Paulus Jonathan

Pemicu 3

Adams & Victor’s Principles of Neurology, 9th Edition

• Definitions:– Seizures: an excessive and disorderly discharge

of cerebral nervous tissue on muscles– Epilepsy: a condition of recurrent unprovoked

seizures (≈seizure; lay term)– Convulsion: an intense paroxysm of involuntary

repetitive muscular contractions– Status epilepticus: convulsive seizures

prolonged or repeated every few minutes

Seizures

Epidemiology

Clinical Neurology 7ed

Etiology

EtiologyPrimary Neurologic Disorder Systemic Disorder

Benign febrile convulsions of childhood Hypoglycemia

Idiopathic / cryptogenic seizure Hyponatremia

Cerebral dysgenesis Hyperosmolar states

Symptomatic epilepsy Hypocalcemia

Head trauma Uremia

Stroke or vascular malformation Hepatic encephalopathy

Mass lesions Porphyria

CNS infections Drug toxicity, drug withdrawal

Encephalitis, meningitis Global cerebral ischemia

Cysticercosis Hypertensive encephalopathy

HIV encephalopathy Pre-eclampsia, eclampsia

PERBEDAAN ANTARA KEJANG & SERANGAN yg MENYERUPAI KEJANGKEADAAN KEJANG MENYERUPAI KEJANG

Onset Tiba2 Mungkin gradual

Lama serangan Detik atau menit Beberapa menit

Kesadaran Sering terganggu Jarang terganggu

Sianosis Sering Jarang

Gerakan ekstremitas Sinkron Asinkron

Stereotipik gerakan Selalu Jarang

Lidah tergigit atau luka lain Sering Sangat jarang

Gerakan abnormal bola mata

Selalu Jarang

Fleksi pasif ekstremitas Gerakan tetap ada Gerakan hilang

Dapat diprovokasi Jarang Hampir selalu

Tahanan thp gerakan pasif Jarang Selalu

Bingung paska serangan Hampir selalu Tidak pernah

Iktal EEG abnormal Selalu Hampir tidak pernah

Paska iktal EEG abnormal Selalu Jarang

Potensial Pascasinaptik• 3 jenis neurotransmiter :

– Eksitatori/E (glutamat & aspartat,glisin) : • Depolarisasi (dlm sel paskasinaptik [+] atau

kurang negatif) membran paskasinaptik (mdekati threshold)

• Potensial paskasinapstik yg didepolarisasi EPSP (excitatory postsynaptic potential)

– Inhibitori/I (GABA) :• Hiperpolarisasi (dlm sel paskasinaptik [-]) pd

membran paskasinaptik• Potensial paskasinapstik yg dihiperpolarisasi

IPSP (inhibitory postsynaptic potential)- Eksitatori & inhibitori asetikolin

Impuls saraf sampai di synaptic end bulb dr akson presinaptik↓

Voltage-gated Ca2+ channel tbuka (fase depolarisasi impuls saraf)↓

[ ] Ca2+ ↑ (neuron prasinaptik)↓

Eksositosis vesikel sinaps (neurotransmiter)↓

Dikeluarkan ke dalam synaptic cleft (difusi)↓

Ikat reseptor neurotransmiter (ionotropik atau metabotropik)↓

Ligand-gated channel tbuka↓

Ion2 mengalir ke membran (potensial paskasinaptik)↓

Depolarisasi atau hiperpolarisasi

• E > I EPSP # capai threshold impuls saraf (depolarisasi)

• I > E hiperpolarisasi (IPSP) inhibisi neuron paskasinaptik & # impuls saraf

PATOFISIOLOGI

Paroxysmal depolarization shift (PDS)

Depolarisasi potensial pasca sinap berlangsung lama

Muatan listrik yang berlebihan pada neuron – neuron

Merangsang sel neuron lain melepaskan muatan listriknya

- Gelombang paku iktal pada EEG- Gejala klinis: tergantung luas sel neuron yang tereksitasi

1. Kemampuan membran sel sebagai pacemaker neuron untuk melepaskan muatan listrik yang berlebihan

2. Berkurangnya inhibisi untuk neurotransmiter asan gama amino butirat (GABA)

3. Meningkatnya eksitasi sinaptik oleh transmiter asam glutamat dan aspartat melalui jalur eksitasi yang berulang

Pathophysiology•Abnormal structure•Disruptions of focal metabolic or biochemical function

↑ electrical activity

An ictrogenic focus

Sensitive neurons(acetylcholine, GABA)

Produce sustained membrane depolarization

Local hyperpolarization and recruitment

Deeper and cross the midline

KLASIFIKASI KEJANG• KEJANG PARSIAL

– sederhana– Kompleks (kejang

lobus temporal / psikomotor)

