Unexpected improvement after prolonged posttraumatic vegetative state.

An 18-year-old girl suffered a severe head injury in a traffic accident. As a result, she was vegetative for about 21/2 years. She then showed signs of a gradually returning responsiveness. Six years after the accident, she is now able to comprehend and communicate, shows considerable interest in her surroundings and is able to establish interpersonal relationships. Moreover, the improvement is still continuing. Her severe contractures, however, prevent her from making full use of these regained mental capacities. Such a mental recovery after such a long-lasting vegetative state of traumatic origin has not previously been described.     

The Diagnostic Yield of a Second EEG After Partial Sleep Deprivation: A Prospective Study in Children with Newly Diagnosed Seizures

Summary: Purpose: To assess the diagnostic yield of a repeated EEG (REPEEG) after partial sleep deprivation (SD) in children and adolescents with one or more seizures who previously had had a standard EEG (STDEEG) without epileptiform abnormalities (EAs). In the literature, 32–75% of such REPEEGs after SD were reported to show EA. Methods: In a prospective, multicentred study, we selected children aged 1 month to 16 years with one or more idiopathic or remote symptomatic newly diagnosed seizures. A REPEEG was recorded in children without EAs in their STDEEG. Results: Of 552 children and adolescents who entered the study, 243 (44%) had a STDEEG without EAs. In 177 (73% of eligible children), REPEEGs were recorded after SD. We found EAs in 61 (34.5%) REPEEGs and new nonepileptiform abnormalities in five (1%). In 552 children in the total cohort, the REPEEG thus added 11% with EAs to the 56% with EAs in the STDEEG. Of REPEEGs, 81% included sleep compared with 20% of STDEEGs. In about half the REPEEGs, EAs occurred during sleep only. One child had tonic-clonic seizures probably related to the SD. Conclusions: One third of REPEEGs yielded new diagnostic information. Partial, age-dependent SD was highly effective in inducing sleep, which is important because in many cases EAs were found only during EEG recording in sleep. The procedure was safe and convenient.     

Prenatal ultrasound diagnosis of the Holt-Oram syndrome

The Holt-Oram syndrome is an autosomal dominant disorder consisting of a congenital heart defect in combination with characteristic upper limb abnormalities. This report presents the ultrasonographic follow-up of two fetuses at risk for the Holt-Oram syndrome. In the first fetus, the existence of Holt-Oram syndrome was suspected at 22 weeks of gestation; a ventricular septal defect, an atrial septal defect, and a minor skeletal defect were found. In the second fetus, no structural abnormalities were discovered until the 30th week, when a small atrial septal defect was detected. In both pregnancies, it was possible to exclude early in gestation the more severe forms of the Holt-Oram syndrome.     

Follow-Up of 146 Children with Epilepsy after Withdrawal of Antiepileptic Therapy

The relapse rate after discontinuation of antiepileptic drug treatment was investigated in 146 children with epilepsy, in whom medication was withdrawn according to a predesigned protocol, after a seizure-free period of at least 2 years and normalization of the EEG. The cumulative probability of remaining seizure-free in this series was 74.5%. Three-quarters of the relapses occurred during the withdrawal period and in the 2 years thereafter. From multivariate analysis, the factors indicating a significantly higher relapse risk were seizures with a known cause and female sex. In primary generalized epilepsy, no factor significantly increased the likelihood of a recurrence. In partial epilepsy, significant factors predictive of recurrence were the presence of a neurological deficit (focal neurological signs and/or mental retardation), female sex, a positive family history for epilepsy, and the number of drugs necessary for control of the seizures. The present results are compared with the available literature data. It is argued that using multivariate analysis after elimination of EEG variables uncovers significant clinical predictive factors that in other studies may have remained hidden. Finally, it is argued that statistical analysis may be used to enable the clinician to predict the likelihood of recurrence in individual children with a given set of relevant predictive factors.     

