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PubMed1997

Vigabatrin as first line therapy in infantile spasms: review of seven patients.

OBJECTIVE: To study the efficacy of vigabatrin as initial therapy in infantile spasms. METHODOLOGY: We reviewed seven patients with newly diagnosed infantile spasms referred to Tuen Mun Hospital, Hong Kong from 1994 to 1995. Vigabatrin was given as the initial monotherapy at therapeutic dose of 50-100 mg/kg. Efficacy was evaluated at the end of second week of treatment. RESULTS: Significant improvement was observed in four patients, 50-75% seizure reduction in two and no response in one patient. Efficacy was noted within the first week of treatment in five of the six patients who responded. No adverse effects were observed. Vigabatrin monotherapy could be continued in five patients. CONCLUSION: Vigabatrin could be considered as the first line treatment in infantile spasms. A period of 2 weeks may be sufficient to evaluate its efficacy.

Anticonvulsants↗
PubMed1998

Facial muscle spasms: an Australian study.

PURPOSE: A group of patients suffering from blepharospasm, hemifacial spasm and Meige's syndrome were surveyed to determine the delay from the onset of their condition until a correct diagnosis was reached, the attitudes of practitioners towards them and their condition, the effect of their condition on their lifestyle and the effects of different types of treatment on their conditions. METHODS: Questionnaires were offered to all patients with blepharospasm, hemifacial spasm and Meige's syndrome presenting to three ophthalmologists licensed to treat patients with botulinum toxin injections over a 12 month period. RESULTS: Patients consulted an average of 4.4 practitioners before a correct diagnosis was made and many waited a number of years before obtaining satisfactory treatment. Approximately two-thirds of all practitioners consulted were unaware of their condition. Ten per cent of patients reported a family history of similar conditions. Most patients received relief from their symptoms with treatment using injections of botulinum toxin. More than 55% of patients considered themselves to have psychological problems (usually relating to stress and trauma) that they associated with the onset of their condition. CONCLUSIONS: Facial muscle dystonias are rare and patient experiences suggest that they are poorly appreciated in the medical community. From the time they first see a practitioner with symptoms of facial dystonia, patients typically wait 2 years and see four practitioners before a correct diagnosis is made. Stress may be a factor in the symptomatic onset of this condition. Many patients describe pain as part of the presenting symptomatology. Botulinum toxin seems to be effective in the management of facial spasm.

Adult↗
PubMed1981

Infantile spasms: a neuro-ophthalmological study.

A complete neuro-ophthalmological examination was made of 80 children affected by infantile spasms. Ocular features were inconstant and aspecific in the idiopathic, perinatal and postnatal groups. However, infantile spasms in the prenatal symptomatic group frequently were associated with ocular findings related to an ectodermic defect, as well as with brain defects. It is suggested that ocular findings are helpful in the investigation of infantile spasms, both in detecting associated brain malformations, and as an indication of whether further examination by computerized tomography is necessary.

Diagnosis, Differential↗
PubMed1990

Tuberous sclerosis and infantile spasms.

The short- and long-term outcome and special problems of 24 children with infantile spasms and tuberous sclerosis (TS) was studied. The diagnosis of TS is frequently missed: white spots on the skin have to be carefully looked for. In the present study, these spots were always found, and calcifications or hypodense areas of the brain were revealed by CT scans before one year of age. In addition to epilepsy, manifestations of TS included: giant cell astrocytoma, iridic heterotopia, cardiac rhabdomyoma, brain cysts, polycystic kidneys with severe arterial hypertension, retinal phakomas, angiofibroma and white spots on the skin. Long-term outcome was poor. None of the children had normal intelligence and all but one had epilepsy, which was often intractable. Behavioural problems were common. The prognosis, in terms of later intelligence, epilepsy and behavioural problems, was worse than in those with 'idiopathic' infantile spasms or in those with simultaneous neurological disorders. An early diagnosis of TS in patients with infantile spasms clarifies the prognosis and avoids some of the hazards of ACTH therapy. TS children have a high relapse rate after ACTH therapy, so prolonged courses may be indicated.

Adolescent↗
PubMed1983

Infantile spasms: some new theoretical aspects.