– Parsial berubah menjadi generalisata

• KEJANG GENERALISATA (umum)– Tonik-klonik /grand mal– Absence / petit mal– Mioklonik– Atonik– Klonik– Tonik

Seizure types & characteristics

No Syndrome Age of Onset Seizure Type

Associated Findings

EEG Findings

Treatment

1 Lennox-Gastaut Syndrome

Childhood Tonic, atonic,myoclonic, GTC, absence

Mental retardation

Slow (1 to 2 Hz) spike and wave

Valproic acid, Lamotrigine, felbamate

2 Benign Rolandic Epilepsy

Childhood Simple partial involving mouth & face, GTC

Nocturnal preponderance of seizure

Centrotemporal spikes

Carbamazepine

3 Absence Epilepsy

Childhood & adolescence

Absence, GTC

Hyperventilation as trigger

3 Hz spike and wave

Ethosuximide, valproic acid

4 Juvenile Myoclonic Epilepsy

Childhood & young adulthood

Myoclonic, absence, GTC

Early morning preponderance of seizures

4-6 Hz polyspike and wave

Valproic acid, lamotrigine

Partial Seizure

Simple partial seizure• begin with motor, sensory, or autonomic phenomena, depending on

the cortical region affected. • they may be recurrent or continuous or may spread to involve

contiguous regions of the motor cortex (jacksonian march).• During simple partial seizures, consciousness is preserved unless the

seizure discharge spreads to other areas of the brain, producing tonic-clonic seizures (secondary generalization).

• In the postictal state, a focal neurologic deficit such as hemiparesis (Todd paralysis) that resolves over a period of 30 minutes to 36 hours is a manifestation of an underlying focal brain lesion.

• Autonomic symptoms: pallor, flushing, sweating, pupillary dilatation, vomiting, and hypersalivation.

• Psychic symptoms: – déjà vu, cognitive deficits, fear, depression – hallucinations, or illusions.

Complex partial seizures• formerly called psychomotor seizures, are partial seizures in

which consciousness, responsiveness, or memory is impaired.

• The seizure discharge usually arises from the temporal lobe or medial frontal lobe but can originate elsewhere.

• Epigastric sensations are most common, but affective (fear), psychic (déjà vu), and sensory (olfactory hallucinations) symptoms also occur.

• Consciousness is then impaired. Seizures generally persist for less than 30 minutes (on an average, 1-3 minutes).

• The motor manifestations: coordinated involuntary motor activity, termed automatism (orobuccolingual movements

• other facial or neck or hand movements)

Partial seizures with secondary generalized

• Spreading of the epileptiform discharge during CPS can result in secondary generalization with a tonic-clonic convulsion.

• During the spread of the ictal discharge throughout the hemisphere, contralateral versive turning of the head, dystonic posturing, and tonic or clonic movements of the extremities and face, including eye blinking, may be noted.

Generalized Seizures

Generalized Tonic Clonic Seizure

• attacks in which consciousness is lost, usually without aura or other warning. When a warning does occur, it usually consists of nonspecific symptoms

Generalized Tonic Clonic Seizure

• 4 phases1. Tonic phase

- The initial manifestations : unconsciousness and tonic contractions of limb muscles for 10-30 seconds, producing first flexion and then extension particularly of the back and neck

Generalized Tonic Clonic Seizure

1. Tonic phase- Tonic contraction of the muscles of respiration may produce an expiration-induced vocalization (cry or moan) and cyanosis- Tonic contraction of masticatory muscles -> tongue trauma. The patient falls to the ground and may be injured.

Generalized Tonic Clonic Seizure

• 4 phases2. Clonic phase

- alternating muscle contraction and relaxation- phase of symmetric limb jerking that persists for

an additional 30-60 seconds, or longer. - Ventilatory efforts return immediately after cessation of the tonic phase, and cyanosis clears

Generalized Tonic Clonic Seizure

• 4 phases2. Clonic phase

- The mouth may froth with saliva. - With time, the jerking becomes less frequent,

until finally all movements cease and the muscles are flaccid. - Sphincteric relaxation or detrusor muscle contraction may produce urinary incontinence.

Generalized Tonic Clonic Seizure

• 4 phases3. Recovery

- As the patient regains consciousness, there is postictal confusion and often headache.

- Full orientation commonly takes 10-30 minutes, or even longer in patients with status epilepticus or preexisting structural or metabolic brain disorders.