Safety evaluation of the mixture of chemicals at a specific workplace: theoretical considerations and a suggested two-step procedure

Procedures for the selection of compounds with high health hazard potential are reviewed, and major aspects of the assessment of health risks associated with exposure to mixtures of chemicals are discussed. Examples are given of additivity and synergism of effects following exposure to mixtures. Using these data from the literature a two-step procedure for the safety evaluation of the mixture of chemicals occurring at a specific workplace is suggested. The first step consists of estimating the relative health risk associated with each chemical; the estimation is based on the ratio between exposure level and degree of toxicity. Those chemicals representing a high risk are then selected for further consideration. The second step comprises the risk assessment, focusing on prediction of the risk associated with exposure to the mixture of selected chemicals. To allow such prediction the (presumable) mode of action of the selected compounds should be considered. A practical way to find out whether exposure to chemicals at a specific workplace is of serious health concern, could be the conduct of both a 4-week toxicity study in rats and 2 different types of genotoxicity studies with the mixture of selected chemicals, using exposure concentrations related to those occurring in practice.     

Seizure Severity in Children with Epilepsy: A Parent-Completed Scale Compared with Clinical Data

Summary: Purpose: We wished to compare a parent-completed scale quantifying seizure severity (SS) in children with various seizure types with the clinicians' impression of SS and other clinical data. Methods: The parents of 117 children with recurrent seizures completed a 13-item, subjective scale (The Hague Seizure Severity Scale, HASS). Eight treating neurologists quantified SS on a 10-point Visual Analog Scale (VAS) and supplied other clinical data. Results: Both the HASS and the VAS assessments of SS showed considerable variation within one seizure type. Significant differences were noted between groups with (a) absences and simple partial seizures (SPS), (b) complex partial seizures (CPS), and (c) generalized tonic-clonic seizures (GTCS). The correlation coefficient between the neurologists' and the parents' scores was 0.45 but did not exceed 0.26 after stratification for seizure type. The parents' score was not substantially influenced by various other clinical variables. The neurologists' score was correlated with resistance to treatment and presence of mental retardation. Conclusions: The SS ratings of the parents and the neurologists were not substantially correlated. The consideration that parents, as eyewitnesses to the seizures, are probably better judges of SS than clinicians may favor the use of a parent-completed scale to quantify SS. The HASS is a valid and reliable measure of parent-perceived SS that can be useful as an outcome measure in childhood epilepsy.     

Influence of intrapyloric botulinum toxin injection on gastric emptying and meal-related symptoms in gastroparesis patients

BACKGROUND: Recent observations in limited numbers of patients suggest a potential benefit of intrapyloric injection of botulinum toxin in the treatment of gastroparesis. AIM: To characterize the effect of botulinum toxin on solid and liquid gastric emptying and on meal-related symptoms. METHODS: In 20 gastroparesis patients (17 women, mean age 37 +/- 3 years, three diabetic and 17 idiopathic), gastric emptying for solids and liquids was measured before and one month after intrapyloric botulinum toxin 4 x 25 units. Before the meal and at 15-min intervals up to 240 min postprandially, the patient graded the intensity of six gastroparesis symptoms, and a meal-related severity score was obtained by adding all intensities. Data (mean +/- S.E.M.) were compared using paired Student's t-test. RESULTS: Treatment with botulinum toxin significantly enhanced solid (t(1/2) 132 +/- 16 vs. 204 +/- 35 min, P < 0.05) but not liquid (92 +/- 10 vs. 104 +/- 11 min, N.S.) emptying. This was accompanied by a significant decrease in cumulative meal-related symptom score (73.5 +/- 16.3 vs. 103 +/- 17.1 baseline, P = 0.01) as well as individual severity scores for postprandial fullness, bloating, nausea and belching (all P < 0.001, two-way anova). CONCLUSIONS: Botulinum toxin improves solid but not liquid gastric emptying in gastroparesis, and this is accompanied by significant improvement of several meal-related symptoms.