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ABSTRACT: Myoclonic epilepsy in infancy (MEI) is a primary generalized epilepsy. According to the literature, the outcome of MEI is usually benign. Here we report a patient who developed myoclonic astatic epilepsy at age four, having been seizure free without antiepileptic drug treatment for 2 years after his recovery from MEI. At age four, a video-EEG-recording showed frequent head nodding (atonic seizures) and myoclonic astatic seizures associated with diffuse spikes or polyspikes and waves. The interictal EEG revealed frequent bursts of generalized 100-200 μV, 2-4 Hz spike-and-slow-wave complexes. Despite a general favorable outcome, more severe epilepsy syndromes may develop after MEI, and mental retardation is sometimes observed. Our case and the previous literature suggest that epilepsies following on from MEI often involve myoclonic seizures.
Seizure 02/2012; 21(4):300-3. · 1.80 Impact Factor
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ABSTRACT: Video-EEG monitoring (v-EEG) was originally restricted to the evaluation for epilepsy surgery. It is now widely available and often utilized to clarify the nature of paroxysmal events or to identify the epileptic syndrome. It is important to define carefully the diagnostic value of this high-cost and time-consuming procedure. Few data on children are available. In this study, we have evaluated the utility of this procedure and the factors leading to a successful recording in children. We retrospectively reviewed 380 v-EEG done in 320 children. The rate of event detection was 59%. The v-EEG recorded a seizure in 40% (n=150), a non-epileptic event in 19% (n=73), and both seizure and non-epileptic events in 3% (n=11). Only 9% remained without diagnosis after v-EEG. The frequency of the usual events was the only factor contributing to a successful recording. This procedure confirmed the diagnosis of epilepsy in 43% of patients but excluded it in 25% of them. In children with epilepsy, the v-EEG allowed to define a new syndrome (30% of patients) or to improve clinical description and to identify the origin of the seizures (30%). The treatments were modified in 66% of patients following the v-EEG. Continuous video-EEG monitoring is an efficient and valuable procedure in the diagnosis and management of epilepsy and paroxysmal disorders in children.
Seizure 10/2010; 20(1):18-22. · 1.80 Impact Factor
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ABSTRACT: BACKGROUND: Malformations of the cerebral cortex may be associated with severe epilepsy and status epilepticus. It has been shown that status epilepticus models induce excitotoxic cell death. In humans, very few data are available. CASE AND RESULTS: We report a case of a multifocal disorder of the lamination diagnosed in a neonate, born at 30 weeks' gestation, who died from a refractory status epilepticus at two months and half. This abnormality was not detected by repeated MRI studies. Only microscopic investigations permitted to identify this disorder of the lamination. We found also little cell death or cell loss. DISCUSSION: Our report highlights the possible false negative results of MRI in a newborn. We can also discuss that immature human brain maybe less sensitive to neuronal injury than mature as described in animal models.
Brain & development 05/2010; 33(4):339-44. · 1.74 Impact Factor
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ABSTRACT: Late-onset spasms (LOS) are epileptic spasms starting after the first year of life. Our aim was to assess the electroclinical features and the follow-up of the patients with this particular type of epileptic seizure.
We retrospectively included all patients with LOS confirmed by electroencephalography between 1989 and 2008. Clinical and electroencephalographic findings at diagnosis and during follow-up were collected. The Vineland scale was used to evaluate the neuropsychological outcome.
We report 19 patients with LOS of 240 patients with recorded epileptic spasms. Eighteen patients had an epileptic encephalopathy with late-onset spasms. The ictal electroencephalography (EEG) showed a focal or generalized discharge of triphasic slow-waves, slow-spikes, or slow spikes-waves with fast activities. The interictal EEG usually showed focal or generalized slow-waves or slow spikes-waves without hypsarhythmia. LOS were controlled in only six patients. Three developed typical Lennox-Gastaut syndrome and 10 had a severe epileptic encephalopathy. Neuropsychological outcome was evaluated in 15 patients with the Vineland scale. Cognitive functions were normal in only one patient, whereas severe cognitive delay was observed in 12 of 15.
Epileptic spasms may appear after the age of one. They are more frequently observed in patients with epileptic encephalopathy. In few patients this type of seizure was observed before the patients fulfill Lennox-Gastaut syndrome criteria. In one patient, we diagnosed a focal epilepsy with seizures occurring in cluster. When LOS are related to an epileptic encephalopathy, this epileptic syndrome seems to be linked to refractory epilepsy and severe cognitive outcome unrelated to the etiology.
Epilepsia 03/2010; 51(7):1290-6. · 3.96 Impact Factor
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Epilepsia 11/2009; 50(11):2501-3. · 3.96 Impact Factor
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ABSTRACT: Epilepsia partialis continua (EPC) is characterized by continuous myoclonic or clonic jerks repeated at short intervals followed by a slowly progressive neurological disorder. We report three patients with EPC and a defect in the mitochondrial respiratory chain.
Clinical, neuroradiological, and biochemical data were reported.
