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Reappraisal of interictal electroencephalograms in infantile spasms.

To delineate interictal electroencephalographic (EEG) features before treatment of patients with clinically defined infantile spasms, EEGs of 82 infants having tonic spasms in clusters were analyzed by type of paroxysmal abnormalities, continuity, interhemispheric synchrony, topography, and wave component of hypsarrhythmia during wakefulness and sleep. Hypsarrhythmia occurred less frequently in wakefulness than in non-rapid eye movement (NREM) sleep at any age, least frequently in wakefulness after 1 year of age, and disappeared in rapid eye movement (REM) sleep at any age. The continuity of hypsarrhythmia changed with states, but did not change with age, and was greatest in wakefulness and stage 1 and decreased in stage 2-3. Interhemispheric synchrony increased with increasing age but decreased with advancing sleep stage. The term modified hypsarrhythmia should be discarded, and unusual features, if present, should be specified.

Child, Preschool↗

Treatment of infantile spasms with high-dosage vitamin B6.

High-dose vitamin B6 (pyridoxine-HCl, 300 mg/kg/day orally) was introduced as the initial treatment of recently manifested infantile spasms in 17 children (13 symptomatic cases with identified brain lesion and 4 cryptogenic cases). 5 of 17 children (2 cryptogenic, 2 with severe pre/perinatal brain damage and one with Sturge-Weber syndrome) were classified as responders to high-dose vitamin B6. In all 5 cases the response to vitamin B6 occurred within the first 2 weeks of treatment and within 4 weeks all patients were free of seizures. Two patients developed other seizures (partial seizures, etiologically unclear blinking attacks), but no relapse of infantile spasms was observed among the five responders to vitamin B6. No serious adverse reactions were noted. Side effects were mainly gastrointestinal symptoms, which were reversible after reduction of the dosage. Considering the life-threatening side effects of treatment with ACTH/corticosteroids or valproate, a controlled clinical trial with high-dose vitamin B6 would appear justified to either prove or disprove efficacy.

Administration, Oral↗

Long-term otucome of West syndrome: a study of adults with a history of infantile spasms.

To our knowledge, ours is the first study to evaluate the outcome of infantile spasms (IS) in adult patients. We analyzed 214 children born between 1960 and 1976 who had been followed for 20-35 years or until death at 3 months to 30 years of age. Mortality was 31% (67 of 214 patients). Thirty-six of the surviving patients (24%) had normal (25 patients) or only slightly impaired (11 patients) intelligence as assessed by their educational abilities. Four had academic occupations. Eight were married or living unmarried with a partner. Five had healthy children. At follow-up, the EEGs of the 25 normal persons were either normal or slightly abnormal, demonstrated focal findings in 9 (36%), and had unspecific changes in 1. Focal abnormalities were not more common in patients with less good outcomes (37%). In patients with normal neurological outcomes, IS had been classified as cryptogenic only in 9 of 25 (36%) cases. Therefore, some patients with IS apparently have normal intelligence and socioeconomic status as adults, including patients whose spasms were either symptomatic or associated with focal EEG findings.

Adrenocorticotropic Hormone↗

ACTH therapy for infantile spasms: a combination therapy with high-dose pyridoxal phosphate and low-dose ACTH.

Combination therapy consisting of high-dose pyridoxal phosphate (40-50 mg/kg/day) and low-dose synthetic ACTH (0.01 mg/kg/day) was prescribed in 28 children with infantile spasms. Monotherapy with pyridoxal phosphate provided excellent seizure control in 3 of the 28 (11%) patients. ACTH was subsequently added to the regimen of the remaining 25 patients. As of 1 month after discontinuing the ACTH treatment, 21 of the 25 (84%) patients had experienced no seizures. The mean interval until seizure control was achieved was 4.1 days after the start of treatment with ACTH. The 21 patients have been monitored for a mean of 34.9 months (range 2-81 months); 6 patients (29%) have had recurrences of infantile spasms, and 10 (48%) have experienced normal development. Fourteen of the 28 patients (50%) have had transient increases in liver enzymes, but none of the patients developed more serious side effects.

Age of Onset↗

The risk of lower mental outcome in infantile spasms increases after three weeks of hypsarrhythmia duration.

To assess the correlation between hypsarrhythmia duration and mental outcome in infantile spasms (IS) the medical records of 48 infants with IS were reviewed retrospectively and psychological assessments undertaken at follow-up at the age of 3 to 13 years. We found 18 (38%) cryptogenic IS cases with typical hypsarrhythmia and 30 symptomatic with modified hypsarrhythmia-further classified into 15 cases as multifocal, 10 as pseudoperiodic and 5 as unilateral hypsarrhythmia. A short treatment lag (one to two weeks) occurred in 25, three to four weeks in 10 cases. Spasms ceased within one month after treatment in 23 infants. At follow-up 15 children had normal mental outcome (borderline included). A correlation between hypsarrhythmia duration longer than three weeks and lower mental outcome was found using the logistic regression model. The duration of hypsarrhythmia represents a sensitive prognostic parameter in IS; the risk of mental retardation increases after three weeks of hypsarrhythmia.

