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PubMed2002

Infantile spasms: criteria for an animal model.

Infantile spasms is an epilepsy syndrome with several distinctive features, including age specificity during infancy, characteristic semiology (epileptic spasms), specific electroencephalographic patterns (interictal hypsarrhythmia and ictal voltage suppression), and responsiveness to the adrenocorticotropic hormone (ACTH). There is no adequate animal model of infantile spasms, perhaps due to these clinically unique features, that is specific for the developing human brain. An informative animal model would provide insights into the pathophysiology of this syndrome and form the basis for the development of innovative therapies. This chapter considers criteria for an "ideal" animal model of infantile spasms, as well as "minimal" criteria that we consider essential to yield useful information. Two animal models of infantile spasms have been described in rodents: seizures induced by corticotropin-releasing factor and N-methyl-D-aspartic acid. Neither of these models conforms exactly to the human analog, but each possesses intriguing similarities that provide testable hypotheses for future investigations.

Adrenocorticotropic Hormone↗
PubMed1998

Long-term efficacy of local doxorubicin chemomyectomy in patients with blepharospasm and hemifacial spasm.

OBJECTIVE: This study examines the long-term follow-up of all patients treated with doxorubicin injections in the eyelids. DESIGN: Nonrandomized clinical trial. PARTICIPANTS: Eighteen patients with blepharospasm (12 female; 6 male) and nine patients with hemifacial spasm (4 female; 5 male). INTERVENTION: Eyelids were repeatedly injected at intervals of 10 or more weeks until the spasms were ameliorated or the patient requested discontinuation. MAIN OUTCOME MEASURE: Clinical "cure" defined as sufficient symptomatic relief to defer further paralytic treatment. All patients have been followed for more than 1 year since the last injection. RESULTS: Nine of 18 patients with blepharospasm completed the full course of treatment and are considered "cured" for more than 1 year (median, 3 years; maximum, 6 years). Six of nine patients with hemifacial spasm completed treatment. Five of these six patients are considered "cures," lasting for more than 4.5 to 6 years. Two additional patients, one with blepharospasm and one with hemifacial spasm, had significant amelioration and were untreated for more than 3 years after the last doxorubicin injection, but occasionally request botulinum toxin supplementation. The minimum effective dose per treated eyelid ranged from 1.0 to 4.2 mg (median, 2.25 mg). The treatment-related discontinuations and complications were related to skin inflammation. Four of the 14 "cured" patients required some surgical "touch-up" on 1 eyelid. However, all the patients who completed treatment are either cured or have had significant amelioration of symptoms. CONCLUSIONS: Doxorubicin chemomyectomy is an evolving technique and an effective treatment for essential blepharospasm and hemifacial spasms symptomatically localized to the eyelids. Sixteen (59%) of the initial series of 27 patients completed the treatment. Of these, all are apparently cured or their symptoms significantly ameliorated. In the future, an even higher proportion would be expected to complete the treatment due to improvements in the selection criteria and treatment protocols developed during this 8-year trial. While the treatment appears to be reasonably safe compared with surgical myectomy in its present form, the authors are continuing to explore and introduce additional cotreatments to minimize the acute skin changes and maximize the long-term effectiveness of the myectomy.

Aged↗
PubMed2000

The treatment with alendronate in hemifacial spasm associated with Paget's disease of bone.

The association of Paget's disease of bone and hemifacial spasm has rarely been reported. Hemifacial spasm is often associated with compression of the facial nerve by a vascular loop at the point where the nerve leaves the brainstem before traversing the cerebellopontine angle. It is believed that narrowing of the cerebellopontine angle cistern caused by Paget's disease increases the chance of vascular compression of the facial nerve. Whilst specific antipagetic therapy such as calcitonin has been used with good response in hemifacial spasm associated with Paget's disease, the usefulness of the newer bisphosphonates is not clear. A 65-year-old woman with hemifacial spasm associated with Paget's disease was treated with alendronate, and the hemifacial spasm became very infrequent 4 months after commencement of the therapy.

Aged↗
PubMed1986

[Benign epileptic infantile spasms].

Among 100 infants with infantile spasms studied before treatment, 14 had idiopathic spasms with a favourable outcome. They had a normal development, including reaching for objects before the age of 5 months and moderate regression without loss of eye following. Although it was hysarhythmic, the EEG tracing showed identifiable basic activity and sleep spindles; there was no slow waves focus even after diazepam administration and the spasms were "independent" even during a cluster. This type of infantile spasms seems to be a particular type of non-lesional epilepsy. It contrasts with idiopathic spasms of unfavourable outcome that apparently result from a preexisting and overlooked focal lesion responsible of later cognitive troubles.

