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At least 19 recordsLinked to original sources
PubMed1999

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.

Brain↗
PubMed1997

Hypsarrhythmia: frequency of variant patterns and correlation with etiology and outcome.

To determine the frequency and significance of the EEG features of hypsarrhythmia, we analyzed the pre-ACTH records of 53 consecutive patients with infantile spasms for the severity of the following abnormalities: disorganization of background, slowing, high amplitude, spike activity, and for the presence or absence of each of the following patterns and variants: electrodecremental discharges, absence of normal sleep activity, relative normalization, hemihypsarrhythmia, burst suppression (BS), occipital hypsarrhythmia, interhemispheric asymmetry, and interhemispheric synchronization. We calculated a total score indicating the severity of the hypsarrhythmia for each record. The hypsarrhythmia variant patterns occurred frequently in up to 69% of the records. Patients with cerebral dysgenesis were more likely to have hemihypsarrhythmia or BS pattern persistent throughout the EEG. Patients with history of perinatal hypoxia-ischemia were more likely to have absence of normal sleep activity. The occurrence of each of the other variant patterns did not correlate with etiology. Favorable outcome did not correlate with the occurrence, or absence, of any of the variant patterns but was associated with faster background activity (< 75% delta), a lower total hypsarrhythmia score (< or = 10), and with absence of electrodecremental discharges on the pre-ACTH EEG. We conclude that variant patterns of hypsarrhythmia are frequent, generally do not correlate with prognosis, and thus are best included within the definition of hypsarrhythmia. The severity of the hypsarrhythmia, however, does have significant prognostic implications.

Adrenocorticotropic Hormone↗
PubMed1984

Hypsarrhythmia: variations on the theme.

Prolonged monitoring studies of patients with infantile spasms have shown that hypsarrhythmia is a highly variable and dynamic electroencephalographic pattern. Variations of the prototypic pattern (modified hypsarrhythmia) include hypsarrhythmia with increased interhemispheric synchronization, asymmetrical hypsarrhythmia, hypsarrhythmia with a consistent focus of abnormal discharge, hypsarrhythmia with episodes of attenuation, and hypsarrhythmia comprising primarily high-voltage slow activity with little sharp-wave or spike activity. Marked changes in the hypsarrhythmic pattern usually occur during sleep, chiefly during rapid eye movement sleep, when there is a marked reduction in, or total disappearance of, the hypsarrhythmic pattern. Relative normalization of the hypsarrhythmic pattern can also be seen immediately on arousal and during clusters of infantile spasms. Thus, the specific EEG features seen in a given patient depend on multiple factors, including the duration of the EEG recording, the clinical state of the patient, and the presence of various structural abnormalities of the brain.

Arousal↗
PubMed2006

The risk of lower mental outcome in infantile spasms increases after three weeks of hypsarrhythmia duration.

To assess the correlation between hypsarrhythmia duration and mental outcome in infantile spasms (IS) the medical records of 48 infants with IS were reviewed retrospectively and psychological assessments undertaken at follow-up at the age of 3 to 13 years. We found 18 (38%) cryptogenic IS cases with typical hypsarrhythmia and 30 symptomatic with modified hypsarrhythmia-further classified into 15 cases as multifocal, 10 as pseudoperiodic and 5 as unilateral hypsarrhythmia. A short treatment lag (one to two weeks) occurred in 25, three to four weeks in 10 cases. Spasms ceased within one month after treatment in 23 infants. At follow-up 15 children had normal mental outcome (borderline included). A correlation between hypsarrhythmia duration longer than three weeks and lower mental outcome was found using the logistic regression model. The duration of hypsarrhythmia represents a sensitive prognostic parameter in IS; the risk of mental retardation increases after three weeks of hypsarrhythmia.

Age of Onset↗
PubMedDate not supplied

Reduced ACTH content in cerebrospinal fluid of children affected by cryptogenic infantile spasms with hypsarrhythmia.

