Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Spasm”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 541 records · Page 30Linked to original sources
PubMed1997

Developmental outcomes in children receiving resection surgery for medically intractable infantile spasms.

Two-year postsurgical developmental outcomes were assessed in 24 children with infantile spasms who underwent resective surgery. The mean age of onset of infantile spasms was 12.0 weeks and the mean age at surgery was 20.8 months. Developmental outcomes were assessed using the Vineland Adaptive Behavior Scales (VABS). There was a significant increase in developmental level at 2 years postsurgery compared with presurgical levels. At 2 years postsurgery only one of the children in this series was severely retarded. The developmental outcomes of patients in the series were better than those in prior studies of symptomatic patients receiving medical treatment for infantile spasms. It is surprising that the children in the UCLA series frequently had developmental outcomes equal to and sometimes superior to other groups of children with infantile spasms, since all the UCLA patients were symptomatic, had neurologic deficits and had failed to respond to adrenocorticotrophic hormone (ACTH) and antiepileptic drugs. The 2-year postsurgery developmental outcomes were best for the children who received surgery when they were relatively young and who had the highest level of developmental attainments presurgically.

Activities of Daily Living↗
PubMed1984

Adrenocorticotropic hormone controls infantile spasms independently of cortisol stimulation.

Infantile spasms constitute a severe seizure disorder unresponsive to standard anticonvulsants. Both prednisone and adrenocorticotropic hormone (ACTH) have produced remission of seizures in some patients. The mechanisms of action of these hormones are not known. Eight infants with infantile spasms were treated with prednisone for 2 weeks. This controlled the seizures in two patients. In the remaining six patients, prednisone was continued and ACTH was added. This treatment produced cessation of spasms in four patients. Serum prednisone and cortisol were measured at a number of points during treatment. In infants receiving prednisone and then prednisone plus ACTH, serum cortisol was suppressed to about one-quarter of baseline levels with the initiation of prednisone, and remained suppressed during ACTH administration. We conclude that ACTH can exert its effect on infantile spasms in the setting of adrenal suppression, and can act without stimulating endogenous cortisol production. A CNS site of action is suggested and should be sought.

Adrenocorticotropic Hormone↗
PubMedDate not supplied

Therapy of infantile spasms with valproate: results of a prospective study.

In a prospective study, 22 children with recently manifested infantile spasms (18 patients with symptomatic and 4 with idiopathic infantile spasms) were treated with sodium valproate (VPA). Before VPA was instituted, a loading test was performed to exclude abnormal patterns of VPA metabolites by gas chromatography and mass spectroscopy of serum and urine. This test was repeated during VPA therapy; an abnormal pattern of VPA metabolites was not observed. VPA was started in increasing dosage until infantile spasms were controlled or a maximum dose of 100 mg/kg/day was reached. If VPA did not control seizures or at least reduce frequency significantly after a trial of 4-6 weeks, dexamathasone was added to VPA. If focal seizures occurred in association with localized epileptogenic EEG discharges, carbamazepine (CBZ) was added to VPA. After 4 weeks of VPA monotherapy, infantile spasms were controlled in 11 children. After 3 months of therapy, 16 children were free of seizures (14 patients VPA monotherapy), and 4 children had reduction of seizure frequency to less than 25%. VPA doses varied between 40 and 100 mg/kg/day (mean 74). The mean plasma concentration was 113 micrograms/ml (range 46-177). After 6 months of therapy, total seizure control was achieved in 20 of 22 patients (16 children VPA monotherapy). The mean observation time was 16 1/2 months (range 6-36 months). There were seven relapses in six children during the first 7 months of therapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adrenocorticotropic Hormone↗
PubMedDate not supplied

Surgery for intractable infantile spasms: neuroimaging perspectives.

Twenty-three infants and children underwent cortical resection (n = 15) or hemispherectomy (n = 8) for intractable infantile spasms. Infantile spasms were present at the time of surgery in 17 of the 23 patients; in six, spasms had evolved to other seizure types during surgical evaluation. Children with a remote history of infantile spasms were excluded from this study. Focal or hemispheric lesions were identified by magnetic resonance imaging in seven children; an additional two showed focal atrophy without a discrete lesion. Positron emission tomography (PET) showed lateralized or localized abnormalities of cerebral glucose utilization in all patients; in 14, PET was the only neuroimaging modality to identify the epileptogenic cortex. When this occurred, neuropathological examination of resected brain tissue typically showed malformative and dysplastic cortical lesions. Focal interictal and/or ictal electrographic abnormalities were present in all patients, and corresponded well with localization from neuroimaging. None of the patients were subjected to chronic invasive electrographic monitoring with intracranial electrodes. At follow-up (range 4-67 months; mean 28.3 months), 15 children were seizure-free, three had 90% seizure control, one had 75% seizure control, and four failed to benefit from surgery with respect to seizure frequency.