Age of Onset↗

Counting of flexor spasms.

A method is described which is able to convert muscular contraction to a top, which can be counted electronically or visually. Such contractions are found much more frequently in patients with flexor spasms than in normal subjects during studies over 6-9 hours during the night. The method is of value in long-term recording, in evaluating antispastic therapy and in analyzing factors influencing flexor spasms.

Adolescent↗

Organic convergence spasm.

Convergence spasm has frequently been reported as a hysterical manifestation. A case presenting with this sign and diphenylhydantoin intoxication is described. Other previously reported organic associations are discussed. Convergence spasm may be seen in association with significant organic disease of the nervous system affecting the cerebral hemispheres, the brain-stem, or both.

Adolescent↗

Infantile spasms and neuronal heterotopias. A report on six cases.

Six infants with infantile spasms are presented, three primarily considered idiopathic and three symptomatic. Autopsy unexpectedly revealed heterotopias, cortical dyslamination, and other brain malformations in all six cases. No history of any etiologic moment during the neuronal migratory period of early gestation was found. It is concluded that wide-spread disturbances of CNS morphology should always be considered in cases of infantile spasms.

Abnormalities, Multiple↗

Cranial computed tomography in infantile spasms. Primary findings related to long-term mental prognosis.

Out of 109 children with infantile spasms (IS), prospectively tested during the years 1976 to 1979 in Denmark, 52 children were examined by cranial computed tomography (CT). The classification of IS into cryptogenic (CR), symptomatic (SY) and doubtful (DO) was done clinically without considering the CT-findings. Sixty per cent of the scannings were abnormal. Only 6/30 (20%) of the children in ACTH treatment were found to develop cerebral atrophy which means that this finding is not an obligatory side-effect of ACTH treatment of children with IS. Normal CT-findings were found in 50% of the CR and 50% of the SY + DO-groups, and could not be used as a prognostic tool for estimating the mental development. This was also the case for children with cerebral atrophy. Abnormal CT-findings (minus atrophy) were highly correlated to the group with clinical symptoms and indicate an extremely unsatisfying long-term mental prognosis. CT-scanning is a valuable tool for the examination of clearing children with infantile spasms.

Adrenocorticotropic Hormone↗

Esophageal motor disorders: achalasia and esophageal spasm.

PURPOSE: To define the esophageal motor disorders of achalasia and esophageal spasms and describe their presentation in the clinical setting. DATA SOURCES: Selected research-based articles, textbooks, and expert opinion. A case study is presented. CONCLUSIONS: The presentation of esophageal motor disorders may not be clear, particularly when the presenting symptom is chest pain. Determining whether the pain is cardiac or digestive in origin is crucial. IMPLICATIONS FOR PRACTICE: Progressive dysphagia for both solids and liquids is the major symptom of achalasia; other symptoms include regurgitation, chest pain, and nocturnal cough. Diffuse esophageal spasm typically causes substernal chest pain with nonprogressive dysphagia and odynophagia for both liquids and solids. Dysphagia related to esophageal motility is characterized by a sensation of swallowed food "sticking" in the throat or chest; there is no problem initiating the act of swallowing.

Adult↗

Cardiovascular changes associated with skeletal muscle spasm in tetraplegic man.

1. A study has been made of the effect of spontaneous and induced muscle spasms on blood pressure, heart rate, hand blood flow, calf blood flow and occluded vein pressure in the hand and foot in non-bedridden patients with chronic, closed, complete, localized transection of the cervical spinal cord.2. Both types of spasm produced a similar response consisting of an increase in blood pressure and occluded vein pressure and a decrease in heart rate and hand and calf blood flow. The changes commenced within 2-3 sec and usually reached maxima in 20-30 sec. On some occasions there was a temporary initial increase in heart rate and calf blood flow.3. The changes occurred independently of changes in bladder pressure.4. It is concluded that the changes in peripheral blood vessels result from a spinal sympathetic reflex to somatic afferents associated with muscular contraction.

Adult↗

Diagnosis of oesophageal spasm by ergometrine provocation.

Oesophageal spasm may mimic the pain of myocardial ischaemia. Forty-two patients who were thought to have angina until investigations failed to show any cardiovascular abnormality, were examined for oesophageal disease. Ergometrine provocation during oesophageal manometry caused significant deterioration in oesophageal motility, associated with familiar pain, in 24 patients. Ten age-matched controls were examined in a similar way and ergometrine produced motility changes in four and pain in two. Six volunteers with coronary artery stenosis and exercise-induced angina did not develop oesophageal motility changes during the pain. Ergometrine provocation is useful in establishing the diagnosis of oesophageal spasm in patients with recurrent angina-like pain but no cardiac abnormality.

