Subacute corticobasal syndrome following internal carotid endarterectomy.

The present report is of two patients who, immediately after internal carotid endarterectomy, presented with unexplained hemiplegia, despite normal findings on repeated MRI scans, which secondarily evolved into homolateral subacute corticobasal syndrome (CBS), with asymmetrical hemispheric hypometabolism and evidence of dopaminergic denervation. This prompted us to propose an hypothesis of transient cerebral hypoxia arising during the surgical clamping period that might have provoked a prolonged or permanent functional lesion of the left hemisphere and basal ganglia, with no visible infarction on MRI but only synaptic rearrangement of the neural networks, thereby revealing or exacerbating a potentially preexisting silent impairment.

[Cognitive impairment and quality of life in multiple sclerosis patients]. / Déficit cognitif et mesure de la qualité de vie dans la sclérose en plaques.

INTRODUCTION: Cognitive disorders may bias perception of quality-of-life in patients with multiple sclerosis (MS). METHODS: Neuropsychological tests (WAIS-vocabulary, Gröber and Buschke, Stroop, WCST, WAIS-similitude, fluency, Hamilton's depression scale) along with a French health-related quality of life self questionnaire for MS patients (the SEP59) were performed by 117 MS patients with a minor cognitive impairment (Mini Mental State over 24) in order to explore the links between quality of life perception and cognitive functioning. RESULTS: Main Component Analysis and correlation analysis showed that cognitive impairment produced an overrating of the self perception of quality of life. Threshold analysis, splitting patients between the most and the less cognitively impaired, confirmed this relationship. CONCLUSION: The level of cognitive functioning must be considered with caution when analyzing health-related quality of MS patients.

Lack of pharmacokinetic interaction between the selective dopamine agonist cabergoline and the MAO-B inhibitor selegiline.

The addition of a dopamine agonist and of a monoamine oxidase type B inhibitor to I-dopa has been suggested in the therapy of Parkinson's disease. The plasma pharmacokinetics of both cabergoline and I-dopa have previously been shown to remain unaffected when the two drugs are given concomitantly. This study aimed at examining whether the plasma pharmacokinetic parameters of cabergoline and selegiline are modified when given in combination. Selegiline is hardly detectable in plasma. Therefore, the plasma levels of its metabolites amphetamine, methamphetamine and desmetylselegiline were used to assess the effect of cabergoline co-administration. Plasma levels of the selegiline metabolites were determined first after selegiline administration (10 mg/day) for 8 days, and then after administration of both drugs for 22 additional days (day 30). Cabergoline plasma levels were measured on day 30, and then after administration of cabergoline (1 mg/day) alone for further 22 days. No statistical difference was found between the Cmax.ss, tmax.ss, AUC0-24h.ss, C0h.ss, C24h.ss values of cabergoline and of the selegiline metabolites when the two drugs were given alone or in combination, indicating the absence of pharmacokinetic interaction between cabergoline and selegiline.

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.

Spontaneous internal carotid artery dissection with lower cranial nerve palsy.

BACKGROUND: Typical presentation of spontaneous internal carotid artery (ICA) dissection is an ipsilateral pain in neck and face with Horner's syndrome and contralateral deficits. Although rare, lower cranial nerve palsy have been reported in association with an ipsilateral spontaneous ICA dissection. CASE STUDIES: We report three new cases of ICA dissection with lower cranial nerve palsies. RESULTS: The first symtom to appear was headache in all three patients. Examination disclosed a Horner's syndrome in two cases (1 and 2), an isolated XIIth nerve palsy in two patients (case 1 and 3) and IX, X, and XIIth nerve palsies (case 2) revealing an ipsilateral carotid dissection, confirmed by MRI and angiography. In all cases, prognosis was good after a few weeks. CONCLUSIONS: These cases, analysed with those in the literature, led us to discuss two possible mechanisms: direct compression of cranial nerves by a subadventitial haematoma in the parapharyngeal space or ischemic palsy by compression of the ascending pharyngeal artery.

[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.

[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.

[Pyridoxine neuropathies. Review of the literature]. / Neuropathies à la pyridoxine. Revue de la littérature.

Daily needs of vitamin B6 are very low (2 mg per day) and widely covered by normal feeding. Pyridoxine deficiencies are exceptional (congenital metabolic abnormalities, drug or toxic-induced perturbations). First described in animal models, human cases of neuropathy had been encountered in the "megavitamin"-syndrome. They are confirmed by rare case-reports of very high doses given in toxic indication. Sensory peripheral neuropathies can also occur with lower doses taken over a long period of time.

