[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.

[Value of single photon emission computed tomography in the characterization of fronto-temporal dementia subtypes]. / Intérêt de la tomographie d'emission monophotonique dans l'identification de sous-types de démences fronto-temporales.

DFT (fronto-temporal dementia) was diagnosed in 33 patients (17 males and 16 females), mean age 74.9 years, using the criteria from Neary et al. (1998). The severity of the disease was evaluated based on the presence or absence of 4 clinical groups of signs (behavioral, affective, neurological signs and language) allowing the calculation of a global clinical ratio on 20. A CT-scan and a Single Photon Emission Computed Tomography (SPECT) scan using Tc99m-ECD with quantification of brain perfusion were then performed. This study confirmed the classical clinical forms of DFT: a psycho-affective form (21%), where negative symptoms are predominant, and a behavioral disinhibited form (9%). Most of our cases (40%) corresponded to a global form. Finally we also identified a fourth clinical form: cognitive form because of predominance of language disturbances (30%). Concerning SPECT, we found 4 topographic forms: frontal global (46%), frontal superior (24%), frontal inferior (15%) and frontal unilateral (12%). In the frontal inferior form, behavioral disturbances were correlated to the intensity of frontal inferior hypoperfusion, corresponding to an orbito-frontal dysfunction. In the frontal superior form, psycho-affective disturbances (apathy) were correlated with frontal superior hypoperfusion (anterior cingulum and superior dorso-lateral cortex). Finally, in global and frontal inferior SPECT forms, language disturbances were correlated with a frontal inferior hypoperfusion (prefrontal and inferior left frontal cortex).

Absence epilepsy with fast rhythmic discharges during sleep: an intermediary form of generalized epilepsy?

PURPOSE: To describe a particular form of absence epilepsy (AE) characterized by an atypical EEG pattern (fast rhythmic discharges) during sleep and an unfavorable course with the persistence of seizures at a late age and drug resistance. METHODS: We reviewed the medical files of 31 patients diagnosed with AE at our institution been 1995 and 1999 for whom an EEG during sleep had been recorded. Patients with fast rhythmic discharges during sleep were selected for the study. RESULTS: Five patients showed the usual criteria of childhood absence epilepsy (CAE) or juvenile absence epilepsy (JAE), but with fast discharges of rhythmic spikes (10-15 Hz) during sleep. such as those typically observed in Lennox-Gastaut syndrome. Four patients were of adult age at the time of the last examination. Intellectual capacities were "borderline" in each case, with visible social and learning handicaps. Absences were the initial seizure event in all patients, but four patients developed generalized tonic-clonic seizures during the course of the disease. Treatment using a combination of antiabsence drugs such as valproate and lamotrigine led to only marginal improvement. CONCLUSIONS: These findings illustrate the utility of sleep EEG in detecting the fast rhythmic discharges that are markers of drug resistance in AE and suggest the existence of transitional forms of AE that are intermediate between idiopathic and cryptogenic/symptomatic generalized epilepsies.

[Epileptic seizures, hemiplegia and hyperglycemia: late discovery of a localized cortical dysplasia]. / Crises épileptiques, hémiplégie et hyperglycémie: révélation tardive d'une dysplasie corticale localisée.

A 56-year-old woman was hospitalized with a right hemiplegia and aphasia evoking a cerebral infarction. In fact the neurologic deficits were of post-ictal origin, secondary to a partial epilepsy which began a few weeks before, at the same time as a polyuria-polydipsia syndrome revealing diabetes mellitus. This case illustrates the possibility for a partial epilepsy to occur in relation with a nonketotic hyperglycemia. If in most of those cases there is no underlying cortical lesion, in some observations the hyperglycemia is associated with an infarction. In our case the MRI revealed another type of lesion: a cortical dysplasia in form of a unilateral micropolygyria with a perisylvian distribution centered around the insula. The discovery of a cortical dysplasia at such an age is very unusual.

