CONVULSIVE DISORDER

. . LECTURE BY

H. IBNU MAS`UD

MEDICAL FACULTY BRAWIJAYA UNIVERSITY

MALANG 2008

CONVULSIVE DISORDER

HIPPOCRATES:

20 ABAD YG SILAM MELAPORKAN TENTANG PENYAKIT SERIUS ATAU INSIDENTAL SYMPTOM YANG SIFATNYA CURABLE DISEASE ie:

THE CAUSE OF TENDENCY IS STILL ENIGMATIC, AND PROBABLY RELATED WITH:

PREDISPOSED TO CONVULSIVE SEIZURE IS INFANTS DAN YOUNG CHILDREN, PARTICULARLY IN RELATION TO THE ONSET OF AN ACUTE FEBRILE ILLNESS IN RESPONSE TO SOME PROFOUND EMOTIONAL OR METABOLIC DISTURBANCES ABNORMAL FUNCTION OF THE BRAIN TISSUE, THAT WHICH IN EALRY LIFE AS
ITS LACK OF MYELIN ITS GREATER WATER CONTENT ITS RAPID METABOLISM

GREATER IRRITABILITY OF THE NERVOUS SYSTEM

IMMATURE DEVELOPMENT IN ASPECT OF THE FUNCTION PHYSIOLOGICAL HOMEOSTASIS (IS PROGRESSIVELY STABILIZED WITH INCREASING MATURITY

RELATED WITH IMMATURITY IN THE DEVELOPMENT OF THE CEREBRAL INHIBITORY MECHANISM

NEUROPHYSIOLOGY

CLASSIFICATION OF CONVULSIVE DISORDER

IN INFANCY AND CHILDHOOD ACCORDING TO

ETIOLOGY AND PATHOGENESIS CHARACTER OF THE CONVULSIVE ATTACK 9CLINICAL & EEG) ANATOMIC LOCATION OF THE POINT OF ORIGIN OF THE SEIZURE PATIENT`S AGE

CONVULSION ARE FAR MORE COMMON DURING THE FIRST TWO YEARS THAN AT ANY OTHER PERIODE OF LIFE

THE CAUSED ARE:

INTRACRANIAL BIRTH INJURY (INCLUDING THE EFFECTS OF ANOXIA AND HEMORRHAGE)

CONGINETAL DEFECTS OF THE BRAIN (MOST FREQUENTS IN VERY YOUNG INFANTS) ACUTE INFECTION ( INTRACRANIAL OR EXTRA CRANIAL).

MOST FREQUENT CAUSE IN INFANCY AND EARLY CHILDHOOD ( IN LATER CHILDHOOD IS CAUSED BY IDIOPATHIC EPILEPSY)

LESS FREQUENT CAUSES OF CONVULSIONS IN INFANTS ARE TETANY, TRUE EPILEPSY, SPONATNEOUS HYPOGLYCAEMIA, BRAIN TUMOR, RENAL INSUFFICIENCY, POISONING, ASPHYXIA, SPONTANEOUS HEMORRHAGE, POSTNATAL TRAUMA. THE CHIEF CAUSES OF CONVULSIVE SEIZURE IN MIDDLE AND LATER CHILDHOOD ARE :

INFECTION, IDIOPATHIC EPILEPSY, CONGINETAL DEFECTS OF THE BRAIN, RESIDUAL CEREBRAL DAMAGE FROM EARLY TRAUMA, LEAD POISONING, BRAIN TUMOR, ACUTE AND CHRONIC GLOMERULONEPHRITIS, DEGENERATIVE DISEASE OF THE BRAIN.

TETANY: IS A SYMPTOM COMPLEX OR SYNDROME AND THIS MANIFESTATION OF WHICH RESULT FROM A STATE OF INCREASED NEORMUSCULAR IRRITABILTY

TETANY CAN BE PRODUCE EXPERIMENTALLY IN ANIMAL BY IV ADMINSTRATION OF KALIUM PHOSPAHTE AND CALCIUM IS LOWERED AFTER INJECTION GARAM ALKALIN AND SALT

CEREBRAL PALSIES IS CAUSED BY LESSIONS OF VARIOUS PART OF THE BRAIN, FOR EXAMPLE: DISTURBANCES OF A MOTOR FUNCTIONS OF THE BRAIN, ARE DIVIDED IN THREE GENERAL GROUPS:

PHYSIOLOGICAL SEIZURE

1/. LESIONS OF THE MOTOR CORTEX ( RESULT SPASTICITY) 2/. LESIONS OF THE BASE OF THE BRAIN ( ATHETOSIS, TREMOR, SOME TIME RIGIDITY) 3/. LESIONS OF THE CEREBELLUM ( ATAXIA AND INCOORDINATION)

