PADACZKA - INFORMACJE OGÓLNE
Definicje napadów
Napady częściowe
Napady częściowe (napady o początku ogniskowym) - występują wówczas, gdy wyładowanie napadowe pojawia się w określonej okolicy mózgu i - przynajmniej początkowo - ogranicza się tylko do tej okolicy. W tej grupie rozróżnia się:
Napady częściowe z objawami prostymi w czasie których chory zwykle nie traci świadomości. W zależności od struktur mózgowych objętych wyładowaniem napadowym wyróżnia się napady:
Napady ruchowe (niekiedy sprowadzające się zaledwie do drgań kciuka i/lub kąta ust, czasem bardziej skomplikowane - ze zwrotem gałek ocznych i głowy do boku, lub bardzo rzadko, innymi specyficznymi zaburzeniami ruchowymi.np. mowy);
Napady częściowe ruchowe proste (napady Jacksona) Ognisko padaczkowe znajduje się w tym przypadku w okolicy ruchowej płata czołowego mózgu. Napad zaczyna się zazwyczaj rytmicznym skurczem kciuka, palca wskazującego, kącika ust lub palucha po przeciwnej stronie ciała w stosunku do umiejscowienia ogniska. W trakcie trwania ataku drgawki pogłębiają się i zaczynają obejmować inne grupy mięśni. Mogą one zająć drugą kończynę po tej samej stronie, potem stać się obustronne i napad spowodować może utratę przytomności (uogólnienie napadu). Często może wystąpić po napadzie osłabienie mięśni biorących udział w ataku, które to trwać może do dwóch dni.
Napady zmysłowe polegające na rozmaitych doznaniach wzrokowych, słuchowych, smakowych, węchowych lub czuciowych; mają one zwykle dość charakterystyczny, stereotypowy przebieg)
Napady czuciowe proste Charakteryzują się one napadami halucynacji dowolnych rodzajów czucia. Zazwyczaj jest to drętwienie lub mrowienie pewnych części ciała. Także taki napad może przejść w atak Jacksonowski lub drgawki uogólnione.
Napady wegetatywne z takimi objawami ze strony układu wegetatywnego jak nagle występujące nadmierne wydzielanie śliny, ostre bóle brzucha u małych dzieci, itp.;
Napady mieszane tj. składające się z objawów zaliczonych do dwu lub trzech powyższych podgrup.
Napady częściowe z objawami złożonymi. W przeciwieństwie do poprzednich napady te przebiegają zwykle z zaburzeniem świadomości, choć nierzadko tylko z jej przymgleniem. Oznacza to, że pacjent w czasie trwania napadu może częściowo odbierać i zapamiętywać bodźce płynące z otoczenia, a nawet reagować na nie w sposób zupełnie właściwy. Te napady cechują się szczególnym bogactwem objawów; ich źródłem są zwykle nadmierne wyładowania bioelektryczne, pojawiające się w płacie skroniowym (stąd potoczna nazwa "napady skroniowe"). Wśród najczęściej spotykanych postaci tych napadów należy wymienić:
Napady intelektualne - przebiegają one wśród doznań marzeniowych, snopodobnych, z poczuciem czegoś już przeżytego, znanego, lub też odwrotnie - dobrze znane sytuacje czy otoczenie (np. własny pokój) wydają się zupełnie nowe, nieznane; nierzadko są to jakby napływy niezwykle żywych wspomnień z dawnych lat;
Napady z zaburzeniami psychosensorycznymi, tj. z rozmaitego rodzaju zaburzeniami widzenia, słuchu, orientacji przestrzennej, co do własnego ciała itp.;
Napady z zaburzeniami psychoruchowymi tj. automatyzmami ruchowymi, polegającymi zwykle na stereotypowym wykonywaniu dość prostych czynności (np. ruchy żucia, odpinanie i zapinanie guzików, szukanie czegoś w torebce lub kieszeni itp.);
Napady mieszane, na które składają się objawy z różnych podgrup napadów.
