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At least 271 records · Page 15Linked to original sources
PubMed2001

Mechanism of tonic spasms in West syndrome viewed from ictal SPECT findings.

To clarify the pathophysiology of tonic spasms, 21 patients with West syndrome were analyzed using ictal and interictal single photon emission computed tomography (SPECT). We focused on whether ictal perfusion changes were observed in the focal cortical region. Eight of the patients studied showed definite focal cortical ictal hyperperfusion, indicating that there is a unique subset of West syndrome that can be classified as infantile localization-related epilepsy. Of those eight patients, only two showed asymmetric spasms, suggesting that seizure symptomatology in infants gives only limited information on the localization-related nature of epilepsy. Furthermore, the activation of subcortical structures by focal cortical regions might be attributable to the symmetric seizure phenomena. Thirteen patients showed a diffuse pattern in their ictal SPECTs; this probably included patients with diffuse hyperperfusion and those with no changes. The following have yet to be determined: (1) whether West syndrome is divided into subgroups based on the origin of spasms, in that some patients have the origin in the cortical hemisphere and some have the origin in structures other than the cortical hemisphere, such as the brain stem; (2) whether differences in ictal SPECT patterns reflect a unique nature of tonic spasms in West syndrome, where tonic spasms appear in clusters and the interval of each spasm is different among each patient.

Brain↗
PubMed1997

Randomized trial comparing vigabatrin and hydrocortisone in infantile spasms due to tuberous sclerosis.

Vigabatrin has been shown to be efficient in infants with infantile spasms and tuberous sclerosis, in open studies. In order to compare vigabatrin to oral steroids, a prospective randomized multicenter study was implemented using both drugs as monotherapy in newly diagnosed patients with infantile spasms and tuberous sclerosis. Eleven infants received vigabatrin (150 mg/kg per day) and 11 hydrocortisone (15 mg/kg per day) for 1 month. Spasm free patients continued vigabatrin or progressively stopped hydrocortisone in 1 month, non-responders were crossed to the other drug for a new 2 month-period. All vigabatrin patients (11/11) were spasm-free versus 5/11 hydrocortisone infants (P < 0.01). Seven patients were crossed to vigabatrin (six for inefficacy, one for adverse events) and became also totally controlled. Mean time to disappearance of infantile spasms was 3.5 days on vigabatrin versus 13 days on hydrocortisone (P < 0.01). Five patients exhibited side effects on vigabatrin but nine on hydrocortisone (P = 0.006). Vigabatrin should therefore be considered as the first choice treatment for infantile spasms due to tuberous sclerosis.

Anticonvulsants↗
PubMed1998

[Treatment of infantile spasms with vigabatrin as first-line therapy and in monotherapy: apropos of 70 infants].

BACKGROUND: Steroids (hydrocortisone or ACTH) still remain the usual treatment for infantile spasms (IS). However, since 1990, some authors have reported the efficacy of vigabatrin (VGB), especially in cases related to tuberous sclerosis. POPULATION AND METHODS: Seventy children with infantile spasms were treated by VGB first line monotherapy. Modalities of treatment and monitoring were the same for all children. VGB was given at the daily dose of 100 mg/kg during 1 week. If spasms persisted, the daily VGB dose was increased to 150 mg/kg during a 2nd week. In case of persistence of IS, on the 15th day, hydrocortisone was then added to VGB. Of the 70 infants, 39 were symptomatic and 31 cryptogenic. RESULTS: On VGB, 37 children (54%) stopped having IS within a mean 3.5 days. Response to VGB was different according to etiology. Among cryptogenic cases, 22 infants (71%) definitively stopped having spasms and only one relapsed. Among symptomatic cases, only 15 infants (38%) stopped having IS, and half (8/15) relapsed. VGB mean daily dose at cessation of spasms was 114 mg/kg. Side effects were transient drowsiness (27%) and agitation (12%). Mean follow-up was 10 months (1-24 months). Seventy-five percent of the infants presenting with a focus of spike after the 1st month of treatment relapsed. CONCLUSION: Infants with cryptogenic spasms have a good response to VGB monotherapy. When mental retardation is noticed before IS, and MRI is normal, there is no efficacy. In these cases, the best treatment seems to be prolonged corticotherapy associated with VGB.

