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The prognostic value of EEG patterns in epilepsies with infantile spasms.

By scoring EEG patterns (hypsarrhythmia = 10, absence of sleeping patterns = 10, focal epileptic discharge = 5, general-treatment or in whom infantile spasms never disappeared even during ACTH. A low voltage EEG did not have any ending ACTH therapy free of seizures showed lower scores compared to those infants relapsing after the end of ACTH treatment or in whom infantile spasms never disappeared even during ACTH. A low voltage EED did not have any prognostic significance. Using EEG scores it might be possible to separate non-responders and responders after 3 weeks of ACTH therapy, thus shortening ACTH treatment in non-responding infants.

Adrenocorticotropic Hormone↗

Relief by naloxone of morphine-induced spasm of the sphincter of Oddi in a post-cholecystectomy patient.

Spasm of the sphincter of Oddi is a well-recognized effect of the narcotic class of drugs. Although it is usually clinically silent, such spasm occasionally causes debilitating pain that may be mistaken for more serious disorders. We present the case of a patient who had undergone cholecystectomy previously, but in whom morphine given in the Emergency Department precipitated pain consistent with biliary colic; the pain resolved promptly after administration of naloxone. This entity may considered in the differential diagnosis of acute onset of colicky abdominal pain in the patient given narcotics.

Adult↗

Risk factors associated with infantile spasms: a hospital-based case-control study in Taiwan.

We investigated the risk factors associated with infantile spasms (IS) by a hospital-based case-control study in Taiwan. Twenty-five patients with IS were recruited from one medical center (National Taiwan University Hospital) between 1990 and 1997. Based on a close-structured questionnaire, standardized interviews were carried out to obtain information on risk factors associated with IS. Two comparison groups are used, including a total of 106 subjects in the Disease Control group, and 139 subjects in the Normal Control group. Unconditional logistic regression is used to calculate odds ratios (OR) and 95% confidence interval (CI). Univariate analysis revealed gestational age, congenital cerebral anomalies, tuberous sclerosis (TS), asphyxia, febrile seizure, and developmental delay (before onset of spasm) were at increased risk of IS. After adjustment of multiple risk factors through unconditional logistic regression, significant risk factors for IS include congenital cerebral anomalies, TS, asphyxia, postterm, and developmental delay were highly associated with IS. The risk factors of IS may closely relate to underlying neurological abnormalities. Our results are consistent with the previous findings.

Brain Diseases↗

[Focal lesions in infantile spasms].

Among 174 cases of infantile spasms examined by CT-scan between January 80 and December 85, 17 (9.8%) had a focal lesion without microcephaly. It consisted of an angioma (1 case), a cortical dysplasia (1 case) and porencephalic lesions (15 cases). In 11 cases with porencephaly, an acute event had occurred between 28 weeks of gestation and 3 months of life. In 7 cases, the lesion extended to the major part of one hemisphere. In the 10 others, it was localized either to the rolandic or to the temporo-occipital regions. In all the cases, the lesion concerned at least one of these areas. It involved the cortex in 15 cases but in the 2 others it seemed to only concern the white matter. The lesion was on the left in 14 cases. After steroids, the epilepsy recurred in 6 cases of which 5 involved the frontal lobe (P less than 0.05). This study seems to show that it is mainly the rolandic and the temporo-occipital areas that favour the occurrence of infantile spasms, whereas extension to the frontal area is responsible of a later partial epilepsy.

Brain↗

The management of oromandibular motor disorders and facial spasms with injections of botulinum toxin.

Although much work is yet to be done in this area, nine general conclusions can be derived: 1. Local site-of-injection side effects from botulinum toxin injections are rare, assuming proper technique is used. 2. The two most common medication-related side effects from botulinum toxin orofacial injections are alterations in salivary consistency and inadvertent weakness of the swallowing, speech, and facial muscles. These complications are injection site-specific (eg, more common with lateral pterygoid injections and palatal and tongue muscle injections) and dose-dependent problems. These problems are bothersome but are not contraindications for the therapy if it is needed. 3. The data presented in this article are mostly case series-based and open trial-based information that is promising, but randomized, blinded, controlled trials are needed to establish the true efficacy of this method for the orofacial motor and pain disorders. 4. The novice should begin with injection of muscles he or she can inject with low risk of incorrect placement. The hard-to-find muscles should be avoided when starting out. The novice clinician should inject and dissect a few cadavers to improve injection technique. 5. The general latency for botulinum toxin type A is 1 week, its duration is 2 to 3 months, and it is recommended that injection be done no more than once every 12 weeks to avoid development of antibodies against the toxin. 6. Depending on the target muscle, injection dose is 10 to 50 U of Botox type A per site with a total dose of 200 U in the masticatory system. More than this can be used (400 U maximum) if other sites in the head and neck are included in the injection protocol. 7. Regarding injecting painful muscles that do not exhibit palpable muscle hardness or EMG-determined spasticity or observable involuntary movements but have chronic myofascial trigger points or the patient localizes them as the site of their chronic daily headache pain, botulinum toxin injections might be helpful used in this manner, but conclusive data for this controversial application of botulinum toxin are still missing. 8. Hemifacial spasm has the largest number of open-label, clinical trials, some of which have a 10-year follow-up. The conclusions reached by all of these reports is that treatment of hemifacial spasm with repeated injections of botulinum toxin has been highly successful and that the dose and relative effect of the injections are stable over time. 9. Although EMG-guided injection may be useful, EMG is neither practical nor needed in most situations for orofacial injections because most of the orofacial muscles are easily palpable muscles or have definitive bony landmarks to help with the localization process.