Child, Preschool↗
PubMed1998

Adrenocorticotropic hormone therapy for infantile spasms alters pyruvate metabolism in the central nervous system.

To clarify the mechanism of action of adrenocorticotropic hormone (ACTH) in treating infantile spasms, we evaluated the effects of ACTH on the metabolism of pyruvate in the central nervous system (CNS) of children with infantile spasms. We measured the levels of lactate and pyruvate in cerebrospinal fluid (CSF) and serum, before and during ACTH treatment in 12 children with infantile spasms. We evaluated statistically any correlation between the observed metabolic changes and the clinical response of ACTH. ACTH therapy significantly elevated the levels of lactate and pyruvate in the CSF and serum. The effect was not dose-dependent. During ACTH therapy, the serum levels of lactate and pyruvate and the ratio of lactate to pyruvate (L:P ratio) were unrelated to these levels in CSF. Patients who showed a good initial response to treatment had a significantly higher CSF level of pyruvate and a lower L:P ratio during therapy than did those with a poor initial response. This is the first report that ACTH therapy administered for infantile spasms alters pyruvate metabolism in the CNS. This metabolic change may be involved in part in the action of ACTH in relieving infantile spasms.

Adrenocorticotropic Hormone↗
PubMed2002

Prolactin levels in cerebrospinal fluid of patients with infantile spasms.

Infantile spasms are an age-related epileptic syndrome of infancy and are characterized by the combination of clusters of epileptic spasms and specific electroencephalographic findings. The etiology and the pathogenesis of the disease is still unclear. Prolactin has been thought to be specifically related to epileptic seizures. To investigate the possible mechanism of prolactin secretion in infantile spasms cerebrospinal fluid prolactin levels were examined. Fifteen patients with infantile spasms (10 females and five males), 3-16 months of age, were evaluated and compared with age- and sex-matched control subject. Cerebrospinal fluid samples for prolactin were obtained before and after treatment. The mean prolactin levels in the cerebrospinal fluid of the patients before therapy (3.25 +/- 1.48 ng/mL) was higher than the control group (2.38 +/- 0.89 ng/mL), and the difference between the two groups was statistically significant (P < 0.001). The mean prolactin level in the cerebrospinal fluid of the patients after therapy (4.69 +/- 1.47 ng/mL) was demonstrated to be higher than the mean prolactin level before therapy (3.25 +/- 1.48 ng/mL) and the difference between the two groups was statistically significant (P = 0.037). Elevation of cerebrospinal fluid prolactin levels before and after treatment in patients with infantile spasms provided evidence that the cerebrospinal fluid prolactin level is related with neuronal injury.

Female↗
PubMed2001

Gabapentin in the treatment of hemifacial spasm.

OBJECTIVES: To evaluate the efficacy of gabapentin in the treatment of hemifacial spasm. MATERIAL AND METHODS: Twenty-three patients with hemifacial spasm not suitable for surgery or therapy with botulinum toxin were treated with gabapentin. The main efficacy parameter was the percentage of spasm reduction. RESULTS: A clinically significant reduction of spasms was obtained by 16 patients. CONCLUSION: Gabapentin was effective and safe in reducing hemifacial spasm in 16 out 23 (69.6%) patients.

Acetates↗
PubMed2002

Ictal pattern of EEG and muscular activation in symptomatic infantile spasms: a videopolygraphic and computer analysis.

PURPOSE: To investigate ictal muscular phenomena characterizing symptomatic infantile spasms (ISs) and their relation to ictal EEG. METHODS: Four children with severe encephalopathy, neurologic impairment, and refractory ISs related to different dysplastic lesions, underwent videopolygraphic recordings collecting surface electromyogram (EMG) activity from several cranial and limb muscles to evaluate the pattern of muscular recruitment, duration, and side-to-side asymmetry of ISs. Acquired data were stored for off-line analysis by a computerized polygraphic system. RESULTS: Spasms were characterized by a complex pattern of muscular activation. A constant or rostrocaudal propagation pattern was lacking in all patients. Intervals between the onset of EMG activity in different muscles in each spasm were very long: </=100-200 ms. Two patients, with hemispheric cortical dysplasia and agenesis of the corpus callosum, had asymmetric and asynchronous spasms in which the EMG onset of the muscles contralateral to the affected hemisphere constantly anticipated that of the ipsilateral ones. Backaveraging of EEG activity disclosed a high-amplitude EEG complex on the same side as the dysplastic lesion, preceding or succeeding the contralateral muscle activity onset. In the other two patients with diffuse cortical dysplasia, no EEG transient was related to EMG activity onset. CONCLUSIONS: Despite clinical similarity between spasms in the same patient, our data demonstrate the complexity and heterogeneity of these motor phenomena. Our findings cannot be explained simply in terms of cortical or reticular generators like other motor phenomena such as cortical myoclonus or startle reflex.

