[Extensive rehabilitation needed in epilepsy. Different models for varying needs]. / Rehabiliteringsbehovetär stort vid epilepsi. Olika modeller für varierande behov.

Many people with epilepsy experience the need for more information concerning their condition and some people require, at times, greater access to rehabilitation services than has previously been available. Information and support may also be required by the family, friends and work colleagues of the patient. Research indicates that rehabilitation needs, for the patient, can arise in relation to poor self-esteem, overprotection as a child, low expectations, reduced cognitive abilities and stigma perceived or experienced in the family, in society, and at work. Successful rehabilitation is individualistic and should therefore be based on an assessment of the individual's needs. Only on the basis of this can realistic goals be identified. In the cases where intensive multidisciplinary rehabilitation is required a thorough medical assessment will also be needed in order to formulate realistic goals. Achievement of these goals may involve input from psychological, neuropsychological, pedagogical, social, employment and family orientated perspectives. Specialists in these areas must be included within, or co-ordinated by, the epilepsy team or rehabilitation service. In Sweden hospital based epilepsy-teams have added psycho-social rehabilitation aspects in their responsibilities and other rehabilitation services have recently begun to focus on the needs of people with epilepsy. This has led to a range of different rehabilitation models being available. The need to develop improved epilepsy rehabilitation services was identified in the 1970s and has recently received short-term funding support to develop new services and build up expertise. It is hoped that in the future these services will continue to find support at both the county and national levels.

Incidence of epilepsy in rural central Ethiopia.

PURPOSE: To study the incidence of epilepsy in a rural area of Ethiopia. METHODS: A community-based study was performed in a random sample of villages with 61,686 inhabitants in a rural area of central Ethiopia. In a door-to-door survey, all inhabitants in the study area were interviewed about seizures. A standardized protocol was used. All new cases with epilepsy that had occurred since a previous study was made 3.5 years earlier were included. Fifty-three of the subjects were investigated with EEG. RESULTS: One-hundred thirty-nine incident cases were identified, corresponding to an annual incidence of 64 in 100,000 inhabitants [95% confidence interval (CI) 44-84]. The corresponding rate for males was 72 (CI 42-102); for females, it was 57 (CI 31-84). The highest age-specific incidence occurred in the youngest age groups (0-9 years); the next highest was in the group aged 10-19 years. Generalized convulsive seizures occurred in 69%, partial seizures occurred in 20%, and unclassifiable seizures occurred in 11%. Seizures occurred daily in 10% and weekly in another 14%; 33% had monthly seizures. Twenty-two percent had a family history of epilepsy. A history of head trauma was ascertained in 5.7% and was the most common possible etiologic factor identified. Thirteen percent were treated with antiepileptic drugs (AEDs). CONCLUSIONS: The incidence of epilepsy in Ethiopia is high. A high incidence in combination with a prevalence of epilepsy in the study area comparable to that in the rest of the world may be explained by a high degree of spontaneous remission of epilepsy and/or a high mortality due to epilepsy. Despite health education on epilepsy given to the community, a minority of subjects were treated with AEDs, which may reflect the inadequacies of the health services and transportation difficulties faced by the patients.

A model of neurological disorder: a basis for logical and structured thinking and reasoning in neurology.

A model of neurological disorder in graphical form, based upon a model proposed by the World Health Organisation, is presented. This model is intended to be a basis for a logical and structured thinking and reasoning in neurology, not least in diagnosis. The model is a chain of basic elements in which each element causes the following one. The chain can be divided in two parts. The first part is: Etiology → Pathology → Manifestation. Pathology is described by the properties of the lesion in the nervous system. Manifestation is the symptoms and signs, that can be summarised as Semiology, in fact, synonymous with Impairment. The second part of the model is then: Manifestation (= Impairment) → Disability → Handicap. The terms disease and disorder are not contained within the chain of basic elements. The manifestation gives the patient an awareness of disorder and the condition may be classified as syndrome or disease. Neither the awareness of disorder nor the classification influences the chain of basic elements.

Regional cerebral blood flow, white matter abnormalities, and cerebrospinal fluid hydrodynamics in patients with idiopathic adult hydrocephalus syndrome.