Anti-Dyskinesia Agents↗

Human herpesvirus-6 associated encephalitis with subsequent infantile spasms and cerebellar astrocytoma.

A 14-month-old girl presented after 3 days of fever, floppiness, and diffuse urticarial exanthem. She developed encephalitis and carditis and 1 week later, intractable seizures. Initial CT and MRI showed no changes in the brain parenchyma. On days 14 and 34 after the onset of symptoms, a human herpesvirus-6 (HHV-6) genome in cerebrospinal fluid was identified by polymerase chain reaction (PCR). Convulsions became more frequent and 11 weeks from the onset, they changed to typical infantile spasms with hypsarrhythmic electroencephalogram. She gradually lost her social contact and ability to walk and sit. Eleven months after the primary infection, a repeated MRI of the brain revealed a cystic tumour of 2 cm in diameter near the vermis. The tumour was surgically removed, and shown to be a pilocytic astrocytoma on histopathological examination. HHV-6 DNA was detected by PCR in new tumour tissue. This is the first reported case of HHV-6 encephalitis associated with carditis, infantile spasms, and a subsequent brain tumour containing the HHV-6 genome.

Astrocytoma↗

Hemifacial spasm: operative treatment.

This paper describes experience with a transtympanic facial nerve needling operation in 62 patients with hemifacial spasm, over a 15-year period. The procedure is simple and effective, but unfortunately it is usually marred by recurrence of the spasm. Repeat operations are easy to perform, and usually valuable the first time. Despite recurrence, most patients are finally less troubled than before operation. A small number have proved very resistant to treatment, and the aetiological implications of this are presented. Other operative options are also discussed, together with our experience in a small number treated by facial nerve needling at other sites.

Adult↗

Hemifacial spasm: results of microvascular relocation.

Twenty patients with hemifacial spasm were treated between February 1980 and June 1981. All presented with typical disabling unilateral contractions of the face. Computerized Tomographic Scan and angiograms ruled out structural mass lesions. All patients underwent posterior fossa microsurgical relocation of a vessel from the root entry zone of the facial nerve. An offending arterial loop was found in each case. Nineteen patients are free of symptoms and the remaining one is improved. One patient has permanent loss of hearing and two developed mild facial weakness. There has been no recurrence in 18 months follow up. These results given further support to the theory that hemifacial spasm is an affliction of the seventh nerve in the cerebello-pontine angle, most commonly caused by vascular cross compression at the root entry zone. Surgical relocation of the offending vessel relieves the symptoms and there appears to be no recurrence. This procedure is not difficult and carries acceptable risk for the patient with this disabling condition.

Adult↗

Long term follow up of a hemimasticatory spasm.

OBJECTIVES: To describe the clinical and neurophysiological findings in a case of hemimasticatory spasm (HMS) followed during 14 years of evolution. MATERIAL AND METHODS: A woman suffered from very frequent paroxysmal episodes of painful involuntary occlusion of the jaw. Neurophysiological studies were performed at the 3, 12 and 14 years after the onset of symptoms. They included a needle electromyographic (EMG) evaluation of the main jaw closing and opening muscles, the jaw reflex (JR), the masseteric silent period (MSP) and the masseteric inhibitory reflex (MIR). RESULTS: Clinical symptoms remained unchanged throughout the period of observation. Conventional EMG never disclosed neurogenic signs. Voluntary closure of the jaw systematically provoked an abnormal activity with muscle cramps characteristics, restricted to the left masseter muscle. Left JR response was normal in the first evaluation and became delayed and of reduced amplitude in the second. The MSP and MIR were abolished on the left side during the spasmodic episodes whereas they were strictly normal out of them. The MIR abnormalities showed the characteristic pattern of an efferent lesional type. CONCLUSIONS: Hemimasticatory spasm probably is the consequence of an abnormal trigeminal hyperexcitability likely induced by the demyelinating lesion of its peripheral motor pathway. The main neurophysiological abnormalities may persist unmodified over a long course of the disease and allow the differential diagnosis of HMS from oromandibular dystonia and temporomandibular dysfunction (TMD).