Difinition: paroxysmal events thought to represent abnormal electrical activity in cerebral neurons Pathogenesis: usually idiopathic

CEREBRAL PALSY

Definition inclusive term to designate a number of static neuromotor disorders due to a previous central motor system insult Pathogenesis often unknown prenatal insults: genetic, malnutrition, infection, anoxia, toxemia perinatal insults: prematurity, anoxia, hyperbilirubinemia, postnatal: infections, trauma, toxins CLASSIFICATIONS:

Spastic cerebral palsy

spastic quadriplegia: involves all 4 extremities spastic hemiplegia: involves both extremities on one side (upper greater than lower) spastic diplegia: involves all 4 extremities (lower greater than upper) spastic paraplegia: involves both legs with normal upper extremities

Athetoid cerebral palsy:

associated with lesions of the basal ganglia or uncontrolled and uncoordinated movements associated with a cerebellar lesion early hypotonia and decreased tendon reflexes

extrapyramidal tract

Ataxic cerebral palsy:

Mixed cerebral palsy

EEG PATTERN
EEG recording of a normal brain showing no unusual activity

http://www.epilepsyfoundation.org/answerplace/Medical/treatment/eeg.cfm

DEFINITIONS OF SEIZURE & ITS FEATURES

Seizure: a sudden attack, spasm, or convulsion, as in epilepsy or another disorder Epileptic seizure: a transient episode of abnormal and excessive neuronal activity in the brain that is apparent either to the subject or an observer. Epilepsy: a chronic disorder of the brain characterized by recurrent, unprovoked epileptic seizures. FEATURES OF EPILEPTIC SEIZURE ARE:

The abnormal neuronal activity during an epileptic seizure may be manifested as a motor, sensory, autonomic, cognitive, or psychic disturbance. The neurophysiological basis is inferred on clinical grounds. A convulsion is a subtype of seizure in which motor activity occurs. Can be provoked in individuals who do not have epilepsy

CLASSIFICATIONS OF EPILEPTIC SEIZURE Generalized seizures: the first clinical change indicates initial involvement of both cerebral hemispheres (left & right) Partial seizures: there is initial activation of pare of one cerebral hemisphere

There are many paroxysmal disturbances that mimic epileptic seizures.

For examples of provoking insults including fever,trauma, hypoglycaemia and hypoxia)

INTERNATIONAL CLASSIFICATION OF:

EPILEPTIC SEIZURES: Partial Seizures Simple Partial Complex Partial Simple or Complex Partial which generalize Sensory Motor Autonomic EPILEPTIC SEIZURE ~ GENERALIZED: Absence (typical and atypical) Myoclonic Tonic Clonic Atonic-astatic

Christopher M. Inglese, M.D. Regional Epilepsy Center St. Luke's Medical Center Milwaukee,Wisconsin

EPILEPTIC SEIZURE ~ UNCLASSIFIED:

Febrile Seizures Reflex Epilepsies Status Epilepticus

EEG PATTERN
EEG recording of an absence seizure showing the distinctive 3-per-second spike and wave discharge

http://www.epilepsyfoundation.org/answerplace/Medical/treatment/eeg.cfm

GENERALIZED OF SEIZURES

ABSENCE SEIZURE (PETIT MAL):

MYOCLONIC SEIZURE:

brief unawareness lasting a few seconds; no loss of posture; immediate recovery; may be very frequent; associated with automatisms. repaid, brief, usually isolated jerks of the limbs, neck or trunk. a generalised increase in tone

TONIC SEIZURES:

TONIC-CLONIC SEIZURES (GRAND MAL SEIZURE):

ATONIC SEIZURE (ASTATIC):

tonic phase of rigidity with loss of posture followed by clonic movements of all four limbs; loss of consciousness; duration 2-20minutes; postictal drowsiness. a trainsient loss of muscle tone caused a sudden fall to the floor or drop of the head.

GENERALIZED SEIZURE

PARTIAL SEIZURE

SIMPLE PARTIAL SEIZURE (FOCAL SEIZURES):

when the child will retain awareness with consciousness unpaired

COMPLEX PARTIAL SEIZURE:

when there is an altered conscious state or confusion due to the abnormal electrical discharge spreading from the originating site to become generalised when there is a focal seizure manifest clinically or on an ictal EEG followed by a generalised tonic clonic seizure. FEBRIL SEIZURES acute seizure associated with fever in the absence of intracranial infection or an identifiable neurological disorder EPILEPSY chronic disorder of the brain charaterized by recurrent, unprovoked epileptic seizures

PARTIAL SEIZURE WITH SECONDARY GENERALIZATION:

CAUSES OF EPILEPTIC SEIZURE

Absence seizures

http://professionals.epilepsy.com/page/generalized_absence.html

Adapted from: Holmes GL. Classification of seizures and the epilepsies. In: Schachter SC, Schomer DL, eds. The comprehensive evaluation and treatment of epilepsy. San Diego, CA: Academic Press; 1997. p. 1-36.