OBJECTIVES--(1) to evaluate regional cerebral blood flow (rCBF) with single photon emission computed tomography and 99mTc-hexamethylpropyleneamine oxime in patients with the idiopathic adult hydrocephalus syndrome (IAHS); (2) to examine regional cerebral blood flow (rCBF), gait, and psychometric functions before and after CSF removal (CSF tap test); (3) to assess abnormalities in subcortical white matter by MRI. METHODS--Thirty one patients fulfilling the criteria for IAHS (according to history and clinical and neuroradiological examination) were studied. Quantified gait measurements, psychometric testing, and rCBF before and after removal of CSF were obtained. Pressure of CSF and CSF outflow conductance were investigated with a constant pressure infusion method. Brain MRI was used to quantify the severity of white matter lesions and periventricular hyperintensities. In IAHS a widespread rCBF hypoperfusion pattern was depicted, with a caudal frontal and temporal grey matter and subcortical white matter reduction of rCBF as the dominant feature. Removal of CSF was not accompanied by a concomitant increase in rCBF. Significant white matter lesions were detected only in a minority of patients by MRI. An altered CSF hydrodynamic state with a higher CSF pressure and lower conductance was confirmed. IAHS is characterised by an abnormal CSF hydrodynamic state, associated with a widespread rCBF reduction with preference for subcortical white matter and frontal-temporal cortical regions. Furthermore in most patients MRI did not show white matter changes suggestive of a coexistent subcortical arteriosclerotic encephalopathy. At least in the idiopathic group of patients with AHS, measurements of rCBF before and after temporary relief of the CSF hydrodynamic disturbance will not provide additional information that would be helpful in the preoperative evaluation but is suggestive of a preserved autoregulation of rCBF.

Perceptions of blindness.

A study in a mainly rural area of Ethiopia has revealed that sighted, unilaterally blind and totally blind people have many misconceptions about blindness. The work of cure and prevention demands that widespread ignorance about the causes and implications of blindness be overcome.

Migraine, chronic tension-type headache, and cluster headache in an Ethiopian rural community.

Fifteen-thousand-five-hundred adults (> or = 20 years) in a rural district in Ethiopia with a population of 250,000 were studied for chronic headache. Door-to-door survey was performed by trained lay health workers using a questionnaire with a high degree or reliability and validity. The 1-year prevalence of migraine headache was 3.0% (4.2% for females and 1.7% for males) with the peak age specific rate in the fourth decade. Migraine headache was about three times more common in females than in males at any decade. Two-thirds of migraine sufferers had rather frequent attacks. The most frequent trigger factors were emotional stress (90%), changes of weather (78%), physical exhaustion (75%), and smell (70%). Migraine with aura was rare. Family occurrence of migraine in first-degree relatives was 30%. The 1-year prevalence of chronic tension-type headache was 1.7%, while cluster headache was found to be extremely rare. Compared to similarly performed surveys, the prevalence of migraine among rural Ethiopians was less than among Nigerian Africans.

The predictive value of cerebrospinal fluid dynamic tests in patients with th idiopathic adult hydrocephalus syndrome.

OBJECTIVE: To evaluate the predictive value of the cerebrospinal fluid (CSF) tap test and CSF outflow conductance in the selection of patients with the idiopathic adult hydrocephalus syndrome, defined exclusively on a clinical basis, for shunt surgery. DESIGN: A prospective, consecutive case series. All patients were assessed before surgery and at 3 months after shunt placement. Preoperatively, CSF pressure, conductance, and CSF formation rate were assessed by a constant-pressure infusion method. Improvement in gait and cognitive functions after removal of CSF was noted (ie, with the CSF tap test). Postoperatively, the infusion method was used to evaluate shunt function. SETTING: Tertiary, academic referral center. PATIENTS: Thirty-five patients with idiopathic adult hydrocephalus (normal-pressure hydrocephalus) syndrome based on conservative clinical criteria. No predictive tests were used for inclusion. All patients had a typical gait disturbance and a communicating hydrocephalus. Twenty-eight patients also had dementia or incontinence, or both. INTERVENTION: The CSF diversion (Cordis, [Orbis-Sigma]) valve, six patients; Hakim's standard system (Cordis Hakim standard system), 29 patients. OUTCOMES OF SURGERY: Serial videotaping of gait, a comprehensive neuropsychologic battery, and the Bartel index of activities of daily life. RESULTS: Gait was improved in 25 (72%) of the 35 patients, whereas the Bartel index remained unchanged. The conditions of five of seven patients with gait disturbance as the sole symptom improved. The spatial function (37% improved) and the findings from the Fuld object memory tests (29% improved) were significantly improved. Shunt dysfunction could not explain the lack of effect of an operation in the remaining patients. It should be noted that these results obtained at 3 months postoperatively may not be applicable in a long-term perspective. The CSF outflow conductance or CSF tap test were not able to identify those patients who would or would not benefit from a CSF diversion procedure. However patients had a lower conductance and a higher CSF pressure than did control subjects. CONCLUSIONS: Considerable improvement in gait was seen, but cognitive function was little affected. The CSF tap test or conductance does not provide additional information that is necessary to distinguish between patients whose conditions will or will not respond to shunting, when selection for surgery is based on conservative clinical criteria.

