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PubMed1983

Homocarnosine levels in cerebrospinal fluid of patients with infantile spasms under ACTH therapy.

The alteration of homocarnosine (dipeptide of GABA and histidine) levels in cerebrospinal fluid (CSF) was studied on 8 cases with infantile spasms under ACTH therapy. CSF was obtained by lumbar puncture before and during ACTH therapy. Homocarnosine was determined by the amino acid autoanalyzer. The attacks of infantile spasms were well controlled in all 8 cases by ACTH therapy. Homocarnosine levels in cerebrospinal fluid of each case decreased from 1.09 to 0.55, 0.92 to 0.41, 1.23 to 0.90, 0.79 to 0.49, 1.06 to 0.75, 1.06 to 0.89, 0.84 to 0.33 and 2.17 to 0.97 mumol/dl, respectively. The decrease of homocarnosine in CSF were 49.5%, 55.4%, 26.8%, 38.0%, 29.2%, 16.0%, 60.7% and 55.3%, respectively. This finding suggests that the decrease of homocarnosine in CSF may be related to the disappearance of attacks of infantile spasms.

Adrenocorticotropic Hormone↗
PubMed1987

Advances in therapy of infantile spasms. Current knowledge of actions of ACTH and corticosteroids.

The anticonvulsant actions of ACTH and corticosteroids in the treatment of infantile spasms have been well documented during the past 29 years. In the past decade neuropeptides have been studied intensively and an understanding of their actions has been gained. Most of the actions of ACTH are well documented in animal experiments and cytochemical studies. Some proceedings of modern steroid research are here reviewed. In the treatment of infantile spasms, the principal mechanism of the therapeutic action of ACTH and corticosteroids is unknown. Clinical data concerning their effects, site and mode of action, on brain and CSF neurochemical activity are still scant and controversial. Some mechanisms probably involved in the therapeutic effects are reviewed. It seems that a disturbance of the central neural transmitter regulation at a specific phase of brain development may be the underlying cause for infantile spasms.

Adrenal Cortex Hormones↗
PubMedDate not supplied

Adrenocorticotropic hormone and vigabatrin treatment of children with infantile spasms underlying cerebral palsy.

Nine infants with an underlying static encephalopathy (confirmed as cerebral palsy in a later follow-up examination) and newly diagnosed infantile spasms were entered in an open study with adrenocorticotropic hormone (ACTH) and vigabatrin as the initial therapy regimen. The ACTH was discontinued after 4-6 weeks and the infants were maintained on vigabatrin alone. Following an initial response with complete suppression of spasms in all patients, a long term response maintained for a mean of 19.2 months was confirmed in all but one child. Tolerability appeared excellent with 7 of 9 patients reporting no side effects; vigabatrin related hypotonia presented in all patients and turned out to be a 'positive' side-effect on the abnormally increased muscle tone of these infants. Given the very poor prognosis of infantile spasms especially in such conditions as cerebral palsy, the combination of ACTH and vigabatrin appears to be an interesting therapy advance with very few side effects.

Adrenocorticotropic Hormone↗
PubMed1999

Infantile spasm: the effect of corticotropin (ACTH) on the free amino acid profile in cerebrospinal fluid.

Increased excitatory amino acid neurotransmission has been implicated in the pathogenesis of infantile spasm. In this study we studied the profile of free amino acids in cerebrospinal fluid (CSF) from 16 patients with infantile spasm before corticotropin (ACTH) treatment. After 10 weeks ACTH therapy the profile of amino acids in CSF was studied once more in eight of the patients. Eleven patients were in the symptomatic group, and five in the cryptogenic group. Increased aspartate levels were measured in CSF following ACTH therapy (P<0.05). It was concluded that aspartate might have an important role in hypothalamic-hypophyseal axis in patients with infantile spasm.

Adrenocorticotropic Hormone↗
PubMed1999

Treatment of infantile spasms with zonisamide.