Anti-Inflammatory Agents↗
PubMed1999

Spectral properties of EEG fast activity ictal discharges associated with infantile spasms.

OBJECTIVE: The aim of this study was to evaluate the characteristics of the ictal EEG event accompanying infantile spasms. METHODS: Quantitative analysis was used, based on the application of a bivariate autoregressive (AR) parametric model; autospectra, coherence, phase functions and inter-hemispheric time differences were estimated on homologous EEG channels in 18 infants presenting with either cryptogenic or symptomatic West syndrome. RESULTS: The AR analysis of the 500 ms EEG epochs preceding spasm onset revealed the presence of a short discharge of fast activity restricted to a narrow frequency band in 13 of the 18 cases included in the study. The fast discharge peaked at 17.5+/-2.1 Hz, with rather low inter-hemispheric coherence values (0.52+/-0.17) and asymmetric amplitude on homologous EEG derivations. It persisted briefly after spasm onset, reaching a higher coherence value (0.71+/-0.16). The inter-hemispheric time difference, estimated in those cases with the coherence values significantly different from zero, ranged from 9.1 to 14.3 ms (11.4+/-1.9) in the epoch preceding spasm onset. CONCLUSION: The data obtained from the analysis of the ictal EEG events, compared with clinical and interictal EEG features, indicate that an asymmetric EEG pattern (mainly consisting of a rhythmic burst of fast activity) consistently preceded both symmetric and asymmetric spasms, thus suggesting a localized cortical origin of the ictal discharge giving rise to the spasms.

Age of Onset↗
PubMed2001

Accommodative esotropia: an unrecognized cause of hemifacial spasm in children.

Pediatric hemifacial spasm can be a sign of an intracranial tumor. We examined two females, ages 2 and 6 years, who presented with hemifacial spasm as an early sign of accommodative esotropia. Initial ophthalmologic examination disclosed high hyperopia without detectable esotropia. Both children developed an intermittent esotropia over the following month. In the first child, the hemifacial spasm resolved concurrent with the onset of amblyopia. In the second child, spectacle correction produced immediate cessation of the spasms. In both children, squinting to avoid diplopia produced an overflow facial movement that manifested as hemifacial spasm. Accommodative esotropia should be considered in the differential diagnosis of pediatric hemifacial spasm.

Amblyopia↗
PubMed1977

Critical evaluation of the role of immunization as an etiological factor of infantile spasms.

One hundred and ten cases of infantile spasms with detailed information about immunization available were selected as the masterial to evaluate the significance of immunization as an etiological factor of infantile spasms. In 80% of the cases, immunization could not be considered to have any relation with causation of infantile spasms, because 44 cases (40%) never had innoculations as yet, and other 44 cases (40%) had been immunized by some vaccines over one month before or after the onset of the disease. The remaining 22 cases, in whom immunization had been performed within one month before the onset of the disease, constituted the candidates for further study. The age of onset of the disease of the candidate group ranged from 3 to 9 months of age with peak incidence at 4 to 5 months old, exactly identical with that of patients with infantile spasms in general. The kind of vaccines concerned included DPT triple vaccines in 15 cases, smallpox vaccine in 4, antipolio live vaccine in 1, anti-Japanese encephalitis vaccine in 2. Causal relationship of immunization with infantile spasms in each patient was evaluated on the basis of the following three apsects: 1. Any other causal factors except immunization should be absent. 2. Psychomotor development before onset of the disease should be normal. 3. Interval from immunization to onset of the disease should be lied: a) Within 48 hours, in the case of pertussis vaccine, b) within 18 days, in the case of smallpox vaccine or anti-Japanese encephalitis vaccine or antipolio vaccine. Only five cases (4.8% of the total) were able to classify into the compatible group which should fulfill all the above three criteria. The small figure may easily be explicable on the assumption that the natural onset of spasm is chronologically superposed by chance over immunizations which have to be done within the first year of life.

Age Factors↗
PubMed1998

[Unilateral accommodation spasm: a diagnostic pitfall!].

