Abstracts from studies presented at the Brazilian Congress of Neurology 2000

Abstracts from studies presented at the Brazilian Congress of Neurology 2000


Prognosis of symptomatic epilepsies due to neurocysticercosis.

Paulo César Trevisol-Bittencourt
Alan Luiz Eckeli
Fabíola Dach
Felipe Eduardo Broering
Carlos Fernando Collares
Sílvia Kochen

Rationale. In our society about 80% of epilepsies are represented by symptomatic epilepsies and neurocysticercosis (NC) is the most frequent etiology. In the present study we evaluated the prognosis of epilepsies due to NC. Methods. The Multidisciplinary Epilepsy Clinic (MEC) is the only specialized out-patient clinic attending patients with epilepsy in our state, linked to the Brazilian public health service (SUS). There is a computerized attending protocol for the patients in the MEC and a data base has been created. Until now, 405 patients were randomly registered. In the present study we have accomplished an observational, descriptive, and transversal cut study based in this data bank, and it has been analyzed the following variables: remission epilepsy, AED withdrawal and seizures frequency before and after admission on the MEC. Results. The time of attendance was X +/-Y and the age of first seizure was 16,7±10,9 years old. We observed that, among these patients, 28,8% are in seizure remission and others 16,3% are in AED retreat. The frequency of partial seizures before the admission on the MEC was >1 seizure/month=65,1%, none or <1seizure/month=34,9%. After the admission on the MEC: >1 seizure/month=16,9%, none or <1 seizure/month=83,1%. About the frequency of the secondary generalized seizures before the attendance on the MEC: >1trimestral seizure=63,9%, none or <1 trimestral seizure=36,1%. And after: >1 trimestral seizure=31,3%, none or <1 trimestral seizure=68,7%. In this study only 1,2% of epilepsies due to NC are hardly controlled ones. Conclusions. Nowadays many doctors still believe in the classic aphorism: symptomatic epilepsy due to NC is easy to treat but very difficult to cure. In spite of that, our study showed that epilepsy secondary to NC overall has a very good prognosis. A clear tendency to go into remission was identified in the most patients of this sample.


CADASIL (Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalophaty) in a family from Santa Catarina, Southern Brazil.

Paulo César Trevisol-Bittencourt
Alan Luiz Eckeli
Fabíola Dach
Felipe Eduardo Broering
Carlos Fernando Collares
Salmo Raskin
Maria Verônica Muñoz Rojas
Hélio Afonso Ghizoni Teive

Objective. CADASIL is a vascular disease with autosomal dominant inheritance caused by a mutation of Notch3 gene on chromosome 19. The main symptoms are: migraine, depression, and progressive dementia due to multiple cerebral infarcts. Epileptic seizures may also occur. It usually starts on 4th decade of life. The classic vascular risk factors are absent. MRI on T2-WI shows hyper intensity areas more frequent in deep white matter. This report will describe members of a brazilian family with probable diagnosis of CADASIL. Methods. Clinical and neuroimaging (CT and MRI) exams were made in 3 members of a family of azorian origin, living at Santa Catarina State, Southern Brazil. The assessment of their mental status was by Mini-Mental State Examination. Results. Patient 1: female, 54 years-old, started with migraine in the adolescence time. When she was 40 years-old , she began with memory troubles and few years later transient ischemic attacks were noticed. During her 6th decade, asymmetric weakness and global cognitive dysfunctioning were obvious. Presently, she has severe dementia and epilepsy. Patient 2: female, 63 years-old, started with migraine during menopause. Despite of its slow progression, her clinical evolution has been similar to the patient 1, except for the epileptic seizures. Currently, she presents mild progressive dementia. Patient 3: male, 45 years-old, previously asymptomatic, except for very rare migraine attacks. Recent MRI exhibited frontal ischemic lesion. All patients had cerebral lesions in the white matter, and most of them were small subcortical infarcts. Conclusions. Doctors should pay attention to this cause of vascular dementia that will become even more important in the next few years. It must be suspected in all adult individuals suffering from ischemic cerebral disease associated with migraine and/or depression in the past. These peculiar patients usually do not have any vascular risk factors. Finally we would like to point out that cerebral angiography must not be performed in patients under suspicion of suffering from CADASIL. Nowadays, genetical analysis is already the best approach to confirm its diagnosis.


