[Topography of secondary dystonia lesions].

Biological causes provoking dystonia can not be systematized, with the exception of the small group of levodopa-responsive dystonia. Therefore the pathophysiology of the dystonic syndrome can be approached by considering the site of the lesions. In 40 cases of uni or bilateral symptomatic dystonias, this site could be identified with CT Scan or MRI.

Visual cognitive dysfunction in depression: an event-related potential study.

The P3(00) event-related potentials (ERPs) elicited by visual stimuli in two visual tasks were assessed in depressed patients (12 patients with major depression and 11 patients with bipolar disorder) and compared with those of 20 age-matched normal controls. At remission, the ERPs from 18 of the depressed patients were again recorded. The visual oddball (VO) paradigm presented both target and standard visual stimuli and the simple visual (SV) paradigm presented a target but no standard visual stimulus.

Clinical assessment of olfactory dysfunction in Parkinson's disease.

We used two simple tasks to test the capacities of patients with Parkinson's disease to discriminate and identify olfactory stimuli. The patients presented defective odor identification abilities whereas their capacity to discriminate between odors was apparently unaffected. This raises a question about the nature of olfactory dysfunction in Parkinson's disease.

[Juvenile GM2 gangliosidosis with progressive spinal muscular atrophy onset].

GM2 gangliosidosis are caused by a beta-hexosaminidase A enzyme deficiency. Mutations in the gene leaving residual enzyme activity give rise to juvenile and adult forms of the disease which have a great clinical heterogeneity. We report three cases which have been considered for some time as Kugelberg-Welander disease.

Intracerebral recording of movement related readiness potentials: an exploration in epileptic patients.

Readiness potentials (RPs) preceding voluntary self-paced limb movements were recorded intracerebrally in 13 patients suffering drug resistant, intractable epilepsy. Multilead depth electrodes were positioned using the Talairach's coordinate system; they allowed simultaneous recording from the external and mesial cortices and from the interposed white matter during self-paced unilateral hand or plantar flexions. Our intracerebral explorations have shown RPs in the primary motor cortex (MC) contralateral to the movement and in both supplementary motor areas (SMAs), indicating that at least 3 cortical sites become active before the movement.

Abnormal movement related potentials in patients with lesions of basal ganglia and anterior thalamus.

Movement-related cortical potentials (MRCPs) were recorded from scalp electrodes during wrist flexion in 15 dystonic patients with bilateral (nine) or unilateral (six) circumscribed lesions in the striatum (eight), pallidum (six), or anterior thalamus (one). The results were compared with those of 10 age-matched healthy volunteers. The early (BP) and late (NS') MRCP components were assessed in terms of their gradients and distribution on the scalp in Cz, C3', and C4'.

Botulinum antibodies in dystonic patients treated with type A botulinum toxin: frequency and significance.

We measured serum antibodies to botulinum toxin (ABT) in 96 patients with focal dystonia who had been treated with type A botulinum toxin. The frequency of detectable ABT was 3% (three patients). Patients with ABT had received more than 50 ng of botulinum toxin, and the shortest time between two injections was significantly less than in patients without ABT.

[Positron-emission tomographic study of the dopaminergic system in a case of secondary unilateral tremor after mesencephalic hematoma].

A 30 year-old woman developed a postural and rest tremor of the left hand following a right peduncular post-traumatic hematoma. Two years later, positron emission tomography showed a marked decrease in [18F] fluorodopa uptake contrasting with a normal [76Br] bromolisuride uptake in the right striatum. This suggests that: 1) chronic unilateral dopaminergic striatal denervation may occur without persistent D2 dopaminergic receptor upregulation in humans; and 2) symptomatic mesencephalic tremor may be, at least in part, related to dopaminergic striatal denervation.

Chronic administration of L-dopa affects the movement-related cortical potentials of patients with Parkinson's disease.

The chronic effect of L-Dopa administration on the movement-related cortical potentials (MRCPs) was studied in two groups of patients with Parkinson's disease (PD): patients de novo (DN) and patients with on-off fluctuations. The BP and NS' premovement components of MRCPs associated with wrist flexion were assessed by their gradients and by their distribution on the midline (CZ) and the ipsilateral and contralateral hand sensorimotor areas. The treatment efficacy was controlled by a decrease in PD score (Columbia University Rating Scale).

[Movement-related cortical potentials in aged subjects].

Cortical potentials related to freely-executed voluntary wrist flexion (MRPs) were studied in 35 subjects aged 23-80 years. The characteristics of the MPRs in aged subjects were determined in comparison data from 14 young subjects aged 23-40 years. The analysis concerned 3 components of the MRPs: the slow negative shifts (NS1 and NS2) before the movement onset and the motor potential (MP).

