Dropped head syndrome in severe hypotension.

INTRODUCTION: Dropped head syndrome (DHS) is characterized by a chin-on-chest deformity, correctable by passive neck extension. METHODS: Case report. RESULTS: A patient with a heavy nephrological history (dialyzed since 5 years) complained for twenty months about a falling head. The symptomatology was punctuated by dialysis sessions, with aggravation secondary to dialysis. Clinical and paraclinical exploration for neurological, neuromuscular or orthopedic disease was negative. Analysis of the post-dialysis blood pressure showed a slow and gradual decline. From the date the patient became symptomatic, blood pressure was below 80/40mmHg. The correction of blood pressure by increasing midodrine posology resulted in a cure of DHS. DISCUSSION: Considering the negativity of explorations, the cure of symptoms following the correction of arterial hypotension, the rhythmic nature of symptomatology by dialysis, and the recurrence of symptoms concomitantly with drops in blood pressure, we suggested that hypotension was the only etiology explaining this DHS.

French validation of the Quality of life in Essential Tremor Questionnaire (QUEST) and the Essential Tremor Embarrassment Assessment (ETEA).

Two scales have been developed and validated in English to evaluate the impact of tremor on daily life, namely Quality of life in Essential Tremor Questionnaire (QUEST) and Essential Tremor Embarrassment Assessment (ETEA). The psychometric properties of the French version of these two scales were assessed for 117 patients with head tremor. Both scales showed excellent acceptability, very good internal consistency (Cronbach's alpha coefficient>0.8) and reproducibility (Lin concordance coefficient>0.8), satisfactory external validity and satisfactory sensitivity to change. In conclusion, the French versions of QUEST and ETEA are comprehensive, valid and reliable instruments for assessing patients with head tremor.

The Cortical Processing of Sensorimotor Sequences is Disrupted in Writer's Cramp.

Evidence for pre-existing abnormalities in the sensory and motor systems has been previously reported in writer's cramp (WC). However, the processing of somatosensory information during motor planning has received little attention. We hypothesized that sensorimotor integration processes might be impaired partly due to a disruption in the parieto-premotor network. To test this assumption, we designed 2 nonwriting motor tasks in which subjects had to perform a 4-finger motor sequence either on the basis of sensory stimuli previously memorized (SM task) or freely generated (SG task). Brain activity was measured by combining event-related functional magnetic resonance imaging and coherency electroencephalography in 15 WC patients and 15 normal controls. The bold signal was decreased in patients in both tasks during sensory stimulation but not during movement execution. However, the EEG study showed that coherency was decreased in patients compared with controls, during the delay of the SM task and during the execution of the SG task, on both the whole network and for specific couples of electrodes. Overall, these results demonstrate an endophenotypic impairment in the synchronization of cortical areas within the parieto-premotor network during somatosensory processing and motor planning in WC patients.

Differential role of visuospatial working memory in the propensity toward uncertainty in patients with obsessive-compulsive disorder and in healthy subjects.

BACKGROUND: Obsessive-compulsive disorder (OCD) is associated with visuospatial working memory deficits. Intolerance of uncertainty is thought to be a core component of OCD symptoms. Recent findings argue for a possible relationship between abilities in visuospatial memory and uncertainty. However, this relationship remains unclear in both OCD patients and healthy subjects. To address this issue, we measured performance in visuospatial working memory and the propensity to express uncertainty during decision making. We assessed their relationship and the temporal direction of this relationship in both OCD patients and healthy subjects. METHOD: Baseline abilities in visuospatial working memory were measured with the Corsi block-tapping test. A delayed matching-to-sample task was used to identify explicit situations of certainty, uncertainty and ignorance and to assess continuous performance in visuospatial working memory. Behavioural variables were recorded over 360 consecutive trials in both groups. RESULTS: Baseline scores of visuospatial working memory did not predict the number of uncertain situations in OCD patients whereas they did in healthy subjects. Uncertain trials led to reduced abilities in visuospatial working memory to 65% of usual performance in OCD patients whereas they remained stable in healthy subjects. CONCLUSIONS: The present findings show an opposite temporal direction in the relationship between abilities in working memory and uncertainty in OCD patients and healthy subjects. Poor working memory performance contributes to the propensity to feel uncertainty in healthy subjects whereas uncertainty contributes to decreased continuous performance in working memory in OCD patients.

Evolution of brain gray matter loss in Huntington's disease: a meta-analysis.