Features of juvenile myoclonic epilepsy in the State of Santa Catarina, Southern Brazil

Paulo César Trevisol-Bittencourt
Alan Luiz Eckeli
Fabíola Dach
Felipe Eduardo Broering
Carlos Fernando Collares

Rationale. Despite being juvenile myoclonic epilepsy (JME) a relatively common condition, it is usually not properly recognized in our society. Its world prevalence is roughly 3-12% of all epilepsies. Commonly, specialist doctors show difficulties to establish early diagnosis of this syndrome, probably due to the diverse seizure presentations observed on these peculiar patients and to the focal abnormalities occasionally seen in EEGs. We also describe the pattern of seizures in patients with JME. Methods. The Multidisciplinary Epilepsy Clinic (MEC) is the only specialized out-patient clinic attending patients with epilepsy in our state, linked to the brazilian public health service (SUS). There is a computerized attending protocol for the patients in the MEC and a data base has been created. Until now, 405 patients were randomly registered. In the present study we have accomplished an observational, descriptive, transversal study, and it has been analyzed the following variables: number of patients with JME, age of the first seizure and its classification, and the prescribed treatment before admission on the MEC. Results. Among 405 evaluated cases, we found 17 (4,1%) patients with the JME. The age of the first epileptic seizure was 11,8± 4,2 years old (SD). All of them presented myoclonic seizures. Absence seizures were present in 58,8% of the patients and 94,1% had tonic-clonic seizures. Before admission on MEC, 40% had been treated with phenobarbital (PB), 24% with carbamazepine, 8% with phenytoin, 4% with clonazepan, 4% with diazepam and 20% were treated with diverse politherapy regimens, in which PB was the common drug. Conclusions. Many general practitioners and some neurologists as well have certain difficulty in the early diagnosis of this condition, as we can notice by the improper treatment prescribed for typical Janz syndrome.


Epileptic seizures precipitating factors

Paulo César Trevisol-Bittencourt
Alan Luiz Eckeli
Fabíola Dach
Felipe Eduardo Broering
Carlos Fernando Collares

Rationale. Epileptic patients not rarely complain about seizure precipitating factors. Some of them are quite curious. We observed which are the most common precipitating factors related by the patients, connecting them with seizure type and epileptic syndrome presented by patients attending a multidisciplinary epilepsy clinic. Methods. The Multidisciplinary Epilepsy Clinic (MEC) is the only specialized out-patient clinic attending patients with epilepsy in our state, linked to the brazilian public health service (SUS). There is a computerized attending protocol for the patients in the MEC and a data base has been created. Until now, 405 patients were randomly registered. In the present study we have accomplished an observational, descriptive, transversal study, and it has been analyzed the following variables: seizure precipitating factors occurrence, epilepsy etiologic classification and kind of precipitating factor. Results. In the 405 analyzed cases, 73 (18%) reported to have a kind of seizure precipitating factor. Among them, 42 patients had symptomatic epilepsy (57,5%), 20 had idiopathic epilepsy (27,3%) and 11 had cryptogenic epilepsy (15%). It was found out as seizure precipitating factors: anxiety, menstruation, alcohol, headache, full moon, feeling of extreme happiness and contraceptive drugs. Conclusions. All the seizure precipitating factors are related by the patients own words, and should be interpreted with caution, but must not be underestimated or be a motive to jocosity by the health personnel. Undoubtedly, while some of them belong to epileptic syndrome as their simple partial component, others are well known precipitating factors in susceptible individuals. Consequently, essential information for a better clinical approach to the epileptic patient can be obtained by asking this simple question.


Number of hospitalizations before and after admission in a multidisciplinary clinic of epilepsy

Paulo César Trevisol-Bittencourt
Alan Luiz Eckeli
Fabíola Dach
Felipe Eduardo Broering
Carlos Fernando Collares

