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Resume Introduction La carence en vitamine B12 est frequemment associee a des manifestations neurologiques dont la degenerescence combinee de la moelle est la forme la plus classique. Observation Nous rapportons le cas d’une patiente qui presenta une ataxie cerebelleuse, une deterioration cognitive associee a une leucoencephalopathie a l’IRM cerebrale secondaire a une carence en vitamine B12 dans un contexte de maladie de Biermer. Une supplementation vitaminique a initialement ameliore la symptomatologie, mais son interruption a conduit a un etat grabataire irreversible malgre la reprise du traitement. Conclusion Une carence en vitamine B12 doit etre recherchee devant toute symptomatologie neurologique centrale progressive inexpliquee, une supplementation precoce permettant de limiter les sequelles irreversibles.

Resume Introduction La prise en charge des patients ayant un accident vasculaire cerebral (AVC) dans les unites neuro-vasculaires (UNV) diminue la proportion de deces et de handicap, independamment de l’acces aux traitements d’urgence comme la thrombolyse intraveineuse. Au CHU de Tours, une filiere d’accueil direct des patients atteints d’AVC permettant l’utilisation du traitement par thrombolyse intraveineuse a ete mise en place en juin 2003. Methodes Nous avons conduit une etude prospective pendant 18 mois, pour evaluer le fonctionnement de la filiere neuro-vasculaire, en recueillant les donnees de prise en charge et de devenir de tous les patients qui y etaient admis. Dans un second temps, ces donnees ont ete comparees a celles recueillies pendant 6 mois aux urgences en 2002, pour les patients suspects d’AVC. Nous avons egalement etudie les conditions d’utilisation du traitement par thrombolyse intraveineuse. Resultats Trois cent soixante-quatre patients ont ete inclus dans l’etude. La creation de la filiere s’est accompagnee d’une diminution significative des delais d’admission des patients et des delais d’imagerie, et d’une plus grande implication des services de transport d’urgence (SAMU). La proportion de patients ayant une mauvaise evolution est moindre dans la filiere, cette diminution ne restant significative apres ajustement que pour les patients ayant un AVC hemorragique. Les conditions d’utilisation du traitement thrombolytique dans notre centre sont comparables a celles de la litterature en terme de securite et d’efficacite. Conclusion Cette etude montre que la creation d’une filiere neuro-vasculaire d’accueil direct et de thrombolyse s’est globalement accompagnee d’une amelioration de la qualite des soins, avant meme la creation d’une unite neuro-vasculaire repondant aux recommandations.

Summary: Background: Continuous spike-waves during slow sleep syndrome (CSWSS) is a rare epileptic syndrome occurring in children, which is characterized by the association of epilepsy, neuropsychological disorders, and abnormal paroxysmal electroencephalographic (EEG) discharges activated by sleep. Language can be affected but, to date, language disorders and their long-term outcome have been documented only rarely. Purposes: Description of language impairment in patients with the CSWSS. Methods: We performed a detailed language testing in 10 right-handed children and adolescents with the CSWSS. Their pragmatic performance was compared to that of a control population of 36 children aged 6—10 years. Results: Patients with CSWSS had lower scores in tests measuring their lexical, morphosyntactic, and pragmatic skills compared to controls. Comprehension remains unaffected. In addition, language impairment was found to be just as severe in patients in remission as those still in an active phase. Conclusions: We found severe language impairments in lexical and syntactic skills. The language profile is different from that observed in the Landau‐Kleffner syndrome. Moreover patients in remission and those in an active phase of the CSWSS have the same language impairment profiles. This confirms the poor long-term neuropsychological prognosis. Our results raise points about the relationship between epileptic activity and language development. This pilot study underscores the need to assess language, and especially pragmatic skills, and to study long-term outcome in such childhood epileptic syn

Resume Objectifs. – L'utilite de l'electroencephalogramme (EEG) dans differentes situations cliniques d'urgence a ete precisee par une conference de consensus (Tours, mai 1996). Afin d'etudier la place de l'EEG en urgence en pratique clinique, les demandes concretes d'EEG dans le cadre de l'astreinte d'electroencephalographie du CHU de Tours ont ete confrontees aux recommandations de la reunion de Tours de 1996. Materiel et methode. – Realisee de maniere retrospective, l'analyse portait sur les indications et les resultats de tous les traces EEG consecutifs sur une periode d'astreinte de six mois. Resultats et conclusion. – Les indications les plus frequentes etaient : la presomption de mort cerebrale (13%) ; la prise en charge des etats de mal epileptiques (12,1%) ; la suspicion d'etat de mal non convulsif (10,6%). Plus d'un tiers des demandes (38,6 %) n'etaient pas conformes aux conclusions de la conference de consensus. L'utilite de l'EEG est optimale quand les criteres cliniques consensuels sont appliques, notamment pour la prise en charge des etats de mal epileptiques, pour rechercher un etat de mal larve ou un etat de mal non convulsif. En revanche, l'EEG urgent n'a pas d'utilite devant une perte de connaissance breve ou un deficit focal transitoire ou constitue non febrile.

