1/157. Infantile spasms associated with a histamine H1 antagonist.Some anti-allergic agents act as histamine h1 antagonists and induce seizure discharges in epileptic patients. Of these agents, ketotifen has an especially potent effect. We have experienced 2 cases of 4-month-old boys who developed infantile spasms 8 to 10 days after ketotifen administration. They showed almost the same clinical course with regard to their age, the interval between ketotifen administration and the onset of seizures, their symptoms and EEG abnormalities. These cases suggest that the administration of ketotifen to young infants may be hazardous with regard to inducing infantile spasms.- - - - - - - - - - ranking = 1keywords = spasm (Clic here for more details about this article) |
2/157. Surgical treatment of a case of early infantile epileptic encephalopathy with suppression-bursts associated with focal cortical dysplasia.We report a surgically treated case of early infantile epileptic encephalopathy (EIEE) with suppression-bursts associated with focal cortical dysplasia. Tonic-clonic seizures followed by a series of spasms occurred about a hundred times a day at a few days of age. Interictal electroencephalogram (EEG) revealed a suppression-burst pattern that was predominant in the left hemisphere. magnetic resonance imaging (MRI) suggested focal cortical dysplasia in the left prefrontal area. Combination therapies with antiepileptic treatments showed only partial efficacy. The patient underwent lesionectomy at age 4 months, after which he gradually showed psychomotor development and a decrease of spasms to 0-2 series daily. In cases of EIEE with focal cortical dysplasia, surgical treatment may have beneficial effects on both psychomotor development and seizure control.- - - - - - - - - - ranking = 0.33333333333333keywords = spasm (Clic here for more details about this article) |
3/157. Vein of Galen malformation and infantile spasms.Recently, there has been increasing interest in the study, characterization, and management of infantile spasms. With technological advances, it is now possible to determine a specific diagnosis on many patients with infantile spasms, thus broadening the spectrum of conditions associated with this electroclinical syndrome. We report a child with infantile spasms who also had a vascular malformation of the vein of Galen. Both clinical and electroencephalographic findings improved rapidly after endovascular treatment of the vascular anomaly, suggesting a possible association.- - - - - - - - - - ranking = 1.1666666666667keywords = spasm (Clic here for more details about this article) |
4/157. A case of West syndrome well controlled by very short and low-dose ACTH therapy.The case of a 5-month-old boy with tuberous sclerosis and West syndrome is reported. Tonic spasms were noted from the age of 4 months. High-dose pyridoxal phosphate could not control the seizures completely. Very short and low-dose adrenocorticotropic hormone (ACTH) therapy (i.e. 0.011 mg/kg per dose, 12 times in 20 days) controlled the seizures, while pyridoxal phosphate was on. Early tapering of ACTH was successfully done while abnormal electroencephalogram (EEG) findings remained. Although side effects such as hypertension and brain shrinkage were transiently observed, both the cognitive and seizure prognoses were excellent at the age of 3 years and 2 months. The good response to a small dosage of ACTH might be due to some responsiveness of the high-dose pyridoxal phosphate and the underlying cause of tuberous sclerosis with normal development before onset. The present case illustrates that the duration and dosage of ACTH therapy in West syndrome should be modified according to the individual's requirements.- - - - - - - - - - ranking = 0.16666666666667keywords = spasm (Clic here for more details about this article) |
5/157. The origin of hypsarrhythmia and tonic spasms in West syndrome: evidence from a case of porencephaly and hydrocephalus with focal hypsarrhythmia.We report on a 3-year-old girl with West syndrome and with focal hypsarrhythmia. The left hemisphere of the patient was virtually completely defective and continuous hypsarrhythmia was only seen in the residual right frontal cortex, where an interictal single photon emission computed tomography (SPECT) showed hyperperfusion. Despite a focal epileptic pattern, the tonic spasms were quite symmetrical. In our patient, spasms might not require the sensorimotor cortex, but the brainstem containing the descending pathways that control spinal reflexes and other infratentorial structures seem to be essential for the occurrence of spasms. This is in accordance with the result of an ictal SPECT that showed hyperperfusion of the brainstem and cerebellum. These findings suggest that hypsarrhythmia originates from cortical lesions, while subcortical structures may be primarily responsible for the tonic spasms in this patient.- - - - - - - - - - ranking = 1.3333333333333keywords = spasm (Clic here for more details about this article) |
6/157. Interhemispheric interactions analyzed by coherence during flexor spasms.OBJECTIVE: We used coherence analysis to test for leading discharges on an ipsilateral right mesial temporal lesion in a 5 year old boy with flexor spasms. METHOD: Digital EEG analysis with video-EEG telemetry was performed preparatory to epilepsy surgery. RESULTS: Study of 10 spasms with head drop and subsequent flexion of both arms demonstrated an interhemispheric time lag with secondary bilateral synchrony, with a mean difference of 17 ms. The right hemisphere led. After a lesionectomy with resection of epileptic regions (performed with electrocorticographical guidance), the patient has been seizure-free for 4 years. pathology confirmed a low-grade mixed glioma and cortical dysgenesis. CONCLUSION: The coherence analysis demonstrated a pathway of secondary generalization, confirming that the lesional side was leading during ictal generalized discharges in flexor spasms.- - - - - - - - - - ranking = 1.1666666666667keywords = spasm (Clic here for more details about this article) |
