[Spasticity and botulinum toxin in 2003. An update]. / spasticité et toxine botulique. Le point en 2003.

After the spastic foot in cerebral palsy, there are now wider indications for botulinum toxin injections in spasticity. Post stroke upper limb spasticity has been usefully treated by botulinum toxin in several studies, including double blind placebo-controlled studies. Two serotypes and one serotype B are marketed, with various properties. Botulinum toxin has been studied in multiple etiologies of spasticity. In multiple sclerosis, few studies revealed an efficacy in angulations and comfort. In spinal cord injuries, gait and sphincter disorders can be improved. In post stroke spasticity, lower limb angulations are improved, but gait remained difficult to evaluate. In upper limb spasticity, angulation, function and quality of life were improved in double blind, placebo controlled studies. Comparisons of costs and efficacy are made between botulinum toxin and the other antispastic methods.

Rat seminal vesicle FAD-dependent sulfhydryl oxidase. Biochemical characterization and molecular cloning of a member of the new sulfhydryl oxidase/quiescin Q6 gene family.

Rat FAD-dependent sulfhydryl oxidase was purified; partial sequencing indicated that it was homologous to human quiescin Q6. A cDNA (GenBank accession no. AF285078) was cloned from rat seminal vesicles, and active recombinant sulfhydryl oxidase was expressed in Chinese hamster ovary epithelial cells. This 2472-nucleotide cDNA has an open reading frame of 1710 base pairs, encoding a protein of 570 amino acids including a 32-amino acid leader sequence and two potential sites for N-glycosylation. One of them is used and the 64,000 M(r) purified protein was transformed to 61,000 by the action of endoglycosidase F. Northern blotting and reverse transcription-polymerase chain reaction analyses showed that there were small amounts of sulfhydryl oxidase in the rat testis, prostate, lung, heart, kidney, spleen, and liver, and that the gene was highly expressed in seminal vesicles and epididymis. Rat sulfhydryl oxidase cDNA corresponds to the human cell growth inhibiting factor cDNA, which could be a differently spliced form of quiescin Q6. Comparing sulfhydryl oxidase sequences with those of human quiescin Q6 and mammalian and Caenorhabditis elegans quiescin Q6-related genes established the existence of a new family of FAD-dependent sulfhydryl oxidase/quiescin Q6-related genes containing protein-disulfide isomerase-type thioredoxin and yeast ERV1 domains.

Effects of propofol on H-reflex in humans.

BACKGROUND: Depression of spinal cord motoneuron excitability has been proposed to contribute to surgical immobility. The H-reflex, which measures alpha-motoneuron excitability, is depressed by volatile anesthetics, whereas the action of propofol is unknown. The objective of this study was to determine the effects of propofol anesthesia on the H-reflex. METHODS: In 13 patients (group 1), H-reflex was measured before (T0), 3 min after (T1), and 10 min after (T2) a 2-mg/kg bolus dose of propofol, followed by an infusion of 10 mg x kg(-1) x h(-1). Ten patients (group 2) were studied when propofol was given via a programmable pump set to a propofol blood concentration of 6 microg/ml, and 10 patients (group 3) were studied with the pump set to 9 microg/ml. Latencies and amplitudes of H-reflexes (H0, H1, H2) and M-responses (M0, M1, M2) of the soleus muscle were recorded, and H/M ratios (H0/M0, H1/M1, H2/M2) were calculated. RESULTS: In group 1, H-reflex amplitudes and the H/M ratio were diminished after induction with propofol (H0 vs. H1, P = 0.033; H0/M0 vs. H1/M1, P = 0.042). After 10 min of propofol infusion, the H2/M2 ratio was still decreased versus H0/M0 (P = 0.031). In group 2, no difference was detected. In group 3, propofol depressed H-reflex amplitudes at T2 (H0 vs. H2, P < 0.01), and amplitudes were also lower at T2 than at T1 (H1 vs. H2, P < 0.01). In this group, the H/M ratio decreased from T0 to T2 (H0/M0 vs. H2/M2, P < 0.002). CONCLUSIONS: During steady state conditions using propofol as the sole agent, a depression of the H-reflex is observed only at a high blood concentration of 9 microg/ml. The authors suggest that immobility during propofol anesthesia is not caused by a depression of spinal motoneuron circuit excitability.