In view of the therapeutic efficacy of adrenocorticotropic hormone (ACTH) in the treatment of infantile spasms (IS) with hypsarrhythmia, we studied the cerebrospinal fluid (CSF) levels of ACTH in 15 children (4-10 months) affected by IS with hypsarrhythmia (eight cryptogenic forms, seven secondary to perinatal distress) and in age-matched controls. Lumbar puncture was performed in all but one case before any kind of treatment. In another case, CSF was collected 3 weeks after a spontaneous remission. Both ACTH and beta-endorphin (beta-EP), the other peptide related to the same precursor (proopiomelanocortin), were measured by specific radioimmunoassay after gel chromatography. While beta-EP levels were unchanged in the two groups of patients, ACTH concentrations of cryptogenic (3.75 +/- 2.40 fmol/ml, Mean +/- SD p less than 0.05) and secondary (6.36 +/- 3.70, NS) forms were lower than in controls (10.90 +/- 5.79). On the other hand, ACTH was higher in the case studied after therapy (9.0) and in the case presenting a spontaneous clinical and EEG remission (15.0). These data indicate that in children affected by IS with hypsarrhythmia (mainly of cryptogenic type), CSF levels of ACTH are lower, while levels of beta-EP remain normal. It would therefore appear that central ACTH content may play a possible role in the pathogenesis of IS with hypsarrhythmia.

Adrenocorticotropic Hormone↗
PubMedDate not supplied

Callosotomy in West syndrome suggests a cortical origin of hypsarrhythmia.

Hypsarrhythmia and spasms of West syndrome have been postulated by some investigators to have a brainstem origin. Cortical involvement is also postulated because of the frequent association with cortical lesions. Cortical discharges have been proposed to stimulate subcortical structures (i.e., brainstem) and hypsarrhythmia is a secondarily generalized phenomenon produced by the brainstem. Two children with cryptogenic West syndrome refractory for several years to steroids and conventional antiepileptic drugs underwent total callosotomy. Hypsarrhythmia that was bilateral before operation became unilateral after the procedure and spasms became asymmetrical, suggesting that a corticocortical pathway through the corpus callosum is important in the generalization of hypsarrhythmia.

Brain Stem↗
PubMed1995

Asymmetric hypsarrhythmia: clinical electroencephalographic and radiological findings.

Twenty-six children (16 boys and 10 girls) with hypsarrhythmia and infantile spasms (IS) were studied at the University of Michigan EEG Laboratory in a 4-year period. Six (2 boys, 4 girls), had asymmetric hypsarrhythmia with a preponderance of both slowing and epileptiform activity over one hemisphere. All 6 had the symptomatic form of IS, 4 with dysplastic conditions, 1 with porencephaly from a cerebral infarct, and 1 with hypoxicischemic encephalopathy. Five children had focal abnormalities on either physical examination or imaging studies. Four had the highest amplitude slowing and most epileptiform activity ipsilateral to the lesion, in 1, it was contralateral. Asymmetric hypsarrhythmia constituted 23% of cases with hypsarrhythmia examined at our EEG laboratory. The significant success in surgical therapy for some children with IS indicates the importance of identifying focal hemispheric abnormalities even if they are not apparent clinically. EEG may suggest focal changes not detected clinically or radiologically.

Brain↗
PubMed1994

Asymmetric hypsarrhythmia and infantile spasms in west syndrome.

The video encephalograms (EEGs) of 77 consecutive infantile spasms patients were evaluated for the presence of focal or asymmetric hypsarrhythmia and infantile spasms, to determine whether these findings were useful in predicting the presence of focal structural brain disease and were of any additional diagnostic or prognostic significance. Of the 77 patients with infantile spasms, 38% had focal or lateralized features present on video-EEG studies. Unilateral hypsarrhythmia and asymmetric ictal EEG changes during infantile spasms often occurred together: each always indicated the side of a focal or asymmetric structural cerebral lesion that was visible on computed tomographic or magnetic resonance imaging brain scan and was usually large. Clinically asymmetric infantile spasms were less common, always occurred in the presence of asymmetric ictal EEG changes, and did not appear to have additional localizing value. Lateralized hypsarrhythmia, with or without asymmetric infantile spasms, occurred in the presence of bilateral structural lesions that were more abnormal in the area of the greater EEG abnormality. Partial seizures also indicated symptomatic etiologies but were less localizing to visible focal lesions. Patients with symmetric hypsarrhythmia and infantile spasms rarely had focal/lateralized lesions visible on imaging studies. Although the majority of the symmetric group had structural brain disease, these brain lesions were diffuse, not lateralized. This group also included all patients who had cryptogenic etiology and normal development.

Brain↗
PubMedDate not supplied

Epilepsy and the electroencephalogram in progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy (the PEHO syndrome).

Progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy (PEHO) syndrome) is an apparently autosomal recessive disorder manifested by infantile spasms, severe hypotonia, and early arrest of psychomotor development. Subcutaneous edema in the limbs, typical facial features, and blindness with optic atrophy are also present. Neuropathologic and radiographic studies show progressive brain atrophy, which is accentuated infratentorially. We recorded 85 EEGs from 10 patients between the ages of 3 weeks and 12.7 years; follow-up ranged from 7 months to 12.1 years. The infantile spasms were preceded by other neurological symptoms in all patients. Seven of nine patients showed focal or generalized epileptiform activity or abnormal EEG background. All patients developed hypsarrhythmia, first recorded between 3 and 11 months of age, that was resistant to therapy with ACTH and antiepileptic drugs. After the hypsarrhythmia disappeared, five patients showed slow spike-wave activity generally seen in the Lennox-Gastaut syndrome, and three patients showed background EEG abnormality with generalized or diffuse paroxysmal activity. There were no specific EEG features that could help in the diagnosis of PEHO. The PEHO syndrome should be borne in mind in the diagnostic work-up of patients with infantile spasms, so that potentially harmful treatment can be avoided, and the parents can be counseled about the inheritability of the disorder.

Age Factors↗
PubMed1995

Multifocal independent Spike syndrome: relationship to hypsarrhythmia and the slow spike-wave (Lennox-Gastaut) syndrome.

During a 3 year period EEGs were performed in 64 children with multifocal independent spikes (MIS), 17 with slow spike-wave complexes (SSWC), 22 with MIS and SSWC and 15 with hypsarrhythmia. Only EEG records containing adequate wakefulness and sleep were analyzed in 40 children with two or more serial EEGs at least 5 months apart. Transitions from one pattern to another occurred in 25/40 patients, consistent in all cases with the following sequence: Hypsarrythmia-->MIS-->MIS and generalized discharges-->SSWC. Patients with and without transitions did not differ in their age at presentation or duration of follow-up. Eleven of 12 patients whose initial EEG showed hypsarrhythmia or multifocal independent spikes underwent transitions, compared to 0/8 patients with SSWC (p < 0.001), indicating that SSWC is a stable pattern in children. Over a 6 month period, we also prospectively analyzed EEGs of 20 patients with multifocal spikes, hypsarrhythmia, and slow spike-wave complexes. Sleep activated additional spike foci, increased the frequency of generalized spike discharges and produced synchronization of bitemporal and bifrontal spike-wave discharges at 1.5-2.5 Hz the same as SSWC.

Adolescent↗
PubMed2003

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].

Anticonvulsants↗
PubMed2005

Cri du chat syndrome and complex karyotype in a patient with infantile spasms, hypsarrhythmia, nonketotic hyperglycinemia, and heterotopia.

Seizures are rarely reported in association with deletion or duplication syndromes of the short arm of chromosome 5, or with chromosome 5 rings. We report on the clinical and cytogenetic findings in a girl with Cri du chat syndrome associated with complex abnormalities in chromosome 5, dysmorphic features, flexor infantile spasms, hypsarrhythmia, nonketotic hyperglycinemia, and heterotopia in her brain. Peripheral blood cytogenetic analysis indicates a mosaic karyotype with de novo deletion of varying amounts of 5p and pericentric inversion of the same chromosome 5. The deleted segment on 5p includes the region implicated in the catlike cry as well as sequences implicated in development of facial dysmorphism and mental retardation. This is the first case with Cri du chat syndrome associated with nonketotic hyperglycinemia, infantile spasms, hypsarrhythmia, and heterotopia.

Abnormalities, Multiple↗
PubMed2000

Successful zonisamide treatment for infants with hypsarrhythmia.

We determined that zonisamide was effective in three epileptic infants with hypsarrhythmia. Two patients had the electroencephalographic pattern of hypsarrhythmia associated with developmental delay but lacked the characteristic tonic spasms. A third patient exhibited the typical triad of infantile spasms but had a poor general condition requiring controlled ventilation. We therefore chose zonisamide instead of adrenocorticotropin for these patients. Our data suggest that zonisamide may be a useful treatment in patients falling short of the diagnostic triad of infantile spasms or complicated by a poor overall medical condition.

Anticonvulsants↗
PubMed1995

Progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy (PEHO syndrome) in two Japanese siblings.