Brain↗
PubMed1996

Hemifacial spasm: magnetic resonance angiography.

Twenty-three consecutive patients with hemifacial spasm were studied. Magnetic resonance imaging angiography of the brain was performed in 20 patients and 15 controls. The angiograms were evaluated by two independent observers and blinded for side-location of the spasm. Contact between an artery from the vertebrobasilar circulation and the intracranial part of the facial nerve was observed ipsilaterally to the spasm in 17 patients (85%) and in two of 30 control half-brains (7%), respectively. Treatment is discussed. The study confirms that arterial relation to the facial nerve root is the most frequent cause of hemifacial spasm. Magnetic resonance imaging is recommended to exclude mass lesions in the posterior cranial fossa, and magnetic resonance angiography is recommended in preoperative evaluation and in research.

Adult↗
PubMed1996

Botulinum toxin A injection in the treatment of hemifacial spasm.

UNLABELLED: There are conflicting reports concerning the variation in duration of symptoms relief for patients with hemifacial spasm who have undergone several injections of botulinum A toxin (BOTX-A). We present our experience of BOTX-A injections in Taiwanese patients to analyze this issues, and to inspect whether the efficacy of treatment depends on the pre-injection severity. MATERIAL AND METHOD: From July 1992 to December 1994, 137 patients received injections of BOTX-A. We used objective and subjective score system to evaluate the efficacy and side effects of BOTX-A injection. RESULTS: The overall successful rate of substantial relief of spasm was 88%. The mean duration of response was 20 weeks. Patents with more severe spasm tended to have shorter duration of improvement. The effects of consecutive injections remained fairly constant over the first 4 injections. CONCLUSION: The BOTX-A injection is an effective and safe treatment for patients with hemifacial spasm and the effect could be sustained over the consecutive injections.

Adult↗
PubMed2005

Health-related quality of life in blepharospasm or hemifacial spasm.

OBJECTIVES: Health-related quality of life (HRQL) has become an important outcome criterion of medical interventions, but was hardly studied in patients with facial spasms. MATERIALS AND METHODS: Patients with blepharospasm or hemifacial spasm treated with botulinum toxin were included. A healthy control group sociodemografically matched to patients was established. Instruments applied included the SF-36 (global HRQL), the NEI-VFQ (disease-specific HRQL) and the Blepharospasm Rating/Disability Scale, the latter to patients only. RESULTS: Thirty-one patients with blepharospasm and 21 patients with hemifacial spasm took part. The Blepharospasm Rating/Disability Scale revealed minor functional limitations, but identified some symptoms to be improved. In both patient groups global (both SF-36 Component Summaries) and disease-specific (eight of 12 subscales) HRQL were significantly impaired compared with controls. CONCLUSION: HRQL should be integrated as core outcome criterion in treatment of patients with facial spasms. Assessment by a generic and a disease-specific instrument is recommended.

Aged↗
PubMed1990

Long-term treatment of involuntary facial spasms using botulinum toxin.

Botulinum toxin, a powerful pre-synaptic neurotoxin produced by Clostridium botulinum, interferes with the release of acetylcholine from nerve terminals. Since September 1985, we have been using this toxin to treat altogether 62 patients with benign facial spasms. Most of the patients had been on drugs or psychotherapy, 2 had received alcohol injections, 2 had undergone surgery of the orbicular branch, and 2 electrocoagulation of the facial nerve. In essential blepharospasm the duration of the beneficial effect after each treatment with botulinum toxin was about 3 1/2 months. In patients with hemifacial spasm the response was clearly longer, nearly 5 months in most cases. The treatment gave the best and longest-lasting relief of symptoms in patients suffering from disturbing myokymia. Response was poorest in patients suffering from facial spasms who simultaneously had a severe psychiatric disease. The most frequent side effect was mild or moderate ptosis (22.6%). Some patients complained of dry eyes and a few cases displayed facial nerve paresis. Side effects caused by botulinum toxin injections are transient but so also, unfortunately, is the beneficial effect on facial spasms.

Adult↗
PubMed1978

Infantile spasms and HLA antigens.

21 new cases of infantile spasms were reported in 1976 from paediatric departments in Denmark. The connection between infantile spasms and the Lennox-Gastaut syndrome is mentioned, because of reports of a significantly higher incidence of HLA-B7 in children with Lennox-Gastaut syndrome. The HLA antigen distribution in 19 of the 21 children was compared with that of 1967 healthy adults. No difference in the HLA antigens was demonstrated between children with infantile spasms and controls, whether in the material as a whole, or in the cryptogenic or symptomatic groups. However HLA typing of children with infantile spasms should continue in the search for a potential genetic marker in this grave disease, particularly in view of the reported high incidence of HLA-B7 in children with the Lennox-Gastaut syndrome.