[Lower limb spasticity in adults. Clinical evaluation with motor block]. / La spasticité des membres inférieurs chez l'adulte. Evaluation clinique et blocs moteurs.

Clinical assessment of spastic lower limbs in adults requires identification of neurologic components (motor deficit, spasticity, co-contraction, sensory deficit) and non-neurological components (contracture) of the motor disorder and the intrinsic mechanism of the "spastic" gait. In order to determine the nature of a complex motor disorder, the clinical can call on different diagnostic tools such as motor blocks or gait analysis measuring time-distance parameters, kinematic, kinetics, gait dynamic electromyography and energy expenditure for gait performance. Clinical assessment is guided by the therapeutic goal (function, gait, mobility, pain relief, nursing), both to propose treatment and objectively monitor treatment results in terms of deficit (strength, flexibility, appropriate contraction, muscle activation) and handicap (gait, transfers, activities of daily living).

[Peripheral neurotomy in the treatment of spasticity. Indications, techniques and results in the lower limbs]. / Les neurotomies périphériques dans le traitement de la spasticité. Indications, technique et résultats aux membres inférieurs.

INTRODUCTION: The aim of this study was to review the principle of peripheral neurotomy for the treatment of lower limb spasticity. The importance of clinical assessment is stressed (with particular focus on motor block) for determining the best surgical indications. MATERIAL AND METHOD: Between 1989 and 1997, 392 neurotomies were performed in 277 patients in the neurosurgery department of the Henri Mondor Hospital, Créteil, France. Surgical technique consisted in partial and segmental resection, involving each motor collateral branch of muscles with excessive spasticity. For the lower limb, the most frequent neurotomy was performed on collateral branches of the posterior tibial nerve (66%) for the spastic foot. RESULTS: Preoperative motor block provided the advantage of "mimicking" the effect of the neurotomy and therefore gave the patients an idea of the expected result of surgery. For the spastic foot, posterior neurotomy led to the disappearance of ankle clonus in all patients. When antagonist muscles were present, splints were no longer needed after neurotomy. Cinematic analysis showed that tibial neurotomy improved angular variations of the second rocker of the ankle during stance. CONCLUSION: Peripheral neurotomies are simple and safe procedures for the treatment of lower limb spasticity and should be routinely used in general neurosurgery practice.

[Treatment of spasticity with injections of botulinum toxin. Review of the literature]. / Traitement de la spasticité par injections de toxine botulique. Revue de la littérature.

Botulinum toxin injections are a new treatment for limb spasticity. Intramuscular injections can be performed in spastic muscles; efficacy occurs one or two weeks later, with a mean duration of three months. Clinical action is related to chemical denervation of presynaptic motor end nerves by the botulinum toxin. Double blind studies versus placebo have demonstrated the improvement of limb spasticity after injections of botulinum toxin. Ashworth scales, articular angulations, pain and spasms improve both in upper and lower limb spasticity. Functional scores are not changed in the upper limb, but quality of life improves. Kinematic parameters of gait are improved in lower limb spasticity, especially in children with cerebral palsy disorders. There were no reports of serious side effects. Botulinum toxin is a safe and effective treatment of localized spasticity in adults and children.

Improvement of action tremor by chronic cortical stimulation.

The authors present a patient who had long-term improvement of a severe upper limb action tremor after chronic cortical stimulation. A 40-year-old woman complained of facial pain and tremor of the left arm after removal of an acoustic neurinoma. A motor cortex stimulation was performed to treat the deafferentation facial pain in 1993. Chronic cortical stimulation induced complete relief of both pain and tremor and allowed the patient to recover functional capacity of the limb. These effects persisted throughout a 32-month follow up. Differential effects on pain and tremor were observed when parameters of stimulation were varied, suggesting different mechanisms for the relief of pain and tremor. Attention was focused on control of the tremor. This effect could be the result of the inhibition of subcortical structures which are involved in tremor. Chronic cortical stimulation appears to be an effective treatment for controlling severe action tremors.

