Karin Edebol Eeg-Olofsson

Neuropsychological findings in children with benign childhood epilepsy with centrotemporal spikes

Benign childhood epilepsy with centrotemporal spikes (BCECTS) is a well‐known idiopathic age‐ and localization‐related epileptic syndrome with characteristic clinical and EEG manifestations. Due to the reported benign evolution of this epilepsy syndrome, neuropsychological assessment has been considered unnecessary. However, the benign nature of BCECTS has recently been challenged: verbal dysfunction as well as impaired visuomotor coordination, specific learning disabilities, and attention deficit have been noticed. These findings prompted this research study in which all children with BCECTS attending our epilepsy clinic underwent neuropsychological assessment. Seventeen children (10 boys and seven girls) aged 7 to 14 years were investigated with a neuropsychological test battery focusing on immediate and delayed recall of auditory‐verbal and visual material, verbal fluency, problem‐solving ability, and visuospatial constructional ability. Ravens coloured matrices and questionnaires regarding school functioning and behaviour were also administered. The children were matched with control subjects for age, sex, and school. Children with BCECTS had significantly lower scores than their control subject partners on the neuropsychological items. Intellectual abilities did not differ and neither did school functioning or behaviour according to teachers. Parents, however, recognized greater difficulties with concentration, temperament, and impulsiveness in children with BCECTS.

BILATERAL SPASTIC CEREBRAL PALSY‐A COMPARATIVE STUDY BETWEEN SOUTHWEST GERMANY AND WESTERN SWEDEN. II: EPIDEMIOLOGY

Epidemiological data of a collaborative study on children with bilateral spastic cerebral palsy (BSCP) 3 between south‐west Germany and western Sweden are reported. The study period covered the birth 5 years 1975 to 1986. Overall, the rate of BSCP increased during the birth year periods 1975‐77 and 1978‐80, but decreased thereafter. The rise was due to an increase of BSCP in low‐birthweight (LBW) children, especially very LBW (VLBW) children. Mortality rates in LBW, and particularly VLBW, children decreased significantly during the whole study period in both countries. The BSCP rate, after the initial increase, showed a decrease during the second half of the study period in LBW children. Results are interpreted in favour of a predominantly prenatal aetiology in normal birthweight and of a predominantly peri‐ and neonatal aetiology in LBW children.

Hippocampal asymmetries and white matter abnormalities on MRI in benign childhood epilepsy with centrotemporal spikes.

Summary: Purpose: To look for brain abnormalities by using magnetic resonance imaging (MRI) in patients with benign childhood epilepsy with centrotemporal spikes (BCECTS), which is the most common epilepsy syndrome in children.

Analysis of motor pathway involvement in Konzo using transcranial electrical and magnetic stimulation

To elucidate the involvement of motor pathways in konzo, 21 konzo subjects (mean age 22 years) underwent transcranial electrical stimulation (TES) in 1998. Fourteen konzo subjects (mean age 21 years) underwent transcranial magnetic stimulation (TMS) in 2000. Three subjects underwent both TES and TMS. Motor evoked potentials (MEPs) were recorded in the abductor pollicis brevis (APB) muscle with TES, and in the abductor digiti minimi (ADM) and tibialis anterior (TA) muscles with TMS. APB‐MEPs were normal in 2 of 21 subjects and absent in 9; central conduction time (CCT) was prolonged in 10. Resting ADM‐MEPs were absent in 9 of 14 subjects with clinically preserved upper limbs. Among these nine, seven subjects responded after facilitation. Most subjects (13 of 14) failed to show TA‐MEPs. Of the subjects who underwent both types of stimulation, one had normal TES‐MEP but abnormal ADM‐MEP with TMS. These findings suggest involvement of both corticomotoneurons and motor descending pathways in konzo.