Angina Pectoris↗

Diffuse oesophageal spasm: diagnosis by ambulatory 24 hour manometry.

BACKGROUND: Diffuse oesophageal spasm (DOS) is a potential cause of intermittent chest pain and/or dysphagia. In the past, the diagnosis of DOS has relied on criteria obtained from standard oesophageal manometry (more than one simultaneous contraction in a series of 10 wet swallows with the rest being peristaltic). As symptoms are intermittent, however, 24 hour manometry may well be more suited to its investigation. AIMS: To determine the ability of 24 hour manometry to detect the symptomatic contractions of DOS and to compare standard, laboratory based manometry with 24 hour manometry in its diagnosis. PATIENTS: Three hundred and ninety consecutive patients referred with suspected oesophageal disorders. METHODS: Standard laboratory based manometry and 24 hour outpatient manometry. RESULTS: Sixteen patients were classified by 24 hour manometry as having DOS on the basis of painful contractions (spasms) of excessive duration and increased amplitude. Laboratory based manometry failed to detect the majority of these patients with DOS (14/16), and 53/55 were incorrectly labelled as having DOS on the basis of asymptomatic manometric findings. CONCLUSION: The detection of symptomatic DOS requires 24 hour manometry.

Adult↗

Dyspnoea during generalised spasms in tetraplegic patients.

Dyspnoea during generalised spasms is reported in three tetraplegic patients. Intraoesophageal electromyographic evidence strongly suggests that the dyspnoea is a result of diaphragmatic spasms. Utilisation of this EMG technique in other tetraplegic patients may add to our knowledge of variations in the distribution of the phrenic nerve nucleus within the spinal cord.

Adult↗

The association of hemifacial spasm and facial pain.

The recognition of an association between trigeminal neuralgia and ipsilateral hemifacial spasm has been delayed by confusion over the nomenclature of the two conditions. Three patients are presented who had facial pain associated with hemifacial spasm. The findings on investigation of these patients, and an analysis of the literature, suggests that the combination is almost inevitably associated with pathological processes in the posterior fossa, particularly anomalous, ectatic or aneurysmal blood vessels.

Facial Muscles↗

Long term results of botulinum toxin type A (Dysport) in the treatment of hemifacial spasm: a report of 175 cases.

OBJECTIVE: To describe the long term efficacy and side effects of the treatment of hemifacial spasm with Dysport and to evaluate two different sites of injection to hopefully reduce side effects. METHODS: This study was designed as a prospective descriptive study. Injections were made subcutaneously around the eye. Peak improvement was subjectively assessed by using a visual analogue scale and reported in percentages (0-100%). Duration of improvement was assessed subjectively and reported in months. RESULTS: Of 175 cases, 17 were lost to follow up and were excluded. 855 treatments were injected in the remaining 158 patients with a median of 4 treatments. The response rate was 97%. Of 855 treatments, the adjusted mean peak and duration of improvement was 77.2 (95% confidence interval (95%CI) 74.7-79.4)% and 3.4 (95%CI 3.2-3.6) months respectively. In 158 patients (complete group), the long term results from the first to the 12th treatment showed that the mean peak improvement ranged from 72.70 to 80.10% and the duration of improvement was 2.60 to 3.71 months. It remained constant throughout (p=0.40, p=0.87 respectively). The most common side effect was ptosis. Of the 158 patients, 21 completed 12 treatments (subgroup). A separate analysis of this group disclosed a mean peak and duration of improvement from the first to 12th treatments ranging from 70.00 to 78.10% and 2.65 to 4.31 months respectively. Analysis of variance with repeated measures showed no significant variation of peak and duration of improvement over the first to the 12th treatments (p=0.38, p=0.38 respectively). Only 3% of the treatments were unsuccessful but responded to subsequent treatments. The incidence of ptosis was reduced from 27.17% to 9.68% by moving the injection site to the lateral part of orbital orbicularis oculi without any loss of efficacy. The yearly cost of Dysport is considerably less than Botox. CONCLUSION: This study is the first to show, in detail, the long term results of treatments of hemifacial spasm with Dysport. The efficacy is constant throughout orbicularis oculi. The efficacy of Dysport is comparable with Botox in long term follow up.

Aged↗

Arterial spasm during renal angioplasty.

Spasm of the renal arteries during transluminal angioplasty is a well-documented phenomenon with serious potential sequelae, particularly in young patients with fibromuscular dysplasia. The authors report their experience in 98 cases (105 arteries). Tolazoline, lidocaine, nitrates (or calcium blockers, if available), and heparin should be administered either directly into the renal artery or systemically prior to angioplasty to decrease the incidence and severity of spasm.

Adolescent↗