Peripheral selective neurotomy of the brachial plexus collateral branches for treatment of the spastic shoulder: anatomical study and clinical results in five patients.

A new type of peripheral selective neurotomy involving the collateral branches of the brachial plexus has been perfected for treatment of the spastic shoulder. Anatomical study of six cadaveric shoulders led to the specification of a surgical approach to the pectoralis major and teres major nerves, which innervate the main muscles implicated in shoulder spasticity. Between August 1994 and September 1995, five patients (four men and one woman) underwent two to four associated neurotomies of the upper limb, which included neurotomies of the pectoralis major (all five patients) and the teres major (two patients). The average follow-up period was 11 months, during which there were no local or general complications. The spasticity of the treated muscles resolved in all five patients (Held score range 3-0). The neurotomies led to statistically significant average amplitude increases in shoulder mobility, especially in abduction (+30 degrees), antepulsion (+50 degrees), retropulsion (+20 degrees), and external rotation (+20 degrees). The functionally useful active amplitude scores increased from 2.66 to 5.16/6. This functional improvements mainly involved the standing position and walking stability, as well as improvement in the range of motion of the lower limb. These results encourage the increasing use of this new type of neurotomy in treatment of the spastic upper limb.

Treatment of deafferentation pain by chronic stimulation of the motor cortex: report of a series of 20 cases.

Twenty patients with deafferentation pain were treated by chronic stimulation of the motor cortex. The central fissure was localized using stereotactic MRI and the motor cortex was mapped using intra-operative somatosensory evoked potentials. Seven patients with trigeminal neuropathic pain experienced definite pain relief varying between 40 and 100%. Ten patients had central pain secondary to central nervous system lesions. A satisfactory long-lasting pain control (pain relief > 40%) was obtained in five of them (50% of cases). One patient with pain from peripheral nerve injury obtained more than 80% pain relief. Two patients had pain from spinal cord lesions. One did not respond but the other obtained an excellent long-term result. The location of the effective stimulation plots was in agreement with the somatotopic maps of the primary motor cortex. One patient developed a small extradural haematoma which resolved spontaneously. None of the patients developed seizure activity. This study confirms the potential value of motor cortex stimulation in the treatment of certain forms of intractable pain, especially in cases with trigeminal neuropathic pain.

Physiological effects of selective tibial neurotomy on lower limb spasticity.

OBJECTIVES: To assess by electrophysiology the effect of tibial selective neurotomy on muscle imbalance of the spastic ankle. METHOD: The amplitudes of the H reflexes, M responses (muscle contractions recorded after stimulation of the tibial nerve), and Hmax:Mmax ratio were recorded in 12 patients with chronic lower limb spasticity, before and one month after tibial selective neurotomy. Recordings were done on medial and lateral gastrocnemius and soleus muscles. Clinical evaluation was done with both global (Held's score) and analytical tests (step measurements, gait velocity, and ankle angulation during active and passive movements). RESULTS: After neurotomy, gait improved in all patients. Held's score of spasticity was better in all patients. Active dorsiflexion of the ankle was unchanged in three patients, but the others improved by 5 degrees to 12 degrees. Hmax, Mmax, and Hmax:Mmax ratios were lower. The Hmax on the gastrocnemius muscle, clinical strength, Mmax of all the muscles, and Hmax:Mmax ratio for the soleus and lateral gastrocnemius muscle were significantly lower after surgery. CONCLUSION: There was an improvement of clinical and electrophysiological spastic indices after selective tibial neurotomy. Neurotomy acted not only on motor neurons by decreasing strength, but also the reflex enlargement by decreasing sensory afferents.

Is electrostimulation preferable to surgery for upper limb ataxia?

Ataxic syndromes of the upper limbs are observed in various situations but are usually a result of lesions involving the efferent pathways of the cerebellum, the superior cerebellar peduncles and midbrain. The commonest causes are multiple sclerosis, brain injury and focal neoplastic or vascular lesions. Cerebellar tremor, which usually comprises a postural and intentional component, is the commonest clinical form. In this review, we assess the value of the various surgical techniques in the treatment of this particular form of ataxia of the upper limbs.