Infantile spasms begin in a very active phase of brain development. The new neuropathological techniques have shed light on changes in the fine structure of brain in children with infantile spasms. An understanding of the action of adrenocorticotropic hormone on the biochemistry and development of the brain provides, in an indirect way, a partial explanation of the biochemical events in this syndrome; most of these actions are well documented in animal experiments. Some anticonvulsants are also effective in treating infantile spasms, and modern research has explained many actions of these agents as well. The principal mechanism causing retardation in most children is unknown. Some future aspects of the research are reviewed.

Adrenal Cortex Hormones↗
PubMed1984

CSF protein profile in infantile spasms. Influence of etiology and ACTH or dexamethasone treatment.

CSF proteins in 107 children ranging from 3 to 24 months of age were analyzed by means of quantitative zone electrophoresis on agarose gel. Subjects included 50 children with infantile spasms, 41 children without CNS disease serving as controls, and 16 infants with acute aseptic meningitis who demonstrated the protein pattern of blood-CSF barrier disturbance. Children with infantile spasms were subdivided into several groups according to etiological categories: symptomatic (pre-, peri-, and postnatal), doubtful, and cryptogenetic. Before any treatment was started, these children showed the protein profile of increased permeability of the blood-CSF barrier, especially for albumin. There was an association between the severity of the changes and the etiological category. Changes were most marked in the symptomatic group, intermediate in the doubtful group, and slight in the cryptogenetic group. No child with infantile spasms of doubtful or unknown etiology revealed changes of the immunoglobulin-containing gamma fractions. Ten children who had received adrenocorticotropic hormone (ACTH) or dexamethasone for 2-11 weeks no longer showed any protein leakage into the CSF. The period of ACTH or dexamethasone treatment was characterized by the following findings: the disappearance or reduction of hypsarrhythmia; the reappearance of normal cerebrovascular permeability for protein; and the occurrence of reversible dilatation of the subarachnoid and intraventricular spaces.

Acute Disease↗
PubMedDate not supplied

Infantile spasms following near-drowning: a report of two cases.

We report the occurrence of infantile spasms following near-drowning in two children with previously normal development. In both cases, the episode of near-drowning occurred well after the 1st year of life, and the age at onset of spasms (20 and 34 months, respectively) was unusually late. The possible significance of these two cases in relation to the pathophysiological mechanism underlying infantile spasms is briefly discussed.

Age Factors↗
PubMedDate not supplied

Partial seizures evolving to infantile spasms.

We describe the clinical and electroencephalographic (EEG) manifestations of four patients with simultaneous EEG-video-telemetry recording (VTR) documented partial seizures evolving to infantile spasms. Clinical manifestations of the partial seizures included cessation of activity, staring, automatisms, increased limb tone, and laughter. In each case, partial seizures were followed by a cluster of infantile spasms. Infantile spasms preceded by partial seizures have not been previously documented.

Electroencephalography↗
PubMedDate not supplied

A retrospective study of spontaneous remission and long-term outcome in patients with infantile spasms.

The spontaneous remission rate and long-term outcome in 44 patients with infantile spasms not treated with hormonal drugs were studied. The cumulative spontaneous remission rate during the first 12 months after onset of infantile spasms, as determined by retrospective analysis, was as follows: 1 month, 2%; 2 months, 2%; 3 months, 5%; 4 months, 7%; 5 months, 9%; 6 months, 11%; 7 months, 11%; 8 months, 14%; 9 months, 16%; 10 months, 18%; 11 months, 25%; 12 months, 25%. The average age at follow-up was 80 months. Nine percent of patients had normal development or only mild impairment. The remainder showed various degrees of retardation. These data should be useful in the design and interpretation of therapeutic drug trials in patients with infantile spasms.

Adrenal Cortex Hormones↗
PubMedDate not supplied

Twenty-four-hour ambulatory EEG monitoring in infantile spasms.

