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Biomedical subjects

B de Toffol

Publications and source records attributed to B de Toffol.

At least 37 records · Page 2Linked to original sources
PubMed2001

Sleep and brain lesions: a critical review of the literature and additional new cases.

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 disorganization. Sleep waking disorganization 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 after 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.

Animals↗
PubMed2001

[Primary hypereosinophilia syndrome manifesting as encephalopathy and vision disorders].

We report the case of a 60-year-old man who developed visual and cognitive disorders. Investigations confirmed the diagnosis of idiopathic hypereosinophilic syndrome even though the patient had a history of rectal cancer. The olinical course was favorable after treatment. We discuss the different clinical forms, imaging data and treatments of eosinphilic syndrome.

Brain Ischemia↗
PubMed2000

[Amyotrophic lateral sclerosis manifesting as cognitive disorders. Value of brain perfusion scintigraphic tomography in intensive care].

BACKGROUND: Cognitive disorders have been described in amyotrophic lateral sclerosis, but usually after the diagnosis has ben established. CASE REPORT: A 57-year-old man was intubated for acute respiratory distress subsequent to pneumonia and diaphragm palsy. He had a 2-year history of drug-resistant depression and deterioration of cognitive functions. A pyramidal syndrome associated with biopsy-proven chronic neurogenic atrophy led to the diagnosis of amyotrophic lateral sclerosis. The electromyogram did not contribute to diagnosis. Brain MRI only evidenced moderate bilateral frontal-temporal atrophy. Brain SPECT demonstrated major perfusion defects in the frontal lobes. DISCUSSION: This patient had amyotrophic lateral sclerosis and frontal-temporal dementia with an unusually late onset clinical presentation: cognitive disorder was the inaugural sign. Brain SPECT and muscle biopsy enabled us to identify the cortical and peripheral motor neurone involvement in this uncooperative intensive care patient totally dependent on mechanical ventilation.

Biopsy↗
PubMed2000

Continuous spikes and waves during slow sleep (CSWS): outcome in adulthood.

We report a longitudinal, electroencephalographic and neuropsychological analysis of epilepsy with continuous spikes and waves during slow sleep (CSWS) in a 19 year-old boy. The clinical course fluctuated, with temporary worsening or improvement of the paroxysmal abnormalities, epilepsy and cognitive functions. At the end of the follow-up period, seizures persisted. Evaluation of the boy's behaviour, language and cognitive function suggested a dysexecutive syndrome. We discuss the relationship between paroxysmal abnormalities and neuropsychological disorders.

Adult↗
PubMed2000

[Primary lateral sclerosis with breast cancer, a potential paraneoplastic neurological syndrome].

Few reports indicate that motor neuron diseases may have paraneoplastic origin. A 70 year-old woman suffering from progressive upper motor neuron disease is presented. Laboratory, radiological and neurophysiologic studies were compatible with primary lateral sclerosis. Six years later a routine screening led to the discovery of a breast cancer, suggesting that the upper motor neuron syndrome could be paraneoplastic. So, in female patients with primary lateral sclerosis, a mammography should be recommended to search for breast cancer.

Aged↗
PubMed2000

Gamma knife surgery for epilepsy related to hypothalamic hamartomas.

