Long-term therapy of benign essential blepharospasm and facial hemispasm with botulinum toxin A: retrospective assessment of the clinical and quality of life impact in patients treated for more than 15 years.

Botulinum toxin type A (BoNT-A) is recognized as the treatment of choice for patients with blepharospasm and facial hemispasm. We report the results of long-term BoNT-A therapy (15-20 years) in a group of patients with blepharospasm (9 patients) and hemifacial spasm (18 patients). We evaluated the number of treatment sessions, duration of therapeutic effects, side effects and their frequency during long-term therapy, and the differences between these two groups of patients. We used patient self-assessment and a patient questionnaire to evaluate the influence of the treatment on their quality of life. We have concluded that BoNT-A is an effective and safe long-term treatment of these facial dyskinesias. Despite the different pathophysiology of blepharospasm and facial hemispasm, the therapy effectiveness is comparable. The only differences were in the side effects. In patients with blepharospasm, the side effect frequency was higher and manifested by double vision or eyelid ptosis. In patients with facial hemispasm, the most frequent side effect was lower facial weakness. No association between therapy duration and side effect frequency was determined. Based on patient questionnaires, all patients believed that the treatment was safe and effective with a positive impact on their quality of life, especially in social communication.

Transcranial magnetic stimulation of the cerebellum.

INTRODUCTION: The cerebellum is a very complex structure with many motor/non-motor functions and direct and indirect connections with almost the entire central nervous system. Transcranial magnetic stimulation (TMS) is a non-invasive electrophysiological method for studying, diagnosing, and treating disorders of the nervous system. The aim of the present review is to summarise the research and potential clinical uses of cerebellar TMS. METHODS: PubMed literature search using the key words "cerebellum TMS". RESULTS: TMS of the cerebellum is used in two types of protocols. The first type involves the separate stimulation of the cerebellum while tracking its clinical or electrophysiological influence on motor and non-motor functions. The second involves stimulation of the cerebellum as a conditioning stimulus before stimulating the motor cortex, to monitor the electrophysiological impact of cerebellar stimulation on the motor cortex. Most studies are performed on small groups of healthy volunteers; isolated studies are performed on patients with neurological disorders (spinocerebellar ataxia, migraine, dystonia, Miller Fisher syndrome). It has been shown that cerebellar TMS is able to influence motor systems, memory, and perception of time, and there is evidence of its electrophysiological effects in the frontal cortex. CONCLUSION: Published studies suggest that cerebellar TMS is currently only important in research. There is not yet any clear or reliable evidence of the therapeutic effects of cerebellar TMS. However, its use as a treatment method can be anticipated.

Cognitive potentials in the basal ganglia-frontocortical circuits. An intracerebral recording study.

We studied cognitive functions related to processing sensory and motor activities in the basal ganglia (BG), specifically in the putamen and in cortical structures forming the BG-frontocortical circuits. Intracerebral recordings were made from 160 brain sites in 32 epilepsy surgery candidates. We studied P3-like potentials in five different tests evoked by auditory and visual stimuli, and two sustained potentials that are related to cognitive activities linked with movement preparation: BP (Bereitschaftspotential) and CNV (contingent negative variation). We compared the presence of a potential with a phase reversal or an amplitude gradient to the absence of a generator. All of the studied cognitive potentials were generated in the BG; the occurrence in frontal cortical areas was more selective. The frequency of all but one potential was significantly higher in the BG than in the prefrontal and in the cingulate cortices. The P3-like potentials elicited in the oddball paradigm were also more frequent in the BG than in the motor/premotor cortex, while the occurrence of potentials elicited in motor tasks (BP, CNV, and P3-like potentials in the CNV paradigm) in the motor cortex did not significantly differ from the occurrence in the BG. The processing of motor tasks fits with the model by Alexander et al. of segregated information processing in the motor loop. A variable and task-dependent internal organisation is more probable in cognitive sensory information processing. Cognitive potentials were recorded from all over the putamen. The BG may play an integrative role in cognitive information processing.

Cortical and subcortical distribution of middle and long latency auditory and visual evoked potentials in a cognitive (CNV) paradigm.