Summary: Purpose: The aim of this study was to determine the clinical, social, and/or professional and cognitive outcomes in adulthood of the continuous spike–waves during slow sleep (CSWS) and Landau–Kleffner syndromes, which are two rare epileptic syndromes occurring in children. Methods: We enrolled seven young adults, five who had a CSWS syndrome, and two, a Landau–Kleffner syndrome in childhood. We evaluated their intellectual level as well as their oral and written language and executive functions. Results: This study confirmed that the epilepsy associated with these syndromes has a good prognosis. Only one patient still had active epilepsy. However, the neuropsychological disorders particular to each syndrome persisted. Only two patients had followed a normal pathway in school. Three of the five patients with a CSWS syndrome during childhood remained globally and nonselectively mentally deficient. We found no evidence of the persistence of a dysexecutive syndrome in this study group. The intellectual functions of the two patients with Landau–Kleffner syndrome were normal; however, their everyday lives were disrupted by severe, disabling language disturbances. We discuss the role of some prognostic factors such as the location of the interictal electric focus and the age at onset of CSWS. Conclusions: These two epileptic syndromes of childhood are very similar in many respects, but their clinical outcomes in adulthood are different.

The etiology of amyotrophic lateral sclerosis remains unknown in the majority of cases. Homozygous SMN1 (survival motor neuron) gene deletion causes spinal muscular atrophy, and SMN2 gene deletions are possible risk factors in lower motor neuron disease. We studied SMN1 and SMN2 genes copy numbers in 167 amyotrophic lateral sclerosis patients and in 167 matched controls. We noted that 16% of amyotrophic lateral sclerosis patients had an abnormal copy number of the SMN1 gene (1 or 3 copies), compared with 4% of controls. An abnormal SMN1 gene locus may be a susceptibility factor for amyotrophic lateral sclerosis.

We studied the morphometric MRI findings and their correlation with cognitive functions in a population of 10 young adults shunted for congenital hydrocephalus related to spina bifida. Morphometric MRI analysis included measurement of the ventricular dilatation index, frontal and occipital parenchymal thickness and the size of the corpus callosum. The neuropsychological status was evaluated, notably to look for a discrepancy between verbal and performance skills, a finding which has previously been described in hydrocephalic children. We also investigated whether there was a correlation between cognitive function and cerebral morphometric indexes. In each case, MRI demonstrated the structural changes associated with the Chiari II malformation. The size of the lateral ventricles varied, ranging from important dilatation to small ventricles. Six patients had only partial development of the corpus callosum. All patients had a normal global IQ. In our population of young adults, we did not observe any discrepancy between verbal and visuospatial performances as has been described in children with hydrocephalus. We found no relationship between cognitive function and ventricle dilatation or parenchymal thickness or between the size of the corpus callosum and callosal transfer.

123I-Iodolisuride has high specific affinity for binding on dopamine D2 receptors in the striatum and has been used in a few single photon emission computed tomography (SPECT) studies of extrapyramidal disorders. The diagnosis of Parkinson's disease (PD) is very difficult in the first 5 years of evolution, with 15-25% false positive diagnoses. The aim of this study was therefore to determine the value of iodolisuride SPECT in discriminating Parkinson's from the most frequent Parkinson-plus syndromes (PPS). Seventeen patients with an extrapyramidal syndrome had a SPECT examination 1 h after injection of 180-185 MBq of 23 I-iodolisuride They were followed under dopaminergic treatment for at least 2 years. After 2 years, they were separated in two groups according to specific clinical criteria and sensitivity to dopaminergic treatment: nine patients had PD (age = 59.8 ± 8.8 years; Hoehn and Yahr = 1.8 ± 0.7; evolution = 4.3 ± 3 years) and eight had PPS (age = 71.6 ± 7.3 years; Hoehn and Yahr = 2.9 ± 2.0; evolution = 4.1 ± 1.5 years). The binding potential of iodolisuride in the striatum was assessed by considering the striatum (S)/occipital lobe (O) ratio at the pseudo-equilibrium 1 h after injection. The S/O ratio was statistically different between PD and PPS (1.97 ± 0.3 vs 1.65 ± 0.2 (P < 0.02)). Iodolisuride SPECT could differentiate both groups with a sensitivity of 88.8% and a specificity of 75%. Iodolisuride is a good specific D2 receptor ligand for SPECT and complements specific clinical criteria for the diagnosis of Parkinson's disease and differentiation between different extrapyramidal disorders.