OBJECTIVE: Drug-resistant epilepsy associated with hypothalamic hamartomas (HHs) can be cured by microsurgical resection of the lesions. Morbidity and mortality rates for microsurgery in this area are significant. Gamma knife surgery (GKS) is less invasive and seems to be well adapted for this indication. METHODS: To evaluate the safety and efficacy of GKS to treat this uncommon pathological condition, we organized a multicenter retrospective study. Ten patients were treated in seven different centers. The follow-up periods were more than 12 months for eight patients, with a median follow-up period of 28 months (mean, 35 mo; range, 12-71 mo). All patients had severe drug-resistant epilepsy, including frequent gelastic and generalized tonic or tonicoclonic attacks. The median age was 13.5 years (range, 1-32 yr; mean, 14 yr) at the time of GKS. Three patients experienced precocious puberty. All patients had sessile HHs. The median marginal dose was 15.25 Gy (range, 12-20 Gy). Two patients were treated two times (at 19 and 49 mo) because of insufficient efficacy. RESULTS: All patients exhibited improvement. Four patients were seizure-free, one experienced rare nocturnal seizures, one experienced some rare partial seizures but no more generalized attacks, and two exhibited only improvement, with reductions in the frequency of seizures but persistence of some rare generalized seizures. Two patients, now seizure-free, were considered to exhibit insufficient improvement after the first GKS procedure and were treated a second time. A clear correlation between efficacy and dose was observed in this series. The marginal dose was more than 17 Gy for all patients in the successful group and less than 13 Gy for all patients in the "improved" group. No side effects were reported, except for poikilothermia in one patient. Behavior was clearly improved for two patients (with only slight improvements in their epilepsy). Complete coverage of the HHs did not seem to be mandatory, because the dosimetry spared a significant part of the lesions for two patients in the successful group. CONCLUSION: We report the first series demonstrating that GKS can be a safe and effective treatment for epilepsy related to HHs. We advocate marginal doses greater than or equal to 17 Gy and partial dose-planning when necessary, for avoidance of critical surrounding structures.

Adolescent↗
PubMed1999

Sleep and epilepsy.

This review considers the effect of sleep on seizures and interictal electroencephalogram (EEG) paroxysmal activities (PAs), as classified by the International League Against Epilepsy criteria. No type of seizure is, per se, specifically linked with non-rapid eye movement (NREM) or rapid eye movement (REM) sleep. However, in some syndromes, seizures are more frequent in slow wave sleep (SWS) [partial motor or generalized seizure in benign epilepsy with centro-temporal spikes (BECTS), frontal seizures in idiopathic familial or not familial frontal lobe epilepsy and generalized tonic seizure in secondary generalized epilepsy are increased by SWS]. Conversely myoclonia and grand mal seizures are associated with awakening in some forms of generalized idiopathic epilepsy. There is a mean increase in PAs during SWS in generalized and in partial epilepsies on the whole. However, precise analysis shows that in partial cryptogenic or symptomatic epilepsy and, most likely, in the majority of generalized idiopathic epileptic syndromes about 20% of patients have an increase in PA density during SWS, 20% experience an increase in waking, 50% have very few PAs and in 10% there is no significant difference between sleep and waking. BECTS, however, exhibits a definite increase in sleep PA increase and in juvenile myoclonic epilepsy an increase in PAs during the intra-night awakening is reported. There are at least three syndromes, which cause a huge increase in PAs during sleep: the Landau-Kleffner syndrome and the syndromes of continuous focal or generalized spike-waves during SWS. Their physiopathology and neuropsychological consequences are discussed. Neurophysiological animal data are also reported highlighting the relationships between slow sleep oscillations and the generation of spike waves. A biochemical review is also presented.

Journal Article↗
PubMed1999

Opercular reflex seizures: a case report with stereo-electroencephalographic demonstration.

Reflex epileptic seizures of opercular origin have been described previously based on video-electroencephalographic monitoring, but very few patients have been explored with depth electrodes. We report a woman with late-onset epilepsy who had intractable seizures despite trials of several antiepileptic drugs. At the time of the depth-electrode recordings, seizures were usually continuous and occurred either spontaneously or were induced by movements of the jaw and mouth. The seizures originated in the deep central opercular cortex; localization was confirmed by a good surgical outcome.

Cerebral Cortex↗
PubMed1999

Neuropsychologic and adaptive functioning in adolescents and young adults shunted for congenital hydrocephalus.