[During the treatment or after... drug-induced convulsive accidents. Apropos of 70 cases]. / En cours de traitement ou à l'arrêt ... les accidents convulsifs liés aux médicaments. A propos de 70 observations.

During a five year period (1988-1992) 70 cases of seizures were collected by the Adverse Drug Reaction Monitoring Center of Clermont-Ferrand (3.58% of total collected cases). 31 cases (22 M and 9 F) occurred after a drug withdrawal. Benzodiazepines--either alone or in association--were the most often involved. Mean age was 48.8 +/- 2.6 years in this group and additional factors (alcohol abuse and/or association of drugs that lower the seizure threshold) were associated in 26 cases. 39 cases (14 M and 25 F) occurred on the course of various treatments. The most frequently involved drugs were neuropsychiatric, antiinfectious (especially beta-lactam antibiotics, fluorquinolones and isoniazid) and theophylline. Mean age was 56.5 +/- 3.8 years and additional different factors (high dosages, antecedents of epilepsy, underlying diseases) were present in 22 observations.

Apomorphine in treatment of Parkinson's disease: comparison between subcutaneous and sublingual routes.

The efficacy of two routes of apomorphine, subcutaneous (SC) and sublingual (SL), successively administered in 7 Parkinsonian patients with motor fluctuations, was compared in reducing the daily duration of "off" phases. The mean duration of SC and SL treatment was 7.7 and 6.8 months respectively. The mean time spent in "off" phase was 55% after SC and 68% after SL treatment. The mean time before turning "on" after an "off" period was 14 minutes after SC and 28 minutes after SL treatment. Two patients developed stomatitis after SL route. SL apomorphine may be helpful in the treatment of motor fluctuations in PD.

Walk headache: an unusual manifestation of ischemic heart disease.

A 71 year old man sought neurological advice because for two years he had suffered from headache every time he made an effort. A treadmill stress test showed a relation between effort, headache and depression of ST segments on E.C.G. With isosorbide dinitrate and diltiazem, the manifestations improved. This suggests a referred head pain due to myocardial ischemia.

A photoacoustic investigation of the influence of some constituents of the stratum corneum on ultraviolet absorption.

Photoacoustic spectroscopy (PAS) was used to evaluate light absorption by solid samples. Application of this technique to the human stratum corneum (SC) was used to investigate the influence of some constituents of SC (water, lipids, hydrophilic substances) on ultraviolet (UV) absorption by this tissue. The results showed that the water content of SC modified the PAS signal obtained. For example, there was a fall of over 20% in the UV absorption coefficient when the SC water-content rose from 0 to 30%. Epidermal lipids were not shown to have any direct effect on UV absorption by SC. However, indirectly they do seem to have protected some of the hydrophilic substances from water extraction. After removal of these hydrophilic substances, the UV absorption from SC was greatly decreased. Results indicated that these hydrophilic substances were protected in situ by polar lipids enmeshed within the structure of SC.

Relation between plasma concentration and clinical efficacy after sublingual single dose apomorphine in Parkinson's disease.

Five patients with Parkinson's disease were given a single sublingual dose of apomorphine in 3 mg tablets (2 patients received 18 mg and 3 patients took 39 mg). The therapeutic effect appeared within 33.0 min and lasted 137 min. There was a significant correlation between peak concentration, area under the curve, dose (mg/kg) and the duration of the therapeutic effect.

CAG repeat expansion in the TATA box-binding protein gene causes autosomal dominant cerebellar ataxia.

At least 13 loci responsible for autosomal dominant cerebellar ataxia (ADCA) have been identified. Spinocerebellar ataxia 1, 2, 3, 6 and 7 are caused by translated CAG repeat expansions. However, in France, >30% of ADCAs are not explained by the known genes. Recently, analysis of the TATA box-binding protein (TBP) gene, one of the transcription factors known to contain a CAG/CAA repeat, in patients with progressive cerebellar ataxia revealed one sporadic case with 63 repeats. We examined this gene in 162 index cases with ADCA. An expanded repeat with 46 repeat units was detected in a single index case from Belgium. In this family, two affected members and six unaffected, but at-risk, individuals carried expanded alleles. Interestingly, the expanded repeat was stable during transmission. The main clinical features in six patients were cerebellar ataxia, dementia and behavioural disturbances with onset in their fourth to sixth decade. The main neuropathological finding was severe neuronal loss and gliosis in the Purkinje cell layer. Immunohistochemical analysis showed neuronal intranuclear inclusions containing expanded polyglutamine, indicating that this disease shares several features with other polyglutamine diseases. This study demonstrates that CAG/CAA repeat expansion in the TBP gene causes ADCA with dementia and/or psychiatric manifestations.