[Treating writer's cramp: 14 years' experience with botulinum toxin]. / Prise en charge de la crampe de l'écrivain: 14 ans d'expérience de la toxine botulique.

INTRODUCTION: Writer's cramp is a focal dystonia; treatment remains disappointing. We report our 14-year experience with a population of 119 patients aged between 18 and 85 years (average age 43 years). METHODS: Treatment was based on botulinum toxin injections (Dysport) and physiotherapy. Patients were reviewed every four to six months with clinical and video evaluation by three different observers and subjective analysis of the treatment efficiency by the patient (score of 1 to 3). The post-injection deficit, if present, was also quantified. RESULTS: In the group treated with toxin and physiotherapy, cramps improved (score 2 and 3) in 61.6% of patients; a majority of patients (n=14) reported they were moderately satisfied (score 2). In the group treated with toxin alone, 37.9% of patients were improved (score 2 and 3) with a majority (n=18) very satisfied reporting normal writing (score 3). Age was not a predictor of therapeutic response. Good results were obtained with injections of the flexor carpi radialis followed by flexor digitorum profundus II and III and the flexor pollicis longus. Seventy-one per cent of injections caused moderate muscle weakness, minimally disabling compared to the benefit of injections. Twenty-seven patients were followed for more than two years and three patients, who had achieved score 3 with excellent response, were followed for 14 years with very efficient repeated injections. If the injections were not effective the first time, we re-assessed the situation and adjusted the injections; we considered that toxin treatment was unsuccessful after three injections without benefit. CONCLUSION: The choice treatment for writer's cramp remains well-targeted injections of botulinum toxin. Physiotherapy is useful when the toxin injections are ineffective in completely improving writing. This requires close cooperation between the injector, the physiotherapist and the patient.

Phagocytic cell function in aged subjects.

In order to study the activity of phagocytic cells in normal and pathological aging, we compared normal young and aged subjects and patients with Alzheimer's (AD) or Parkinson's (PD) disease. Blood granulocytes and monocytes were separately assayed for ingestion of three different particle species (opsonized zymosan, immunoglobulin-coated sheep red cells (IgG-SRC) and glutaraldehyde-treated sheep red cells (G-SRC]. The superoxide anion production induced by these particles was also measured. All granulocyte responses to zymosan and IgG-SRC were depressed in the three aged groups as compared to young controls. Hence, only functions involving a specific receptor (Fc or C3b receptor) seemed affected. Monocyte activity was slightly decreased in the same groups. No difference was found between AD or PD patients and normal aged subjects. Hence the phagocytic and oxidative defects we found were a consequence of aging.

Chronic progressive spinobulbar spasticity. A rare form of primary lateral sclerosis.

Although it was first described over a century ago (by Charcot in 1865; by Erb in 1875), the concept of primary lateral sclerosis (PLS) is still not universally accepted. Despite this skepticism, several well-documented cases of isolated degeneration with varying degrees of involvement of corticospinal pyramidal pathways have been reported in the literature. The clinical manifestations in these cases can take one of two forms, ie, isolated spasmodic paraplegia or tetraplegia on the one hand or spasmodic tetraplegia associated with a pseudobulbar syndrome featuring severe spastic dysarthria (chronic progressive bilateral spinobulbar spasticity) on the other hand. Obviously, without firm pathologic data, PLS is a hazardous diagnosis for isolated paraplegia or tetraplegia. Conversely, for bilateral spinobulbar spasticity, it would appear to be the only diagnosis possible once investigate findings have eliminated the other possibilities, such as a pyramidal form of amyotrophic lateral sclerosis or a spinal form of multiple sclerosis. To underscore this point, in this report, five cases of chronic progressive bilateral spinobulbar spasticity developed over 5, 10, 12, 10, and 28 years, respectively, for which the only possible diagnosis was PLS. It was concluded that there are three forms of degenerative diseases of the principal motor pathways: one involving both central and peripheral neurons, ie, amyotrophic lateral sclerosis; one involving only peripheral neurons, ie, spinal amyotrophy; and one involving only central motor neurons, ie, PLS.

Isolated, chronic, epilepsia partialis continua in an HIV-infected patient.