Resume L'analyse quantifiee de l'EEG a ete utilisee chez neuf patients epileptiques temporaux droitiers inclus dans une procedure prechirurgicale pour tenter de predire la localisation des centres du langage. Ce travail avait pour but de valider un protocole d'etude des variations de la puissance spectrale de l'alpha dans un paradigme d'activation precedemment developpe chez des sujets sains droitiers : la diminution significative de la puissance spectrale du rythme alpha sur l'hemisphere gauche au cours d'une tâche d'ecriture de la main droite par rapport aux valeurs de repos, et/ou la diminution de la puissance spectrale du rythme alpha sur l'hemisphere droit au cours d'une tâche de reconnaissance de formes de la main gauche par rapport aux valeurs de repos, etaient considerees comme un marqueur electrophysiologique de la specialisation hemispherique gauche pour le langage. Dans ce travail, la localisation du langage obtenue par l'etude des variations spectrales chez les patients etait comparee aux resultats du test de Wada considere comme la technique de reference. Parmi les neuf sujets, huit avaient l'hemisphere gauche dominant pour le langage (pour un hemisphere droit) au test de Wada. Six patients avaient une diminution significative de la puissance de l'alpha en accord avec les resultats du test de Wada ; pour les trois patients restant (incluant le patient dont l'hemisphere droit etait dominant pour le langage), les variations spectrales etaient non significatives. La diminution de la puissance spectrale du rythme alpha sur l'hemisphere actif au cours d'un paradigme d'activation cerebrale semble statistiquement liee a la specialisation hemispherique gauche des centres du langage. Cependant, pour un patient epileptique donne, l'etude des seules variations spectrales ne permet pas de definir de maniere cliniquement pertinente la lateralisation des centres du langage.

Resume Un deficit neurologique focal peut se definir comme le temoignage clinique d'une perturbation, structurelle ou fonctionnelle, transitoire ou durable, localisee a une region particuliere de l'encephale. Dans ce contexte, nous resumons ainsi les indications urgentes de l'EEG: (i) l'EEG n'a pas d'indications en urgence pour le diagnostic, le pronostic ou le traitement d'un accident ischemique transitoire ou d'une migraine ; (ii) en cas de crise partielle complexe, un EEG realise dans les 24 heures est utile, mais pas toujours indispensable au diagnostic positif d'epilepsie. Il a une signification pronostique et therapeutique encore incertaine; (iii) l'interet de l'EEG comme element pronostique a la phase aigue d'un AVC ischemique a ete suggere, mais pas clairement demontre; (iiii) l'EEG est indique en urgence dans les deficits focaux febriles, dans lesquels il presente un interet diagnostique et pronostique specifique et irremplacable.

Resume Introduction La ciclosporine est un medicament immunosuppresseur majeur dans la prevention de rejet des greffes et dans certaines affections auto-immunes. La prescription de cette molecule est limitee par de nombreux effets parmi lesquels on note des cas de neuropathies et de myopathies, rapportees notamment lorsque la ciclosporine est associee a des medicaments inhibant la co-enzyme A reductase. Observation Nous rapportons le cas d’une patiente de 67 ans qui a developpe quelques mois apres une greffe renale un deficit sensitivo-moteur rapidement evolutif en rapport avec une neuromyopathie. Bien que la ciclosporine fut prescrite seule dans les taux therapeutiques, l’elimination des autres etiologies responsables d’un tel tableau conduisait a privilegier l’hypothese d’une toxicite induite exclusivement par la ciclosporine. Cette hypothese fut confirmee par l’amelioration de l’etat neurologique apres arret de cette prescription. Conclusion Cette observation souligne donc la neurotoxicite potentielle de la ciclosporine meme lorsqu’elle est prescrite en monotherapie aux taux therapeutiques.

Ornithine transcarbamylase deficiency (OTCD) is the most common urea cycle disorder. This condition usually presents in neonates or children. This report describes the clinical case of a 21 year old woman who was diagnosed in adulthood during the course of an unexplained coma. After recovery from the coma, she presented very unusual neuropsychological disorders involving memory and the meaning of certain words, suggesting a semantic deficit. The discovery of OTCD in adulthood is rare and the neuropsychological consequences may be unique.

Hemisphere specialization for language was studied in 10 children with expressive developmental dysphasia (DD) (mean age 10 years 4 months) submitted to a dichotic listening task (in a word free-recall task and forced-attention task) and a finger tapping/vocalization dual-task paradigm. A nonsense shape dichaptic task was also introduced to control right hemispheric processing. Performances of dysphasic children were compared to those obtained from 15 normal children. The results showed that controls had a right ear advantage in free-recall (words) dichotic listening task and a significant right ear advantage in forced-right-attention task, with a change in ear asymmetry as a consequence of instruction. In the dysphasic group we observed a significant right ear advantage in the free-recall dichotic listening task and no change in ear asymmetry during forced right or forced left condition. Results in time sharing paradigm and nonsense dichaptic task are more difficult to interpret, because there was no interaction between group and condition. These results cannot support a complete left hemisphere dysfunction in developmental dysphasia.