BACKGROUND: Accommodation-convergence spasm (spasm of the near reflex) is usually bilateral, resulting in increased myopia, convergence, and miosis. Unilateral spasm of accommodation has rarely been reported. PATIENTS: We investigated three females (age range 10-19 years) referred for investigations of retrobulbar optic neuritis (2 cases) and decompensated esophoria (1 case). RESULTS: They all presented unilateral spasm of accommodation with visual loss due to increased myopia (-3.5 to -11.75 diopters). Apart from that, results of neuro-ophthalmological examination were normal. CONCLUSIONS: Unilateral accommodation spasm is rare and can mimic retrobulbar optic neuritis and convergent strabismus. The three components of the accommodation-convergence spasm may not always be present simultaneously. Recognizing such an entity is important to prevent the patient from useless, costful and potentially harmful investigations.

Accommodation, Ocular↗
PubMed2004

Role of motoneurons in the generation of muscle spasms after spinal cord injury.

Motoneurons in the spinal cord have intrinsic voltage-dependent persistent inward currents (PICs; e.g. persistent calcium currents) that amplify synaptic inputs by three- to five-fold in addition to providing a sustained excitatory drive that allows motoneurons to fire repetitively following a brief synaptic excitation. In this study, we examined whether prolonged involuntary muscle spasms in subjects with long-term injury to the spinal cord are mediated by the activation of PICs in the motoneuron. To examine this in the human, we used a paired motor unit analysis technique where the firing frequency of one motor unit of the pair (control unit) was used to estimate the synaptic drive to the motoneuron pool, including the drive to a second higher-threshold motor unit of the pair (test unit). The degree to which a motoneuron PIC helped to sustain the discharge of a test motor unit (self-sustained firing) was determined from the reduction in control unit firing at de-recruitment (DeltaF) compared with recruitment of the test unit. This DeltaF value corresponds to the reduction in synaptic drive needed to counteract the intrinsic PIC and, thus, was used an indirect measure of this current. In the nine motor unit pairs studied, the average estimated synaptic drive, or control unit firing rate, required to recruit a test motor unit at the onset of a muscle spasm was significantly higher (by 43%) than the estimated synaptic drive during de-recruitment at the end of a muscle spasm. This indicated that a motoneuron PIC, and associated self-sustained firing, facilitated the firing of the test units during the prolonged muscle spasms. In addition, in all subjects tested (seven out of seven), we observed that following a muscle spasm or voluntary contraction, spontaneous and self-sustained firing of motor units could continue for many seconds, even minutes, at very low discharge rates (average 5.2 +/- 1.6 Hz) with extremely low spike-to-spike variability (coefficient of variation = 5.4 +/- 1.6%). Moreover, increases in synaptic drive (noise) to the spontaneously firing units with voluntary muscle contractions or muscle spasms increased both the mean firing rate of the motor units in addition to their firing variability. This suggests that the slow spontaneous firing commonly observed in chronic spinal injury likely occurs without appreciable synaptic noise and is likely driven to a substantial degree by PICs intrinsic to the motoneuron because it is self-sustained and very regular.

Chronic Disease↗
PubMed2000

Ketorolac suppresses postoperative bladder spasms after pediatric ureteral reimplantation.

UNLABELLED: We evaluated the efficacy of ketorolac in suppressing postoperative bladder spasms after ureteroneocystostomy (ureteral reimplantation). Twenty-four pediatric patients undergoing intravesical ureteroneocystostomy were enrolled prospectively to receive either ketorolac or placebo via double-blinded randomization. Twelve patients in each group shared similar preoperative characteristics. All were maintained on an epidural infusion of bupivacaine (0.1%) with fentanyl (2 microg/mL) throughout the study. Patients were given either ketorolac (0.5 mg. kg(-1). dose(-1)) or placebo (equivalent volume saline) IV after surgery and every 6 h thereafter for 48 h. Parents were instructed to record bladder spasm episodes prospectively by using a standardized time-flow diary. Three patients (25%) in the ketorolac group experienced bladder spasms, compared with 10 patients (83%) in the placebo group (two-sided P < 0.05). The median severity score for the ketorolac group was 1.2 (mild = 1.0, severe = 3.0), compared with 2.6 for the placebo group (P = 0.003). We conclude that IV ketorolac reduces the frequency and severity of postoperative bladder spasms after intravesical ureteroneocystostomy. IMPLICATIONS: We studied the efficacy of ketorolac, a prostaglandin synthesis inhibitor, in the treatment of bladder spasm after ureteroneocystostomy (antireflux operation). Patients were randomized in a double-blinded manner to receive either ketorolac or placebo after the surgery. We demonstrate that ketorolac reduces the frequency and severity of postoperative bladder spasm.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMed2004

Spasm of the near reflex triggered by disruption of normal binocular vision.