[Long-term treatment of Alzheimer's disease: followup of a cohort of 255 patients treated with lacrine for four years]. / Traitement de la maladie d'Alzheimer au long cours: à propos d'une cohorte de 255 patients traités quatre ans par tacrine.

We describe the follow-up of a cohort of 255 Alzheimer's disease (AD) patients (81 males, 174 females) treated by tacrine during 4 years. We performed the survey of hepatic, cholinergic and general tolérance. Drug efficacy was measured by MMS examination on weeks 0, 18, 30, 52, 104, 156 and 208. A total of 190 patients (74.5 percent) were dropped out of this study, 75 (29 percent) for adverse events. We found 85 hepatic (33 percent), 79 cholinergic (31 percent), 31 (12 percent) neuropsychiatric and 72 general (28 percent) side effects. In term of drug efficacy we observed a global decline of 2.5 MMS points during the first year and 2 MMS points between W52 and W156. Tacrine's symptomatic efficacy, defined as the number of patients improved or stabilized at W30, was present in 50 patients (46 percent) among the 109 patients reaching W30. The intensity of symptomatic efficacy was expressed by a 2.7 MMS points increase in 37 patients improved on W30. The long term effects of Tacrine, measured by the MMS score at one year, showed a positive impact as the MMS was 2.5 points above the expected score in non treated AD patients. This study raises the practical problem of optimal cholinesterase inhibitors use in AD and the theoretical question of long term action of cholinesterase inhibitors on cerebral lesions of AD.

[Charcot Marie Tooth disease: exacerbation in pregnancy]. / Maladie de Charcot Marie Tooth: aggravation lors de la grossesse.

We report a case which illustrates the fact that an exacerbation of Charcot Marie Tooth disease, while rare, is possible during pregnancy. Moreover our case suggests the possibility of a positive effect of corticosteroids on such a complication, with an improvement of clinical symptoms as well as of electrophysiological results.

[Pharmaco-epidemiological study on the prophylactic treatment of migraine. National inquiry on attitude to prescription practices by primary care physicians and neurologists in Franceø]. / Etude pharmaco-épidémiologique sur le traitement de fond de la migraine.

A pharmacoepidemiological survey was conducted in order to understand the pattern of migraine prophylactic drug utilization by French physicians. Neurologists and primary care physicians completed a phone-mail-phone questionnaire which inquired about migraine prophylactic treatment. French neurologists and PCP made the same use of migraine prophylaxis in terms of indication, time interval between treatment onset and evaluation, and duration. The two most commonly chosen migraine prophylactic agents were dihydroergotamine and beta-blockers. This study also showed the importance of considering quality of life to evaluate efficacy of migraine prophylaxis.

Gamma knife surgery for mesial temporal lobe epilepsy.

PURPOSE: Gamma knife radiosurgery (GK) allows precise and complete destruction of chosen target structures containing healthy and/or pathologic cells, without significant concomitant or late radiation damage to adjacent tissues. All the well-documented radiosurgery of epilepsy cases are epilepsies associated with tumors or arteriovenous malformations (AVMs). Results prompted the idea to test radiosurgery as a new way of treating epilepsy without space-occupying lesions. METHODS: To evaluate this new method, we selected seven patients with drug-resistant "mesial temporal lobe epilepsy" (MTLE). The preoperative evaluation program was the one we usually perform for patients selected for microsurgery of TLE [video-EEG analysis of seizures, foramen ovale electrode recording, magnetic resonance imaging (MRI) positron emission tomography (PET) scan, neuropsychological testing]. In lieu of microsurgery, the amygdalohippocampectomy was performed by using GK radiosurgery. RESULTS: Morphologic (MRI) signs of destruction of the target took place at 9 months after GK surgery. Since the treatment day, the first patient has been seizure free. Seizure improvement came more gradually for the following patients, and complete cessation of seizures occurred around the tenth month (range, 8-15 months). MRI shows that the amygdaloentorhinohippocampal target was selectively injured. No significant side effect (except one case of homologous quadrantanopia) or morbidity and no mortality was observed. The current follow-up is 24-61 months, and all (but one) patients are seizure free. CONCLUSIONS: This initial experience proves clearly the short-to middle-term efficiency and safety of GK for MTLE surgery. These results need further confirmation of long-term efficiency, but the introduction of GK surgery into epilepsy surgery can reduce dramatically its invasiveness and morbidity.