The word agrypnia, ie, organic insomnia, was first used to describe a patient with a Morvan fibrillary chorea, which is an ill-delineated syndrome. This review considers the experimental insomnia created by raphe nuclei, anterior hypothalamic, or thalamic lesions. There are some papers reporting REM and non-REM sleep reduction in man after vascular, traumatic or degenerative lesion of the pons. There is only one case of agrypnia due to a bilateral stereotatic thalamic injury. Infectious agrypnia (trypanosomiasis, Von Economo) may exist but has not been documented by polygraphic means. Fatal familial insomnia induces a precocious agrypnia and leads to death with vegetative and motor disturbances. It is associated with an abnormal prion-protein which may interfere with gabaergic synapses. Finally agrypnia in humans corresponds to either lesionnal or infra microscopic synaptic prion linked disorders.

Resume Le sommeil reduit considerablement la survenue des mouvements anormaux observes au cours de la veille dans les affections degeneratives du systeme extrapyramidal. Neanmoins, les enregistrements electrophysiologiques montrent que les contractions musculaires n'ont pas totalement disparu. Cette reduction est parallele a la diminution de la veille qui semble, ainsi, agir comme un systeme d'amplification non specifique. On note neanmoins que le sommeil fait disparaitre les caracteres specifiques du tremblement parkinsonien et que certaines myoclonies du voile perdurent durant le sommeil.

Spinal muscular atrophy (SMA) is caused by SMN1 gene deletions or mutations, and ALS is the most frequent motor neuron condition in adults. The authors describe three families in which ALS and SMA coexist. The authors found that no SOD1 mutation was found within these families; all three ALS cases had at least two SMN1 copies; and an abnormal SMN1 gene locus did not explain the co-occurrence of these two motor neuron disorders in these families.

Polysomnographic recordings were performed in 50 children free from any familial or personal history of seizure or neurologic diseases to evaluate the frequency of epileptiform and unusual electroencephalographic patterns in a normal population. A 9-year-old boy exhibited focal spikes that became bilateral with a density of 24% to 32% during slow wave sleep, and another boy showed a few spikes during slow wave sleep. In seven cases, 14- and 6-Hz rhythms were recorded, mostly in rapid eye movement sleep. A right rhythmic and temporal discharge was observed in one girl. Epileptiform electroencephalographic patterns are not infrequent, and 14- and 6-Hz rhythms during rapid eye movement sleep are common in normal children. (J Child Neurol 1992;7:422-426).

The effects of maintaining lateral gaze (as opposed to looking straight ahead) on electroencephalographic spectral power were tested in 12 right handed male subjects during eye opening (EO) and eye closure (EC). Our working hypothesis, based on Kinsbourne's paradigm, was that maintaining right lateral gaze activates the left hemisphere while maintaining left lateral gaze activates the right hemisphere, this activation resulting in a reduction in the spectral power over the hemisphere in question. Results showed that the variations in spectral power involved mainly the alpha frequency band. In the EC condition, the results were consistent with our working hypothesis: right lateral gaze produced a marked reduction in left hemispheric spectral power. In the EO condition, alpha power was constantly higher in the right hemisphere whether lateral gaze was maintained to the right or to the left. This can be possibly be due to an attentional effect. Results are discussed with regard of the type of alpha rhythm and of the activation of cortical oculmotor censtres. They shed light on the controversy concerning the existence of specific EEG correlates of cognitive activity, which preferentially involve each of the cerebral hemispheres.

Familial hemiplegic migraine (FHM) is a rare autosomal dominant subtype of migraine with aura that is characterized by motor weakness during attacks. FHM1 is associated with mutations in the CACNA1A gene located on chromosome 19. We report a severe, prolonged HM attack in a young pregnant patient who had the S218L FHM1. This CACNA1A mutation has been associated with HM, delayed cerebral oedema and coma following minor head trauma. The case history we report suggests a specific, severe phenotype and the co-occurrence of HM and epilepsy related to the S218L FHM1 mutation.

In order to test the effect of ‘lateralized’ sensorimotor and neuropsychological activities on EEG spectral power (P), we recorded EEGs over the right and left central regions with 3 different derivations C5P3/C6P4, C5Cz/C6Cz, and C5 ears-linked (EL)/C6EL in 14 young, right-handed men who underwent ten 2 min sequences including 4 during rest (3 with eyes closed (EC), 1 with eyes open (EO)), and 6 during tasks reputed to involved preferentially the left (Le) or the right (Ri) hemisphere, i.e., pure left and pure right motor activity of the hand (EC), pure neuropsychological tasks consisting in lexical followed by spatial form analysis and finally mixed (neuropsychological and motor) EC tasks consisting in writing followed by left hand object recognition. Three spectral parameters P, log P and asymmetry index AI = (P Ri − P Le)/(P Ri + P Le) were calculated in 5 frequency bands θ, α1, α2, α3 and β1. We observed a relationship between task complexity and P reduction on both hemispheres which was greater during rest, less during motor activity and least during mixed motor and neuropsychological activities; during EC activities more specifically involving the left hemisphere, only the comparison between rest and mixed sequences was signigicant. During EC activities involving the right hemisphere, both motor and mixed sequences were significantly different from rest sequences. In addition, during the EO sequences, P was usually greater than during rest. As compared to rest conditions, the AI increased during sequences preferentially involving the left hemisphere and decreased during preferentially right hemisphere activities. The number of rhythms involved varied according to the lead type and the parameter chosen. Therefore, this report supports the hypothesis that in the frequency bands studied, P decreases according to the complexity of the cerebral activity, being even lower on the hemisphere directly implicated in the task.