Cerebrospinal fluid shunt dynamics in patients with idiopathic adult hydrocephalus syndrome.

The objective was to assess CSF dynamics of different shunt constructions in patients with adult hydrocephalus syndrome and correlate these findings to clinical outcome, neuroradiology, and the specifications of the shunts provided by the manufacturer. Thirty four patients with idiopathic adult hydrocephalus (normal pressure hydrocephalus) syndrome were included in a prospective, consecutive case series. A differential pressure valve (Cordis Hakim standard system) was used in 28 patients and a variable resistance valve (Cordis Orbis-Sigma) in six. A constant pressure infusion method was used; CSF pressure and conductance were determined before surgery. Three months after shunt placement CSF pressure, the "pressure v flow" curve, and gravity induced flow were measured. There was no difference between mean preoperative and postoperative resting CSF pressures in patients with Hakim shunts. The opening pressures of the Hakim shunts were higher than the value proposed by the manufacturer. A pronounced gravity effect induced CSF flow and decrease of the CSF pressure. In functioning variable resistance valves, CSF dynamics normalised postoperatively. There was no gravity effect and the characteristics shaped "pressure v flow" curve was sometimes seen. Six patients (three differential pressure valves, three variable resistance valves) had non-functioning shunts. Four of these patients were improved after the operation but improvement was transient in three. In all patients, there was no relation between the width of the ventricles and clinical improvement or CSF pressure. In conclusion, the differential pressure valve system does not behave according to the specifications provided by the manufacturer. A decrease in CSF pressure in patients with this shunt was solely due to the effect of gravity. Eleven percent of the differential pressure valves and 50% of the variable resistance valves were non-functioning. In the functioning variable resistance valves, the antisiphon system seems to be effective.

CSF concentration gradients of monoamine metabolites in patients with hydrocephalus.

Concentration gradients of homovanillic acid (HVA), 5-hydroxyindoleacetic acid (5-HIAA), and 3-methoxy-4-hydroxyphenylglycol (MHPG), were assessed in 762 successive CSF fractions (2 ml lumbar CSF) from 15 patients with the adult hydrocephalus syndrome (AHS) and 11 patients with hydrocephalus of other causes (mixed group). A mean volume of 49.6 (SD 11.8) ml CSF was removed in the AHS group and 56.4 (10.2) ml in the mixed group. The CSF was collected with a specially designed carousel fraction collector and the corresponding CSF dynamics were continuously registered by a constant pressure CSF infusion method. Pronounced gradients in CSF HVA and CSF 5-HIAA were seen in both patient groups in the first 25 ml of CSF removed. The concentration curves levelled off, despite the removal of larger amounts of CSF and stabilised at about twice the initial concentrations. This phenomenon has not been described before. Concentrations of HVA and 5-HIAA in the first CSF fraction correlated strongly with concentrations in fractions up to about 40 ml. A positive correlation between the first fraction of CSF HVA and CSF 5-HIAA concentrations and CSF outflow conductance was found in the AHS group. There was no gradient in MHPG. It is suggested that the rostrocaudal gradients in CSF HVA and 5-HIAA may be explained by a downward flow of CSF along the spinal cord with absorption of metabolites occurring during passage. Mixing of CSF from different CSF compartments, extraventricular production sites of CSF, clearance of metabolites to venous blood or extracellular fluid, and CSF outflow conductance are probably important determinants of the plateau phase in patients with hydrocephalus. It is concluded that lumbar CSF does not exclusively reflect the concentrations of HVA, 5-HIAA, or MHPG in the ventricles. It should be noted that these results obtained in patients with hydrocephalus may not be applicable to other groups of patients or normal subjects.