BACKGROUND: Huntington's disease is characterized by neuronal loss throughout the disease course. Voxel-based morphometry studies have reported reductions in gray matter concentration (GMC) in many brain regions in patients with Huntington. The description of the time course of gray matter loss may help to identify some evolution markers. Here, we conducted a meta-analysis of voxel-based morphometry studies of Huntington's disease to describe the evolution of brain gray matter loss. METHODS: A systematic search led to the inclusion of 11 articles on Huntington's disease (297 patients and 205 controls). We extracted data from patients with preclinical Huntington, patients with clinical Huntington, and controls. Finally, anatomical likelihood estimation analyses were conducted to identify GMC changes between preclinical patients and controls, between clinical patients and controls, and between preclinical and clinical patients. RESULTS: Preclinical patients exhibited gray matter loss in the left basal ganglia and the prefrontal cortex. Clinical patients had bilateral gray matter loss in the basal ganglia, the prefrontal cortex, and the insula. The left striatum was smaller in clinical patients than in preclinical patients. CONCLUSIONS: Neurodegenerative processes associated with Huntington's disease, as assessed by GMC reduction, begin in the left hemisphere and extend to the contralateral hemisphere throughout the inexorable course of the disease. Changes in gray matter, especially the volumetric side ratio of the striatum, could represent a relevant biomarker for characterizing the different progression stages of the disease.

Basal ganglia: From the bench to the bed.

The basal ganglia (BG) encompass a set of archaic structures of the vertebrate brain that have evolved relatively little during the phylogenetic process. From an anatomic point of view, they are widely distributed throughout brain from the telencephalon to the mesencephalon. The fact that they have been preserved through evolution suggests that they may play a critical role in behavioral monitoring. Indeed, a line of evidence suggests that they are involved in the building of behavioral routines and habits that drive most of our activities in everyday life. In this article, we first examine the organization and physiology of the basal ganglia to explain their function in the control of behavior. Then, we show how disruption of the putamen, and to a lesser extent of the cerebellum, might lead to various dystonic syndromes that frequently arise during childhood.

Sensory motor mismatch within the supplementary motor area in the dystonic monkey.

Dystonia, a movement disorder characterized by abnormal postures, is associated in primary forms of the disease with subtle proprioceptive troubles and aberrant somatotopic representation in the somatosensory cortex (SC). However, it is unclear whether these sensory features are a causal phenomenon or a consequence of dystonia. The supplementary motor area proper (SMAp), a premotor cortical region, receives strong inputs from both the SC and basal ganglia. We hypothesized that disruption in sensory-motor integration within the SMAp may play a part in the pathophysiology of dystonia. Using a model of secondary dystonia obtained by 3-nitropropionic acid intoxication in rhesus monkeys, we first provide evidence that the SMAp was overexcitable in dystonic animals. Second, we show that proprioceptive inputs processed by SMAp neurons were dramatically increased with wider sensory receptive fields and a mismatch between sensory inputs and motor outputs. These findings suggest that abnormal sensory inputs impinging upon SMAp neurons play a critical role in the pathophysiology of dystonia.

A challenging task for assessment of checking behaviors in obsessive-compulsive disorder.

OBJECTIVE: The present study concerns the objective and quantitative measurement of checking activity, which represents the most frequently observed compulsions in obsessive-compulsive disorder (OCD). To address this issue, we developed an instrumental task producing repetitive checking in OCD subjects. METHOD: Fifty OCD subjects and 50 normal volunteers (NV) were administered a delayed matching-to-sample task that offered the unrestricted opportunity to verify the choice made. Response accuracy, number of verifications, and response time for choice taken to reflect the degree of uncertainty and doubt were recorded over 50 consecutive trials. RESULTS: Despite similar levels of performance, patients with OCD demonstrated a greater number of verifications and a longer response time for choice before checking than NV. Such behavioral patterns were more pronounced in OCD subjects currently experiencing checking compulsions. CONCLUSION: The present task might be of special relevance for the quantitative assessment of checking behaviors and for determining relationships with cognitive processes.

[Deep-brain stimulation of the internal pallidum in multiple system atrophy]. / Stimulation cérébrale profonde du pallidum interne dans l'atrophie multisystématisée.

INTRODUCTION: The experience with deep-brain stimulation (DBS) in multiple-system atrophy (MSA) is sparse and generally disappointing. DBS is currently not recommended in MSA and its use is often related to a misdiagnosis. OBSERVATION: We describe the outcome of bilateral DBS of the internal pallidum in a 46-year-old woman suffering from MSA that initially resembled Parkinson's disease with prominent levodopa-induced dyskinesias. DBS of the left internal pallidum was performed in 1998 after a ten-year clinical course and improved dyskinesias. Six months later, the right side was implanted. A few months after the second surgery, the patient progressively developed signs of cerebellar and dysautonomic impairment and MSA was diagnosed. CONCLUSION: Our observation confirms the ineffectiveness of DBS of the internal pallidum in MSA and even suggests a harmful effect. DBS remains contra-indicated in atypical parkinsonism.