Generalized Tonic Clonic Seizure

• 4 phases3. Recovery

- Physical examination during the postictal state is usually otherwise normal in idiopathic epilepsy or seizures of metabolic origin, except that plantar responses may be transiently extensor (Babinski sign). The pupils always react to light, even when the patient is unconscious.

Generalized Tonic Clonic Seizure

• 4 phases4. Status epilepticus

- seizures that continue for more than 30 minutes without ceasing spontaneously, or which recur so frequently that full consciousness is not restored between successive episodes. - a medical emergency -> can lead to permanent brain damage, from hyperpyrexia, circulatory collapse, or excitotoxic neuronal damage, if untreated.

Absence (petit mal) Seizures

• genetically transmitted seizures • always begin in childhood and rarely persist into

adolescence. • characterized by brief loss of consciousness (for

5-10 seconds) without loss of postural tone.• Subtle motor manifestations : 1. Common -> eye blinking or a slight head turning2. Uncommon -> Automatisms

Absence (petit mal) Seizures

• Full orientation immediately follows cessation of the seizure.

• Several hundred spells daily -> leading to impaired school performance and social interactions, so that children may be mistakenly thought to be mentally retarded before the diagnosis of petit mal epilepsy is made.

• The spells -> inducible by hyperventilation.

Absence (petit mal) Seizures

• EEG -> 3hz spike-and-wave pattern during the seizures

• In most patients with normal intelligence and normal background activity on EEG, absence spells occur only during childhood

• in other cases, however, the attacks continue into adult life, either alone or in association with other types of seizures.

Absence (petit mal) Seizure

Absence (petit mal) Seizures

Tonic Seizures

• characterized by continuing muscle contraction -> fixation of the limbs and axial musculature in flexion or extension

• a cause of drop attacks; the accompanying arrest of ventilatory movements leads to cyanosis

• Consciousness is lost, and there is no clonic phase to these seizures.

Tonic Seizures

Clonic Seizures

• characterized by repetitive clonic jerking accompanied by loss of consciousness. There is no initial tonic component.

Clonic Seizures

Myoclonic Seizures

• characterized by sudden, brief, shocklike contractions that may be localized to a few muscles or one or more extremities or that may have a more generalized distribution.

• Juvenile Myoclonic Epilepsy (JME) is the most common, with onset usually in adolescence. There is a family history of seizures in one-third.

• May be idiopathic or associated with a variety of rare hereditary neurodegenerative disorders,

Myoclonic Seizures

including Unverricht-Lundborg disease, Lafora body disease, neuronal ceroid lipofuscinosis (late infantile, juvenile, and adult forms), sialidosis, and mitochondrial encephalomyopathy (myoclonus epilepsy with ragged red fibers on skeletal muscle biopsy).

• Not all myoclonic jerks have an epileptic basis

Myoclonic Seizures

Atonic Seizures

• Result from loss of postural tone, sometimes following a myoclonic jerk, leading to a fall or drop attack.

• Most common in developmental disorders such as the Lennox-Gastaut syndrome.

Status epilepticus Adalah Kejang berulang yang terjadi selama 30 menit

atau lebih, tanpa pasien mencapai kesadaran kembali diantara serangan.

Klasifikasi : Konvulsif (bangkitan umum tonik-klonik) Non konvulsif (bangkitan bukan umum tonik-klonik)

• partly treated status epilepticus -> cessation of clinical seizures or only subtle symptoms, but the continuance of electrographic seizures.

• Refractory status epilepticus is defined as seizures lasting longer than 2 h or seizures recurring at a rate of two or more episodes per hour without recovery to baseline between seizures, despite treatment with conventional antiepileptic drugs(AEDs)

• However, in clinical practice, status epilepticus is often considered to be refractory in any patient who has not responded to first-line AEDs.

Seizures

• The systemic effects of status epilepticus are a consequence of the massive catecholamine release that occurs together with excessive muscular activity.

• Lothman divided these progressive changes into two phases: – the first phase lasts up to 30 min after seizure

initiation – the second phase continues after the initial 30 min

Treatment

Treatment

Treatment

Prognosis• The mortality of status epilepticus varies from 11% to 34% with rates higher in

adults (15–49%) than in children (3–15%)

• In the adult population, status epilepticus is often secondary to anoxia, hypoxia, stroke, metabolic abnormalities, brain tumor, or head injury, which has the highest mortality.

• In children, higher mortality is associated with severe acute encephalopathies and progressive encephalopathies. The lowest mortality rate is found in febrile and idiopathic status epilepticus

• The risk of unprovoked seizures is 3 × 34 times higher after acute symptomatic status epilepticus (41%) than after single seizures (13%), and the risk of developing a febrile seizure is much higher after status epilepticus than after simple febrile convulsions