Rationale. Many patients suffering from epilepsies are frequently and unnecessarily put in hospital care, increasing the financial expenses in public health care. The aim of this study was to evaluate the number of hospitalizations among epileptic patients due to occasional seizures, status epilepticus or “epileptic psychosis” before and after attending a multidisciplinary epilepsy team. Methods. The Multidisciplinary Epilepsy Clinic (MEC) is the only specialized out-patient clinic attending patients with epilepsy in our state, linked to the brazilian public health service (SUS). There is a computerized attending protocol for the patients in the MEC and a data base has been created. Until now, 405 patients were randomly registered. In the present study we have accomplished an observational, descriptive, transversal study, and it has been analyzed the following variables: number of hospitalizations in general hospitals, due to status epilepticus or unique seizures, and psychiatric institutions, due to “epileptic psychosis”, before and after the admission on the MEC. Results. In the 405 investigated individuals, 184 had been admitted in general hospitals, resulting in 465 hospitalizations. After the admission on the MEC, the number of hospitalized patients had decreased to 22 in 31 occurrences. Concerning psychiatric institutions, 17 patients were hospitalized in 17 occasions before the first assessment on the MEC. After the admission on the MEC it decreased to 6 patients each of them with only one hospitalization. Conclusions. It is possible to infer that a multidisciplinary approach of people suffering from epilepsy may lead to an expressive reduction in the number of unnecessary hospitalizations, either in general or psychiatric hospitals. The diagnostic accuracy results in lower financial expenses in public health care, better treatment efficacy and, in addition, an important improvement on the patients quality of life could be expected.


Partial seizures and the diagnosis of epilepsy

Paulo César Trevisol-Bittencourt
Alan Luiz Eckeli
Fabíola Dach
Felipe Eduardo Broering
Carlos Fernando Collares

Rationale. Frequently the epilepsy diagnosis is only considered when a “grand mal” fit happens. However, partial seizures are the main manifestation of epilepsy and not rarely are the only seizure type. In this study, patients from a multidisciplinary epilepsy clinic were assessed considering the prevalence of partial seizures and identifying which were the most common. Methods. The Multidisciplinary Epilepsy Clinic (MEC) is the only specialized out-patient clinic attending patients with epilepsy in our state, linked to the brazilian public health service (SUS). There is a computerized attending protocol for the patients in the MEC and a data base has been created. Until now, 405 patients were randomly registered. In the present study we have accomplished an observational, descriptive, transversal study, and it has been analyzed the following variables: prevalence and type of partial seizures. Results. Among the 405 analyzed cases, 314 (77,5%) present at least one type of partial seizure. In this sample, 245 (78%) have simple partial seizure (motor 37,1%; psychic 30,6%; autonomic 21,6%; sensitive 18% e another types 9,8%). Complex partial seizures occur in 189 patients (60,1%) (72% with automatism). Partial seizures presenting secondary generalization occurred in 308 patients (98,1%). Conclusions. Despite what many people can think, including health professionals, partial seizures are an important manifestation of epilepsy. Even considering that almost all patients in the sample have secondary tonic-clonic fits, it should be emphasized that partial seizures are very prevalent. Not only can it be the first manifestation, but it can also be the only kind of fit during many years of follow up. Therefore, the diagnosis of epilepsy should not wait for the occurrence of a “grand mal” seizure in order to be established.


Adverse effects of chronic phenobarbital usage

Paulo César Trevisol-Bittencourt
Alan Luiz Eckeli
Fabíola Dach
Felipe Eduardo Broering
Carlos Fernando Collares

Objective. Phenobarbital (PB) is a well known anti-epileptic drug (AED). However, it has undesirable effects as any other drug. Behavioral disturbances are found frequently among its users. Besides, learning difficulties seem to be equally common. Methods. The Multidisciplinary Epilepsy Clinic (MEC) is the only specialized out-patient clinic attending patients with epilepsy in our state, linked to the brazilian public health service (SUS). There is a computerized attending protocol for the patients in the MEC and a data base has been created. Until now, 405 patients were randomly registered. In the present study we have accomplished an observational, descriptive, transversal study, and it has been analyzed the following variables: usage of PB in monotherapy regimen and the main cognitive and behavioral side effects referred by patients and relatives as well. Results: Among 352 patients using PB as their single AED, 51,08% showed evidences of cognitive dysfunction. This expressive number had demonstrated sensible decay in scholarship grades or had failed. In addition, pathological irritability has been detected in approximately half of the sample and 35,29% related drowsiness as the main adverse effect of PB. Conclusions. PB is an AED of indubitable efficacy , however its frequent side effects are unfortunately neglected , leading to an overestimation of its benefits and to an underestimation of its toxicity. In our society the usage of PB as the first choice AED should be reconsidered. Apparently children are more vulnerable to cognitive and behavioral effects, therefore they should be spared from PB prescription, at least, as the first therapeutic option.


An unusual familial progressive myoclonic epilepsy starting with late onset: a new condition?