We determined the efficacy of and tolerability to zonisamide (ZNS) in newly diagnosed patients with infantile spasms. ZNS, 4-20 mg/kg per day, was introduced as an add-on therapy or monotherapy in 27 children with infantile spasms (cryptogenic, 2; symptomatic, 25). The dosage was initially 2-4 mg/kg per day, and then was increased by 2-5 mg/kg every 2-4 days until the seizures disappeared. Nine (33.3%) out of the 27 patients who were administered ZNS exhibited the disappearance of seizures. ZNS was effective in all the cryptogenic cases and seven (28.0%) of the symptomatic cases. The effective daily doses were 5-12.5 mg/kg (mean, 7.8 mg/kg), and the daily dosages were 40-100 mg (mean, 61.1 mg). The steady-state plasma ZNS concentrations were almost within the therapeutic range. The mean time interval between the start of ZNS therapy and the seizure disappearance was 5.0 days. Six (75.0%) of eight effective cases, the exception being one for whom EEG was not performed after the therapy, showed the disappearance of hypsarrhythmia. The recurrence of seizures were observed in four of the nine cases. No adverse reaction to ZNS was noted in any patient. In conclusion, ZNS treatment is considered to be worthwhile trying, for early stage therapy for infantile spasms.

Anticonvulsants↗
PubMed1998

Infantile spasms: a proposal for a staged evaluation.

The purpose of this study was to determine whether a staged, algorithmic evaluation of infantile spasms could be developed to minimize patient discomfort, treatment delay, and overall costs. A retrospective chart review of patients diagnosed with infantile spasms at the authors' institution during a 10-year period was performed, with 29 patients identified; 28 charts were reviewed. By history and physical examination, 21 were classified as symptomatic and seven as cryptogenic. Of the 21 symptomatic patients, 13 had a known etiology at presentation; with further testing the specific etiology was determined in two more. Two in the cryptogenic group were reclassified on the basis of neuroimaging findings. Evaluations included neuroimaging (27, 15 abnormal), cerebrospinal fluid studies (nine, all normal), comprehensive metabolic studies (17, all normal), chromosomal analysis (11, two abnormal), and ophthalmologic evaluation (27, six abnormal). The average cost of the studies per patient was $5,076 at the authors' institution. Etiologic yield was increased by 20% with neuroimaging. The other investigations either confirmed a known etiology or were noncontributory. On the basis of these findings, the authors propose an algorithm for a more focused evaluation of infantile spasms. Using the algorithm, the authors suggest directly proceeding to therapy in patients with specific etiologies determined by history and examination. Further evaluation should start with neuroimaging. Subsequent evaluations should be on the basis of those results. The authors estimate a potential 60-90% reduction in total costs if this algorithm is applied.

Algorithms↗
PubMed1999

Vigabatrin in the treatment of infantile spasms.

Infantile spasms (IS) have been conventionally treated with adrenocorticotropic hormone (ACTH), which is often associated with significant side effects. This study assessed the efficacy of vigabatrin (VGB) as an alternative in the treatment of IS and compared the efficacy of VGB in symptomatic vs cryptogenic patients. The study retrospectively reviewed 25 infants with IS (19 symptomatic, six cryptogenic) who were treated with VGB. Of the symptomatic group, 13 (68.4%) of 19 had clinical improvement, and 15 (78.9%) had electroencephalographic improvement. Three (50%) of six in the cryptogenic group had clinical improvement, and two (33%) had electroencephalographic improvement. Overall, three patients demonstrated clinical spasm control but electroencephalographic deterioration or persistence of hypsarrhythmia coupled with further cognitive decline. Four of the six partial clinical responders had deterioration of spasms with additional VGB dosage increases. VGB is comparable with ACTH in effectiveness for treatment of symptomatic IS. Higher doses of VGB may sometimes cause deterioration rather than further improvement, and therefore an optimum dosage of VGB needs to be titrated for every patient. Persistent electroencephalographic abnormalities and even electroencephalographic deterioration despite clinical control have been observed with VGB treatment; electroencephalographic monitoring during VGB treatment is recommended.

Adrenocorticotropic Hormone↗
PubMed1998

Long-latency response to transcranial magnetic stimulation in patients with hemifacial spasm.