Blood pressure patterns and its correlates in schoolchildren of an Ethiopian community.

This study was undertaken to define blood pressure pattern in a sample of schoolchildren in an Ethiopian rural community. All schoolchildren below the age of 15 years in Butajira town formed the study population. Blood pressure measurement which was made according to the recommendations of the American Task Force on blood pressure control in children was found to be unimodal with almost symmetrical distribution. The mean systolic and mean diastolic blood pressure for both sexes combined was 106 +/- 14.8 and 70.5 +/- 4.5 mmHg, respectively. The mean systolic and mean diastolic blood pressures increased with age in both boys (P < 0.05) and girls (P < 0.01). Girls had significantly higher mean systolic and mean diastolic blood pressures than boys (P < 0.001). Similarly, girls had significantly higher pulse rate and arm-girth than boys which may partly explain the higher mean pressures in the girls. A multiple regression analysis showed that height, body mass index, arm-girth, and heart rate were significant in explaining the variations in systolic and diastolic blood pressures. In conclusion, except for the higher blood pressure in girls, our findings are similar to the majority of the reports from Africa and other parts of the world.

Attitudes of rural people in central Ethiopia towards leprosy and a brief comparison with observations on epilepsy.

To find out public attitudes toward leprosy a door-to-door survey was carried out in 1546 sampled households in the rural farming community of Meskan and Mareko in central Ethiopia, where the prevalence of leprosy is estimated to be 1:1000. Attitudes toward leprosy were compared with attitudes to epilepsy, studied in a previously performed survey in the same community. Eighty-seven per cent of the respondents were above the age of 25, and 59.5% were females. There were slightly more Muslims (54%) than Christians. The majority of the interviewees (87%) were farmers, with an illiteracy rate of 84%. Ninety-five per cent and 83%, respectively, were not willing to employ or work with a person having the disease. Seventy-five per cent would not allow their children to associate with a playmate suffering from leprosy. Comparative analysis of attitudes in the same community showed that negative attitudes toward leprosy were stronger than those toward epilepsy, particularly with regard to matrimonial associations, sharing of accommodation, and physical contact with an affected person. The reasons for these differences appear to be the community's deeply entrenched belief that leprosy is both hereditary and contagious, expressed respectively by 48% and 53% of the respondents. In order to minimize the perpetuation of negative attitudes, there is a need to educate and impress on the population that leprosy is a treatable infectious disease which is not congenitally acquired, and that it is even curable if detected early. The study reinforces previously proposed suggestions that, in developing countries such as Ethiopia, leprosy care should be integrated into the general health services.

CSF hydrodynamics in superior sagittal sinus thrombosis.

Cerebrospinal fluid hydrodynamics were investigated with a constant pressure infusion method in patients with superior sagittal sinus thrombosis. Ten patients were studied with serial examinations up to 15 years after the onset of the disease. A total of 70 CSF hydrodynamic examinations were performed. A clear increase in intracranial pressure due to raised pressure in the major dural sinus was seen in all patients. A striking feature was the persistent intracranial pressure increase that declined only gradually. This had no obvious clinical impact. Change in CSF resorption facility played only a minor role in the intracranial pressure elevation. None of the patients developed hydrocephalus.

CSF hydrodynamics in idiopathic intracranial hypertension: a long-term study.

To examine CSF hydrodynamics, we studied 16 patients with idiopathic intracranial hypertension and 45 control subjects with a constant-pressure infusion method. Fifteen patients had 155 examinations up to 15 years after the onset of disease. In most patients, the disturbances of CSF hydrodynamics persisted for many years. We identified at least two mechanisms for the development of the increased CSF pressure: a rise of sagittal sinus pressure, probably explained by extracellular edema causing partial compression of the major venous sinus (type 1), or a low conductance with a compensatory increase in CSF pressure in order to sustain the bulk flow (type 2).