Paulo César Trevisol-Bittencourt
Alan Luiz Eckeli
Fabíola Dach
Felipe Eduardo Broering
Carlos Fernando Collares
Luiz Carlos Coral
Maria Verônica Muñoz Rojas
João Harold Bertelli

Rationale. To describe an unusual myoclonic progressive familial epilepsy affecting three brothers of a brazilian family with german origin living in the state of Santa Catarina, southern Brazil. Case Reports. The family is composed by eleven brothers, three of them, a woman and two men, had initiated in adult age, respectively at 28, 34 and 37 years old, with identical symptomathology presenting minor myoclonic jerks, mainly in superiors members, frequently atypical absence seizures, and rare tonic-clonic seizures episodes. All patients had responded to treatment with valproic acid associated with clonazepam, however a complete remission was not obtained in any of them. The female patient had presented absence-like epilepticus status associated to minor myoclonic jerks in both arms. There are some evidences that this condition had uninterruptedly affected her for many years. It had been noticed a moderate cognitive dysfunction as well, with a progressive deterioration tendency in all of them. None of the patients had presented miopathy symptoms, neither another movement disorders besides minor myoclonic jerks were found. A plenty of electroencephalografic studies were made, showing a similar presentation represented by generalized discharges of spikes and polispikes slow spike- wave complex. In all of them the neuroimaging studies CT and MRI were normal. Discussion/ Conclusion. These patients had initiated in adult age a unique form of myoclonic epilepsy. The peculiarities of this familial condition do not have any similarity in previously reporting. Despite the fact that they have been responsive to appropriated anti-epileptic therapy, a complete seizure control has not been obtained yet. Besides, it is observed a progressive cognitive deterioration; possibly due to aberrant persistent cerebral electrical activity. Genetic studies have been carried out to define their diagnosis.


Partial epilepsy associated with extensive intracranial calcifications

Paulo César Trevisol-Bittencourt
Alan Luiz Eckeli
Fabíola Dach
Felipe Eduardo Broering
Carlos Fernando Collares
Daniel Chaves

Rationale. To describe the clinical features of a patient with extensive intracranial calcifications seen in routine computerized tomography. Case Report. IJG, 27 years old, male, azorian origin, bricklayer, had initiated with partial epilepsy at 12 years old. Frequently he had partial complex seizures with automatisms and almost every times preceded by a “ghost vision”. Occasionally he presented secondary tonic-clonic seizures as well. There is report of neonatal hypoxia. Familial history is only about a distant cousin with epilepsy. He received bizarre anti-epileptic polypharmacy in the past with no adequate seizure control. Since 10 years ago a better control was achieved using a major anti-epileptic drug in monotherapy regimen. This patient has been able to work and get active social life. The electroencephalografic study demonstrated bilateral temporal irritative activity. The CT had demonstrated an extensive bizarre calcification presentation involving mainly basal ganglia, cortex and cerebellum. Investigations for Fahr´s Disease and hypoparatyreoidism were negatives. Nowadays he has sporadic seizures, only when spontaneously discontinues the medication. Discussion/ Conclusion. Until now, IJG presents an unknown etiologic diagnosis. An initial investigation for Fahr´s Disease and hypoparatyreoidism is negative. Despite his seizures are been relatively controlled the patient still has been submitted to diagnosis tests.


Alternative treatments for epilepsy in Southern Brazil

Paulo César Trevisol-Bittencourt, Márcio Alcides Molinari, Alan Luiz Eckeli, Fabíola Dach, Felipe Eduardo Broering, Carlos Fernando Collares

Rationale. The present study aims to identify the alternative therapies used in our society for the treatment of patients suffering from epilepsy. Methods. The Multidisciplinary Epilepsy Clinic (MEC) is the only specialized out-patient clinic attending patients with epilepsy in our state, linked to the brazilian public health service (SUS). There is a computerized attending protocol for the patients in the MEC and a data base has been created. Until now, 405 patients were randomly registered. In the present study we have accomplished an observational, descriptive, transversal study, and it has been analyzed the following variables: kind of professional and alternative therapies practiced. Results. Among 405 patients, there were found 205 (50,5%) looked for non-medical professional aid, and have used diverse types of treatments (exorcism, spiritual surgery, skunk oil, witchcraft, hand healing, etc.) in order to achieve cure. Conclusions. Nowadays, it can be observed that various non-orthodoxical therapies are still used many times by layman professionals intentioning to deceived these persons. This kind of practice not only harms the wallet, but could also cause serious physical and psychological damage – many times, irreversibly. In this situations, information in overdoses seems to be the only remedy that we can count on. Finally we would like to point out that specialized clinics are essential to avoid this tragic reality.