The major aim of this study was to assess whether the syndrome of nonverbal learning disabilities described in hydrocephalic children is observed in adulthood. Eleven adults shunted for congenital hydrocephalus related to spina bifida and eight adults shunted for hydrocephalus related to aqueductal stenosis were administered an extensive neuropsychologic battery to investigate discrepancies between verbal and visuospatial cognition, verbal and visuospatial long-term memory, and psycho-social adaptive abilities. The results showed no discrepancies between Wechsler Performance IQ or Verbal IQ in either hydrocephalic group. Nevertheless, the subjects with spina bifida appeared more cognitively impaired than the subjects with aqueductal stenosis, who performed normally on the Wechsler Adult Intelligence Scale-Revised. Memory assessment using Signoret's Memory Battery revealed no discrepancy between verbal and visuospatial memory in the hydrocephalic group. Nevertheless, the subjects with spina bifida had poorer verbal and visuospatial memory performance than the subjects with aqueductal stenosis. There were no differences on the Vineland Adaptive Behavioral Scale between subjects with spina bifida and those with aqueductal stenosis in autonomy, socialization, and daily living skills. These results suggest that shunted congenital hydrocephalus is not characterized by nonverbal learning disabilities syndrome in adolescence or in adulthood.

Adaptation, Psychological↗
PubMed1999

[Acute demyelinating motor neuropathy: an atypical form of the Guillain-Barre syndrome?].

A 33-year-old man presented an acute motor demyelinating neuropathy following Campylobacter jejuni enteritis. The patient was improved with an IgIV treatment. Clinical features and course time were compatible with the diagnosis of a Guillain-Barré syndrome. The electrophysiologic studies were in favor of multifocal motor neuropathy with conduction blocks. We discuss the nosologic group of this neuropathy.

Acute Disease↗
PubMed1998

[Emergency EEG and brain injuries].

The aim of this study is to review indications for emergency EEG in case of brain trauma. The authors emphasize the indication of emergency EEG for the diagnosis of either cerebral death or early post traumatic seizures, and for the monitoring of intensive neurological treatments. Emergency EEG and diagnosis of cerebral death has been reviewed in another issue of this journal. Diagnosis of early post-traumatic seizures may be difficult in case of cranial trauma in either the presence or the absence of coma. Emergency EEG help guide the diagnosis of electrical signs of seizures, thus indicating that treatment with antiepileptic drugs is advisable. Severe post-traumatic coma requires barbiturate impregnation and moderate hypothermia. In this last case, emergency EEG is essential for the monitoring of pharmacological treatments. The authors conclude that continuous EEG monitoring could in the future substitute for standard EEG recorded in emergency.

Brain Death↗
PubMed1998

[Anticonvulsants can aggravate idiopathic generalized epilepsy].

Antiepileptic drugs can sometimes worsen idiopathic generalized epilepsies. Relevant data are based on empirical experience of epileptologists and on few published case-reports according to the suspected drug with serious methodological limitations. Some animal models of epileptic syndromes such as genetic models of absence seizures in rat seem to be helpful for understanding the possible mechanisms underlying drug-induced syndrome aggravation. Adequate epileptic syndrome diagnosis is needed before starting antiepileptic drug.

Animals↗
PubMed1998

[Significance of single photon emission computed tomography and akinetic mutism].

Akinetic mutism is a reactive status with permanent opening of the eyes. The accountable lesions are always bilateral. The injured cerebral structures include the frontal gyri, the thalami or the mesencephalic areas. In one case of a 44-year-old patient, magnetic resonance imaging and computed tomography were not contributive. Tc99m brain SPECT imaging was performed and displayed bilateral frontal hypoactivity. This case suggests that this technique could be helpful for diagnosis when clinical features and radiological pattern are opposite.

Adult↗
PubMed1997

Sleep and the epilepsies.

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.

Epilepsy↗
PubMed1997

[Indications for emergency EEG. Focal neurologic deficits and partial epileptic seizures in adults].

A focal neurological deficit is defined as the clinical expression of a structural or functional, transient or permanent abnormality that can be ascribed to a particular brain region. In that context, the indications of an EEG as part of the emergency management may be summarized as follows: i) The EEG in not indicated for the diagnostic, prognostic or therapeutic management of transient ischemic attacks (TIA) or migraine; ii) The possible prognostic value of EEG during the acute phase of stroke has not yet been validated; iii) In partial epileptic seizures, especially complex partial epilepsy, the EEG is useful but not always indispensable for diagnosis. Its therapeutical and prognostic implications are still uncertain. Lastly, EEG is an absolute indication as part of the emergency management of focal deficits in a febrile context, where its diagnostic and prognostic value are unequaled.

Adult↗