia in 2 sisters with a follow-up of more than 30 years]. / Myasthénie familiale auto-immune deux soeurs suivies pendant plus de 30 ans.

The clinical course of two sisters with myasthenia initially published in La Revue Neurologique in 1960 has been followed. Both had secondary severe respiratory impairment requiring a tracheotomy and mechanical ventilation. Thymectomy was performed in both and revealed residual thymic tissue. Complete remission was observed long after thymectomy even with the persistence of antiacetylcholine receptor antibodies which are still present in the older sister despite intercurrent autoimmune thyroiditis and in the younger sister despite the development of mediastinal lymphosarcoma leading to death 32 years after the onset of myasthenia.

[Sublingual administration of apomorphine in the treatment of motor fluctuations in Parkinson disease]. / Administration de l'apomorphine par voie sublinguale dans le traitement des fluctuations motrices de la maladie de Parkinson.

Apomorphine, a mixed dopaminergic agonist was given sublingually to 12 patients with Parkinson's disease disabled by severe on-off fluctuations. The patient's mean age was 57 years and the duration of Parkinson's disease was 12 years. All patients were also given domperidone (60 mg/day). Apomorphine was administered as soon as the off periods appeared. On periods were observed in 11 patients, with a mean apomorphine dose of 40 mg for each administration (extremes values: 20-60 mg). One patient had no motor benefit after an apomorphine dose of 120 mg. The mean duration of daily off periods was reduced by 64 per cent in 11 patients, for a mean duration of 8 months (extremes values: 2-12 months). Four patients developed stomatitis or gingival edema and stopped treatment. This pilot study shows that sublingual apomorphine, during a mean period of 8 months, significantly decreases off periods in parkinsonian patients. Others studies are necessary to confirm these results.

[Demyelinating involvement in Borrelian neuropathies]. / Les atteintes démyélinisantes au cours des neuropathies borréliennes.

Five patients (4 men, 1 woman, aged 51-67), were hospitalized for peripheral painful neuropathies. The serological survey and the dramatic responses to penicillin confirmed the responsibility of Borrelia burgdorferi in all 5 cases. Electromyographical studies showed evidence of demyelination: prolonged distal latency, slowing down of nerve conduction velocity. In 3 patients this pattern was related to axonal degeneration as shown by fibrillation potentials and reduced voluntary motor unit potentials recorded on needle examination. The other two showed no axonal degeneration. One also had motor conduction blocks. Such a demyelination could be due to vasculitis of the vasa nervorum described in tick-bone meningoradiculitis.

[Therapeutic problems posed by hemiballism of metastatic origin]. / Problèmes thérapeutiques posés par un hémiballisme d'origine métastatique.

A 40 year old woman undergoing treatment for cancer of the breast presented with metastatic hemiballism. Diagnosis was supported by the presence of bone and liver metastases and by CT-scan showing an enhanced rapidly growing lesion in the subthalamic region contralateral to hemiballism. Treatment by corticotherapy and neuroleptics was ineffective. Radiotherapy was unsuccessful. Metastatic hemiballism is a rare disease with no effective treatment.

[Neurogenic muscle hypertrophy: 3 cases]. / L'hypertrophie musculaire neurogène: 3 cas.

Three cases of neurogenic muscular hypertrophy are reported. First case presented a hypertrophy of one calf following S1 radiculopathy; the second had a hypertrophy of tibialis anterior muscle, five years after a compressive injury of the common peroneal nerve. The third case is a global hypertrophy of leg muscles after intensive care or legionella neuropathy. Previous cases of the literature are reviewed and the various pathophysiological hypothesis are considered. Among these hypothesis, the role of abnormal electrophysiological activities in most previous cases, as well as in ours (namely complex repetitive discharges and fasciculations), seems the most consistent.

[Neurologic involvement in campylobacter infections. 5 cases]. / Atteintes neurologiques au décours d'infections à campylobacter. Cinq cas.