Female↗
PubMed2005

CDKL5/STK9 is mutated in Rett syndrome variant with infantile spasms.

BACKGROUND: Rett syndrome is a severe neurodevelopmental disorder, almost exclusively affecting females and characterised by a wide spectrum of clinical manifestations. Both the classic form and preserved speech variant of Rett syndrome are due to mutations in the MECP2 gene. Several other variants of Rett syndrome have been described. In 1985, Hanefeld described a variant with the early appearance of convulsions. In this variant, the normal perinatal period is soon followed by the appearance of seizures, usually infantile spasms. We have observed two patients with signs of Rett syndrome showing acquired microcephaly and stereotypic midline hand movements. The disease started with generalised convulsions and myoclonic fits at 1.5 months in the first patient and with spasms at 10 days in the other, suggesting a diagnosis of the Hanefeld variant. In these patients, MECP2 point mutations and gross rearrangements were excluded by denaturing high performance liquid chromatography and real time quantitative PCR. The ARX and CDKL5 genes have been associated with West syndrome (infantile spasms, hypsarrhythmia, and mental retardation). METHODS: Based on the clinical overlap between the Hanefeld variant and West syndrome, we analysed ARX and CDKL5 in the two girls. RESULTS: We found frameshift deletions in CDKL5 in both patients; one in exon 5 (c.163_166delGAAA) and the other in exon 18 (c.2635_2636delCT). CDKL5 was then analysed in 19 classic Rett and 15 preserved speech variant patients, all MECP2 negative, but no mutations were found. CONCLUSION: Our results show that CDKL5 is responsible for a rare variant of Rett syndrome characterised by early development of convulsions, usually of the spasm type.

Amino Acid Sequence↗
PubMed1983

Hemimasticatory spasm--a peripheral paroxysmal cranial neuropathy?

The clinical and electrophysiological features of a case of hemimasticatory spasm are presented. The findings are in many respects similar to those described in hemifacial spasm. A peripheral cranial neuropathy as proposed in hemifacial spasm also may be responsible for hemimasticatory spasm.

Blinking↗
PubMed1999

Gabapentin as treatment for hemifacial spasm.

Hemifacial spasm, a life-long condition characterized by involuntary unilateral contractions of the facial muscles, is a disabling disorder often resulting in patient irritation and social embarassment. Its probable etiology is neurovascular compression of the facial nerve at its root exit zone. The current medical treatment consists of either baclofen or anticonvulsant drugs, with limitation due to side effects or low efficacy. In recent years botulinum toxin injection and microvascular decompression of the facial nerve have been shown to be highly successful. However, both procedures share some complications and require special techniques. We present 5 patients affected by hemifacial spasm who responded well to the novel anticonvulsant drug gabapentin. Gabapentin was administered at a dose ranging from 900 to 1,600 mg daily, with rapid and clear improvement of spasms and absence of any remarkable adverse effects. Our findings suggest that gabapentin may be an effective treatment for patients with hemifacial spasm with a very good ratio of therapeutic effects to side effects when compared with other drugs currently used.

Acetates↗
PubMed2003

Lactic dehydrogenase isoenzyme in cerebrospinal fluid of children with infantile spasms.

Increased levels of lactic dehydrogenase (LDH) in cerebrospinal fluid (CSF) have been reported in association with several intracranial pathologies. We studied LDH isoenzymes in the CSF of children with infantile spasms. CSF samples collected from 12 patients (aged 4-9 months) with infantile spasms were analyzed for total LDH isoenzymes activity, and were compared to samples from 15 normal children. Mean total LDH activity in the CSF was 34.62 +/- 6.52 U/l. Patients with infantile spasms had a lower LDH-1 percentage and higher LDH-3 percentage; the differences from the control group were statistically significant (p < 0.01). LDH-4 and LDH-5 had similar values in both groups. Infantile spasm is apparently associated with a distinct LDH isoenzyme pattern in the CSF. More studies are needed to confirm the rise in LDH-2, LDH-3 and to determine the optimum time of analysis.

Humans↗
PubMed1988

Effects of spinal cord stimulation on spasticity and spasms secondary to myelopathy.

16 subjects with severe spasms secondary to traumatic and nontraumatic myelopathy underwent epidural spinal cord stimulation. 4 patients had a complete motor and sensory spinal cord lesion. 6 of the subjects with an incomplete spinal cord lesion were ambulatory. All patients had previously undergone extensive trials with medications and physical therapy. All 14 subjects in whom a satisfactory placement of the electrode could be obtained had a reduction in the severity of the spasms. In 6 patients, the spasms were almost abolished. Extremity, trunkal and abdominal spasms were affected. Clonus in the upper extremities was consistently reduced. Marked improvement in bladder and bowel function was observed in each of 2 subjects. In over 1-year follow-up, 5 subjects show persistence of the results, with less stimulation required to maintain the therapeutic effects. No neurological deterioration occurred following the procedure or after long-term spinal stimulation. 1 patient showed after several months of continuous stimulation increased voluntary motor control present only when spinal cord stimulation was activated. Complications included 1 system infection, 1 electrode migration, 1 wire breakage and skin breakdown at a connector site, development of high impedance in 1 electrode and 1 skin breakdown over the lead.