A 26-year-old healthy female was referred by her optometrist to the binocular vision clinic of our institution for the investigation of an accommodative spasm occurring during monocular conditions. Corrected binocular visual acuity was 20/20 (6/6), with normal pupils and good ocular alignment. When the fellow eye was covered, visual acuity was <20/200 (6/60) in each eye, miosis was present in both eyes, and the occluded eye was in esodeviation, indicating a spasm of the near reflex. The spasm disappeared when a translucent occluder was used instead of an opaque black occluder. Further investigation permitted us to establish that dioptric and nondioptric blur, as well as reduced light transmission, also triggered the spasm of the near reflex, but only for specific power, opacity, or density. Cycloplegia did not eliminate the spasm. Comparisons are made with the single other similar case found in the literature. Assumptions are made as to the possible causes of that intermittent spasm.

Accommodation, Ocular↗
PubMed2005

The latest on infantile spasms.

PURPOSE OF REVIEW: This article reviews the most significant advances in the field of infantile spasm during the past year, with emphasis on best practise for treatment, and on some new etiological genetic and metabolic causes for the spasms, and new advances in the knowledge of tuberous sclerosis. RECENT FINDINGS: Up-to-date information comparing corticotrophin, oral steroids and vigabatrin shows that hormonal treatment is the most effective therapy in the short term. In a recent randomized trial, large doses of prednisolone were as effective as corticotrophin. There are insufficient data to recommend any treatment schedule for infantile spasms. Vigabatrin is the choice for infants with tuberous sclerosis. Visual field defects in (older) children seem to be as common as in adults. In animals, vigabatrin can induce apoptosis of the neurons in the developing brain. New rare factors associated with infantile spasms are mitochondrial diseases, mutations of the Aristales-related homeobax gene and posterior quadrantic dysplasia syndrome. The outcome in children with tuberous sclerosis and infantile spasms is better understood. SUMMARY: The accurate determination of etiology is now becoming increasingly possible. There is still a lack of consensus about the treatment of first choice for infantile spasms. However, recent data show that hormonal treatment is the most effective therapy in the short term. Frequency of visual field defects in children treated with vigabatrin should be studied in addition to the long-term outcome in general. Advances in our understanding of brain maturation, etiologies, mechanisms and genetics underlying catastrophic epilepsy may facilitate more effective pharmacologic interventions.

Anticonvulsants↗
PubMed2004

Very fast rhythmic activity on scalp EEG associated with epileptic spasms.

PURPOSE: Very fast activity was investigated on the ictal EEGs of epileptic spasms to elucidate the pathophysiology of West syndrome (WS) and related disorders from a novel point of view. METHODS: The traces of scalp ictal EEG of spasms temporally were expanded in 11 patients whose clinical diagnosis was symptomatic WS in six, cryptogenic WS in two, Aicardi syndrome in one, and symptomatic generalized epilepsy after WS in the remaining two. Time evolution of averaged power spectra of the ictal fast activity also was analyzed in each patient. RESULTS: Rhythmic gamma activity with frequency ranging from 50 to 100 Hz was detected in a total of 345 of 537 spasms. Fast activity was seen bilaterally in nine patients, was lateralized to one hemisphere in another, and appeared independently on each hemisphere in the remaining infant with Aicardi syndrome. Power spectra showed a clear peak corresponding to spasm-associated gamma rhythm, with frequency centering approximately 65 Hz and ranging from 51 to 98 Hz. The morphology and spectral characteristics of ictal gamma rhythm were completely different from those of muscle activity or alternating current (AC) artifacts. CONCLUSIONS: Spasm-associated gamma activity was clearly detected on the scalp. This observation may provide a clue to the pathophysiology of spasms.

Cerebral Cortex↗
PubMedDate not supplied

Management of post-operative bladder spasm.