Side-effects of subthalamic stimulation in Parkinson's disease: clinical evolution and predictive factors.

Chronic bilateral high-frequency stimulation of the subthalamic nucleus (STN) is an alternative treatment for disabling forms of Parkinson's disease when on-off fluctuations and levodopa-induced dyskinesias compromise patients' quality of life. The aim of this study was to assess the evolution of side-effects during the first year of follow-up and search for clinical predictive factors accounting for their occurrence. We compared the frequency of side-effects at 3 and 12 months after surgery in a cohort of 44 patients. The off-medication scores of Unified Parkinson's Disease Rating Scale (UPDRS) II, III, axial symptoms, disease duration and age at surgery were retained for correlation analysis. Dysarthria/hypophonia, weight gain and postural instability were the most frequent chronic side-effects. Whereas dysarthria/hypophonia remained stable over time, weight gain and postural instability increased during the first year post-op. High axial and UPDRS II scores at surgery were predictive of dysarthria/hypophonia. Age and axial score at surgery were positively correlated with postural instability. Despite the occurrence of side-effects, the benefit/side-effects ratio of STN stimulation was largely positive during the first year of follow-up. Age, intensity of axial symptoms and UDPRS II off-medication score before surgery are predictive factors of dysarthria/hypophonia and postural instability after surgery.

From experimentation to the surgical treatment of Parkinson's disease: prelude or suite in basal ganglia research?

Parkinson's disease remains one of the greatest challenges facing those who work in the field of neurological research. Although the development of levodopa treatment revolutionised management of this debilitating diseases, no effective long-term therapy has yet been found. With recent advances in the understanding of basal ganglia physiopathology and the experimental demonstration of the efficacy of certain surgical procedures, there is a renewed interest in the surgical approach. This paper provides a chronological overview of the history of parkinsonian surgery and discusses the principal surgical options at our disposal today. These take three main forms: ablation (thalamotomy, pallidotomy and subthalamotomy); cell graft and gene therapy (mainly in the striatum); and deep brain stimulation (of the thalamus, globus pallidus pars internalis and the subthalamic nucleus). Our knowledge of basal ganglia function and our conception of how motor information is processed by this network have evolved parallel to the development of surgical techniques. Recent results from both clinical and experimental studies underline the complexity of the physiopathological mechanisms which generate parkinsonian symptomatology and lead us to question our assumption that each class of clinical signs (tremor, akinesia, rigidity, levodopa-induced dyskinesias...) is produced by a specific and separate mechanism. In the same way, comparison of the electrophysiological and biochemical effects of the different techniques induced in brain function vary considerably. This complex world of interaction and interconnection is a labyrinth that we are still far from comprehending in its entirety. All the more reason, in consequence, for extending experimental investigation into the impact of any new therapy before proposing its clinical application.

Gait and attentional performance in freezers under methylphenidate.

BACKGROUND: Attentional resources appear to be involved in the occurrence of FoG. The Parkgait study recently reported that methylphenidate reduces gait hypokinesia and freezing of gait (FoG) in advanced PD patients receiving STN-DBS in the off-dopaminergic drug condition. Methylphenidate is considered to improve attention. The primary objective of the present ancillary study was to determine whether methylphenidate reduced the interference between a cognitive task and gait in patients with FoG. The study's secondary objective was to compare attentional performance in methylphenidate-treated and placebo-treated patients. METHODS: A total of 24 patients (from two centers) were included in the study. Patients were randomly assigned 1:1 to a three-month course of methylphenidate (1mg/kg/day) or placebo. Patients were assessed after an acute L-dopa challenge. The primary outcome criterion was the stride length ratio ((dual-task stride length minus free gait stride length)/free gait stride length). Trials with FoG episodes were excluded from the analysis. Secondary outcomes included changes in reaction times for computerized attention tasks and FoG severity. RESULTS: When comparing patients receiving methylphenidate with those receiving placebo, we did not observe any significant differences in the interaction between the dual task and gait or in attentional performance. CONCLUSION: As in the main Parkgait study, methylphenidate did not reduce gait hypokinesia in patients receiving dopaminergic treatment. Our present results suggest that the reduction in the number of FoG episodes previously observed in patients on methylphenidate was neither due to interaction between a dual-task and gait nor an increase in attentional performance.

Somatosensory evoked potentials in the assessment of peripheral neuropathies: Commented results of a survey among French-speaking practitioners and recommendations for practice.