Brain Damage, Chronic↗
PubMed2001

Intraoperative monitoring of facial EMG responses during microvascular decompression for hemifacial spasm. Prognostic value for long-term outcome: a study in a 33-patient series.

Lateral spread responses (LSR), an electrophysiological characteristic of hemifacial spasm (HFS), can be recorded during surgery. This work aims at evaluating the prognostic value of the persistence or suppression of the LSR at the end of the microvascular decompression (MVD) procedure of the facial nerve. Thirty-three patients with HFS, which had been evolving for 5.5 years, underwent MVD with intraoperative EMG. Monitoring required the placement of a needle in the frontalis and mentalis muscles. Responses were recorded after stimulation of inferior or superior branches of the facial nerve to search for abnormal ephaptic LSR. Preoperative abnormal LSRs were present in all patients. In 23 patients, LSR disappeared with vascular decompression and was not present upon closure. Among those patients, 20 were considered clinically cured and three still presented with mild/moderate spasm at 3-month follow-up. At late follow-up, 22 patients were free of spasm. One patient had recurrence of spasm at month 10. On the contrary, 10 patients had persistent abnormal LSR upon closure. Among those, seven were cured at early follow-up (3 months on average), whereas spasm disappeared at late follow-up (12 to 36 months) in the other three patients. The prognostic value of LSR monitoring is questionable; a good clinical result may be obtained in patients who presented with persistent LSR at the end of MVD. Delayed cure strongly supports the hypothesis that HFS is not only due to the mechanical pulsations of the elongated artery against the root exit zone of the facial nerve, but also to demyelination of the nerve and/or hyperactivity of the facial motornucleus generated by the neurovascular compression.

Adult↗
PubMed2001

Delayed resolution of residual hemifacial spasm after microvascular decompression operations.

OBJECTIVE: After microvascular decompression to treat hemifacial spasm (HFS), resolution of the HFS is often gradual. We carefully investigated the course of the gradual resolution of HFS and examined the differences between patients with and without postoperative HFS. METHODS: One hundred seventy-five patients with HFS were monitored, for observation of 1) whether postoperative HFS occurred, 2) when it occurred, and 3) when it disappeared after microvascular decompression. For two groups of patients, with (Group I) and without (Group II) postoperative HFS, we investigated age, sex, spasm side, preoperative facial nerve block (botulinum toxin treatment), decompression material, preoperative HFS period, offender (compressing vessel), temporary and permanent postoperative complications, and electromyographic findings. RESULTS: In Group I (88 patients), postoperative HFS began within 4 days after surgery, a period that we have termed the silent period of postoperative HFS; the median value for the time to resolution was 28 days. The other 87 patients exhibited no postoperative HFS (Group II). There was a significantly higher incidence of postoperative facial weakness in Group II (Group II, 41.3%; Group I, 25.5%; P = 0.02 by logistic regression analysis). In Group I, there was no statistically significant relationship between the investigated parameters and the silent period or the postoperative HFS period, as determined by Cox proportional-hazards regression analysis, except for the number of preoperative facial nerve blocks. Electromyographic investigation of F waves revealed facial paresis during the silent period in a patient. CONCLUSION: Approximately 50% of patients with HFS exhibited residual spasm postoperatively. An immediate postoperative silent period of 4 days without spasm was characteristic. One-quarter, one-half, and 90% of the residual spasm resolved by 1 week, 1 month, and 8 months after surgery, respectively.

Adult↗
PubMed1995

Infantile spasms.

The recent intense focus of attention on further characterization and management of infantile spasms is due, in part, to the resistant nature of these seizures and the frequently poor cognitive outcome, even when the seizures are controlled. Technological advances have increased our ability to diagnose specific brain disorders associated with infantile spasms, and have led to new therapeutic approaches. Most infantile spasms previously classified as cryptogenic have now been shown to be associated with various types of brain malformations and can, therefore, be reclassified as symptomatic. Infantile spasms are probably initiated by cortical epileptic discharges which propagate to the brainstem and other subcortical areas. When the epileptogenic cortex is unilateral in an infant with resistant spasms, resective surgery may be considered. For most infants, however, surgery is not a viable option, and the search for the most effective and least toxic anticonvulsant continues.