Napady częściowe złożone (padaczka skroniowa) Napady te są efektem wyładowań w płacie skroniowym mózgu. Przybierają one różne postacie. Mogą to być omamy węchowe, smakowe, urojenia wzrokowe czy słuchowe. Pojawiać się może zjawisko deja vu - choremu wydaje się, że coś, co dzieje się w rzeczywistości pierwszy raz, on już wcześniej doświadczał. Padaczka skroniowa objawiać się może atakami wspomnień z przeszłości lub silnymi, nieprzyjemnymi doznaniami emocjonalnymi. Pojawiają się także zróżnicowane objawy ruchowe, czasem przechodzące w uogólnione drgawki.
Schemat podziału napadów częściowych i uogólnionych
"On the Sacred Disease" – O świetej chorobie kilka zdań: tłumaczenie z oryginału na język angielski
"It is thus with regard to the disease called sacred: it appears to me to be in no way more divine nor more sacred than other diseases.The brain is the cause of this affliction.When the [surplus] phlegm [from the brain] runs down through the veins, the patient loses his speech and foams at the mouth, his hands are contracted, the eyes contorted, he becomes insensible, and in some cases the bowels are emptied. The patient kicks with his feet. The patient must endure all these symptoms when the cold phlegm flows into the warm blood."Medycyna rzymska: Galen (129 - ok. 200): "On diseased parts of the body"
Według Galena istnieja trzy formy padaczki:
"In all forms it is the brain which is diseased; either the sickness originates in the brain itself, or it rises in sympathy into the brain from the cardiac orifice of the stomach. Seldom, however, it can have its origin in any part of the body and then rises to the head in a way which the patient can feel.
Case description: I heard the boy say that his condition began in his lower leg and then moved up through the thigh, the groin and side of the chest above the affected thigh up to the neck and then to the head. As soon as [the condition] reached this part, he said that he was no longer aware of himself. When the doctors asked what the movement into the head was like, [another] boy said the movement upwards was like a cold breeze (aura)."Medycyna Bizancjum: Alexandros of Tralleis (ok. 525 - 605):
Z dzieła "12 ksiąg o medycynie" - (Volume I, Chapter 15)
"The proof that epilepsy originates in the stomach lies in the fact that a feeling of restlessness and gnawing begins in the stomach and then the patient feels the affliction approaching.
As soon as the patient gets up in the morning and has emptied his bowels, he should drink an infusion of hyssop, which will do him a lot of good, as many have been healed simply by drinking this, and were only taken ill two or three times.
It is forbidden to drink undiluted wine after taking a bath as nothing can set off a seizure more easily than this - and indeed undiluted wine is in general dangerous for all epileptics."Medycyna arabska: Avicenna (980 - 1037):
Z dzieła "Canon medicinae" (The Canon of Medicine)"Epilepsy is a disease which prevents those organs affected from using the senses, moving and walking upright.And this is caused by a blockage. Usually it is a general seizure, caused by some damage, which affects the front cerebral ventricle; and it is impossible for the person affected to remain standing upright. "Wczesne Średniowiecze - okres mroczny dla medycyny a dla chorych na choroby psychiczne i padaczkę szczególnie ciężki
Ilustracja z 1250 przedstawia Świętego Walentego leczącego chorego na padaczkęMedycyna Śtredniowiecza:
Modlitwa chorego na padaczke Falling Sickness Blessing (14th/15th century): "As convulse and bewitch are walking across the heath,
they meet the Holy Virgin Mary.
the Virgin Mary asks convulse and bewitch:
'convulse and bewitch, where are you going?'
convulse and bewitch say: 'We are going to him and him.'
The Virgin Mary asks: 'What are you going to do there?'
convulse and bewitch say
'We're going to tear flesh, drink blood and break legs.'
The Virgin Mary says: 'You must not do that:
you must go where there are bare rocks,
there you can tear flesh, drink blood and break legs.'
May god the father, god the son and god the holy ghost help us. Amen."Renesans (od około roku 1500):
The Renaissance physician (from around 1500) gradually rejects the medical beliefs of the Middle Ages, which were influenced by the Christian faith and by superstition; he begins to make tentative steps towards practising natural "scientific" medicine. One of the most famous and influential Renaissance physicians was Paracelcus, who devoted much time to the study of the "falling sickness", epilepsy. The painting shows the Renaissance physician fighting death and his companions-diseases (depicted as black birds) with "new weapons": with rational (scientific) thought (symbolised by the owl); mwith the experience and knowledge of ancient Greek and Roman physicians (symbolised by books); with natural, specially prepared substances (symbolised by the mortar and pestle); with chemical-physical experiments (symbolised by chemical apparatus)."Lord, have mercy on my son," he said. "He has seizures and is suffering greatly. He often falls into the fire or into the water."