Twenty-four-hour ambulatory EEG (AEEG) recordings were performed in 74 infants with West Syndrome (WS) who had not received corticosteroids before the recording. EEG analysis was performed visually for interictal background activity as well as for ictal events: spasms (isolated or in clusters) and other seizures either generalized or partial. Six hundred fifty-four seizures were recorded in 67 patients. Partial seizures (PS) were noted in 31 infants (51% of symptomatic WS cases, 33% of cryptogenic WS cases). In 14 patients, PS were immediately followed by a cluster of spasms consisting of a single ictal event. Patients with PS had an asymmetrical interictal background activity in 85% of cases, with no return to hypsarrhythmia between spasms in a given cluster. AEEG is a reliable method to detect and analyze ictal events in infants with WS. In this population, patients with unfavorable outcome of both epilepsy and psychomotor development have PS. Therefore, the existence of PS may contribute to etiologic diagnosis and prognostic evaluation.

Child, Preschool↗
PubMedDate not supplied

Neuropathologic study of resected cerebral tissue from patients with infantile spasms.

Studies to date on the neuropathologic substrates of infantile spasms have largely utilized autopsy material of children who die after a long and complicated seizure history. This makes the interpretation of primary versus secondary changes in the cerebral tissue difficult if not impossible. We have recently had the opportunity to review the neuropathologic changes in cortical tissue resected from infants and children with a history of infantile spasms. The major identifiable abnormalities were destructive lesions, sometimes classifiable as cystic-gliotic encephalomalacia, and dysplastic changes of varying degree. The cortical dysplasias had some similarity to cerebral changes described in tuberous sclerosis, including the presence of bizarre gemistocytic "balloon" cells, and secondary cytoskeletal changes within neuronal cell bodies. Such material provides an opportunity to apply immunohistochemical and molecular techniques to epileptic tissue in an attempt to understand the morphologic substrates of infantile spasms and other types of generalized epilepsy.

Brain↗
PubMed1996

Early clinical and EEG features of infantile spasms in Down syndrome.

The combination of West syndrome (WS) and Down syndrome appears not to be coincidental. Fourteen patients free of cardiac malformation or history of perinatal hypoxia were referred and investigated before they had received any treatment and were followed to the mean age of 4.5 years (range 19 months to 14 years). Spasms had onset at the mean age of 8 months (range 4-18 months) in cluster and were symmetrical. Hypsarrhythmia was symmetrical and, after intravenous diazepam (4 patients, 0.5 mg/kg) it disappeared, without any remaining focus. Recorded spasms during a cluster were "independent," with recurrence of hypsarrhythmia between successive spasms, and thus had the ictal and interictal EEG characteristics of idiopathic WS. Seven patients exhibited other types of seizures after WS, consisting of myoclonic jerks, atonic, tonic-clonic or absence seizures, which proved quite easy to control with valproate and/or ethosuximide.

Adolescent↗
PubMed1997

Zonisamide monotherapy in newly diagnosed infantile spasms.

PURPOSE: We determined the short-term efficacy of zonisamide (ZNS) monotherapy in newly diagnosed patients with infantile spasms (IS). METHODS: Eleven hospitals participated in this open, prospective trial. ZNS 3-10 mg/kg/day was administered as the second-choice drug to 11 newly diagnosed patients with IS (cryptogenic 3, symptomatic 8) who failed to respond to high-dose vitamin B6. RESULTS: Four infants with symptomatic IS had cessation of spasms and disappearance of the hypsarrhythmia. In these responders, the spasms ceased after a few days (1-5 days) of treatment at a dose of ZNS 4-5 mg/kg/day which produced plasma ZNS concentrations ranging from 5.2 to 16.3 microg/ml (mean 9.8 microg/ml). There were two relapses (50%) 4-6 weeks after cessation of seizures, however. Relapse was predicted by effects of ZNS on EEG; the 2 infants in whom an abnormal EEG persisted had relapses, whereas the 2 whose EEG normalized remained seizure-free (follow-up 20 and 26 months). No adverse reactions were noted. CONCLUSIONS: ZNS may be effective in the initial treatment of selected patients with IS.

Adrenocorticotropic Hormone↗
PubMed1999

REM sleep components predict the response to initial treatment of infantile spasms.