Improvement of severe postural cerebellar tremor in multiple sclerosis by chronic thalamic stimulation.

Tremor can be particularly disabling in patients with multiple sclerosis (MS) and is mildly improved by drug treatment. The efficiency of stereotactic thalamotomy has been reported in a small number of patients but was counterbalanced by severe postoperative complications. Stimulation of the thalamic ventral intermediate nucleus, which is a less aggressive surgical method, is efficient in essential and in parkinsonian tremors. We report here the results of thalamic stimulation in 13 patients with MS with tremor. All patients were subjected to clinical examination, videorecording, and quantification of the functional disability before surgery and 3 months postoperatively. The surgical intervention was well tolerated in all cases. A clear improvement of the tremor was observed in 69.2% of the patients. Functional improvement was more varied and depended on the severity of tremor and coexistence of other neurological symptoms. Of the eight most severely affected patients, seven recovered the possibility to easily catch an object and use it. The results indicate that thalamic stimulation may be useful in the treatment of severe postural cerebellar tremor in MS.

[Contribution of position emission tomography in a case of Marchiafava Bignami disease: Morel's laminar sclerosis?]. / Apport de la tomographie par émission de positons dans un cas de maladie de Marchiafava Bignami: sclérose laminaire de Morel?

We report the case of a 26-year old alcoholic woman who abruptly presented with confusion associated with a major hypertonia. She evolved into a chronically vegetative state. Magnetic resonance imaging (MRI) was consistent with the diagnosis of Marchiafava Bignami disease. The corpus callosum first presented an oedematous aspect, then a central atrophy with an axial band. The clinical presentation and the functional imaging strongly suggest an association between the Marchiafava Bignami disease and diffuse cortical lesions, such as the laminar sclerosis of Morel.

[Selective peripheral neurotomy of the hamstring branches of the sciatic nerve in the treatment of spastic flexion of the knee. Apropos of a series of 11 patients]. / Neurotomies périphériques sélectives des collatérales pour les muscles ischio-jambiers dans le traitement de la spasticité en flexion du genou. A propos d'une série de 11 patients.

BACKGROUND: A series of 11 patients (9 adults and 2 children) were operated on by selective peripheral neurotomy for spastic knee in flexion. The objective of surgery was to restore bipodal stance and therefore walking for the adult patients and to improve walking for the diplegic children. METHODS: Spasticity was secondary to stroke or cranial trauma for the adult patients. The two children presented with Little disease. The delay between onset and surgery was on the average of 2 years. All the patients had a passive flexum of the knee of 20 degrees, and an excessive spasticity of the hamstrings (average Held score of 4.55). Peripheral selective neurotomies consist of a partial section of the spastic muscle's motor fascicles. The partial section concerns the afferent fibers to the spinal cord whose interruption leads to the disappearance of spasticity. The section also involves the motoneurones' axons and thus leads to partial denervation. RESULTS: The spasticity of the treated muscles disappeared in the 11 patients. Average follow-up was 16 months. The neurotomies led to improvement of walking without splint for 3 walking patients, and restored walking with a splint for 5 patients. One patient failed to walk. The two children had a dramatic improvement of their walk. CONCLUSION: Our results incite us to increasingly indicate this type of neurotomy in the treatment of the spastic lower limb.

Laryngeal tardive dyskinesia.

Neuroleptic treatment frequently induces movement disorders, the tardive dyskinesias. These are frequently seen in the orobuccolingual region. Although the beginning of neuroleptic treatment can cause acute dystonia and breathing difficulty, chronic neuroleptic treatment has only rarely been shown to affect the laryngeal musculature. Laryngeal abnormal movements were assessed in 12 patients receiving chronic neuroleptic treatment who showed orobuccolingual abnormal movements. The Abnormal Involuntary Movement Scale was systematically assessed in all patients. Clinical examination revealed that 8 had speech disorders, 8 had breathing difficulties, and 5 had swallowing disorders. Laryngeal endoscopy showed that 10 of the patients had intermittent partial obstruction of the glottis, due to repetitive abnormal adduction of the vocal cords. Percutaneous electromyography of the thyroarytenoid muscles showed spontaneous irregular and prolonged muscular contractions, while the patients were at rest and when speaking. The patients were not aware of these movements. In view of this finding, laryngeal dyskinesia should be considered and studied as a possible side-effect of chronic neuroleptic use.