OBJECTIVE: Pain management following bladder surgery in children is often complicated by bladder spasm. The overall severity of spasm can be reduced with opioids, anticholinergic medication and sedatives, although breakthrough spasms often occur. At the Royal Children's Hospital, Melbourne, intravesical bupivacaine has been used to manage postoperative bladder spasm to good effect. The administration of intravesical bupivacaine is analysed in this prospective audit of locally applied intravesical anaesthetic and compared with other methods. METHOD: From February to August 2003, histories of 58 patients who had intravesical bupivacaine were studied and compared with six other methods of management of postoperative bladder spasm. CONCLUSION: Data showed that epidural anaesthesia was the most effective treatment of pain, with a pain score reduction of 6.6, compared with a reduction of 6.1 with intravesical bupivacaine, and 4.5 using intravenous morphine. However, intravesical bupivacaine was the most effective method for the relief of bladder spasm.

Acetaminophen↗
PubMed1995

Decreasing perinatal mortality: unchanged infantile spasm morbidity.

The present study examined whether changes in the incidence of infantile spasms could be used to evaluate changes in the quality of pre- and perinatal care over time. 107 children in 1960 to 1976, and 102 in 1977 to 1991, developed infantile spasms in southern Finland. The number of small-for-gestational age (SGA) infants and infants with neonatal hypoglycaemia decreased significantly. Brain malformations, malformation syndromes and patients with tuberous sclerosis increased; this probably reflects the development of more refined neuroradiological screening methods. SGA infants are probably more apt to develop infantile spasms than preterm infants appropriate for gestational age. Early prenatal factors seem to play a major role in the genesis of infantile spasms. Little can be done to reduce the incidence of infantile spasms, but every effort should be made to reduce the number of SGA infants by good prenatal care. Treatment of hypoglycaemia is important because infantile spasms might be preventable in these infants.

Brain↗
PubMed1996

Localization of focal cortical lesions influences age of onset of infantile spasms.

PURPOSE: To determine whether the localization of focal cerebral lesions affects age of onset of infantile spasms (IS). METHODS: We reviewed 216 cases of IS and identified patients with focal cerebral lesions confined to frontal, centrotemporoparietal, or occipital regions. The age of onset of spasms in each of the three groups was analyzed. RESULTS: Ninety-three patients were found to have focal cerebral lesions confined to one of the three regions previously described. The mean age of onset of IS was 3.36 +/- 1.71 months in patients with occipital lesions, 6.26 +/- 2.68 months in centrotemporoparietal lesions, and 9.83 +/- 2.56 in frontal lesions. CONCLUSIONS: Occipital lesions are found to be associated with earliest onset of spasms, whereas frontal lesions are rare and associated with latest spasms onset. The age distribution pattern of spasms onset according to localization of cortical lesion is in close correlation with that of normal sequence of brain maturation. This supports the hypothesis that cerebral lesions located in critical areas of brain maturation have a role in the genesis of infantile spasms.

Age of Onset↗
PubMed1997

Vigabatrin versus ACTH as first-line treatment for infantile spasms: a randomized, prospective study.

PURPOSE: To compare the efficacy and tolerability of vigabatrin (VGB) and adrenocorticotrophic hormone (ACTH) as first-line therapy in infantile spasms. METHODS: Forty-two infants (22 males, 20 females) aged 2-9 months with newly diagnosed infantile spasms, were included in the trial. Patients were randomized to receive VGB 100-150 mg/kg/day or Depot ACTH 10 IU/day. The alternative drug was given if spasms were not controlled within 20 days or in cases of intolerance to initial therapy. Twenty-three patients (7 cryptogenic, 16 symptomatic) received VGB as first-line therapy; 19 patients (8 cryptogenic, 11 symptomatic) received ACTH as the first drug. RESULTS: Cessation of spasms was observed in 11 (48%) of the patients randomized to VGB and in 14 (74%) of those randomized to ACTH. Response to VGB was observed within 1-14 days, but two-thirds of patients (7/11) responded within 3 days. In the group treated with VGB, side effects such as drowsiness, hypotonia and irritability were observed in 13% of patients, compared with 37% in the group treated with ACTH. VGB was more effective than ACTH as treatment for cerebral malformations or tuberous sclerosis, whereas ACTH proved more effective in perinatal hypoxic/ischemic injury. The efficacy of the two drugs was similar in cryptogenic cases. Disappearance of interictal EEG abnormalities occurred sooner in patients randomized to ACTH than in those who received VGB as initial therapy. During the second phase, the alternative drug was given to the resistant patients. Spasms ceased in 2 of 5 patients treated with VGB and in 11 of 12 patients treated with ACTH. After 3 months, relapses of spasms were observed in 6 patients treated with ACTH and in 1 treated with VGB. VGB produced a therapeutic response in nearly half the patients receiving this drug. CONCLUSIONS: Our data lend further support to the view that VGB may be considered a first-choice drug in the treatment of IS.