BACKGROUND: The characteristic clinical feature of epilepsia partialis continua (EPC) is chronic focal myoclonus, usually involving the distal part of one extremity. A variety of pathogenetic factors have been implicated in EPC. In children, the most common cause is Rasmussen encephalitis; in adults, it is vascular disease or tumor involving the sensorimotor cortex. Epileptic seizures are a relatively common manifestation of central nervous system involvement in patients infected with human immunodeficiency virus (HIV), but, to our knowledge, isolated, chronic EPC has not been previously reported. OBJECTIVE: To describe a case of typical EPC in a patient infected with HIV. DESIGN AND SETTING: Case report from an epilepsy center. PATIENT: A 58-year-old man infected with HIV had continuous myoclonus that involved the right arm and was associated with intermittent motor seizures. The electroencephalographic findings were normal at the onset of the symptoms, but left central theta rhythm appeared later. Serial magnetic resonance imaging scans obtained over a 3-month period showed a progressively increasing left rolandic T2-weighted hypersignal. Histologic study of a stereotactic biopsy specimen demonstrated inflammation characterized by perivascular mononuclear cell infiltration. The only detectable cause was HIV infection. Immunocytochemical tests ruled out JC virus. Neuropsychological testing showed no evidence of cognitive impairment. An electroencephalographic-electromyographic "back-averaging" study showed a reproducible transient left biphasic complex preceding the bursts by about 30 milliseconds on the C3 and F3 electrodes, thus demonstrating that the myoclonus was of cortical origin. High-dose corticosteroid (prednisone, 100 mg/d) and anti-HIV- 1 therapy led to marked radiological and clinical improvement. Infection with HIV enhances the risk of seizures, but, to our knowledge, this is the first reported case of "inflammatory" EPC. CONCLUSIONS: The present case suggests that the possibility of central nervous system involvement by HIV-1 should be taken into account in the diagnostic workup of patients with EPC. This case also indicates that treatment can be effective.

Lipomas of the corpus callosum and epilepsy.

An analysis of four cases of lipomas of the corpus callosum with epilepsy, and a review of the literature, have led to the following conclusions: (1) Epilepsy as an almost constant feature is often severe, nearly always partial, and begins before the age of 15. (2) Pathophysiology of the seizures appears to be essentially an interhemispheric disconnection (rather than the classic theory that seizures depend upon an infiltration of the cingulate gyri by fibrous tissue growing out from the capsule of the lipoma). This disconnection is responsible for a facilitatory and disinhibitory action that favors the appearance of seizures caused by an epileptogenic lesion (the effects of which remain subthreshold in the presence of an intact corpus callosum playing its normal inhibitory role). This hypothesis is clearly applicable in explaining the epilepsies--in every way comparable--that are observed in agenesis of the corpus callosum and in Marchiafava-Bignami disease.

A multicenter proton magnetic resonance spectroscopy study of neurological complications of AIDS.

Human immunodeficiency virus (HIV) infection as seen in Europe and the United States has predominantly been contracted through male homosexual sex or intravenous drug abuse. In infected subjects, the brain is frequently affected both clinically and neuropathologically. The aim of this multicenter study has been to evaluate the value of single-voxel proton magnetic resonance spectroscopy (MRS) in the assessment of the neurological complications of acquired immunodeficiency syndrome (AIDS). MRS (voxel size = 8 ml, TR/TE = 1600/135 msec) was performed in 137 HIV-1-seropositive patients and 64 healthy controls without risk factors at three clinical MR sites operating at 1.5 T. The first result of this multicenter trial is that good reproducibility of results among participating sites was found. This demonstrates the reliability and robustness of MRS in the study of in vivo brain metabolism. In HIV patients, there was no significant correlation between metabolite ratios of brain detected by MRS and CDC grouping of patients or CD4 count. In contrast, the variations of brain metabolite ratios (NA/Cr, NA/Cho, and Cho/Cr) were related to the occurrence of encephalopathy, brain atrophy, or diffuse white matter lesions. There was no significant difference in brain metabolites between male homosexual AIDS patients and male intravenous drug user AIDS patients, whatever their neurological status (neurosymptomatic or neuroasymptomatic). Thus, the mode of transmission of HIV infection does not appear to affect the cerebral changes observed in the proton spectra from AIDS patients. Because of its ease of implementation and high information content, single-voxel proton MRS is likely to play a significant role in the evaluation of HIV-related encephalopathies.

Multiple sclerosis: cell-mediated immunity to human brain gangliosides.