OBJECTIVE: This study concerned sensory processing (post-stimulus late evoked potential components) in different parts of the human brain as related to a motor task (hand movement) in a cognitive paradigm (Contingent Negative Variation). The focus of the study was on the time and space distribution of middle and late post-stimulus evoked potential (EP) components, and on the processing of sensory information in the subcortical-cortical networks. METHODS: Stereoelectroencephalography (SEEG) recordings of the contingent negative variation (CNV) in an audio-visual paradigm with a motor task were taken from 30 patients (27 patients with drug-resistant epilepsy; 3 patients with chronic thalamic pain). The intracerebral recordings were taken from 337 cortical sites (primary sensorimotor area (SM1); supplementary motor area (SMA); the cingulate gyrus; the orbitofrontal, premotor and dorsolateral prefrontal cortices; the temporal cortex, including the amygdalohippocampal complex; the parietooccipital lobes; and the insula) and from subcortical structures (the basal ganglia and the posterior thalamus). The concurrent scalp recordings were obtained from 3 patients in the thalamic group. In 4 patients in the epilepsy group, scalp recordings were taken separately from the SEEG procedure. The middle and long latency evoked potentials following an auditory warning (S1) and a visual imperative (S2) stimuli were analyzed. The occurrences of EPs were studied in two time windows (200-300 ms; and over 300 ms) following S1 and S2. RESULTS: Following S1, a high frequency of EP with latencies over 200 ms was observed in the primary sensorimotor area, the supplementary motor area, the premotor cortex, the orbitofrontal cortex, the cingulate gyrus, some parts of the temporal lobe, the basal ganglia, the insula, and the posterior thalamus. Following S2, a high frequency of EP in both of the time windows over 200 ms was observed in the SM1, the SMA, the premotor and dorsolateral prefrontal cortex, the orbitofrontal cortex, the cingulate gyrus, the basal ganglia, the posterior thalamus, and in some parts of the temporal cortex. The concurrent scalp recordings in the thalamic group of patients twice revealed potentials peaking approximately at 215 ms following S1. Following S2, EP occurred with latencies of 215 and 310 ms, respectively. Following S1, separate scalp recordings in 4 patients in the epilepsy group displayed EP 3 times in the 'over 300 ms' time window. Following S2, EP were presented once in the '200-300 ms' time window and 3 times in the 'over 300 ms' time window. CONCLUSIONS: The SM1, the SMA, multiple sites of the frontal lobe, some parts of the temporal lobe, the cingulate gyrus, the basal ganglia, the insula, and the posterior thalamus all participate in a cortico-subcortical network that is important for the parallel cognitive processing of sensory information in a movement related task.

The impact of motor activity on intracerebral ERPs: P3 latency variability in modified auditory odd-ball paradigms involving a motor task.

The P3 wave of event-related potentials was recorded with intracranial electrodes in 24 epileptic patients during the pre-surgical evaluation of epilepsy surgery. Three different cognitive auditory paradigms were used: (1) odd-ball paradigm with no output required (PGI) where patients had simply to recognize target tones, (2) odd-ball with motor response (PGII), where patients had to press a button in response to target tones, and (3) odd-ball with both counting task and motor response (PGIII), where patients had to recognize target tones, press a button in response to them, and count their number. The occurrence of P3 potential, its latency and amplitude, and the dependence of P3 latency on the task complexity were calculated. Identifiable P3 potentials in all the three paradigms were recorded from locations in mesial cortex (18 locations mesial temporal, eight locations mesial frontal, two locations mesial parietal) and lateral sites (eight sites lateral temporal, five lateral frontal, and two lateral parietal). P3 latency values ranged from 257 to 320 ms in all explored cortical areas when PGI was used; they significantly increased or decreased during PGII and PGIII, depending on the task and structure explored. In the mesial temporal cortex, the changes of P3 latency between paradigms were minimal. In the mesial parietal cortex, there was significant P3 delay in both PGII and PGIII relative to PGI. In the mesial frontal cortex, there was a significant latency decrease in PGII, and practically identical mean latency in PGI and PGIII. In all lateral cortices (temporal, frontal and parietal), there was always a P3 latency increase in PGII and PGIII relative to PGI, the most significant results being observed in the parietal and frontal lateral areas. The results support the multi-generator theory of P3. Prolongation of the mean P3 latency in lateral frontal and parietal cortices when the paradigm involved the execution of a motor task might reflect specific gating in this area during active movements, while the absence of modification in the temporal lobe may reflect minimal involvement of this region in motor planning or processing. The prolongation of mean P3 latency in practically all lateral structures in PGIII suggests that most cortical areas were involved in the cognitive functions needed for this test. The finding of reduction and subsequent prolongation of P3 latency in the mesial frontal cortex might reflect the unique specialization of this area and its specific involvement in motor processing.

Abnormalities of cortical excitability and cortical inhibition in cervical dystonia Evidence from somatosensory evoked potentials and paired transcranial magnetic stimulation recordings.

Cortical excitability and cortico-cortical inhibition were examined in twenty-one patients suffering from idiopathic rotational cervical dystonia. Polymyography of cervical muscles, somatosensory evoked potential recordings, and paired transcranial magnetic stimulation were used to assess the dystonic disorder. The results were compared with those obtained in a group of sixteen healthy age-matched volunteers. Statistically significant differences between the patient group and the control group were found when the amplitude values of the mean P22/N30 component measured at F [3, 4] and C[3, 4]' electrode positions were compared. The mean amplitude of P22/N30 in both of these electrode positions contralaterally to the direction of head deviation was significantly higher in the patient group (p

Levodopa-induced dyskinesias and continuous subcutaneous infusions of apomorphine: results of a two-year, prospective follow-up.

Twelve patients with levodopa-induced dyskinesias were treated with continuous subcutaneous apomorphine. A markedly significant reduction in peak dose dyskinesias occurred over a two-year follow-up.