A patient presented with a right hemiparesis and a left nuclear third nerve syndrome. The CT scan showed a hematoma of the left mesencephalon, near the oculomotor nucleus. After resorption of the hema

Objective Emergency situations require a rapid and precise diagnostic approach. However, the exact role and value of the electroencephalogram (EEG) in emergent conditions have yet to be clearly defined. Our objective was to determine why clinicians order an emergency EEG, to assess to what extent it helps establish a correct diagnosis and to evaluate the result it has on subsequent patient management. Methods We studied all successive emergency EEGs ordered during a 3-month period in our institution. We analyzed the reasons why each EEG was ordered and interviewed the prescribing clinicians in order to determine the impact the result of the EEG had on the diagnosis and subsequent therapeutic management. Results We prospectively studied a total of 111 consecutive recordings. The main reasons for ordering an emergent EEG were: suspected cerebral death (21%), non-convulsive status epilepticus (19.7%), subtle status epilepticus (14%) and follow-up of convulsive status epilepticus (11.2%). In 77.5% of the cases the clinicians considered that the EEG contributed to making the diagnosis and that it helped confirm a clinically-suspected diagnosis in 36% of the cases. When subtle status epilepticus (SSE) or non-convulsive status epilepticus (NCSE) was suspected, the diagnosis was confirmed in 45% and 43.3% of the cases, respectively. In 22.2% of the requests involving follow-up of convulsive status epilepticus after initial treatment, the EEG demonstrated persistent status epilepticus. It resulted in a change in patient treatment in 37.8% of all the cases. When the EEG helped establish the diagnosis, patient treatment was subsequently modified in 46.6% of the cases. Conclusions This prospective study confirms the value of an emergent EEG in certain specific clinical contexts: the management of convulsive status epilepticus following initial treatment or to rule out subtle status epilepticus. An emergent EEG can also be ordered if one suspects the existence of non-convulsive status epilepticus when a patient presents with mental confusion or altered wakefulness after first looking for the specific signs suggesting this diagnostic hypothesis. Significance After 50 years of development and use in daily practice, the EEG remains a dependable, inexpensive and useful diagnostic tool in a number of clearly-defined emergency situations.

Resume Nous rapportons le cas d'un homme de 65 ans atteint d'une neuropathie dysglobulinemique associee a une activite serique anti-Mag. La discussion porte sur l'etiologie centrale (cerebelleuse) ou peripherique (proprioceptive) du trouble de l'equilibre.

We report the case of a young girl who presented severe learning disabilities in oral and written language related to a continuous spike-waves during slow sleep (CSWS) syndrome. A sleep EEG recording obtained in her younger brother, who presented a clinical pattern suggesting developmental dysphasia, also showed a CSWS syndrome. These two clinical cases underscore the need to look for this syndrome in the siblings of an affected child when learning difficulties appear in a child who previously had normal psychomotor development.

Intravascular lymphomatosis (IVL) is a rare form of high-grade malignant non-Hodgkin’s lymphoma, characterized by the proliferation of neoplastic lymphoid cells within the lumen of small-caliber blood vessels, producing localized vascular occlusion. CNS involvement is found in 75 to 85% of cases.1,2⇓ The clinical presentation is variable and can mimic other neurologic conditions.3 We report a patient with IVL presenting with an isolated, rapidly progressive spinal cord syndrome. A 71-year-old retired woman was admitted because of rapidly progressive paraparesis, accompanied by dysuria and urinary incontinence. At admission, neurologic examination revealed paraparesis with a pyramidal syndrome, T12 hypoesthesia, and anal hypotonia. The rest of the examination was normal. Laboratory studies showed an elevated erythrocyte sedimentation rate and C-reactive protein. Spinal MRI revealed an increase in the size of the conus medullaris and a high signal in T2-weighted sequences. There was no gadolinium enhancement in T1-weighted sequences. Spinal ischemia was diagnosed. Cardiac and aortic ultrasonography were normal and a spinal arteriography …

Objective : To describe a case with night terrors (NT) symptomatic of a thalamic lesion. Methods : Videopolysomnography and brain MRI were used to study a 48 year old woman with a recent onset of brief episodes, occurring exclusively during nocturnal sleep, where she suddenly sat up in bed, screamed and appeared to be very frightened. Results : Videopolysomnography recorded an episode suggestive of NT. Sleep fragmentation with frequent brief arousals or microarousals was also evident mainly during slow wave sleep. The brain MRI showed increased T2 signal from the right thalamus suggestive of a low-grade tumor. Conclusions : Our case suggests that NT starting in adulthood can, rarely, be symptomatic of neurological disease, and warrant further investigation with MRI. Significance: A thalamic dysfunction, disrupting at this level the arousal system, may play a role in provoking NT.