WHAT CAUSES A SEIZURE?

[K]o = extracellular potassium AHP = after hyper-polarization NMDA = N-methyl-D-aspartate IPSP = inhibitory post-synaptic potential EC = extracellular Interictal = between seizures

convulsion sudden, violent, involuntary contraction of the muscles of the body, often accompanied by loss of consciousness. It is not known what causes the abnormal impulses from the brain that result in convulsive seizures, since the disturbance may arise in normal brain tissue as well as in diseased or injured tissue. Convulsions may occur in such conditions as epilepsy, poisoning, high fever (especially in young children), disturbances of calcium or phosphorus metabolism, alkalosis, diabetes, oxygen insufficiency, and a low blood-sugar content, as well as in local irritation or injury of the brain. Persons undergoing convulsions should be guarded against self-injury (see epilepsy ). Otherwise, treatment must be directed to the underlying cause. (The Columbia Encyclopedia, Sixth Edition. Copyright 2008 Columbia University Press ) Convulsions are when a person's body shakes rapidly and uncontrollably. During convulsions, the person's muscles contract and relax repeatedly. FEBRILE convulsion is a common medical condition. About ... aged six months to six years have a convulsion when they have a fever or high temperature. And the brain is normal. That is mean: A febrile convulsion is a seizure occurring in a child aged from six months to six years, precipitated by a fever arising from infection outside the nervous system in a child who is otherwise neurologically normal.

CONVULSION

SEIZURE

Definition A seizure is a sudden change in behavior due to an excessive electrical activity in the brain. Seizure: A clinical event in which there is a sudden disturbance of neurological function in association with an abnormal or excessive neuronal discharge. (Lissauer, 2002). There are a wide variety of possible symptoms of seizures, depending on what parts of the brain are affected.

Many types of seizures cause loss of consciousness with twitching or shaking of the body. However, some seizures consist of staring spells that can easily go unnoticed. Occasionally, seizures can cause temporary abnormal sensations or visual disturbances.

Seizures can generally be classified as either "simple" (no change in level of consciousness) or "complex" (change in level of consciousness). Seizures may also be classified as generalized (whole body affected) or focal (only one part or side of the body is affected). Epilepsy is a chronic disorder with recurrent seizures. Some types of epilepsy are hereditary.

SEIZURE

Any medical condition that irritates brain cells may result in a seizure. Common medical conditions that commonly cause seizures include: Hypoglycemia (low blood sugar) Drug use (especially cocaine or stimulants) Alcohol withdrawal Very high fever (fever convulsions in children)

Febrile seizures are convulsions brought on by a fever in infants or small children. During a febrile seizure, a child often loses consciousness and shakes, moving limbs on both sides of the body. Less commonly, the child becomes rigid or has twitches in only a portion of the body. Most febrile seizures last a minute or two; some can be as brief as a few seconds, while others last for more than 15 minutes.

Febrile convulsions occur in young children when there is a rapid increase in their body temperature. It affects up to 1 in 20 children between the ages of one and four but can affect children between six months and about five years old. The child's risk of febrile convulsion rises if: they are genetically predisposed to it They suffer frequent illnesses, which include high temperatures The first attack of febrile convulsion was accompanied by a relatively low body temperature - below 39C. most febrile seizures (fever convulsion) are harmless and do not cause brain damage

FEBRILE SEIZURE

FEBRILE SEIZURE (F S):

the most common seizure disorder in children ( infancy & childhood)

associated with a rapidly rising temperature usually develop when the core temperature reaches 39 C or greater occur with the diseases out side the CNS and associated with fever age dependent: 3-6month ( peak age of onset:14-18 month of age) till 5 years of age With normal CNS structure and function (without evidence intracranial infection or defined cause) Without febrile seizure history Genetic predisposition

CLINICAL MANISFESTATION OF F S

Simple FS:

Occurs in the children: 3 month - 5 yr of age Typically generalized seizure (tonic-clonic) Duration: a few seconds to 10-min Only has once or twice of seizures during a period of disease

Atypical FS Age of seizure onset: < 6 mont or > 6 years Seizure persisting for more than 15 min Repeated convulsions during a febrile period A focal seizure. An EEG is indicated for atypical febrile seizures or for the child at risk for developing epilepsy

FEBRILE SEIZURE

Febrile convulsions are a common paediatric presentation to A&E departments, occurring in about 3% of children between the ages of six months and five years. The seizure usually occurs early on in a viral infection when the temperature is rising rapidly, and typically lasts less than five minutes. It is the abrupt rise in temperature rather than the high level that is important. The seizures are tonic or tonic-clonic, with loss of consciousness and muscular rigidity forming the tonic stage. This may be preceded by a frightened cry from the child. Cessation of respiratory movements and incontinence of urine and faeces may occur during this stage, which lasts about 30 seconds. The clonic stage that follows is characterised by repetitive movements of the limbs and face.