Cell-mediated immunity (CMI) to myelin components has been implicated in Multiple Sclerosis (MS) pathogenesis: two targets were suggested, Myelin Basic Protein with controversial results and, more recently, gangliosides. In order to investigate their possible involvement, we have performed Leukocyte Migration inhibition (LMI) tests in the presence of human brain gangliosides. Thirty nine MS patients (twenty four being "definite", according to McDonald and Halliday's classification), twenty nine patients with Other Neurological Diseases (OND), thirty six patients with Inflammatory diseases (ID) and forty healthy controls were tested. MS patients were divided into two groups, depending on the clinical stage of the disease. The mean migration inhibition percentage of the MS-attack group was found to be significantly different from the four others (p less than 0.01) (24.4 +/- 16.2 versus 10.9 +/- 8.5 in MS without attack, 4.4 +/- 12.9 in OND, 3.9 +/- 13.9 in ID and 11.1 +/- 12.1 in healthy subjects). LMI to gangliosides is therefore significantly increased during the attack stage in MS. These results support the notion of a Delayed Type Hypersensitivity to these glycolipids during the active stage of the disease.

Partial complex epileptic seizures provoked by ingestion of alcohol.

The authors describe three cases of partial complex epileptic seizures precipitated by alcohol consumption. No other causative factor was found. Brain imaging was normal. The role of alcohol in precipitating this type of seizures is discussed.

CT of primary muscle diseases.

Seventy-five patients with a variety of muscular dystrophies were studied using computed tomography (CT). At least 11 slices were taken in each patient, from the forearm to the lower leg. Sufficient information was obtained to provide some CT characteristics of several dystrophies, including Duchenne muscular dystrophy, facioscapulohumeral syndrome, limb-girdle muscle myopathies, and myopathic dystrophies. CT promises to be of increasing value in these areas in the future.

A multiparametric data analysis showing the potential of localized proton MR spectroscopy of the brain in the metabolic characterization of neurological diseases.

We conducted an extended clinical evaluation of localized proton magnetic resonance spectroscopy (MRS) of the brain, performed on various brain diseases using short stimulated echo times. Pathologies studied were mainly multiple sclerosis, stroke, leukoaraiosis, AIDS-related leukoencephalopathies and glial tumors. Other miscellaneous pathologies were also studied. Magnetic resonance examination of the brain was conducted on a Siemens Magnetom SP63 (equipped with a 1.5 T magnet). Localized proton MRS was performed on a routine basis immediately after imaging, using the STEAM (stimulated echo acquisition mode) with a short echo time (20 ms) combined with a CHESS (chemical shift selective excitation) sequence. One or two VOI (8 ml) were examined. Data on 125 spectra were processed by principal component analysis (PCA) and conventional variance analysis. The following metabolite resonances were studied: inositol-glycine, taurine-scyllo-inositol, choline derivatives, phosphocreatine-creatine, aspartate, glutamine glutamate, N-acetylaspartate, acetate and lactate. PCA demonstrates that the different metabolic variables are independent. The analysis of groups of spectra clearly demonstrates that the metabolic profiles detected by localized MRS in various pathologies (i) differ significantly from controls, and (ii) allow a metabolic discrimination between groups of pathologies. Results of PCA are confirmed by variance analysis. Strokes are characterized by an increase in lactate concentration and leukoaraiosis by a decrease in inositol-glycine resonance. AIDS-related leukodystrophies are characterized by increases in lactate and choline concentrations. Reduction in N-acetylaspartate which is observed in most pathologies is not significant in the small lesions of white matter. Lactate has often been found in MS plaques, but no variation in the choline/phosphocreatine ratio was observed. GABA was tentatively assigned in the spectrum of a patient with epilepsy under sodium valproate treatment. This study illustrates the clinical feasibility of the technique, the value of a multiparametric data analysis in the definition of the pertinent variables characterizing the metabolic impairment, and the impact of localized proton MR spectroscopy of the brain in the assessment of cerebral suffering.

Development of focal chronic epilepsy following focal status epilepticus in adult patients.

In several experimental models, status epilepticus (SE) leads to secondary brain hyperexcitability and epileptogenesis. In humans, such phenomena have been rarely demonstrated, particularly in cases of SE involving the neocortical structures. We report a 36 year old woman that presented partial SE in May 1991 involving the right cerebral hemisphere. The patient was then treated in the intensive care unit with artificial ventilation and anesthesia by pentobarbital and clometiazole. MRI showed transient right parietal and temporal posterior cortical hyperintensity. The cause of SE was not determined. Three months later, the patient developed partial complex seizures with aura characterized by vertigo, nausea and auditory hallucination. Ictal video/EEG recording showed a clear right temporal posterior onset of the discharges. We speculate that status epilepticus created the lesions which subsequently caused the focal chronic epilepsy.

[Study of sensory involvement and dysautonomia in HIV infected patients. A prospective study of 55 cases]. / Recherche d'une atteinte sensitive et dysautonomique chez des sujets infectés par le VIH. Etude prospective de 55 cas.