We report on a patient with a 30-year history of left temporal lobe epilepsy who presented with ictal bradycardia followed by cardiac asystole. The EEG during the ictal period was documented and analyzed. Clinical features and therapeutic considerations are discussed.

The etiology of amyotrophic lateral sclerosis remains unknown in the majority of cases. Homozygous SMN1 (survival motor neuron) gene deletion causes spinal muscular atrophy, and SMN2 gene deletions are possible risk factors in lower motor neuron disease. We studied SMN1 and SMN2 genes copy numbers in 167 amyotrophic lateral sclerosis patients and in 167 matched controls. We noted that 16% of amyotrophic lateral sclerosis patients had an abnormal copy number of the SMN1 gene (1 or 3 copies), compared with 4% of controls. An abnormal SMN1 gene locus may be a susceptibility factor for amyotrophic lateral sclerosis.

We present a comprehensive review of sleep studies performed in patients with brain lesions complemented by 16 additional personal selected cases and by discussion of the corresponding animal data. The reader is cautioned about the risk of establishing an erroneous correlation between abnormal sleep and a given disorder due to the important inter and intra variability of sleep parameters among individuals. Salient points are stressed : the high frequency of post-stroke sleep breathing disorders is becoming increasingly recognised and may, in the near future, change the way this condition is managed. Meso-diencephalic bilateral infarcts induce a variable degree of damage to both waking and non-REM sleep networks producing and abnormal waking and sometimes a stage 1 hypersomnia reduced by modafinil or bromocriptine, which can be considered as a syndrome of cathecholaminergic deficiency. Central pontine lesions induce REM and non-REM sleep insomnia with bilateral lateral gaze paralysis. Bulbar stroke leads to frequent sleep breathing disorders. Polysomnography can help define the extent of involvement of various degenerative diseases. Fragmented sleep in Parkinson’s disease may be preceded by REM sleep behavioural disorders. Multiple system atrophies are characterised by important sleep disorganisation. Sleep waking disorganisation and a specific ocular REM pattern are often seen in supra-nuclear ophtalmoplegia. In Alzheimer patients, sleep perturbations parallel the mental deterioration and are possibly related to cholinergic deficiency. Fronto-temporal dementia may be associated with an important decrease in REM sleep. Few narcoleptic syndromes are reported to be associated with a tumour of the third ventricle or a multiple sclerosis or to follow a brain trauma ; all these cases raise the question whether this is a simple coincidence, a revelation of a latent narcolepsy or, as in non- DR16 /DQ5 patients, a genuine symptomatic narcolepsy. Trypanosomiasis and the abnormal prion protein precociously alter sleep patterns. Polysomnography is a precious tool for evaluating brain function provided it is realised under optimal conditions in stable patients and interpreted with caution. Several unpublished cases are presented: one case of pseudohypersomnia due to a bilateral thalamic infarct and corrected by modafinil, four probable late-onset autosomal recessive cerebellar ataxias without sleep pattern anomalies, six cases of fronto-temporal dementia with strong reduction in total sleep time and REMS percentage on the first polysomnographic night, one case of periodic hypersomnia associated with a Rathke’s cleft cyst and four cases of suspected symptomatic narcolepsy with a DR16-DQ5 haplotype, three of which were post-traumatic without MRI anomalies, and one associated with multiple sclerosis exhibiting pontine hyper signals on MRI.

The clinical presentation of stroke usually includes sensory–motor impairment, cranial nerve palsies, or cognitive dysfunction. Disorders in behaviour are less frequently seen. The case of a patient with a very disturbing presentation, which included a disturbance in vigilance, bilateral third nerve palsy and masturbating behaviour, is presented. The topography of the lesions and its implications on the deficits observed are discussed.

We report on a case of palatal myoclonus associated with olivary hypertrophy on magnetic resonance imaging (MRI) in a 63-year-old man. This rare radiological finding must be differentiated from a brainstem tumor.