OBJECTIVE: We studied the long-latency response of the orbicularis oris muscle elicited with transcranial magnetic stimulation in patients with hemifacial spasm (HFS) and evaluated the excitability of the facial nucleus. METHODS: We compared the thresholds on both sides in 8 normal volunteers and 7 patients with hemifacial spasm. The thresholds were determined as the lowest intensity required to produce motor evoked potentials with an amplitude of at least 50 microV in the orbicularis oris muscle. Average values were given as means +/- standard deviation. Wilcoxon's rank sum test was used for comparisons between the sides of normal subjects and of patients with HFS with respect to the threshold stimulus. RESULTS: There was no significant difference between the thresholds on the two sides of the normal subjects (mean 1.88+/-5.30%, P > 0.05). In patients with HFS, there was a significant difference between the thresholds on the spasm side and the normal side (mean 20.7+/-13.0%, P < 0.05) In one patient studied after MVD, the difference between both sides disappeared. CONCLUSION: The difference between the thresholds in patients with HFS and the normalization in threshold after MVD suggested that the mechanism of HFS was hyperexcitability of the facial nucleus.

Adult↗
PubMed1997

Long-term outcomes of conventional therapy for infantile spasms.

Infantile spasms (IS) is an age-specific epilepsy which responds to anticonvulsant therapy but has a generally poor prognosis for normal psychomotor development. The subgroup of infants with a cryptogenic aetiology or whose therapy is initiated promptly is thought to have a more favourable prognosis. We retrospectively reviewed 28 infants with IS treated between 1990 and 1996 with adrenocorticotropic hormone (ACTH), valproic acid (VPA), or both, in order to correlate therapeutic response with long-term outcome. Mean age at onset of treatment was 6.4 months, with 57% of patients started within 1 month of IS appearance. IS was considered cryptogenic in 39%. The majority of infants responded to ACTH or VPA with a reduction in spasms of 75% or more. Total remission of seizures occurred in 52%. Death occurred in eight patients; mean duration of follow-up for survivors was 55 months. All subgroups based on age, aetiology, or treatment had poor outcomes, commonly with residual epilepsy, cerebral palsy or mental retardation. Conventional treatment for IS, even when initially successful in reducing spasms, is inadequate when viewed from a long-term developmental perspective, suggesting the need for novel innovative approaches for treating IS.

Adrenocorticotropic Hormone↗
PubMed1999

Interhemispheric interactions analyzed by coherence during flexor spasms.

OBJECTIVE: We used coherence analysis to test for leading discharges on an ipsilateral right mesial temporal lesion in a 5 year old boy with flexor spasms. METHOD: Digital EEG analysis with video-EEG telemetry was performed preparatory to epilepsy surgery. RESULTS: Study of 10 spasms with head drop and subsequent flexion of both arms demonstrated an interhemispheric time lag with secondary bilateral synchrony, with a mean difference of 17 ms. The right hemisphere led. After a lesionectomy with resection of epileptic regions (performed with electrocorticographical guidance), the patient has been seizure-free for 4 years. Pathology confirmed a low-grade mixed glioma and cortical dysgenesis. CONCLUSION: The coherence analysis demonstrated a pathway of secondary generalization, confirming that the lesional side was leading during ictal generalized discharges in flexor spasms.

Brain↗
PubMed2003

Comparison of the esophageal manometric characteristics of idiopathic and reflux-associated esophageal spasm: evaluation by 24-hour ambulatory esophageal motility and pH monitoring.

Diffuse esophageal spasm (DES) is a typical esophageal dysfunction, but its cause is unknown. DES has been classified into reflux-associated esophageal spasm (RDES), caused by acid exposure, and idiopathic esophageal spasm (IDES), of unknown causes. The differences in esophageal motility pattern between the RDES and IDES are clues to elucidating the pathogenesis of DES. Although 24-hr ambulatory esophageal motility and pH monitoring is considered a feasible method for evaluating gastroesophageal reflux and esophageal function, most researchers previously defined the RDES as DES accompanied by esophagitis and heartburn using standard manometry over a short time frame. To clarify the pathogenesis of RDES, we did this research using 24-hr ambulatory esophageal motility and pH monitoring. The investigation included 25 normal controls and 116 patients with upper digestive symptoms. Among the 116 patients, 45 had DES (11 RDES, 30 IDES, and DES-GERD coexistence). Patients with RDES showed a significantly higher frequency of simultaneous contractions than did patients with IDES. Heartburn has been said to be peculiar to RDES, but also occurred in 12.5% of IDES. No significant differences in the mean pH of the esophagus during acid reflux were found between the RDES and IDES patients. Based on the results, we have proposed a refined definition of RDES.