Anticonvulsants↗
PubMed2003

Magnesium sulfate for control of muscle rigidity and spasms and avoidance of mechanical ventilation in pediatric tetanus.

OBJECTIVE: To describe the use of intravenous magnesium sulfate for the control of muscle spasms and severe generalized rigidity in a child with moderate to severe tetanus without the need for prolonged deep sedation, mechanical ventilation, or neuromuscular blockade. DESIGN: Case report. SETTING: Pediatric intensive care unit in a tertiary care, university-based children's hospital. INTERVENTIONS: A continuous infusion of magnesium sulfate. MEASUREMENTS AND MAIN RESULTS: We describe a 12-yr-old child with moderate to severe tetanus who was treated with a continuous infusion of magnesium sulfate to control painful muscle spasms and severe generalized rigidity initially refractory to moderate sedation. Muscle spasms and severe generalized rigidity were improved with magnesium sulfate. No adverse effects associated with the use of magnesium sulfate were noted during the monitoring of cardiovascular and respiratory function, reflexes, and serum magnesium concentrations. CONCLUSIONS: An infusion of magnesium sulfate can be utilized to treat muscle spasms and severe generalized rigidity without the need for deep sedation, mechanical ventilation, or neuromuscular blockade. We recommend that magnesium sulfate be considered in the armamentarium of therapeutics utilized to treat muscle spasms and rigidity associated with tetanus, provided the patient's neurologic, cardiovascular, and respiratory status can be closely monitored in the pediatric intensive care unit.

Child↗
PubMed1997

Magnesium sulphate for control of spasms in severe tetanus. Can we avoid sedation and artificial ventilation?

A prospective pilot study was undertaken to investigate the ability of magnesium sulphate to control the spasms of severe tetanus without the need for sedation and artificial ventilation. All eight patients admitted with severe tetanus to our intensive care unit within the last year were given magnesium sulphate intravenously as a 5-g loading dose followed by an infusion of 2-3 gh-1. The infusion rate was increased to control spasms while retaining the patella tendon reflex, which proved a valid guide to avoid overdose. Spasms were effectively controlled and serum magnesium concentrations were maintained within the therapeutic range. Spontaneous ventilation was adequate, ventilatory support being required only for the management of lung pathology. There was no evidence of cardiovascular instability due to sympathetic over activity. No supplementary sedation was required for the control of spasms or autonomic dysfunction during magnesium therapy. We conclude that magnesium sulphate can be used as the sole agent for the control of spasms in tetanus without the need for sedation and artificial ventilation.

Adult↗
PubMed1994

Efficacy and side effects of botulinum toxin treatment for blepharospasm and hemifacial spasm.

PURPOSE: To analyse the effectiveness and rate of side effects of botulinum toxin treatment for blepharospasm and hemifacial spasm. METHODS: In a prospective trial, 81 patients with blepharospasm and 70 with hemifacial spasm were treated with botulinum toxin A in the neuroophthalmology clinic at St Vincent's Hospital, Melbourne. Some 989 treatments were given and the mean follow-up time was 28.7 months. RESULTS: The duration of action was longer for patients with hemifacial spasm than for those with blepharospasm (median 12.0 weeks compared with 7.0 weeks, P < 0.0001). There was no change in the duration of effect over time with repeated treatments of the same dose (F = 0.4, P > 0.05). Once an effective dosage was reached, increasing the dose further did not prolong the duration of effect. There were no systemic side effects, but there were a number of local transient side effects. The most significant side effect was ptosis, which occurred in 12% of treatments given to those patients with blepharospasm and hemifacial spasm. CONCLUSION: Botulinum toxin is an effective treatment for blepharospasm and hemifacial spasm, but there are a number of side effects, the most significant being ptosis.

Blepharoptosis↗
PubMed1994

Infantile spasms in children with Down syndrome.

The authors retrospectively identified 17 children with Down syndrome who developed infantile spasms, and analysed their etiology, EEG findings, response to medication, development and prognosis. Compared with symptomatic infantile spasms in the general population, which have a poor prognosis, these patients had a relatively benign outcome with regard to seizure control: only three of 16 survivors currently have seizures and seven of the 16 currently receive anticonvulsants. Developmental outcome was poorest in those with a superimposed hypoxic insult and in those who regressed developmentally at the onset of the spasms and did not regain developmental milestones. The overall neurological prognosis for children with Down syndrome and infantile spasms appears to be better than for children with infantile spasms in the general population.