Presents Christ driving out, with a gesture of blessing, an evil spirit that has attacked a young man. The fact that this exorcism was actually successful is represented by the demon with dragon wings, tail and claws that is seen escaping upwards from out of the patient’s mount.
Miniatura (foliał 166) z książki "Les Tr?s Riches Heures" autor Johann von Frankreich (Duc de Berry, 1340 – 1416)In the Gospels: Matthew, Chapter 17 (verse 1 ff.); Mark Chapter 9 (verse 2 ff.) and likewise Luke, Chapter 9 (verse 28 ff.), the Transfiguration of Christ is described. All three evangelists mentioned, in the same chapter, follow the description of this transfiguration with the story of the healing of the "moonstruck" (namely epileptic) boy. One of the most important Renaissance painters, Raffaelo Santi (generally Raphael, 1483 - 1520), integrated the Transfiguration and the healing episode into one great masterpiece. Of the Transfiguration-themed paintings, his, in which an epileptic person is portrayed, is the most famous, most important, and probably also the one that makes the most impression. The painting, which is 405 x 278 cm in size and was the last to be painted by Raphael (1519/20), shows the Transfiguration of Christ (on Mount Tabor) in the upper half. The lower half portrays a desperate father (on the right side of the picture, dressed in green) bringing his moonstruck (epileptic) son to the disciples of Christ and begging them to help the child, who during his seizures, "often falls into the fire or into the water". "But" as it is then told by Mark, "they did not have the power to do it." It is only when Christ comes there later that he heals the boy by driving out a demon
Medycyna okresu Renesansu: Paracelcus (1493 - 1541):
On ailments which rob us of our reason (1525)
"'And such falling sicknesses have five seats: One is in the brain, the second in the liver, the third in the heart, the fourth in the intestines, the fifth in the limbs. And this is not only so in human beings but also in every living creature, in animals, which also fall down in the same form as in humans, and the earthquake also has the same origin as the falling sickness.
We say that it is impossible to cure the root of the disease, but that it is possible to prevent the root from growing.Medycyna XVIII wieku: Samuel Auguste A. D. Tissot (1728 - 1797):
Treatise on Epilepsy or the Falling Sickness (1771)
"In order to be in a position to cure this disease, one must first take pains to examine whether there is any sympathetic cause which supports it, and what this could be; or whether it is an idiopathic one, that is to say whether it simply stems from an over-sensitivity of the brain.At last, valerian has fortunately become the favourite remedy of all sensible physicians. I am convinced that, if this does not have an effect, then it is because the malady is incurable."Medycyna XIX i XX wieku: John Hughlings Jackson (1835 - 1911): A Study of Convulsions
"The fit usually begins, it is to be observed, in that part of the face, of the arm, and oft the leg, which has the most varied uses.
The fits which begin in the hand begin usually in the index finger and thumb; fits which begin in the foot begin usually in the great toe. It may be that the order of frequency mentioned point merely to an order of frequency in liability of parts to become diseased.Parts which have the most varied uses will be represented in the central nervous system by most ganglion cells."
In 1904, the term "epileptologist" was first used to describe a person who specializes in epilepsy. William Spratling, the neurologist who coined the word, is now regarded as North America's first epileptologist.
In 1912, two groups of chemists on their own created Phenobarbital under the name of Luminal. Phenobarbital is the oldest AED in common clinical use.In 1920, the ketogenic diet is one of the oldest forms of treatment for epilepsy. Created in the 1920s when there were few effective treatments for epilepsy, this special diet, which is high in fat, low in protein, and has negligible amounts of carbohydrate, was created to simulate some of the metabolic effects of fasting, a state known to decrease seizures in some individuals.
In 1929, a German psychiatrist named Hans Berger announced to the world that it was possible to record electric currents generated on the brain, without opening the skull, and to illustrate them graphically onto a strip of paper. Berger named this new form of recording as the electroencephalogram (EEG). In 1953, Schindler at Geigy synthesized Carbamazepine (CBZ) in an effort to try to compete with the new introduced antipsychotic, chlorpromazine. Over the years, CBZ has gained acceptance as a first-line treatment for partial and tonic-clonic seizures.