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.

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.

Late-onset epilepsy associated with regional brain cortical dysplasia.

RATIONALE: Cortical dysplasia (CD) designates a diverse group of malformations resulting from one or more abnormalities in the development of the cerebral cortex. The clinical manifestations of CD are varied, probably depending on the type, location and extent of CD. Epilepsy is a potential late manifestation of any cortical malformation. To our knowledge, however, no study has focused specifically on late onset of epilepsy in patients with localized CD. MATERIAL AND METHODS: We studied patients with localized CD confirmed by MRI. Patients were divided into 2 groups according to age at onset of epilepsy. Group 1 included patients in whom the first seizure occurred up to the age of 12 (early-onset group) and group 2 included patients in whom the first seizure occurred after the age of 12 (late-onset group). The two groups were compared with regard to the type of CD, clinical findings and EEG findings. RESULTS: Thirty-three patients with various forms of CD were studied. Onset of epilepsy occurred in adolescence or adulthood in 9 cases (37%). In 6 of these (17% overall), the first seizure occurred in adulthood. CD were posterior bilateral pachygyria (1), unilateral polymicrogyria (3), focal dysplasia with subcortical gray matter heterotopia (1), perisylvian bilateral polymicrogyria (1), bioccipital polymicrogyria (1) and bilateral nodular periventricular gray matter heterotopia (2). The incidence of neurological signs was lower in the late-onset group. Mental retardation was moderate or absent, thus allowing a fairly normal lifestyle. All patients presented partial seizures with a lower incidence of drug resistance (p < 0.01). EEG demonstrated preservation of background activity and absence of diffuse or multifocal abnormalities. CONCLUSION: Onset of epilepsy with various forms of CD may be delayed until adolescence or adulthood. Prognosis of epilepsy is usually more favorable in these cases.

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.

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.

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.

[Balance sheet of tolerance-efficacy on the use of tacrine in 100 cases of Alzheimer's disease]. / Bilan tolérance-efficacité d'un an d'utilisation de la tacrine dans 100 cas de maladie d'Alzheimer.

We report our first 100 cases of Alzheimer's (AD) patients treated with tacrine (Cognex) for a period of one year. At the beginning of treatment the mean Mini-Mental-Status (MMS) score was 15.1. To date 71 patients are still under treatment (12 for more than 12 months). Forty-three instances of side-effects were observed, of which 31 involved hepatic side-effects with an increase in ALAT > IN (normal value) (6 cases > 3N), the mean date of appearance was 10.4 +/- 6.8 weeks, there were 16 cholinergic side-effects (nausea, vomiting, diarrhoea), plus 4 neurologic and 2 cutaneous side-effects. These side-effects led to the arrest of the treatment in 19 cases (16 for hepatic toxicity). Treatment was reattempted after interruption in 13 cases; successfully in 3 instances only. The measure of tacrine efficacy was based on 52 MMS score re-evaluations in week 18: there was an increase of the MMS score in 22 cases (3.3 points +/- 2.5), a stabilisation in 11 cases and a decrease in 19 cases (3.3 points +/- 2.2.). In week 30, the MMS scores (35 patients) increased in 9 cases (3.6 points +/- 2.4), stabilized in 5 cases and decreased in 21 cases (3.9 points +/- 3.3). At week 52, only 28 per cent of the patients were considered as either improved or stabilized. We conclude that there is a necessity for close follow-up of tacrine-treated patients, and that globally at 8 months there is an improvement or a stabilization in 40 per cent of patients and long term (at one year) a stabilizing effect on AD patients.

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.