7/157. Congenital retarded myelinization in a new-born child with infantile spasms.A new-born child with frequent infantile spasms was investigated. In a cachectic state it died at the age of 11 months. biopsy of the cerebral cortex revealed a retarded myelinization; at autopsy this slight myelinization was found again. The ganglioside pattern suggested a developmental stage of approximately 3 months before birth. This is a case with a congenital failure of myelinization.- - - - - - - - - - ranking = 0.83333333333333keywords = spasm (Clic here for more details about this article) |
8/157. The smith-magenis syndrome: a new case with infant spasms.The smith-magenis syndrome (SMS) is characterized by congenital anomalies, mental retardation and the interstitial deletion of the 17p. 11.2 chromosome. The subjects affected by this syndrome show cranio-facial dysmorphias, brachycephalia, skeletal, ocular, cardiac, genitourinary and otolaryngological anomalies. The central nervous system is affected and this may be shown by psychomotor retardation, intellective deficit, electroencephalographic alterations (reduced/missing REM phase); the neuroradiological tests detect megacisterna magna, cerebellar hypoplasia, cortical dysplasia, ventricular asymmetry. Behavioural troubles are frequent and, among them, self-aggressive conducts (tearing out the nails). The syndrome is associated with the interstitial deletion of the 17p. 11.2 chromosome. The diagnosis can be made in the pre-natal period and a mosaic situation is possible. Even though the cases of SMS reported in the literature allow defining a characteristic phenotype, studies have been carried out to quantify the deletion of the chromosome 17 in order to identify the chromosomic tract which is responsible for the phenotypical induction. The deletion can either appear de novo or come from one of the parents. In addition, these subjects can show peripheral neuropathy, missing or reduced deep tendon reflexes and (rarely) epileptic crises. However, by reviewing the literature, no descriptions of patients affected by infant spasms are pointed out. This report refers to a new case of smith-magenis syndrome in a nine-month-old girl with spasms in extension.- - - - - - - - - - ranking = 1keywords = spasm (Clic here for more details about this article) |
9/157. Surgery for the treatment of medically intractable infantile spasms: a cautionary case.The most appropriate time to consider cortical resection to treat medically intractable infantile spasms has not been clearly defined. The risks that need to be reconciled to make this decision are: What is the risk of loss of developmental potential if surgery is delayed too long versus what is the risk of unnecessary surgery if it is done too soon. We propose that, in addition to evaluation of seizures, developmental assessment is a key factor in the surgical decision. The case report illustrates this concept. CASE REPORT: HC had onset of seizures at 9 days of age and developed infantile spasms due to mild right hemimegancephaly. At 19 months, she was having up to 50 seizures/day and was evaluated and approved for right hemispherectomy but surgery was delayed. Despite the seizures, her development had been much better than most patients with hemimegencephaly and infantile spasms. At 25 months her seizure control was much improved but she had several seizures/week. EcoG at the time of surgery did not demonstrate the usual abnormalities so no resection was performed. She has had only 5 seizures in the 2(1/2) year since. CONCLUSIONS: (a) Hemimegalencephaly is not always associated with severe mental retardation; (b) normal or near-normal development may, in some cases, indicate the possibility of medical control of seizures as the child grows; (c) a localized developmental brain abnormality in a child with intractable seizures should not necessarily lead to cortical resection; and (d) when a child meets developmental milestones, it may be appropriate to delay surgical intervention.- - - - - - - - - - ranking = 1.1666666666667keywords = spasm (Clic here for more details about this article) |
10/157. Concomitant administration of sodium dichloroacetate and thiamine in west syndrome caused by thiamine-responsive pyruvate dehydrogenase complex deficiency.We treated a female patient with West syndrome caused by thiamine-responsive pyruvate dehydrogenase complex (PDHC) deficiency. Infantile spasms occurred in association with elevated blood and CSF lactate concentrations; these symptoms disappeared when lactate concentrations had been lowered by treatment with concomitant sodium dichloroacetate (DCA) and high dose thiamine. Sequencing the patient's PDHC E(1)alpha subunit revealed a substitution of serine for glycine at position 89 in exon 3 (G89S). This mutation must be a de novo mutation because it was not found in either parents' genome dna. To our knowledge, five previously described patients with PDHC deficiency have displayed the West syndrome. All six known patients, including our own, were female, even though an approximately equal number of males and females have been identified with PDHC deficiency and overall West syndrome occurs somewhat more frequently in males. These results indicated that West syndrome occurred more frequently in female patients with PDHC deficiency. It is suggested that lactate concentration should be measured in patients with West syndrome for potential PDHC deficiency, especially in females.- - - - - - - - - - ranking = 0.16666666666667keywords = spasm (Clic here for more details about this article) |
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