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.

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.

Introduction. Cognitive disorders may bias perception of quality-of-life in patients with multiple sclerosis (MS). Methods. Neuropsychological tests (WAIS-vocabulary, Grober 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.

Radicular involvement in Horton's disease is rare. In all but one of the eight documented cases, C5 nerve roots were affected. We report a new case with C5 involvement. Cervical radiculopathy could be due to arteritis of radicular vessels. Preferential involvement of C5 nerve roots may depend on the vascularization of cervical roots but could be due to the inability to diagnose upper cervical involvement (C1-C3). Outcome is favourable in all cases with corticosteroids.

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.

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.Conclusion: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.

CAROLE is a prospective survey of children and adults who experienced epileptic unprovoked seizure(s) diagnosed for the first time between May 1 1995 and June 30 1996 by 243 French neurologists and neuropediatricians. Case records forms at entry allowed to compare patients who had a single seizure or several seizures prior to diagnosis. In patients with recurrent seizures, the time elapsed between the onset of attacks and the diagnosis (diagnostic delay) was looked for.Half of the 1942 included individuals already experienced more than one seizure when diagnosed. Due to natural history of epilepsies, 13 p.100 of the patients did not come to medical attention after a single seizure. Time to diagnosis ranged from 0 day to 52 years. While the overall median delay was 6 days, it ranged from 0 day to 7-8 months according to the type of seizure and the epilepsy syndrome. Two thirds of generalized convulsive seizures were immediately diagnosed versus one third of partial seizures. One half of infantile spasms were identified within 2 weeks versus 6 weeks in complex partial seizures, 4 months in absence seizures, 6 months in simple partial seizures, and 7 months in myoclonic seizures. Three quarters of idiopathic partial epilepsies were diagnosed within 4 weeks versus 3 months in symptomatic generalized epilepsies, 8 months in symptomatic partial epilepsies, 15 months in idiopathic generalized epilepsies, 30 months in cryptogenic partial epilepsies, and 33 months in undetermined epilepsies.So, the time elapsed between a first epileptic event and its diagnosis is epilepsy-dependent seizure type and cause. Other reasons of diagnostic delay do exist: doctor and patient They will be addressed in another study.

Background In patients with acute ischemic stroke, early treatment with thrombolytic agents is thought to permit reperfusion of ischemic neurons and to promote recovery of function. The Multicenter Acute Stroke-Trial - Europe (MAST-E) was designed to assess the efficacy and safety of streptokinase in patients with acute ischemic stroke.Methods Patients with moderate-to-severe ischemia in the territory of the middle cerebral artery were randomly assigned to receive streptokinase (1.5 million units over a period of one hour) or placebo within six hours after the onset of stroke. The primary efficacy outcome was a binary criterion combining mortality and severe disability at six months, with severe disability defined as a score of 3 or higher on the Rankin scale. The primary safety outcomes were mortality at 10 days and cerebral hemorrhage.Results All randomized patients (156 in the streptokinase group and 154 in the placebo group) were evaluated at six months. The incidence of the primary efficacy outcome was similar in the two groups (124 patients in the streptokinase group and 126 in the placebo group died or had a Rankin score greater than or equal to 3). However, the mortality rate at 10 days was significantly higher in the streptokinase group than in the placebo group (34.0 percent vs. 18.2 percent, P=0.002). The higher rate in the streptokinase group was mainly due to the hemorrhagic transformation of ischemic cerebral infarcts. At six months, more deaths had occurred in the streptokinase group than in the placebo group (73 vs. 59, P=0.06).Conclusions In patients with acute ischemic stroke, treatment with streptokinase resulted in an increase in mortality. The routine use of streptokinase cannot be recommended in acute ischemic stroke. (C) 1996, Massachusetts Medical Society.

Three cases of neurogenic muscular hypertrophy are reported. First case presented a hypertrophy of one calf following SI 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.

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.

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.