[Hypoxic cerebral lesions. X-ray computed tomography and MRI aspects. Apropos of 20 cases. Selective vulnerability of the striatopallidum]. / Lésions cérébrales hypoxiques. Aspects TDM et IRM. A propos de 20 cas. Vulnérabilité sélective du striatopallidum.

Brain lesions following hypoxic-ischaemic injuries are known from autopsy studies, but their appearance in live patients has been only occasionally described, and only sporadic reports have been published on their CT and MRI images. Over a 2-year period (1991-93) we studied the clinical, MRI and CT features in 20 patients shortly after a severe hypoxia. Clinical examination showed motor extrapyramidal signs in 13 cases and coma in 7 cases. MR with inversion recovery (IR) and T2-weighted spin echo (SE) sequences was performed in 17 patients and CT in 15. Bilateral lesions were found in 11 cases, but in 13 of them CT was normal. Radiological lesions were always symmetrical and bilateral, located in the pallidum in 10 cases, the striatum in 4 cases and the thalamus in 2 cases. Additional white matter lesions were present in only 4 MRI examinations. No relationship was found between the mechanism of hypoxia and the severity of clinical signs. The course of the clinical signs was correlated with the presence of radiological lesions. In comatous patients there was a relation between parkinsonism and abnormalities of basal ganglia. None of the patients who had perinatal asphyxia had radiological lesions. The presence of pallidal or striatal confirmed the hypoxic origin of neurological symptoms, especially in patients with parkinsonism. MRI, particularly in IR sequences, makes it possible to detect small lesions in basal ganglia after hypoxic injuries.

[History of the basal ganglia system. Slow development of a major cerebral system]. / Histoire du système des ganglions de la base. La lente émergence d'un système cérébral majeur.

Initially, basal ganglia was a descriptive term for onto- and phylogenetic or topographic classifications. A variable list of structures were included as basal ganglia. A major step was made when the thalamus was separated from the "striated bodies" (Vic d'Azyr, 1786) which was sometimes taken into account in the French description of the noyaux gris centraux. Even if the term is not perfect, it is preferable to "the system of basal ganglia". The subdivisions of the putamen, the distinction between the striatum and the pallidum were not really made until the beginning of the twentieth century. Modern tracing methods were needed to demonstrate the main connections. It was not until the end of the 1960s that the importance of the striato-pallido-nigral network within the basal ganglia and the cortico-striatal connections, the main afferent system, were recognized. With the description of the cortico-striatal connections, the sub-cortical system with multiple complex "loops" was questioned. The term "extra-pyramidal system" had an exaggerated success. Initially, it designated descending non-pyramidal afferents (some which do not exist) and their source. In 1992, Spatz based his separation of this heterogeneous group on the iron content. The terms of extra-pyramidal "system" and "syndrome" should be abandoned by clinicians. Physiological interpretations have varied. The role of automatic "habitual" motricity, derived from a concept of hierarchic, Jacksonian cerebral organization, was questioned when the pyramidal network was described. Clinico-pathological analysis (hemiballism, Parkinson's disease ...) has placed new emphasis on the motor role, for a time the only role accepted as real. More recently, debate has centred on other roles, particularly in cognition and motivation. An illustration of functions other than purely motor functions of the basal ganglia is given by the syndromes of loss of psychic auto-activation secondary to bilateral lesions.

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. At variance with the scalp RPs recorded in the same patients, the intracerebral potentials were either negative, or positive, depending on the recording site. No consistent differences in duration and time of onset could be established between the MC and the SMA RPs, at least with the used time resolution. RPs were only occasionally observed in the parietal cortex and hippocampus and none were recorded from the amygdala, the temporal, temporo-occipital, prefrontal, frontal and cingular cortices. The wide topographical distribution of the scalp RPs may not be fully explained by the above intracortical findings, leaving the possibility that other generators exist, whose locations remain to be determined.