Adrenocorticotropic Hormone↗
PubMed1992

Percutaneous delivery of low-level laser energy reverses histamine-induced spasm in atherosclerotic Yucatan microswine.

BACKGROUND: Previous in vitro experiments performed in our laboratory have shown that low-level laser energy may produce prompt reduction in isometric tension of vascular smooth muscle. The present study was designed to extend these previous in vitro findings to an in vivo model and thereby investigate the hypothesis that laser light delivered percutaneously in vivo could successfully reverse arterial spasm. METHODS AND RESULTS: Spasm defined as greater than 50% reversible reduction in luminal diameter persisting for greater than or equal to 5 minutes was successfully provoked by injection of histamine (100-400 micrograms/kg) in 13 arteries among 10 atherosclerotic Yucatan microswine; the magnitude of histamine-induced vasoconstriction was then documented angiographically by repeated injections of contrast media for as long as 30 minutes (controls). After return of angiographic luminal diameter to baseline, spasm was reproduced with a second injection of histamine into the same artery. Representative wavelengths generated by ultraviolet (UV), visible, and infrared lasers were then delivered percutaneously via conventional fiberoptics to the site of spasm, and angiographic assessment was repeated for as long as 30 minutes (treatment trial). In three arteries treated with UV (351 nm) light from an excimer laser, angiographic luminal diameter narrowing decreased from 100% to 23.9%, 50.0% to 9.3%, and 76.0% to 42.3%, respectively. The magnitude of laser-induced increase in luminal diameter was 50.2 +/- 22.7%, which was significantly greater than the magnitude of relaxation observed spontaneously during the control trials (10.9 +/- 9.8%, p = 0.02). Visible light from a helium-neon (632 nm) laser accomplished complete reversal of histamine-induced spasm in two of four arteries; in the remaining two arteries, luminal diameter narrowing percentages were reduced from 57.0% to 20.0% and from 76.5% to 30.8%, respectively. The magnitude of helium-neon laser-induced relaxation (55.8 +/- 17.9%) was again significantly greater than that observed during the control trials (0.9 +/- 1.9%, p = 0.01). Finally, infrared irradiation from a diode-pumped neodymium:yttrium aluminum garnet (1,064 nm) laser decreased histamine-induced luminal diameter narrowing in three arteries from 100% to 21.4%, 56.0% to 8.7%, and 68.3% to 35.3%, respectively. The magnitude of infrared laser-induced improvement in luminal diameter narrowing was 53.0 +/- 23.3%, which was significantly greater than that observed during the control trials (12.9 +/- 10.7%, p = 0.01). In three additional arteries, fiberoptic sham trials (without laser irradiation) failed to produce relaxation of histamine-induced spasm. CONCLUSIONS: These findings document for the first time that light-induced relaxation of vascular smooth muscle, previously documented in vitro, may be reproduced in vivo.

Animals↗
PubMed2001

Psychogenic hemifacial spasm.

Facial spasms that distort facial expression are typically due to facial dystonia, tics, and hemifacial spasm (HFS). Psychogenic facial spasms, however, have not been well characterized. The authors sought to 1) determine prevalence of psychogenic facial spasm in patients referred for evaluation of HFS and 2) draw attention to clinical characteristics and potential diagnostic pitfalls. Among 210 consecutive patients referred for evaluation of HFS, 5 (2.4%) received diagnoses of psychogenic facial spasm. All patients were female; mean age was 34.6 years (range 26-45) and mean symptom duration 1.1 years (range 2 wk-2 yr). Onset was left-sided in 3 patients, and the lid was the initial site affected in 2 patients. This series of patients shows that facial spasms, although usually of neurovascular etiology, may be the initial or only manifestation of a psychogenic movement disorder, often associated with an underlying depression.

Adult↗