Adolescent↗
PubMed1996

P2-purinoceptors mediating spasm of the isolated uterus of the non-pregnant guinea-pig.

1. The isolated uterus of the non-pregnant guinea-pig has been suggested to contain P1-, and possibly P2-purinoceptors mediating spasm. The presence of P1-purinoceptors has been confirmed and these receptors have been further characterized. 2. In the presence of the adenosine uptake inhibitor, S-(4-nitrobenzyl)-6-thioinosine (NBTI, 300 nM) and a pA100 concentration of the P1-purinoceptor antagonist 8-sulphophenyltheophylline (140 microM), the potency order of agonists as spasmogens was: 2 methylthio ATP >> alpha,beta methylene ATP = UTP = ATP >> beta,gamma methylene ATP. This order is not consistent with any single recognised P2-purinoceptor subtype. 3. Indomethacin (1 microM) treatment abolished responses to 2 methylthio ATP, alpha,beta methylene ATP and UTP, while spasm to ATP was significantly inhibited. When the endometrial and circular smooth muscle cell layers were removed, spasmogenic responses to ATP, 2 methylthio ATP, alpha,beta methylene ATP and UTP were significantly reduced. 4. 2-methylthio ATP was able to cause desensitization to itself, but not to UTP, indicating that these agonists act at different receptor sites. 5. The P2-purinoceptor antagonist, suramin antagonized 2 methylthio ATP with a PA2 of 5.9 +/- 0.3. Suramin was also an antagonist of ATP and UTP. In the case of ATP, the antagonism was not dependent on suramin concentration, while for UTP the interaction appeared to be non-equilibrium. Pyridoxalphosphate-6-azophenyl-2',4'-disulphonic acid (PPADS, 10 microM) had no effect on spasm to ATP, UTP or 2 methythio ATP. 6. In the presence of indomethacin, responses to ATP were unaffected by 8-sulphophenyltheophylline (140 microM) or by suramin (100 microM), but PPADS (10 microM) antagonized ATP. 7. These results suggest that the isolated uterus of the non-pregnant guinea-pig contains a mixture of P2-purinoceptors. P2U- (or UTP-selective pyrimidinoceptors) and P2Y-purinoceptors appear to be present, probably mainly located on the endometrial or circular smooth muscle layer. Activation of these receptors leads to spasm via increases in prostanoid generation. There appears also to be a third class of non-P2X-, non p2Y-purinoceptor present, at which ATP is an agonist and PPADS is an antagonist, located on the longitudinal smooth muscle, activation of which causes spasm independent of changes in prostanoids.

Adenosine Triphosphate↗
PubMedDate not supplied

Free amino acids in cerebrospinal fluid from patients with infantile spasms.

Profiles of free amino acids in cerebrospinal fluid (CSF) were determined by high-performance liquid chromatography for 20 nonneurologic control patients and 12 patients with infantile spasms. Statistical comparisons showed significantly elevated levels of lysine (p less than 0.001) and the excitatory neurotransmitter, glutamate, (p less than 0.01) for the infantile spasms group as compared to the nonneurologic control group. When the infantile spasms patients were subdivided according to the presence or absence of etiologic associations, highly elevated amino acid levels were observed only in CSF from patients of the symptomatic subgroup. The idiopathic subgroup showed levels of free amino acids that were not statistically different from those of the nonneurologic control group. These results indicate that while abnormalities of amino acid metabolism often accompany infantile spasms, no specific pattern of the major free amino acids in CSF appears to be directly related to this seizure disorder. Elevated levels of the excitatory amino acids, aspartate and glutamate, do not necessarily accompany infantile spasms, and in this study were only observed in symptomatic patients.

Adolescent↗
PubMedDate not supplied

Serologic HLA typing in infantile spasms.

Serologic HLA typing was performed on 29 patients with infantile spasms and hypsarrhythmic patterns in their electroencephalograms (EEGs). There were no significant increases in the frequencies of HLA-A, B, and C antigens in the infantile spasm group as compared with controls. However, there was a significant increase in the frequency of DRw52 in the infantile spasm patients (90%) as compared with controls (72%) (p less than 0.05). In addition, 3 of 12 white infantile spasm patients demonstrated the complete B18,DR3 (DRw52) haplotype; none of 150 control white subjects showed this haplotype. These findings contribute to evidence that immunological mechanisms may be involved in the pathophysiology of infantile spasms.

HLA Antigens↗