To review the numerous works concerning sleep and epilepsy, this review considers the effects of sleep, firstly on seizures and secondly on paroxysmal interictal EEG activity (PA), in the different types of epilepsy according to the International League against Epilepsy classification. Apart from the exceptions of the definite nocturnal preponderance of seizures in idiopathic rolandic epilepsy and of the mostly nocturnal occurrence of seizures in some types of familial or sporadic frontal-lobe epilepsy, assessing a seizure according to the time of day it occurs is of no diagnostic or predictive value. In generalised idiopathic epilepsy, as in partial symptomatic or cryptogenic epilepsy, only about 20% of the patients had a sleep increase in PA. This percentage is higher (75%) in idiopathic partial epilepsy. Stereoelectroencephalography demonstrates a relative stability of spiking within the focus across the states of vigilance and an increase in transmitted discharges during stages 3 and 4. In the Landau and Kleffner syndrome, as in the syndromes of continuous spike-waves during sleep, there is a huge, unexplained increase in PA during sleep. The neuropsychological consequences of this PA have some relationship with their localisation and the patient's age at the time of occurrence. Sleep PA has also been reported in several groups of non-epileptic subjects. As regards the effect of epilepsy on sleep, sleep may be lighter and abnormally discontinuous in the absence of seizures, particularly in temporal-lobe epilepsy.

A migrainous patient who experienced chest pain attributed to angina pectoris by coronary artery spasm during a migraine attack is reported. Previous reports have already mentioned the association of these two conditions and suggested that it might be the manifestation of a generalized vasospastic disorder. This new report offers an opportunity to review and discuss the available data on such an association.

Neurological syndromes secondary to acute aortic dissection (AAD) are uncommon and usually consist of focal deficits after an embolic cerebral infarction. This article reports the observation of an AAD with the chief complaint of transient acute memory impairment—that is, a non‐usual stroke‐like symptom.

Spectral analysis of the EEG (∝-rhythm) was studied in 8 young right-handed men who underwent 6 experimental tasks which were compared with rest, and included two pure motor activities using only the right and then the left hand, two pure neuropsychological tasks, and two “mixed” tasks with both neurophysiological and motor activities. The electrophysiological parameters studied were the log of spectral power and an asymmetry index (RP-LP)/(RP + LP). In order to demonstrate lateralization of the EEG, one must associate lateralized motor and neuropsychological tasks (which are supposed to preferentially involve one of the cerebral hemispheres). These results may reconcile apparently divergent views published in the literature with respect to lateralized EEG modifications associated with cognitive activities.

Two exceptional cases of mesencephalic lacunar infarcts located both in the anterior vascular territory are reported. In patient 1, the infarct selectively involved the red nucleus, thus resulting in a Claude’s syndrome. In patient 2, the lesion was limited to the external 2/3 of the cerebral peduncle, and was responsible for a pure motor hemiplegia (PMH). CT scan easily demonstrated the lesion in both cases. Claude’s syndrome is very unusual, and PMH has only been reported once before in a mesencephalic infarct. The reasons why these lesions are so uncommon are discussed.

Summary In a population of 11 children with frequent spike waves during non REM sleep who had no neurological symptoms between birth and their first symptom, 3 groups were compared according to their neuropsychological performances. In the first group, the children had no intellectual deficit, in the second group, they had an acquired aphasia as in the Landau — Kleffner syndrome and in the third they had severe behavioural disorder and mental deterioration. The non REM sleep paroxystic activity density tended to be highest in the third group, variable in the second group and moderate in the first group, and their topography was always generalized in the acute phase in groups II and III but asymmetrical in group I. The EEG anomalies disappeared during adolescence but in group II and III children a moderate to severe delay in school work persisted.

We studied the relationships between clinical variables and those related to the states of vigilance in 18 cases of benign partial epilepsy with centro-temporal spike-waves, 22 cases of definite symptomatic partial epilepsy, and 16 cases of undetermined partial epilepsy. The time of day during which the seizures appeared and the paroxysmal activity densities during non-REM and REM sleep are not distributed differently among the 3 electro-clinical types. However, the benign epilepsy with centro-temporal spikes group had more patients with sleep-sensitive paroxysmal activities. Patients who mainly had nocturnal seizures were found to have more frequent generalized seizures and a greater sleep-sensitive paroxysmal activity. Three cases demonstrated continuous spike-waves during sleep. The patients who had little or no paroxysmal activity during sleep were the youngest. This study illustrates that sleep-sensitive seizures and paroxysmal activities are not specific to benign childhood epilepsy with centro-temporal spikes, and that seizures and paroxysmal activities are two manifestations associated with epilepsy, affected in different ways by states of vigilance.

This study tests the effect of maintaining right and left lateral gaze during a writing task which preferentially implicates the left hemisphere using an asymmetry parameter calculated from the spectral power of the alpha rhythm (RP–LP/RP+LP) in a right-handed patient undergoing the same experimental regimen nine times. A six derivation EEG was recorded. Maintaining left lateral gaze (toward the active hemisphere) removes the lateralization found during writing while staring straight ahead whereas maintaining right lateral gaze (toward the side opposite the active hemisphere) results in slightly lower values which are however, not significantly different from those obtained during staring straight ahead. This study adds an electrophysiologic aspect to Kinsbournes's paradigm on gaze position and hemispheric activation.