Results of biofeedback in constipated patients

PURPOSE: The aims of this study were to assess the results of biofeedback treatment in constipated patients and to identify variables that might be used to predict the outcome. METHOD: Twenty-eight patients (5 men; median age, 46 (range, 22–72) years) with any degree of paradoxical activation measured with thin hook needle electromyography in the external sphincter or puborectalis muscle were included. The symptom duration varied between 1 and 30 (median, 9) years. The patients had eight outpatient training sessions with electromyography-based audiovisual feedback. All patients were followed up prospectively with a validated bowel function questionnaire from which a symptom index was created. RESULTS: At three months, nine patients had no improvement and underwent other treatments. The remaining 19 patients were followed up for a median of 14 (range, 12–34) months. Twelve patients (43 percent) stated they had improved rectal emptying. A good result was associated with increased stool frequency (P< 0.05), improved symptom index (P<0.01), and reduction of laxative use (P<0.05). A long symptom duration, a high pretreatment symptom index, and laxative use were related to a poor result (P<0.01–0.05). The improved group had less perineal descent (P<0.05), and a prominent puborectalis impression on defecography tended to be more common (P=0.06). CONCLUSION: With the use of wide inclusion criteria, biofeedback was successful in 43 percent of patients, with a treatment effect lasting at least one year. The results suggest that biofeedback should be used as the initial treatment of constipated patients with a paradoxical puborectalis contraction.

Neuro-ophthalmologic findings in konzo, an upper motor neuron disorder in Africa.

Purpose To investigate the neuro-ophthalmological manifestations in konzo, a non-progressive symmetric spastic para/tetraparesis of acute onset associated with consumption of insufficiently processed bitter cassava roots combined with a low protein intake. Methods Twenty-one Congolese konzo patients underwent neuro-ophthalmological investigations including visual acuity testing, assessment of light pupillary reflexes, evaluation of ocular motility and deviation, direct ophthalmoscopy, and visual field perimetry. Objective refraction including retinoscopy and keratometry, and slit-lamp biomicroscopy were also done. Results Five patients had visual impairment, and 14 had temporal pallor of the optic disc. Fourteen presented visual field defects, the most frequent being concentric constriction and peripheral defects. Overall, 11 subjects had symptoms qualifying for the diagnosis of optic neuropathy. Two had spontaneous pendular nystagmus in primary position of gaze. Visual field defects and pallor of the optic discs were found in mild, moderate and severe forms of konzo. No correlation was found between the severity of the motor disability of konzo and the extent of visual field loss. Conclusions Konzo was associated with optic neuropathy and a few patients had nystagmus. Although the etiopathogenesis of this optic neuropathy remains to be elucidated, the symmetry of the involvement suggests a toxic origin. We suggest that cyanide causes the neuro-ophthalmological damage in konzo. However, the optic neuropathy in konzo patients does not resemble the features of the epidemic optic neuropathy in Tanzania, Cuba or Nigeria, Lebers hereditary optic neuropathy, tobacco amblyopia or vitamin B deficiency.

Is electrostimulation of the pelvic floor an effective treatment for neurogenic faecal incontinence

BACKGROUND This study was designed to evaluate the results of electrostimulation of the pelvic floor for faecal incontinence. METHODS Twenty-four patients with neurogenic (n = 20) or idiopathic (n = 4) incontinence were treated on 12 occasions. Twenty were women, and the mean age was 63 years (range, 26-85 years). The result was evaluated at 3 and 12 months with a validated questionnaire and anorectal manometry. RESULTS Eleven patients (46%) reported improvement after 3 months, and the Millers incontinence score was reduced (P < 0.01). Resting pressures, rectal compliance, and the length of the high-pressure zone were unchanged, but squeeze pressures tended to decrease. A lower threshold was seen for the rectoanal inhibitory reflex (RAIR) (P < 0.01), and the saline infusion test tended to show improved retention (P = 0.07). Nine of 13 patients followed up for 1 year claimed improvement. The most pronounced subjective improvement was seen in patients with moderate incontinence. CONCLUSIONS Electrostimulation was successful in a substantial proportion of the patients. The threshold for RAIR was lowered, and the saline infusion test tended to improve, but variables reflecting sphincter competence did not improve. Moderate incontinence was prognostically favourable, and electrostimulation might therefore be an alternative to surgery in this group.