IS IT FS or EPILEPSY?

Febrile seizures are not classified as epilepsy, about 3% of children with FS go on to develop afebrile recurrent seizures ( epilepsy) Risk factors for epilepsy include: seizures that are focal, prolonged (>15mins) or recur in the same illness; first-degree relative with epilepsy; neurological abnormality Classification of epilepsies

According to the seizure type

Generalized epilepsies and syndromes Localization-related epilepsies and syndromes Idiopathic ( or primary) ---in which there is no apparent cause except perhaps for genetic predisposition Symptomatic---in which the cause is known or suspected

According to aetiology

SEIZURE

Seizures can generally be classified as either:

"simple" (no change in level of consciousness) or "complex" (change in level of consciousness). generalized (whole body affected) or Focal (only one part or side of the body is affected).

Seizures may also be classified as:

Epilepsy is a chronic disorder with recurrent seizures. Some types of epilepsy are hereditary. Causes of seizure: Any condition that results in abnormal electrical excitation of the brain may result in a seizure, including:

Epilepsy Injury or trauma to the head Infection (brain abscess, meningitis) Brain tumor Stroke

CAUSES OF EPILEPSY

Fever 36% Medication change 20% Unknown 9% Metabolic 8% Congenital 7% Anoxic 5% Other(trauma, vascular, infection, tumor, drugs) 15%
DeLorenzo RJ. Epilepsia 1992;33 Suppl 4:S15-25

Basal

nuclei: gray matter yg letaknya didalam (within) white matter td: Corpus striatum:
nucleus. Lentiform nucleus Functions: memperbaiki gerakan motor sadar menjadi lebih baik (in the refinement of voluntary movements.) Parkinsons: penyebabnya adalah degeneration of neurons yg menghubungkan substantia nigra ( midbrain) ke caudate nucleus. Hilangnya kendali GB terhadap aktivitas cortex motor yg berlebihan menyebabkan rentetan rangsngan pada bagian otot tubuh yg berlebihan dan timbul seizure Kejang sesuai rangsangan dari bagian cortex yg mana?kaki, lengan dan bisa menyeluruh.
F:doc.aging immunity/8brainM.ppt/Lt.ibnu/2005

BAGIAN DALAM OTAK:

BASAL NUCLEI

Caudate

MALIGNANT HYPERTHERMIA

Sensory input to the CNS comes from receptors of many different types situated throughout the body. From the sensory cortex to the motor cortex etc and produce a body movement. But the cortex motor can stimulate by some terrible stimulant ie: infection, fever or poison etc.

Stimulation by fever, poison, drugs or infection

Nervous system conveys highspeed electrical signals along neurons such rapid messages control the movement of body parts in response to sudden environmental changes Receptor (sensor) detects a stimulus and sends information to control center Control center = compares the incoming info to a set point (desired value) and sends out a signal that directs an effector to respond Example. ~ a change in blood calcium level
Repetitive stimulation from the motor cortex conveys more electrical signal to the part of body and make a convulsion. Why? Control center fail to regulate set point & signal that directs stimulation to the effector repetitively

Somatosensory and Motor cortex Both of their neurons are distributed according to the part of the body that generates the sensory input or receives the motor commands Primary Motor Cortex helps issue commands that consist of action potentials produced by neurons [located: rear of frontal lobe, adjacent to the primary somatosensory cortex] Action potentials travel along axons to brainstem & spinal cord excite motor neurons excite skeletal muscle cells What happen when stimulation repetitively & uncontrollable to the primary motor cortex?

Management of the fitting febrile child: Clothing should be removed and the child covered with a sheet. The child should be placed on its side, or prone with its head to one side, since vomiting with aspiration is a hazard.

Rectal diazepam is the drug of choice, producing an effective blood concentration of anticonvulsant within ten minutes.
All children with a first febrile convulsion should be admitted to hospital to a) exclude meningitis and b) educate the parents. A urine specimen should be taken to exclude infection, and a blood glucose level should be taken. A lumbar puncture may be performed if the child is less than eighteen months old shows signs of meningitis or sepsis. Treatment of the febrile child: Fever should be treated to promote the comfort of the child and to prevent dehydration. Paracetamol is the preferred anti-pyretic and fluid levels should be maintained. Ibuprofen can be given if the fever does not respond to paracetamol. Rectal diazepam should be administered as soon as possible after the start of the convulsion, and should not be given after the convulsion has stopped.