Adolescent↗
PubMed1999

Confirmation of linkage in X-linked infantile spasms (West syndrome) and refinement of the disease locus to Xp21.3-Xp22.1.

The syndrome of infantile spasms, hypsarrhythmia, and mental retardation (West syndrome) is a classical form of epilepsy, occurring in early infancy, which is etiologically heterogeneous. In rare families, West syndrome is an X-linked recessive condition, mapped to Xp11.4-Xpter (MIM 308350). We have identified a multi-generation family from Western Canada with this rare syndrome of infantile spasms, seen exclusively in male offspring from asymptomatic mothers, thereby confirming segregation as an X-linked recessive trait. Using highly polymorphic microsatellite CA-repeat probes evenly distributed over the entire X chromosome, linkage to markers DXS7110, DXS989, DXS1202, and DXS7106 was confirmed, with a maximum LOD score of 3.97 at a theta of 0.0. The identification of key recombinants refined the disease-containing interval between markers DXS1226 and the adrenal hypoplasia locus (AHC). This now maps the X-linked infantile spasms gene locus to chromosome Xp21.3-Xp22.1 and refines the interval containing the candidate gene to 7.0 cM. Furthermore, this interval overlaps several loci previously linked with either syndromic or non-syndromic X-linked mental retardation (XLMR), including one recognized locus implicated in neuroaxonal processing (radixin, RDXP2). Collectively, these studies lend strong support for the presence of one or more genes intrinsic to brain development and function, occurring within the critical interval defined between Xp21.3-Xp22.1.

Chromosome Mapping↗
PubMed2006

Blepharospasm and hemifacial spasm: a protocol for titration of botulinum toxin dose to the individual patient and for the management of refractory cases.

PURPOSE: To evaluate a protocol for the treatment of facial dystonias. METHOD: A retrospective noncomparative interventional case series of all patients who attended a specialised tertiary referral clinic between January 2000 and January 2003. At the start of treatment, patients were seen and treated at weekly intervals until their symptoms were controlled or they developed complications. The sum of the weekly doses was taken as the individual patient dose and then given at 3-4 monthly intervals as required for the individual patient. Refractory cases of spasm underwent orbicularis muscle reduction. Pretarsal spasm was treated with pretarsal botulinum toxin. If the spasm was relieved but the patient could not open their eyelids, a trial of ptosis props was undertaken and the toxin injections continued, before considering a brow suspension. If patients could not see because of a spastic Bell's phenomenon, they were given centrally acting systemic medication. RESULTS: Of 182 new patients, 63 (35%) required two or more sets of injections to titrate their optimum dose of toxin. Symptoms improved in 169 patients (93%). Of a total 332 new patients and follow-up patients, 47 (14%) underwent surgery during their management, 36 protractor myectomy, and 13 brow suspension. Protractor myectomy improved symptoms in 23 (64%). Brow suspension improved symptoms in 12 patients (92%). CONCLUSIONS: The dose of botulinum toxin can be titrated to the individual patient, and the refractory cases managed satisfactorily if a logical protocol is followed.

Adolescent↗
PubMed2004

Spasms in children with definite and probable mitochondrial disease.