The patients presented continuous myoclonic jerks at age of 8 months, 11 months and 6 years, respectively. Two of the three patients had a previous developmental delay. Neurological examination at first admission revealed extrapyramidal symptoms in all patients. Initial biological investigations suggested mitochondrial dysfunction. Initial EEG showed a continuous discharge of periodic spikes (0.5-1Hz). MRI studies were initially normal then progressed to cerebral hemiatrophia. EEG revealed both correlation and absence of correlation between spikes or sharp waves and myoclonic jerks. The activity of one or several complexes of the mitochondrial respiratory chain was reduced in the muscle samples of the three patients. No mutation of mtDNA was found.
Our report suggests that EPC can be due to mitochondrial respiratory chain disorders. Some clinical findings and initial investigations were indicative of a disorder of mitochondrial metabolism. Previous developmental delay, extrapyramidal symptoms and other organ involvement should suggest a possible mitochondrial etiology of EPC. In case of infant presenting EPC, mitochondrial respiratory chain disorder should be considered first.
Epilepsy Research 02/2008; 78(1):1-6. · 2.29 Impact Factor
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ABSTRACT: A 6-year-old girl had water reflex epilepsy occurring at lower temperature than the core temperature. Seizures episodes consisted of a loss of consciousness absence followed by left predominant hypotonia with right fronto-temporal high voltage slow waves on the ictal-EEG. Seizures were only observed when the water was poured on scalp or face. Neuropsychological evaluation showed frontal dysfunction (Rey's figure). MRI study was normal. Oxcarbazepine permitted the disappearance of seizures and an improvement of executive disorders. In this case, the pathophysiological mechanism cannot be a hyperthermic related event. The temperature control as treatment of hot-water epilepsy could be used after the exploration of its implication in seizure induction.
Brain and Development 06/2006; 28(4):265-8. · 2.12 Impact Factor
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ABSTRACT: Benign myoclonic epilepsy in infants (BMEI) is a rare epileptic syndrome characterized only by generalized myoclonic seizures (MSs) in normal children during the first 2 years. Our aim was to assess the electroclinical features and the follow-up of this syndrome.
BMEI was confirmed by electroencephalogram (EEG) in four neuropediatric units in France between 1981 and 2002. Clinical and electroencephalographic findings at diagnosis and during the follow-up were collected. The Vineland scale or Wechsler scale or both were used to perform neuropsychological evaluations.
We report 34 patients with BMEI characterized by MSs occurring many times a day. The ictal EEG showed a generalized discharge of polyspikes, polyspikes-and-waves, or spikes-and-waves. The interictal EEG was usually normal. A family history of febrile seizures (FSs) or epilepsy was noted in six patients. A history of FSs was noted in 11 patients. Eleven patients had reflex MSs. Monotherapy with valproic acid was effective in 23 of 30 treated patients. The onset of epilepsy was known in all patients. Four patients had seizures after the initial symptoms. Juvenile myoclonic epilepsy developed in two patients, and cryptogenic partial epilepsy in another. Neuropsychological outcome was evaluated in 20 patients (10 with Wechsler scales and 17 with the Vineland scale). Cognitive functions were normal in 17 patients, whereas developmental delay was observed in three others.
BMEI is clinically characterized by myoclonic seizures involving the upper part of the body, occurring many times a day. The ictal EEG showed a generalized discharge of polyspikes, polyspikes-and-waves, or spikes-and-waves. The interictal EEG was usually normal. Reflex MSs were frequently observed, suggesting that two distinctive syndromes are not necessary. BMEI may be followed by juvenile myoclonic epilepsy. Despite a generally favorable neuropsychological outcome, mental retardation can be observed more frequently than in the general population.
Epilepsia 03/2006; 47(2):387-93. · 3.96 Impact Factor
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ABSTRACT: To evaluate and compare the predictive value of history, clinical examination, and biologic and electrophysiologic data regarding the prognosis of children with acute hypoxic-ischemic encephalopathy (HIE).
Prospective cohort of 57 consecutive children who were mechanically ventilated for HIE throughout a 3-year period in a tertiary pediatric intensive care unit at a university hospital in France.
At 24 hours after admission, 12 patients had died, 3 were awake and 42 showed impaired consciousness or were in a coma, of whom 38% had a favorable outcome. In this group, an initial cardiopulmonary resuscitation duration longer than 10 minutes and a Glasgow Coma Scale <5 at 24 hours after admission were associated with an unfavorable outcome (positive predictive value [PPV] 91%, 100%; sensitivity 50%, 54%). A discontinuous electroencephalogram (EEG), the presence of spikes or epileptiform discharges were associated with an unfavorable outcome (PPV 100% for the 2 criteria; sensitivity 27%, 54%). The bilateral absence of the N20 wave on short-latency sensory evoked potentials (SEPs) had a PPV for unfavorable outcome of 100% (sensitivity 63%).
The clinical assessment combined with EEG and SEPs allow an early prediction of the prognosis of children with HIE.
Journal of Pediatrics 07/2002; 141(1):45-50. · 4.11 Impact Factor