Campylobacter are a newly recognized class of human infectious agents. Campylobacter fetus subspecies fetus is a cause of sepsis in immunocompromized hosts. Secondary neurological determinations, meningitis and meningoencephalitis appear to be rare. We report 2 cases, and 8 previously reported cases are reviewed. Campylobacter jejuni appears to be a common bacterial pathogen causing a syndrome of enteritis. Since 1982 it has been associated with Guillain-Barré syndrome (GBS). In one serological retrospective study, Campylobacter jejuni was the most common single pathogen identifiable in association with GBS, and these cases were significantly more severe. We report 3 cases with weakness and amyotrophy of distal limbs. Only 10 other cases have been found in the literature. Diarrhoea antedated the neurological illness by 4 to 21 days. In 4 syndromes of Miller-Fisher the prognosis was good. However electrophysiological axonal loss was reported in 6 GBS with poor functional prognosis. The pathogenic role of bacterial toxins and humoral immune response are discussed. Cross-reactivity between Campylobacter jejuni and human sciatic nerve proteins has not been demonstrated using sera from patients with GBS and serological evidence of Campylobacter enteritis.

[Dexamethasone test in cluster headache]. / Test à la dexaméthasone dans l'algie vasculaire de la face.

Cluster headache and manic depressive illness share in common similarities like: periodic symptomatology, accessibility to lithium therapy, abnormalities in circadian rhythm of cortisol. Though, in contrast to periodic depression, D.S.T. was found normal in 9 patients with cluster headache.

[Delayed contralateral hemiplegia complicating ophthalmic herpes zoster]. / Hémiplégie controlatérale retardée compliquant le zona ophtalmique.

Contralateral hemiplegia may develop a few weeks after the onset of an ophthalmic herpes zoster. This complication, which is not part of the herpetic encephalopathy, generally coexists with lesions of arteritis demonstrable by arteriography, affecting in most cases the proximal segments of the middle and anterior cerebral arteries and resulting in infarcts. The pathophysiology of these lesions is uncertain, but the vessels appear to be invaded by contiguity.

[Benign paralysis of the last 4 cranial nerves. Arguments in favor of an ischemic mechanism]. / Paralysie bénigne des quatre derniers nerfs crâniens. Arguments en faveur d'un mécanisme ischémique.

The last four cranial nerves are fed by the ascending pharyngeal artery, a branch of the external carotid artery. The fact that paralysis of these nerves may occur immediately after arteriography of the external carotid artery demonstrates that ischaemic truncular neuropathies do exist. The deficit is sudden and usually regressive. Ischaemia may account for regressive paralysis of these nerves reported in diabetes, herpes zoster and after some traumas. Apparently idiopathic and benign paralysis might be due to the same mechanism. All 4 nerves may be affected, or only the IXth, Xth and XIth nerves, or the XIth nerve may be spared, being fed by two arteries. Apart from the obvious case of arteriographic accident, the diagnosis of paralysis of ischaemic origin can only be made after other causes, notably compression, have been excluded, since direct evidence of arterial obstruction is exceptionally obtainable; regression at follow-up is a major argument in favour of ischaemia.

[Cervical spinal cord compression caused by ossification of the posterior vertebral ligament. Apropos of a case]. / Compression médullaire cervicale par ossification du ligament vertébral commun postérieur. A propos d'un cas.

Progressive cervical myelopathy due to an ossification of the posterior longitudinal ligament (OPLL) appeared in a sicilian women. Diagnosis was made by lateral conventional tomograms and CT Scan with mielography. Corporectomy of the 5th vertebra and ablation of the ligament dramatically improved the clinical symptoms. If OPLL seems a common cause of cervical myelopathy in Japan, it is very rate in Caucasian people. Instead of the origin of the patients, progressive tetraparesis of the 60 years old people is the usual presentation of this affection. The cause of OPLL remains unknown: genetic factors, vitamin A, association with diffuse idiopathic skeletal hyperostosis, calcium metabolism abnormalities are discussed. As far as surgical treatment is concerned, choice between anterior and posterior decompression is clarified on the basis of the type and the extension of OPLL.