PURPOSE: The phasic inhibition index (PII) is the rate of the simultaneous occurrence of rapid eye movement bursts (RBs) and phasic chin muscle activity (PCMA) during rapid eye movement sleep (REMS). PII is low insofar as physiologically occurring REM-related phasic inhibition acts on chin muscles. Previously we found that PII was significantly higher in patients with infantile spasms (ISs) who had a recurrence of convulsions than in patients with ISs who exhibited no recurrence. We aimed to predict the response of patients with ISs to conventional anticonvulsants (AEDs) by means of REMS components including PII, expecting to facilitate avoidance of potentially hazardous hormonal therapy. METHODS: REMS, recorded before the beginning of any medication, was retrospectively examined in 15 patients with ISs. The patients were classified into two groups according to the response to initial treatment with conventional AEDs. Conventional AEDs were enough to control the spasms in six good responders (GRs), whereas further hormonal therapy was required in nine poor responders (PRs) to control the spasms. RESULTS: The amount of REMS was significantly lower in patients with ISs than in controls. GRs had less REMS than did PRs, although no significant difference was observed. Although the frequencies of RB and PCMA showed no significant differences among GRs, PRs, and controls, the average PII value in PRs (12.6+/-3.4; mean+/-SD) was significantly (p < 0.001) higher than that in GRs (6.1+/-1.7). CONCLUSIONS: PII is a useful parameter for differentiating GRs from PRs.

Anticonvulsants↗
PubMed1999

Surgery for the treatment of medically intractable infantile spasms: a cautionary case.

UNLABELLED: 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.

Age Factors↗
PubMed1999

Epileptic spasms preceded by partial seizures with a close temporal association.

PURPOSE: To investigate the distinctive features of patients with West syndrome who had partial seizures followed by epileptic spasms (PS-ES). METHODS: We examined 45 patients with West syndrome whose epileptic spasms were recorded with simultaneous video-electroencephalography (EEG) monitoring between 1982 and 1996. We investigated the patients who had PS-ES and compared the PS-ES patients with the 37 patients without PS-ES. RESULTS: Of the 45 patients who had epileptic spasms in clusters (ES) and hypsarrhythmia on the interictal EEG, eight (17%) had ES preceded by partial seizures (PS) with a close temporal association. Seven of these eight were female patients. The underlying disorders were tuberous sclerosis (one patient), Aicardi syndrome (one), nonketotic hyperglycinemia (one), and focal cortical dysplasia (one). The etiology was unknown in the remaining four patients, but was suspected to be of prenatal origin in three. Three types of seizure sequence were identified: PS followed several seconds later by ES (two patients), alternating PS and ES starting with PS (three), and PS gradually replaced by ES with overlapping of the two (three). PS-ES disappeared or was replaced by other types of seizures in 1-34 months. Six patients could not walk, and all patients could not speak any sentences at age 3 years. CONCLUSIONS: Compared with patients without PS-ES, those with PS-ES more often had organic brain lesions of prenatal origin, other types of seizures before the onset of ES, asymmetric hypsarrhythmia on the EEG, and poor psychomotor outcome.

Age of Onset↗
PubMed1992

Treatment of idiopathic hemifacial spasm with botulinum toxin.

Twelve patients with idiopathic hemifacial spasm received treatment with botulinum toxin A over a period of 18 months. Of 76 treatments given, most (94.7%) led to successful relief of eyelid spasms and all treatments were successful for perioral and lower facial muscle spasms. An average dose of 9.3 units of toxin per session was given to produce a mean interval of relief of 10.8 weeks. Blepharoptosis was the only ocular side effect; it was mild, reversible and occurred in 2 patients. However, lower facial palsy was frequent (9 patients); it was mild to moderate in severity but only partially reversible in 8 patients. Dosage for lower facial muscles should therefore be reduced.

Adult↗
PubMed1984

Infantile spasms: modern practical aspects.

Data on incidence, aetiology, therapy and prognosis of infantile spasms are reviewed. In a large proportion of cases the aetiological factors of infantile spasms can now be established with some certainty. Especially the new neuroradiological and virological investigative methods have enabled a more accurate diagnosis. There have been changes in the aetiologic pattern over the years. The optimal dosage and duration of ACTH or glucocorticoid therapy have not yet been established. In the Finnish study the large ACTH doses (120-160 IE) generally used in many Nordic countries did not carry a better prognosis than the smaller doses (20-40 IE). Side-effects of ACTH therapy are relatively common. They are more frequent with large doses than with smaller ones. Measures can be taken to prevent or treat part of them. The prognosis and related factors in infantile spasms as well as some recommendations to improve them are reviewed.

Adrenocorticotropic Hormone↗