Rheumatic heart disease prevalence among schoolchildren of an Ethiopian rural town.

Rheumatic heart disease (RHD) is the most important cardiovascular disease in hospital practice in Ethiopia. However, the prevalence rate of RHD at the community level remains unknown. The current survey was undertaken to estimate its prevalence among schoolchildren of a central Ethiopian rural town (Butajira). Accordingly, 92.8% of the entire schoolchildren (n = 3.235) in the town were screened. Their mean age was 13.4 +/- 3.5 years. Fifteen children, accounting for a rate of 4.6/1,000, were noted to have findings consistent with RHD. Females and older children were more frequently affected. Only 2 of the 15 students were aware of their disease, and neither of them were on regular secondary prophylaxis. Our finding is consistent with the hospital-based reports that showed RHD to be a common disorder in clinical practice. At the same time, the lack of awareness about their disease noted among the cases is worrisome and deserves serious attention.

Biochemical effect of liver transplantation in two Swedish patients with familial amyloidotic polyneuropathy (FAP-met30).

Familial amyloidotic polyneuropathy (FAP) is an autosomal dominant inherited disorder characterized by progressive peripheral and autonomic neuropathy, associated with neural and systemic amyloid deposits. The amyloid fibrils contain a variant transthyretin (TTR) molecule (TTR met30), over 90% of which is produced in the liver. After liver transplantation in two patients with severe symptomatic FAP, only normal TTR was detectable in circulation. The two patients are being monitored at regular intervals, and, although in one patient there was no evidence of reduction in the quantity of amyloid present at 6 months, there had been no further progression of the neuropathy.

CSF monoamine metabolites, cholinesterases and lactate in the adult hydrocephalus syndrome (normal pressure hydrocephalus) related to CSF hydrodynamic parameters.

Monoamine metabolites, cholinesterases and lactic acid in lumbar cerebrospinal fluid (CSF) were investigated on patients with the adult hydrocephalus syndrome (idiopathic normal pressure syndrome; AHS, n = 15), Alzheimer's disease (AD, n = 14), multi-infarct dementia (MID, n = 13) and controls (n = 21). Patients had clinical and CSF hydrodynamic investigations. Monoamine concentrations were determined by reversed-phase liquid chromatography, cholinesterases and lactate were determined photometrically. In the AHS patients, CSF monoamine concentrations were not significantly different compared with controls, AD or MID patients. AHS and AD patients showed a similar reduction of CSF acetylcholinesterase activity compared with controls. Positive correlations were found in concentrations of CSF homovanillic acid, CSF 5-hydroxyindoleacetic acid and CSF lactic acid versus CSF outflow conductance (that is, resistance against CSF outflow) in the AHS patients. A similar pattern was observed in a subgroup of MID patients characterised by dilated ventricles and disturbed CSF hydrodynamics. These data suggest that a low CSF outflow conductance may facilitate the clearance of acidic substances from the arachnoid space at the probenecid sensitive active transport site. Alternative explanations would be that a pathologically low CSF outflow conductance is accompanied by an inverse caudorostral flow of CSF or a compromised trans-ependymal diffusion.

Attitudes of rural people in central Ethiopia towards epilepsy.

In the farming community of the sub-district of Meskan and Mareko in central Ethiopia, where the prevalence of epilepsy is known to be 5.2/1000, a door-to-door survey was undertaken in 1546 sampled households to find out public attitudes to epilepsy. Nearly 64% of the respondents were in the age group of 14-50 years, and 58.6% were women. The majority (86%) were illiterate, and 94% had incomes of a subsistence level; 89% had heard or witnessed seizures. Traditional views on the association of evil spirits and superstition was prevalent. By 45% of the interviewees, the disease was believed to be contagious through physical contacts during an attack. Although there was sympathetic concern in the community for the person suffering from epilepsy, negative attitudes were strong on matrimonial associations, sharing of accommodation and physical contacts with affected persons, particularly when there were obvious signs and frequent attacks by seizures. The study demonstrates that the rural community has very poor knowledge of the causes and nature of epilepsy, and this has resulted in social deprivations and at times, rejection of the sufferers.