Cases reported "Spasms, Infantile"

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1/27. Clinical and video-polygraphic features of epileptic spasms in adults with cortical migration disorder.

    The International classification of Epileptic Syndromes considers epileptic spasms to be typical seizures of West syndrome. literature reports show that spasms are present in epileptic syndromes other than West syndrome but there are few data on their characteristics in adults. We describe ictal, clinical and video-polygraphic findings in three patients (aged 21, 32 and 57 years) with epileptic spasms and with diffuse (case 2), focal right fronto-parietal (case 1) and bi-opercular (case 3) pachygyria. Spasms had been present since the ages of 1 month, 11 and 27 years respectively. Only one patient is mentally retarded. Two of our patients (cases 2 and 3) have partial seizures. Ictal polygraphic studies showed a positive, diffuse, high amplitude slow wave activity during spasms, with superimposed fast activity, followed by a diffuse flattening in all cases with a typical muscle pattern. Epileptic spasms, as typically described in West syndrome, can maintain the same semeiological and electroencephalographic features during adulthood in certain patients with cortical dysplasia.
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2/27. Liposteroid therapy for refractory seizures in children.

    Liposteroid is dexamethasone palmitate incorporated into liposomes and was developed as an anti-inflammatory drug for targeting therapy mainly for rheumatoid arthritis. Recently, it was reported that liposteroid might be effective for the treatment of West syndrome, with fewer side effects than those of corticotropin therapy. We describe three patients, a 2-month-old boy with early infantile epileptic encephalopathy, a 4-month-old girl with symptomatic West syndrome, and a 2-year-old girl with symptomatic localization-related epilepsy, whose refractory seizures were treated with liposteroid according to the original method reported by Yamamoto and colleagues in 1998. Uncontrollable seizures ceased completely in two patients and the seizure frequency decreased markedly in the other patient. Electroencephalograms revealed marked improvement in all patients. They showed no relapse of the seizures, and all showed no adverse effects except for mild brain shrinkage in one patient. Our experience with these three patients suggests that liposteroid therapy might be a new option for the treatment of refractory seizures in children, as well as for West syndrome.
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3/27. Gelastic seizures in clusters in a case of West syndrome after perinatal hypothalamic hemorrhage.

    We describe a 4-month-old Japanese infant with West syndrome with gelastic seizures in clusters. Smile developed around 3 months and gradually increased in frequency and intensity. Positron emission tomography showed hypoperfusion in bilateral hypothalamus. Interictal electroencephalogram (EEG) showed hypsarrythmia. Simultaneous video/EEG monitoring was performed. At first, a smile-like episode developed every several seconds, gradually increased to an abrupt flexion of the neck and extremities, and gradually decreased to a smile-like episode at the end. Ictal EEG revealed desynchronization. ACTH was effective. Smiles are common emotional responses in infancy. However, EEG and neuroimaging should be considered in a case of perinatal asphyxia and delayed development.
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4/27. Epileptic negative myoclonus in a newborn with hemimegalencephaly.

    PURPOSE: We report the case of a male newborn with Ohtahara syndrome and right hemimegalencephaly who presented epileptic negative myoclonus in the first days of life. methods: Prolonged polygraphic studies were performed, as well as MRI and a full clinical examination. RESULTS: EEG showed a constant and nonreactive pattern of burst suppression. There were several kinds of electro-clinical seizures (generalized myoclonia, short atonias, typical spasm and tonic spasms) at the beginning of the EEG's burst. The periods of EMG silence, lasting less than 300 ms, were associated with stereotyped EEG transients. CONCLUSIONS: Epileptic negative myoclonus can be observed also in neonatal age. The short transient impairment of motor function observed in the newborn seems linked to the slow component of spike-wave discharge, but its mechanism is still not clear.
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5/27. Benign familial infantile convulsions: a clinical study of seven Dutch families.

    Benign familial infantile convulsions (BFIC) is a recently identified partial epilepsy syndrome with onset between 3 and 12 months of age. We describe the clinical characteristics and outcome of 43 patients with BFIC from six Dutch families and one Dutch-Canadian family and the encountered difficulties in classifying the syndrome. Four families had a pure BFIC phenotype; in two families BFIC was accompanied by paroxysmal kinesigenic dyskinesias; in one family BFIC was associated with later onset focal epilepsy in older generations. Onset of seizures was between 6 weeks and 10 months, and seizures remitted before the age of 3 years in all patients with BFIC. In all, 29 (67%) of the 43 patients had been treated with anti-epileptic drugs for a certain period of time. BFIC is often not recognized as (hereditary) epilepsy by the treating physician. seizures often remit shortly after the start of anti-epileptic drugs but, because of the benign course of the syndrome and the spontaneous remission of seizures, patients with low seizure frequency do not necessarily have to be treated. If prescribed, anti-epileptic drugs can probably be withdrawn after 1 or 2 years of seizure freedom.
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keywords = frequency
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6/27. Epileptic spasms in clusters without hypsarrhythmia in infancy.