Adult↗
PubMed1995

Abnormal esophagocardiac inhibitory reflex in patients with diffuse esophageal spasm.

The aim of this study was to establish if patients with an esophageal dysmotility similar to that found in the majority of patients with 'swallow syncope' may also have a pathologic esophagocardiac reflex similar to that observed in these patients. The effects on heart rate induced by dry and solid swallows and by intraesophageal balloon distension were studied in 8 normal subjects and in 10 patients with diffuse esophageal spasm by simultaneous recording of ECG and intraesophageal pressures. Dry swallows induced a brief increase in heart rate, while deglutition of a solid bolus initially induced an increase followed by a decrease in heart rate, significantly more marked in patients with diffuse spasm. The intraesophageal balloon inflation induced a decrease in heart rate which was significantly more intense in patients with diffuse spasm. In conclusion, the esophageal wall distension either due to solid bolus ingestion or to balloon inflation elicits an inhibitory esophagocardiac reflex which is more intense in patients with diffuse spasm and might induce cardiac arrhythmias in predisposed subjects.

Adult↗
PubMed2005

Reduced visual function associated with infantile spasms in children on vigabatrin therapy.

PURPOSE: To use visual evoked potential (VEP) testing to determine whether visual deficits are present in children with a history of vigabatrin use. METHODS: Contrast sensitivity and visual acuity were assessed by visual evoked potential testing and compared between 28 children (mean age, 4.90 +/- 4.92 years) with seizure disorders who had taken vigabatrin and 14 typically developing children (mean age, 3.14 +/- 1.70 years). Exclusion criteria were heritable eye disease, suspected cortical visual impairment, nystagmus, and prematurity >2 weeks. The effects of the following factors on contrast sensitivity and visual acuity were examined: type of seizure (infantile spasms versus other), ERG result, duration of vigabatrin therapy, cumulative dosage of vigabatrin, and other seizure medications (other versus no other medication). RESULTS: Contrast sensitivity and visual acuity were reduced in vigabatrin-treated children with infantile spasms compared with vigabatrin-treated children with other seizure disorders and typically developing control subjects. The other factors examined had no significant effect on contrast sensitivity or visual acuity, with adjustment for seizure type. CONCLUSIONS: Children with infantile spasms on vigabatrin may have compromised visual function, even in the absence of suspected cortical visual impairment. The children tested in the present study have reduced vision, probably associated with infantile spasms rather than vigabatrin.

Adolescent↗
PubMed2005

A 9-year-old boy with a history of large perinatal stroke, infantile spasms, and high academic achievement.

The prognosis for intellectual development in children with symptomatic infantile spasms is usually poor. We report a 9-year-old boy with a history of a large, presumed perinatal, left middle cerebral artery infarct discovered when he developed infantile spasms at 6 months of age. The infantile spasms responded to treatment with adrenocorticotropic hormone. He attained cognitive milestones at normal times, requiring only speech therapy for dysarthric speech. At 9 years of age, he has seizures and a severe right hemiparesis but is an articulate honor roll student in advanced English classes. The development of infantile spasms after large-branch middle cerebral artery stroke does not always predict future mental retardation.

Aptitude↗
PubMed1996

Nitrazepam for refractory infantile spasms and the Lennox-Gastaut syndrome.

Infantile spasms and the Lennox-Gastaut syndrome are considered to be age-specific pediatric epileptic syndromes and together constitute a significant percentage of medically resistant seizures in childhood. Twenty children, ages 4 to 28 months (median, 12 months), with medically refractory infantile spasms or the Lennox-Gastaut syndrome, were treated with the investigational benzodiazepine nitrazepam in an open-label study. Daily dosage of nitrazepam ranged from 0.5 to 3.5 mg/kg, with a median dosage of 1.5 mg/kg, divided into two doses per day. Side effects included pooling of oral secretions (12 children) and sedation (six children); however, no serious side effects were seen. Responses to nitrazepam were as follows: five complete responses (cessation of all seizures), seven partial responses (greater than 50% reduction of seizures), and eight with no response. Median duration of response was 9 months (range, 4 to 16 months) in children with infantile spasms and 14 months (range, 8 to 26 months) in children with the Lennox-Gastaut syndrome. Nitrazepam is an effective anticonvulsant in this small cohort of children with medically refractory infantile spasms and the Lennox-Gastaut syndrome, resulting in a 25% response rate and only modest side effects.

Administration, Oral↗