Subclinical impairment of the peripheral nervous system (PNS) is frequent during HIV infection. Nevertheless, little attention has been given to the autonomic nervous system (ANS). We undertook a prospective study of the ANS, exploring 55 HIV+ subjects with non-invasive functional tests for the cardiac ANS. We also made a quantitative study of thermic (small fibers) and vibratory (large fibers) sensibilities. The results do not show any statistically significant difference between HIV+ subjects and control subjects or between HIV+ subjects of groups I, II and III and those of group IV of the CDC classification. However, an individual analysis comparing the results of the infected subjects with those of the control group indicated the presence of functional abnormalities of the ANS (small fibers) in 34.5% of HIV+ patients with an equally frequent impairment of the thermic perception (small fibers) and of the vibratory perception (large fibers). Moreover, the signs of subclinical dysautonomia were more frequent in group IV (48.3%) than in groups I, II and III (19%) showing a progressive accentuation of the troubles during the course of the infection.

[The significance of quantified EEG in Alzheimer's disease. Changes induced by piracetam]. / Intérêt de l'EEG quantifié dans la maladie d'Alzheimer. Modifications induites par le piracétam.

One study was performed in 12 patients with presenile Alzheimer's disease (group I), the other one in 16 patients with mild senile dementia of Alzheimer type (group II). In each study, patients were divided into two randomized parallel groups, one receiving placebo, the other piracetam (9 g daily in group I piracetam and 2.4 g daily in group II piracetam) during three months, piracetam induced a decrease in EEG power on the 2-6 Hz range (group I piracetam), 3-5 Hz and 7 Hz (group II piracetam) and an increase of EEG power in the 9-11 Hz range (group I piracetam) and in the 10 Hz and 13 Hz frequencies (group II piracetam). There was also a significant improvement in the Trail Making Test part A in group II piracetam. Correlations between decreased EEG low frequency components and improvement in some psychometric tests were found significant in the two groups. It seems that the main effect of piracetam was to induce increased alertness. The same results were found in both studies; the good reproducibility suggests that EEG spectral analysis is a reliable tool in the assessment of psychotropic drug effects.

Facial reflex myoclonus induced by language: a neuropsychological and neurophysiological study.

We studied a 53 year old right-handed patient who presented isolated myoclonus of right facial muscles induced exclusively by language. Twitching significantly hindered speaking and reading performance. MRI and CT-scan revealed no brain lesion. Conventional EEG showed a few spike-waves predominantly in the left hemisphere. Spike-waves increased during drowsiness. An EEG-EMG polygraphic study was performed during stimulation tests which included linguistic tasks and non-verbal/non-linguistic tests. Myoclonus was triggered by speaking and writing but not by non-linguistic tasks. The severity of myoclonus was dependent on the complexity of the language task. Back-averaging of right facial EMG bursts failed to show a reliable EEG-EMG correlation. However, the facial reflex myoclonus might have originated from the left rolandicopercular cortex, as it was triggered by complex language activities. Findings in this case are compared with those reported for other forms of reflex seizure and myoclonus.

Devic's neuromyelitis optica: a clinicopathological review of the literature in connection with a case showing fatal dysautonomia.

A new clinicopathological case of Devic's neuromyelitis optica, including unusual predominant clinical features (fatal dysautonomia) is described herein: pathological examination showed extensive and severe demyelination mainly involving the optic nerves, the medulla, and spinal cord, which was particularly pronounced in the thoracic segments, and thus explained the unusual vegetative symptomatology. In a review of 45 clinicopathological cases described in the literature as Devic's disease (DD), it turned out that only 22 cases, including the patient described here, fulfilled the recently defined diagnostic criteria [Devic 1980]. Among the other 23 cases, 15 did not fulfill the criteria because of the occurrence of relapses, 3 others had 2 separate pathological locations within the spinal cord, and the remaining 5 showed evidence that a disease other than DD was involved. Among the 22 cases which were definitely taken to be DD, the mean age at clinical onset was 39 years (+/- 14); it was characterized by acute bilateral visual loss and transversal myelitis which gradually led either to death or to partial or complete recovery. The pathological lesions, which mostly were located along the optic tracts and the spinal cord, were characterized by demyelination with inflammation and necrosis; in some cases the pathological process diffused into the medulla (8/22 cases) or the whole brainstem (4/22 cases). The cavitation of the spinal cord was not a key feature of the diagnosis, since it was observed on only 9/22 cases. Devic's neuromyelitis optica may be a separate nosological entity which differs from multiple sclerosis.