We report on two Japanese siblings (one female and one male) with PEHO syndrome (progressive encephalopathy with edema, hypsarrhythmia, and optic atrophy). They showed profound generalized hypotonia early in infancy and developed infantile spasms with hypsarrhythmia within the first year of life. Abnormal eye movement and visual failure with optic atrophy were also observed early in infancy. Psychomotor development was arrested and serial neuroradiological studies showed slight progressive brain atrophy, dominantly of the brainstem. This is the first case report of PEHO syndrome, other than those dealing with the Finnish population.

Brain↗
PubMed1971

Prognostic implications of electroencephalographic findings of hypsarrhythmia in first year of life.

One hundred and five children who had shown the electroencephalographic (E.E.G.) features of hypsarrhythmia in the first year of life (1956-62) were assessed in 1969 in respect of death and mental development. Irrespective of the presenting symptomatology, and to some extent irrespective of therapy, mortality in the group was of the order of one in four (mostly before the age of 3 years) and the incidence of mental subnormality in the survivors was 77%. Only 18 children attained fairly normal standards of mental development and could attend ordinary schools. It seems that whatever the clinical picture at the time, the presence of hypsarrhythmia in the E.E.G. of an infant is a physical sign of grave prognostic implications.

Adrenocorticotropic Hormone↗
PubMed1986

[An anatomo-clinical case of sequelae of acute encephalopathy. Infantile spasm with hypsarrhythmia].

Clinico-pathological studies of West syndrome are rather rare. A case of sequelae of acute encephalopathy which involved a nine-month-old boy with post-mortem data is reported. Birth and postnatal development had been normal until the onset of illness. Laboratory examinations ruled out bacterial or viral meningo-encephalitis and metabolic disorders. After the recovery from a coma lasting several days, spastic quadriplegia, severe mental retardation and intractable epileptic attacks were present. The latter were made of tonic spasms and myoclonic seizures. EEG records showed hypsarrhythmia. Neuropathological examination revealed almost symmetrical bilateral cystic cavities in the pontine tegmentum and lateral nuclei of both thalami. The corpus callosum was very thin. No finding suggested a congenital anomaly. In a search of the pathological basis for infantile spasms, this case was compared with the published data. It would appear that the lesions of the pontine tegmentum play a significant role in the pathogenesis of hypsarrhythmia or infantile spasms.

Acute Disease↗
PubMed1993

Infantile cerebello-optic atrophy. Neuropathology of the progressive encephalopathy syndrome with edema, hypsarrhythmia and optic atrophy (the PEHO syndrome).

Uniform neuropathological changes are described in eight cases of the progressive encephalopathy syndrome with edema, hypsarrhythmia and optic atrophy (PEHO syndrome). Two of the autopsied patients were sisters and two other cases were familial. Macroscopically, cerebral and pronounced cerebellar atrophy was seen, the essential histopathological lesions being confined to the cerebellar cortex and the optic nerve. There was a severe neuronal loss in the inner granular layer of the cerebellum. The Purkinje cells were relatively preserved in number although reduced in size, deformed and slightly disaligned. Their dendrites were horizontally oriented and the proximal axons contained abundant torpedoes. The molecular layer was narrow. The optic nerves were atrophic. Serial neuroimaging studies showed that the disease process is operative during the postnatal period, although a prenatal onset cannot be excluded. An aberrant expression of immunoreactivity against the 200-kDa neurofilament polypeptide in Purkinje cell perikarya indicated disorganization of the cytoskeleton of these cells. The combination of clinical and pathological features of our patients differs from that observed in the few published cases of so-called primary degeneration of the granular layer. Infantile cerebello-optic atrophy, clinically characterized by seizures, blindness and early arrest in psychomotor development, thus seems to constitute a new autosomal recessive disorder.

Atrophy↗
PubMed1997

[18F]-fluorodeoxyglucose-positron emission tomography findings in protein infants with severe periventricular leukomalacia and hypsarrhythmia.

UNLABELLED: Two preterm infants with extensive periventricular leukomalacia (PVL) were examined by [18F]-fluorodeoxyglucose-positron emission tomography (FDG-PET) at the corrected ages of 18 and 34 days. They showed similar clinical courses including oculoclonic seizure, hypsarrhythmia and severe mental retardation, in addition, to spastic quadriplegia. FDG-PET study of these two infants with severe PVL disclosed poorly developed metabolic activity in the primary sensorimotor cortex, while the MRI images displayed only periventricular white matter lesions. CONCLUSIONS: Positron emission tomography may disclose cortical involvement in infants with severe periventricular leukomalacia.

Blood Glucose↗