Disease Progression↗

Reduction of hemifacial spasm and dysarthria following EMG biofeedback.

In this case study, a geriatric patient who had an 18-year history of hemifacial spasm was given EMG-biofeedback-assisted relaxation training. No formal speech therapy was provided. Our results confirmed two hypotheses: (a) The patient would learn to reduce frontalis EMG and facial spasm with and then without biofeedback, and (b) as a result, speech would be markedly improved. At both the 1-month and 15-month follow-up the patient retained the ability to relax his facial muscles with similar carry-over to speech. Possible neurophysiologic mechanisms of action mediating the feedback training are discussed.

Aged↗

Informative value of magnetic resonance imaging and EEG in the prognosis of infantile spasms.

PURPOSE: To investigate the informative value of EEG and cranial magnetic resonance imaging (cMRI) in the prognosis of infantile spasms (ISs); 86 patients with ISs were included in this study. METHODS: All cases had epileptic spasms, psychomotor retardation, and hypsarrhythmia in at least one of their EEGs. cMRIs and laboratory tests necessary for etiologic diagnosis were completed in all cases. Patients were followed up periodically both clinically and by video-EEGs for >1 year. Clinical information was categorized on the basis of four spheres as epilepsy, psychosocial development, motor development, and overall clinical condition, with each category being evaluated under three levels of involvement as good, moderate, and severe, depending on selected parameters. A similar scale was applied for the EEG results and for the cMRI findings. Clinical parameters were correlated to EEG and cMRI results, by Spearman test. Other statistical tests used were Kruskal-Wallis chi(2) and Mann-Whitney U analysis as multiple comparison by post hoc Bonferroni correction. RESULTS: A severe overall clinical course was observed in 64% of patients, whereas this incidence was 58% and 44% in the EEG follow-up and cMRI parameters, respectively. In regard to prognosis, a significant correlation was determined between the clinical and the EEG course. This relation was the most prominent in psychosocial developmental parameters and least prominent in the motor development. cMRI findings, however, were correlated only with motor development. CONCLUSIONS: cMRI and repeated EEG recordings, especially when assessed together, may provide complementary information regarding the prognosis in ISs.

Brain↗

Near reflex accommodation spasm: unusual presentation of generalized photosensitive epilepsy.

We report on a 9 year old girl admitted with prolonged and recurrent episodes of near reflex accommodation spasm (NRAS), which was the sole clinical manifestation of primary generalized photosensitive epilepsy. Four days prior to admission, the patient started to experience intermittent episodes of adduction of both eyes and miosis, namely near reflex accommodation spasm, which apparently became fixed three hours prior to admission, with no other perturbations. On examination she was fully alert showing a fixed NRAS, but otherwise unremarkable neurological examination. Time locked with NRAS, the EEG demonstrated generalized spike/wave discharges, also induced with photic stimuli, compatible with generalized photosensitive epilepsy. Assuming an epileptic etiology for the diagnosed NRAS, she was started on a small dose of valproate and became asymptomatic within the first day of treatment, along with EEG normalization. She remained asymptomatic within seven months when the valproic acid was erroneously discontinued. After a few days she began experiencing short lived episodes of NRAS, which could also be induced by photic stimulation and a concurrent EEG again demonstrated generalized epileptiform discharges. Searching the literature, this is the first report associating generalized photosensitive epilepsy with NRAS, and hence complete resolution of NRAS in response to antiepileptic therapy along with normalization of the EEG. An epileptic disorder should be therefore taken into account in any patient with new onset NRAS, especially when other epileptic phenomena are absent.

Accommodation, Ocular↗

Autosomal recessive polymicrogyria with infantile spasms and limb deformities.

We describe two siblings, a girl and a boy, aged 4 and 2 years and 10 months respectively, born from non-consanguineous parents,with diffuse polymicrogyria, lower limb deformities, infantile spasms and developmental delay. Spasms had a good outcome under antiepileptic drug treatment. Clinical and imaging features were of identical severity in both siblings. Muscle biopsy,creatine kinase, metabolic investigations and chromosomal analysis were normal. This combination of anatomo-clinical features and their occurrence in siblings of both sexes suggests an autosomal recessive malformation syndrome.

Cerebral Cortex↗

[Injection of botulinum toxin for diffuse esophageal spasm].