[Changes in movement-related cortical potentials in Parkinson's patients before and after treatment with levodopa].

Cortical potentials associated with voluntary, self-paced wrist flexion (MRPs) were recorded from 3 scalp locations (Cz and psi contralateral hand motor area) in patients with Parkinson's disease (9 de novo patients and 30 L-Dopa treated patients). The analysis concerned 3 components of the MRPs: the 2 slow negative shifts (NS1 and N1) before the movement onset and the motor potential (MP). The NSI amplitude was measured at Cz, the peak negativity N1 and MP from contralateral hand motor area location.

Therapeutic response to progabide in neuroleptic- and L-dopa-induced dyskinesias.

The results of two trials conducted in human dyskinesia with progabide, a specific gamma-aminobutyric acid (GABA) receptor agonist, are reviewed. In one trial, 13 parkinsonian patients with L-DOPA-induced dyskinesia (LDD) and "on-off" fluctuations were included in a double-blind controlled trial progabide versus placebo. No change was observed during this trial in the severity of dyskinesia on progabide treatment but the drug significantly extended the "on" period as compared with placebo.

Postural adjustments associated with rapid voluntary arm movements in patients with Parkinson's disease.

To determine the extent to which deficits in coordination between posture and movement are influenced by postural disorders, postural adjustments associated with rapid voluntary arm movement were studied in PD patients and controls. EMG activity in postural muscles of the lower limbs and the trunk and local anteroposterior accelerations of the upper part of the leg were recorded in subjects who rapidly raised their arms to a horizontal position in response to a visual signal. The arm movement was characterized electromyographically by EMG activity from the deltoid muscle (anterior portion) and kinetically by acceleration of the arm.

[Bilateral thalamic infarcts with abnormal movements and permanent amnesia].

A 45 year-old woman with mitral valve disease developed bilateral thalamic infarcts presenting as vigilance disorders, with mutism and downwards gaze paralysis during several days, followed by involuntary rhythmic movements over 13 days. These movements affected the four limbs with a rhythm variable with time as alternate or synchronized agonist-antagonist periods. Subsequent examinations demonstrated global, lasting amnesia and a subclinical disorder of vertical ocular movements.

EMG patterns in abnormal involuntary movements induced by neuroleptics.

Electromyographic (EMG) activity of abnormal involuntary movements and their modifications after Piribedil, a dopaminergic agonist, were analysed in patients presenting with tremor or tardive dyskinesia induced by treatment with neuroleptics. Quantitative analysis of EMG bursts and of their phase relationships with bursts of antagonist muscles revealed differences between tremor and tardive dyskinesia; three separate EMG types of the latter were found. In tremor, EMG activity was coordinated between agonists and antagonists.

Conduction of the sciatic nerve in its proximal and distal segment in patients with ALS (amyotrophic lateral sclerosis).

Conduction in the proximal segment of the sciatic nerve was assessed by recording the latencies of M and H responses from soleus muscle, in the distal segment by comparing the latencies of M waves from the abductor hallucis muscle, obtained by stimulating the tibial nerve in the popliteal fossa and at the ankle. Data from 30 patients with amyotrophic lateral sclerosis (ALS) were compared with an age-matched group of healthy subjects. We observed an increase in the difference between the latencies of M and H responses from soleus, and a lengthening of the distal latency of the M wave from abductor hallucis.

[Activation motor response in depressive states. Changes in the Hoffmann reflex during psychomotor testing].

Psychophysiological response of activation was explored both in control and depressed patients before and after antidepressant treatment. Hoffmann reflex (H reflex) and heat rate were recorded. Two psychomotor tests were studied in order to modify the level of vigilance.

The abdominal cutaneous reflex circuit in the rat: a spinobulbo-spinal reflex in phenobarbital-anesthetized animals.

A description is given of an abdominal reflex (ACR) in the rat evoked by cutaneous stimulation. Latency of the ACR in response to stimulation of the contralateral hindlimb was 28.5 +/- 3.

Peripheral neuropathies and tremor.

We studied 14 patients with tremor and acquired peripheral neuropathies of different origin. Minimal weakness was the only clinical finding common to all patients. Sensory changes, etiology, and course of the neuropathy varied.

[Electrophysiological and pharmacological analysis of L-dopa-induced dyskinesia and tardive dyskinesia (author's transl)].

Electrophysiological and pharmacological analysis of L-Dopa-induced dyskinesia and tardive dyskinesia (L.DD) due to neuroleptics was performed on 12 patients with Parkinson's disease and on 12 others with psychotic diseases. This analysis included the examination of spinal reflexes, monosynaptic H reflex, polysynaptic cutaneous reflex of the lower limb, muscular responses to passive movement [stretch reflex and shortening reaction (SR)] and the study of the motor response to a dopaminergic stimulus (I.