BACKGROUND: Somatosensory evoked potentials (SSEPs) are increasingly performed for the assessment of peripheral neuropathies, but no practical guidelines have yet been established in this specific application. STUDY AIM: To determine the relevant indication criteria and optimal technical parameters for SSEP recording in peripheral neuropathy investigation. METHODS: A survey was conducted among the French-speaking practitioners with experience of SSEP recording in the context of peripheral neuropathies. The results of the survey were analyzed and discussed to provide recommendations for practice. RESULTS: SSEPs appear to be a second-line test when electroneuromyographic investigation is not sufficiently conclusive, providing complementary and valuable information on central and proximal peripheral conduction in the somatosensory pathways. CONCLUSIONS: Guidelines for a standardized recording protocol, including the various parameters to be measured, are proposed. CLINICAL RELEVANCE: We hope that these proposals will help to recognize the value of this technique in peripheral neuropathy assessment in clinical practice.

Minimal tissue damage after stimulation of the motor thalamus in a case of chorea-acanthocytosis.

Autopsy findings are reported from a patient with chorea-acanthocytosis treated for 2 years by deep brain stimulation (DBS) of the motor thalamus. Postoperative testing showed a progressive improvement in axial truncal spasms. Although relatively high currents were used for 2 years in this patient, postmortem analysis showed minimal tissue damage in the vicinity of the electrode tip. It is concluded that DBS has little impact on the surrounding tissues.

Movement disorders induced by gamma-aminobutyric agonist and antagonist injections into the internal globus pallidus and substantia nigra pars reticulata of the monkey.

Injections of bicuculline into the medial segment of the globus pallidus (GPi) of the monkey induced dose-dependent hypokinesia with dystonic attitudes in contralateral limbs whereas muscimol injections elicited choreiform movements. Injections of the same drugs in substantia nigra pars reticulata (SNr) provoked severe axial postural anomalies with rotational behavior. Conversely, contralateral hypertonia after bicuculline and contralateral hypotonia after muscimol injections were observed. These data suggest that GABA inputs into GPi and SNr play different roles in terms of motor and postural control and add new insights into the pathophysiology of dystonias.

Reorganization of area 5 neuron activity in trained deafferented monkeys.

A command function is attributable to certain area 5 neurons which clearly fire before movement in trained monkey. Statistical analysis allowed us to define two categories of spontaneous firing mode for these cells: type I which exhibits a random pattern of discharge (14%), and type II displaying markedly "bursty firing". After deafferentation, both categories were still observed in the same proportion. However, the discharge pattern and frequency in type II cells remained altered for 5 months. This paralleled rises in neural latency response (RS) and reaction time (RT). Beginning the 6th month, there was a progressive reorganization of the spontaneous activity along with normalization of RS and RT. Our results support the idea that an enhancement of the excitability of these area 5 neurons, initially depressed by the suppression of sensory inputs, occurs over time. This sensitivity gain could be due to neural network rearrangements induced by repetitive operant solicitation.

Effects of L-DOPA on neuronal activity of the globus pallidus externalis (GPe) and globus pallidus internalis (GPi) in the MPTP-treated monkey.

We studied the effects of L-DOPA on the firing patterns of pallidal neurons in experimental parkinsonism. After a unilateral injection of MPTP, we observed a decrease in the firing rate of GPe neurons, and a slight increase in their bursting activity. In the GPi, there was a considerable augmentation of both neuronal firing frequency and the number of bursting cells. During l-DOPA treatment (10 mg/kg), GPe neurons.pattern is almost unmodified. The firing frequency of GPi neurons, on the contrary, decreased even lower than the control level. A slight reduction was observed in bursting activity. These unexpected results would show that the normalizing effect of L-DOPA on GPi output is limited.

Influence of cognitive strategies on the pattern of cortical activation during mental subtraction. A functional imaging study in human subjects.

Functional magnetic resonance imaging (fMRI) at 1.5 T was used to investigate the influence of cognitive strategies on cortical activation during mental calculation. Twenty-nine right-handed subjects performed a serial subtraction of prime numbers. Even though a common corpus of brain areas was activated during this mental calculation, differences appeared between subjects in function of their spontaneous cognitive strategy. In subjects using a so called verbal strategy (n=15), the main activation was located in the whole left dorsolateral frontal cortex with a little activation of the inferior parietal cortex. In subjects using a so called visual strategy (n=14), a bilateral activation in the prefrontal cortex and a high activation in the left inferior parietal cortex were observed. These results demonstrate that numbers are processed through a distributed network of cortical areas, the lateralization of which is clearly influenced by subject strategy.