HISTORY AND ADMISSION FINDINGS: A 67-year-old man complained of recurrent retrosternal pain for 3 - 5 minutes as the only symptom for 3 - 4 years. Only when dysphagia occurred in the course of the disease was a gastroenterologist consulted. INVESTIGATIONS: The diagnosis of diffuse esophageal spasm was confirmed by esophageal manometry and radiology. Upper gastrointestinal endoscopy revealed no further pathological findings. TREATMENT AND COURSE: 100 units of botulinum toxin were injected in 10 divided doses, into the tubular esophagus, 1 cm apart, beginning at the z-line of the gastroesophageal junction, deeply into the muscular coat of the posterior part of the esophageal wall. This achieved improvement of symptoms as well as esophageal transit time measured by scintigraphy. The symptomatic benefit has now lasted for several months. CONCLUSION: The treatment of patients with diffuse esophageal spasm with botulinum toxin is a promising therapeutic option, which can be safely performed in such patients. But this regime needs evaluation in controlled prospective clinical studies.

Aged↗

[Thoracoscopic longitudinal myotomy of the esophagus. Successful alternative treatment for diffuse esophageal spasm].

HISTORY AND ADMISSION FINDINGS: A 55-year-old woman had for 5 years been suffering from severe burning thoracic pain. She had typical risk factors for cardiovascular disease, namely hypertension, chronic smoking, obesity and hypercholesterolaemia. She had been hospitalized several times for suspected myocardial infarction, but coronary heart disease had been excluded by cardiac examination, including angiocardiography. The only contributory admission finding was epigastric pain on pressure. INVESTIGATION: Gastroscopy revealed a non-erosive duodenitis and chronic antral gastritis. The cardia was tightly closed and there were no signs of reflux oesophagitis. Manometry in the oesophagus showed frequent simultaneous contractions and marked increase in pressure amplitude, predominantly in the distal segment, indicating diffuse oesophageal spasms. TREATMENT AND COURSE: Drug treatment with calcium channel blockers gave only brief relief from the thoracic pain. But longitudinal oesophageal myotomy via thoracoscopy brought about complete pain relief, which has now persisted for 3 years. CONCLUSION: Thoracoscopic longitudinal oesophageal myotomy can provide a therapeutic alternative in patients with oesophageal spasms when drug therapy and pneumatic oesophageal dilatation have failed.

Chest Pain↗

Infantile spasms associated with a histamine H1 antagonist.

Some anti-allergic agents act as histamine H1 antagonists and induce seizure discharges in epileptic patients. Of these agents, ketotifen has an especially potent effect. We have experienced 2 cases of 4-month-old boys who developed infantile spasms 8 to 10 days after ketotifen administration. They showed almost the same clinical course with regard to their age, the interval between ketotifen administration and the onset of seizures, their symptoms and EEG abnormalities. These cases suggest that the administration of ketotifen to young infants may be hazardous with regard to inducing infantile spasms.

Electroencephalography↗

[How effective is the treatment schedule in children with infantile spasms?].

A fatal incident during combined anticonvulsive therapy with ACTH and Dexamethason prompted us to reevaluate our treatment results in 19 children suffering from infantile spasms. We followed Doose's recommendation using Clonazepam as a means of first choice. Failing that, than Dexamethason and Primidone was used. A combination of ACTH, Dexamethason and Primidone was administered to patients failing both treatment courses. Our results question the necessity, the tolerance limit and the efficacy of this treatment schedule. Therefore we propose a prospective multicentric study to reinvestigate the therapeutic guideliness for infantile spasms.

Adrenocorticotropic Hormone↗

Infantile spasms: infectious disorders.

Infections were considered to be etiological factors in 29 patients (10%) with infantile spasms; congenital CMV (n = 5), congenital or acquired CMV (n = 1), acquired CMV (n = 5), congenital rubella (n = 2), herpes simplex virus (n = 5), enterovirus (n = 1), adenovirus (n = 1), viral encephalitis of unknown agent (n = 3), meningococcus (n = 4), pneumococcus (n = 1) and pertussis (n = 1). The children with congenital infections had long-lasting tremor and convulsions from birth. Early EEG pattern was characteristic for children with herpes encephalitis but not for other patients. Infantile spasms appeared only some weeks after viral encephalitis. One patient with enterovirus and another with probable adenovirus infection had necrotic changes in their brain CT resembling those of herpes encephalitis. The response to ACTH was poor (38%) compared to the whole series (60%). The long-term outcome was also poor compared to the whole series; mental retardation in 90%, convulsions in 62%, abnormal EEG in 89%. Four children died during the follow-up of 7 years. Autopsy showed disseminated CMV infection in one patient and chronic CMV infection in another. The outcome of children with infectious etiology appears to be particularly poor. Thus, the prevention and specific diagnosis and treatment are important. Steroid therapy should be avoided in children with a history of herpes virus encephalitis (CMV, herpes simplex) in the past.

Adolescent↗