    Spasms are defined as epileptic seizures characterized by brief axial contraction, in flexion, extension or mixed, symmetric or asymmetric, lasting from a fraction of a second to 1-2s, and are associated with a slow-wave transient or sharp and slow-wave complex, followed or not by voltage attenuation. Epileptic spasms usually appear in clusters and are age-dependent. This type of epileptic spasms associated with the particular EEG pattern, hypsar rhythmia, constitutes the basis for the diagnosis of West syndrome. The question is, how to nosologically define those patients who clearly present epileptic spasms in clusters without modified or typical hypsarrhythmia and with or without focal paroxysmal discharges on the interictal EEG. In the present series, the four patients show that epileptic spasms in clusters may occur in infancy, without hypsarrhythmia. They all presented the following features: normal neuropsychological development before onset of epileptic spasms, clusters of epileptic spasms, focal clinical and/or EEG abnormalities, normal neuroradiological imaging, neurometabolic investigations and karyotypes. In three of the patients, seizures were refractory to AEDs. Epileptic spasms in clusters without hypsarrhythmia that start in the first year of life represent a subtype of infantile spasms that generally are refractory to AEDs. It is not yet clear whether it should be considered as a variant of West syndrome or not [Published with Video sequence].
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7/27. epilepsy in angelman syndrome associated with chromosome 15q deletion.

    We report eight sporadic cases of typical angelman syndrome (AS) associated with chromosome 15q12 deletion. Age at first visit was 3-35 months (average 18 months), and follow-up period was 4-20 years (average 14.1 years). The characteristic features of epilepsy in AS are (a) seizure onset in early childhood (8 of 8); (b) evolution of seizure type with age (8 of 8); (c) EEG abnormality changes from high-voltage slow bursts (HVS) in infancy to diffuse spike and waves in middle childhood (4 of 5); (d) atypical absence seizures (8 of 8), often occurring as atypical absence status (4 of 8); and (e) diminution of seizure discharges and clinical seizures after puberty (7 of 7). We believe that AS may frequently exist in the intractable epilepsies of childhood with severe mental retardation. We stress the importance of AS as one of the main etiologic background diseases of the intractable epilepsies with infantile onset such as West syndrome, Lennox-Gastaut syndrome, and others.
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8/27. Total callosotomy for a case of lissencephaly presenting with West syndrome and generalized seizures.

    CASE REPORT: An 11-month-old girl had an onset of oculogyric crisis at 2 months and she presented with epileptic spasms and generalized tonic seizures with series formation at 3 months. Her seizures were medically intractable and her development had gradually regressed after that. MRI showed severe lissencephaly in bilateral hemispheres. Interictal EEG showed hypsarrhythmia periodically and sporadic spike waves in the right hemisphere. The generalized tonic seizures began with bi-frontal polyspikes on EEG. Our diagnosis was lissencephaly presenting with West syndrome and generalized tonic seizures. A total callosotomy was performed at 11 months.OUTCOME: Postoperative frequency of seizures decreased prominently and developmental progression was resumed.CONCLUSIONS: This case showed that the corpus callosum may play an important role in some types of symptomatic West syndrome. Corpus callosotomy for treating symptomatic West syndrome should be considered as an option after careful selection and consideration of the timing of surgery.
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ranking = 1.0668388704509
keywords = wave, frequency
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9/27. EEG recognition of holoprosencephaly and aicardi syndrome.

    Two cases of alobar holoprosencephaly and two of aicardi syndrome are reported for their highly specific electroencephalographic (EEG) changes. The asynchronous sharp waves and spikes over the frontal regions with decreasing gradient of potential to the occipital leads were seen in alobar holoprosencephaly while burst suppression pattern with total asynchrony between the two hemispheres was seen in aicardi syndrome. Even though EEG changes cannot be pathognomic of any abnormality, it is suggested that as in conditions like subacute sclerosing panencephalitis and petitmal epilepsy, so also in alobar holoprosencephaly and aicardi syndrome, the characteristic features seen on EEG may provide the initial clue to the correct diagnosis.
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keywords = wave
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10/27. Non-ketotic hyperglycinemia as the cause of infant seizures--the case study.

    Non-ketotic hyperglycinemia is a disease that causes serious functional disorders of CNS and the degradation of its structure. The frequency of recognition of this disease is disproportionally low with relation to the number of unexplained deaths of the neonates. We present a case of a neonate in whom the seizures occurred during the first 24 hours of life. A detailed subsequent diagnosis of the seizures by excluding inflammatory reasons, electrolyte disorders and primary neurological reasons was done. The final diagnosis of NKH was based on high concentration of glycine in the blood serum, craniospinal fluid and elevated value of Scriver index, lack of ketosis, changes in EEG tests, (initially the record of "discharge-silence" type, and next the record of hypsarrhythmia type), hypotony of the muscular system and imaging tests of CNS. The taken up treatment decreased the intensity and the frequency of seizure attacks, caused the increase of muscular tone and of motor activity of the child.
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ranking = 0.13367774090179
keywords = frequency
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