The aim of the present work was to study the activity of a new antihypertensive drug, a synthetic furopyridine, cicletanine, upon hypertensive morphological lesions of the retina. The stroke-prone spontaneously hypertensive rat strain (SHR-SP), known to develop hypertensive retinopathy, was a particularly suitable material in this view. The experiment was carried out in 39 rats (SHR-SP/A3N Iffa Credo), initially at the age of 11 weeks, divided into 3 groups: one as control group, the other two treated orally with 100 and 150 mg/kg cicletanine, respectively. All rats had free access to tap water containing 1% NaCl. For 46 days, blood pressure, body weight and death rate were recorded, then the rats were sacrificed. The eyes were removed, the posterior pole collected and fixed with Trump's liquid for transmission electron microscopy. In the control group, the capillaries showed marked hypertensive lesions. Multivesicular bodies were found in the different layers, particularly in the innermost layers; photoreceptor impairments could also be observed. In contrast to this group, both cicletanine-treated groups showed only rare and minimal lesions.

Introduction L’hemosiderose superficielle (HS) est due a un saignement sous arachnoidien repete dont la cause reste inconnue dans la moitie des cas. Classiquement, elle se presente par une ataxie, une hypoacousie et des signes pyramidaux. Observation Un patient, âge de 84 ans, droitier, ancien medecin, fut hospitalise pour une deterioration cognitive evoluant depuis plusieurs semaines, associee a des troubles de la marche, un syndrome dysexecutif essentiellement visuo-constructif, et des hallucinations visuelles elaborees. Les explorations biologiques, et le scanner cerebral, etaient normaux. Il existait une augmentation isolee des hematies (250/mm3) dans le LCR. L’EEG montrait une souffrance hemispherique droite. L’IRM retrouva une hemorragie sous arachnoidienne subaigue hemispherique droite, et des depots spontanement hyperintenses en T1 caracteristiques d’une hemosiderose superficielle droite diffuse. Aucune lesion sous jacente ne fut detectee et le deces survint quelques semaines plus tard. Discussion Les symptomes de l’HS resultent de depots d’hemosiderine dans la region cerebelleuse et medullaire haute. Le scanner cerebral, et le LCR peuvent etre normaux. L’aspect typique de l’IRM en T1 et echo de gradient pose le diagnostic. On peut proposer un traitement par chelateur en fer mais seule l’ablation de la cause est efficace. Les explorations etiologiques doivent etre completes chez le sujet jeune. Conclusion L’HS peut etre hemispherique, unilaterale, et entrainer une encephalopathie severe avec troubles psychiatriques. En l’absence d’etiologie retrouvee, la lesion hemorragique peut rester evolutive et l’issue fatale.

Introduction Malgre le developpement de filieres d’accueil specifiques, la proportion de patients AVC traites par thrombolyse intraveineuse est inferieure a 5 p. 100 en France. Objectifs Determiner pourquoi une thrombolyse n’est pas effectuee chez les patients admis dans les 3 premieres heures apres le debut des symptomes d’AVC ischemique. Methodes Une etude prospective a ete effectuee pour chaque patient admis dans la filiere neuro-vasculaire pendant 18 mois. Les donnees suivantes ont ete recueillies : âge, heure de debut des symptomes, mode d’admission, delai d’imagerie, traitement, devenir a la sortie. Les raisons pour lesquelles les patients admis en moins de 180 minutes n’etaient pas traites par thrombolyse intraveineuse etaient notees et verifiees apres examen du dossier. Resultats En 18 mois, 364 patients furent admis dans la filiere : 30 patients entre 150 et 180 minutes, d’emblee exclus, 170 patients avant 150 minutes, pour lesquels les causes d’exclusion etaient : un deficit mineur 3 h (28 p. 100), un diagnostic non vasculaire (24 p. 100), une hemorragie (13 p. 100), l’âge > 80 ans (10 p. 100), une CI pour 12 patients, un dysfonctionnement de la filiere dans 13 cas. Une thrombolyse intraveineuse a ete effectuee dans 17 cas. Discussion Les principales causes d’abstention sont celles retrouvees dans la litterature, notamment le delai d’admission pre-hospitalier d’emblee trop important, et un deficit mineur ou regressif (1/3 de ces patients ayant eu une evolution defavorable). Neanmoins des dysfonctionnements pourraient etre corriges : diminution des delais d’acheminement, du delai « door-to-needle », sensibilisation des intervenants. Conclusion Dix-huit pour cent des patients avec un AVC ischemique adresses dans la filiere furent traites par thrombolyse intraveineuse, mais les causes d’abstention therapeutique semblent evitables dans un grand nombre de cas.