The diagnosis of mitochondrial encephalomyopathies is complex and a system for classification of the diagnosis as definite, probable, and possible has been proposed. The objective of this study was to explore the spectrum of epileptic disorders associated with probable and definite mitochondrial disease in children using this classification system. The patient population with mitochondrial disease and epilepsy was selected from a tertiary care children's hospital. Interictal electroencephalograms and video-EEG recordings were used to characterize seizure types. Ten children fulfilled the criteria for probable or definite mitochondrial disease and had epilepsy. Four had siblings with a similar clinical phenotype. Spasms were the most common seizure type and were the initial seizure type in seven patients and two siblings. Four patients had only partial seizures, with or without generalization, and one patient had seizures that were difficult to classify. Blood lactate concentrations were elevated consistently in patients with partial seizures alone but were occasionally normal in children with spasms. Spasms were the most common presenting seizure type in children with probable and definite mitochondrial disease.

Child↗
PubMed2003

Infantile spasms in a patient with williams syndrome and craniosynostosis.

A patient with Williams syndrome, craniosynostosis, and infantile spasms is described. At age 6 months, the infant demonstrated infantile spasms and craniosynostosis and was operated on for craniosynostosis and treated with adrenocorticotropic hormone (ACTH) for the infantile spasms. ACTH completely controlled the seizures, but was halted because of the progression of ventricular hypertrophy. The seizure returned, and he was found to have elfin face, failure-to-thrive, developmental delay, and dental malformation in addition to congenital heart defects. High-resolution chromosome analysis revealed interstitial deletion of 7q11.22-q11.23. Therefore his clinical and cytogenetic diagnosis was Williams syndrome. Thyrotropin-releasing hormone (TRH) therapy reduced his seizures and improved the findings of EEG without cardiac side effects. In addition, his psychomotor development was slightly improved.

Adrenocorticotropic Hormone↗
PubMed2002

Eating epilepsy characterized by periodic spasms.

Seizures induced by eating are generally considered rare. Eating epilepsy is a kind of reflex epilepsy. We report two patients aged 11 and 16, with seizures provoked by eating. The eating-provoked seizures in both patients were a series of tonic spasms. Both ictal EEGs showed a periodic pattern characterized by diffuse high voltage slow wave. These findings were consistent with periodic spasms described by Gobbi et al. The ECD were widely distributed in both patients, although that of Patient 1 was partially clustered in the right frontal region. This is the first report of patients with periodic spasms induced by eating.

Adolescent↗
PubMed2003

Infantile spasms with basal ganglia MRI hypersignal may reveal mitochondrial disorder due to T8993G MT DNA mutation.

PURPOSE: To report three cases of infantile spasms (IS) with an abnormal magnetic resonance imaging signal in the basal ganglia (Leigh-like syndrome), due to T8993G mt DNA mutation. PATIENTS AND RESULTS: The first sign was, at the end of the first year of life, IS in one case and the combination of IS with behavior changes in the two other cases. Video EEG polygraphy demonstrated both spasms and hypsarrhythmia, but no other kind of seizures. Vigabatrin or steroids controlled the spasms with a follow-up of several years. All 3 patients had hyperlactatorrhachia (3.47 to 7 mmol/l). Axial hypotonia and dystonia appeared by the end of the first year of life. As in cases with the NARP mutation and onset later in life, neuropathy and retinopathy could also be demonstrated. DISCUSSION: Although it is well established that symptomatic IS with hypsarrhythmia mainly result from cortical lesions, this epileptic encephalopathy may also be generated by lesions in the basal ganglia without evidence of cortical damage. This finding suggests that West syndrome is likely to be caused by age-related dysfunction at any level of a cortico-putaminal loop of hyperexcitability.

Adenosine Triphosphatases↗
PubMed1986

Blood-induced arterial segmental spasm.

Blood-induced arterial segmental spasm has been noticed clinically, and is thought to result from contact between a main feeding artery and extravasating blood from one of its side branches. This localized spasm can persist long enough to compromise the survival of a replantation or flap transfer. A partially amputated rat hind limb model was used to investigate this phenomenon. Bleeding from a side branch was shown to cause severe vasoconstriction and reduced limb perfusion only on direct contact with the main artery. Pretreatment with heparin and minimization of endothelial damage resulted in decreased vasospasm, but aspirin pretreatment had no effect on the degree of spasm and resulting reduced blood flow. This study demonstrates the clinical importance of careful ligation of side branches to reduce vasospasm that may compromise the survival of a replanted part or tissue flap.

Animals↗