Radiosurgery for trigeminal neuralgia and epilepsy.

The role of Gamma Knife surgery in the field of functional surgery recently has evolved dramatically. For treatment of trigeminal neuralgia, Gamma Knife surgery is the least invasive procedure, with a low rate of hypesthesia. If a rate of complete relief similar to that of other surgical techniques could be achieved, this approach will become one of the main techniques used to treat the disease initially. The authors present their experience with a group of 16 patients with mesial temporal lobe epilepsy who have been treated successfully (15 completely seizure-free and 1 with rare, nondisabling seizures) without significant complication. After additional follow-up to demonstrate the absence of long-term consequences, this fascinating new approach could change epilepsy surgery practice dramatically.

Epilepsy and perisylvian lipoma/cortical dysplasia complex.

Intracranial lipomas are congenital malformations composed of mature adipocytes. They are usually located in the midline, particularly in the pericallosal region, a hemispheric location accounting for only 3 to 7% of cases. Review of the literature found 21 previous cases of hemispheric lipoma. Although hemispheric cerebral lipomas are rare, association with epilepsy appears to be frequent. We have recently studied two patients in whom epilepsy was the first clinical manifestation of hemispheric cerebral lipoma in the sylvian region. The patients presented with simple motor partial seizures as the first manifestation of the lesion. Neurological examination was normal. MRI disclosed in both cases a lesion involving the sylvian fissure with characteristics of the lipid signal. MRI also demonstrated abnormalities involving the cerebral cortex in the vicinity of the lesion (pachygyria-like aspect). Partial excision of the lesion was achieved in one patient but was followed by a worsening of seizures and neurological condition (hemiparesis). According to the literature, the prognosis for epilepsy in patients with hemispheric lipoma appears good. Several other arguments support non-surgical management: the lesion is benign and can be identified with a high degree of certainty by imaging; surgery is technically difficult due to adherence to adjacent vascular and cerebral structures and hypervascularity; location near functional brain tissue increases the risk of postoperative sequelae. In addition, mechanisms of epilepsy probably involve vascular and cortical dysplasic abnormalities. In consideration of the complexity of the lesion, hemispheric lipomas are more appropriately classified with localized cortical malformations rather than as simple extracerebral malformations.

[CT scan in acute vascular disorders of the cerebellum (author's transl)]. / Intérêt du C-T scan dans le diagnostic des accidents vasculaires du cervelet.

The clinical diagnosis of acute vascular disorders of the cerebellum (AVDC) is uneasy. Classical views emphasized poor prognosis and need for cranial decompression. These views were probably due to the fact that, in the past, a correct diagnosis was made in the cases with the most dramatic symptoms only. The authors report their findings in 13 cases of AVCD. The diagnosis was confirmed or established by CT scan, which showed cerebellar hemorrhage in 6 patients and cerebellar infarctus in 7 patients. CT scan lead to the recognition of AVCD cases with a more benign course than traditionally thought. In 12 out of 13 patients, there was no need for neurosurgical treatment. Finally, CT scans allowed the diagnosis of hemorrhage versus ischaemia and repeat scans demonstrated the evolution of the lesion.

[Comparison of the antiemetic effectiveness of high-dose corticosteroids with synacthene in nausea induced by chemotherapy: results of a randomized study]. / Comparaison de l'efficacité anti-émétique de corticoïdes à fortes doses à celle du synacthène lors de nausées induites par la chimiothérapie: résultats d'une étude randomisée.

One hundred two patients submitted to intensive chemotherapy were included in a randomized study with cross over comparing for the second course two anti-emetics: tetracosactide (D1: 3 mg, D2 and D3: 2 mg/d) and methylprednisolone (D1: 240 mg, D2 and D3: 160 mg/d). Most patients presented with malignant lymphoma. All patients experienced nausea and emesis during first course of chemotherapy. Results were similar in both groups, respectively for tetracosactide and methylprednisolone: no nausea 37 versus 40%, less than 3 emesis 69% versus 73%. Secondary effects were observed in 5 and 8% of cases. Tetracosactide with a schedule of 7 mg for 3 days gives the same results than methylprednisolone 560 mg for 3 days in chemotherapy induced nausea and emesis prevention.

[Painful legs and moving toes. A drug-induced case]. / Jambes douloureuses et orteils instables. Un cas d'origine médicamenteuse.

In this case of painful legs and moving toes the essential characteristics were to be secondary to a neuropathy due to vincristine and metronidazole. The disorders regressed in six weeks after the arrest of chemotherapy. To our knowledge, this is the first case due to a toxic, and more precisely to a drug.