In 1958, Ethosuximide (ESM) was introduced as an AED and has been the drug of choice for children with absence seizures who do not also have tonic-clonic or myoclonic seizures. ESM is also effective for atypical absence seizures.
In 1963, Sodium Valproate (VPA) anticonvulsant property was recognized serendipitously when it was used by Pierre Eymard as a solvent for a number of other compounds. VPA is effective over the complete range of seizures.
In 1968, The Epilepsy Foundation is a national, charitable organization, founded in 1968 as the Epilepsy Foundation of America. The only such organization devoted to the well-being of people with epilepsy, there objective is to work for children and adults affected by seizures through research, education, advocacy and service.There national office is in Landover, MD, a suburb of Washington, D.C. More than 60 affiliated Epilepsy Foundations serve people with seizures, and their families, in hundreds of communities nationwide. A volunteer board of directors governs their work; a distinguished board of physicians and scientists oversees the scientific and medical programs. The Foundation is a member of the National Health Council and the International Bureau for Epilepsy. The organization is supported almost entirely by private donations. In 1970, the Veterans Administration spearheads a movement toward creating epilepsy centers, introducing a new breed of neurologists who began to specialize in the treatment and research of epilepsy.
In 1990, even in the twentieth century, some U.S. states had laws forbidding people with epilepsy to marry or become parents, and some states permitted sterilization. To establish a clear and comprehensive prohibition of discrimination based on disability, Congress passed the American’s Disability Act of 1990.
In 1993, Felbatol (felbamate) and Neurontin (gabapentin) are FDA approved. The first new epilepsy drug treatment in more than 10 years received FDA approval July 29.
In 1994, Lamictal (lamotrigine) is FDA approved. The FDA has approved Lamictal Tablets (generic: lamotrigine) as an additional therapy for partial seizures in children aged two and up. This expands the already approved use in adults with partial seizures and for the generalized seizures of Lennox-Gastaut syndrome in children.
In 1996, the FDA approves Topamax (topiramate).
In 1997, FDA approves Gabitril (Tiagabine). In 1997, The U.S. Food and Drug Administration (FDA) approved vagus nerve stimulation in combination with seizure medication for partial epilepsy in adults.
In 1999, FDA approves Keppra (levetiracetam). The FDA approved Keppra for use in adults as a secondary treatment for epilepsy in 1999. A new study presented today at the Child Neurology Society meeting suggests that Keppra may also be effective as a first-line treatment for children who do not respond to traditional therapies.
In 2000, Trileptal (Oxcarbazepine) and Zonegran are approved by the FDA.
Also in 2000, A landmark conference, "Curing Epilepsy: The Promise and the Challenge," organized by the Epilepsy Foundation of America, sets confident goals for future treatment as well as prevention and cure of epilepsy; no seizures or side effects for those with the disorder; and discovering ways to stop epilepsy caused from injury, infection, or errors of development. There is no other disorder or disease that has been given so many different names in the course of history as epilepsy. From this, we can conclude that throughout the ages people have been preoccupied with this disease. There are two main reasons for this interest: First, epilepsy has always been a common disorder: zero, 5-1% of all people has been diagnosed with it.
Polski wkład do medycyny w diagnostyce padaczki i neurofizjologii w załączonej prezentacji dr Ludmiły Czarkwiani i profesora Tadeusza Baci: „Miejsce neurofizjologii klinicznej w naukach medycznych w praktyce klinicznej.”
FARMAKOTERAPIA PADACZKI
Rycina 1 i 2. Historyczne ujęcie wprowadzanie kolejnych leków przeciwpadaczkowych
Rycina 3 i 4 Budowa chemiczna powszechnie znanych leków przeciwpadaczkowych
Rycina 5. Budowa chemiczna nowych leków przeciwpadaczkowych.
Rycina 6 Schemat ustalania się poziomu terapeutycznego w surowicy krwi.
Podstawowe informacje w angielskojęzycznej prezentacji An Introduction to Epilepsy » Neuropharmacology of Antiepileptic Drugs