Impairments, disabilities and handicap pattern in konzo - a non-progressive spastic para/tetraparesis of acute onset

Purpose: To assess impairments, disabilities and handicap pattern in konzo. Method: The study included 17 konzo subjects, of which three were males and 14 females (mean age 21, median 18 years). A detailed neurological examination was performed on all subjects. Subsequently, an assessment of impairments, disabilities and handicap was done with a constructed rating scale partially based on the ICIDH-2 framework. Results: The overall disablement picture in all subjects consisted of motor dysfunction in lower limbs leading to limitations in walking and movement activities, and restrictions in mobility. Hip mobility was severely impaired in most cases (15/17). Although konzo subjects showed normal muscle power in upper limbs (13/17), they had impaired fine motor function (10/17). Conclusion: Further studies are needed to assess the effectiveness of the WHO criteria for konzo in defining its forms. The applicability of the ICIDH-2 framework in this study demonstrates the possibility of its use as a common language among researchers in the field of motor disorders. However, a revision is suggested of its taxonomy, and a definition of operational criteria to clarify the content of different qualifiers provided to assess the level of functioning or disability.PURPOSE To assess impairments, disabilities and handicap pattern in konzo. METHOD The study included 17 konzo subjects, of which three were males and 14 females (mean age 21, median 18 years). A detailed neurological examination was performed on all subjects. Subsequently, an assessment of impairments, disabilities and handicap was done with a constructed rating scale partially based on the ICIDH-2 framework. RESULTS The overall disablement picture in all subjects consisted of motor dysfunction in lower limbs leading to limitations in walking and movement activities, and restrictions in mobility. Hip mobility was severely impaired in most cases (15/17). Although konzo subjects showed normal muscle power in upper limbs (13/17), they had impaired fine motor function (10/17). CONCLUSION Further studies are needed to assess the effectiveness of the WHO criteria for konzo in defining its forms. The applicability of the ICIDH-2 framework in this study demonstrates the possibility of its use as a common language among researchers in the field of motor disorders. However, a revision is suggested of its taxonomy, and a definition of operational criteria to clarify the content of different qualifiers provided to assess the level of functioning or disability.

Association between sociodemographic status and antiepileptic drug prescriptions in children with epilepsy

Purpose:  We investigated whether in Sweden sociodemographic differences are associated with access to expert health care and antiepileptic drug (AED) prescriptions in children with epilepsy.

Physiological and structural anorectal abnormalities in patients with systemic sclerosis and fecal incontinence

Abstract Objective. Fecal incontinence is common in systemic sclerosis (SSc), but the underlying mechanisms are not fully understood. The objectives of this study were to characterize anorectal physiological and morphological defects in SSc patients and to correlate the results with incontinence symptoms. Materials and methods. Twenty-five SSc patients underwent anorectal neurophysiological investigations, anal manometry, and ultrasound. Results. Eleven patients (44%) reported incontinence to solid or liquid feces, but no patient reported diarrhea. Increased fiber density (FD) was recorded in 78% of patients with and in 86% of patients without fecal incontinence not significant (NS). Incontinent patients had lower squeeze pressure (SP; median 49.5 mm Hg) in the high-pressure zone (HPZ) than continent patients (median 72 mm Hg; p = 0.01). In two of the incontinent patients, sonographic abnormalities of the internal anal sphincter (IAS) and the external anal sphincter (EAS) were present, whereas in another two patients isolated IAS abnormalities were seen. These four individuals had lower resting pressure at 1 cm and in the HPZ, and lower SP at 2 cm than patients with normal anorectal sonographic findings (p < 0.05). Conclusion. Lower voluntary SP in incontinent patients and EAS sonographic abnormalities only in patients with incontinence suggest that the EAS is more important in maintaining fecal continence in SSc patients than has previously been reported. The finding of increased FD in most patients further supports involvement of the EAS function in SSc and could indicate previous nerve injury with consequent incomplete reinnervation.