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Epilepsy is a chronic disease which spasm occur repeatedly more than 2 times as brain nerve cell excite super normally. In case there occur sudden onset of consciousness disorder, dyskinesia/impaired sensation and there is epidemical spasm, possibility of epilepsy is high. Nerve cell in the brain is acting with regularly concerted electric signal. But when excessed electric disturbance or supernormal excitability occur, epilepsy may be developed.
Symptoms are various like, convulsion, spasm, feeling foggy, jerking, walk around without consciousness (automatism), impaired vision, impaired sensitivity, abdominal discomfort, psychological symptom. Variety of symptoms appears depend on which range of brain nerve cell produces supernormal excitability.
With sufficient clinical history and to witness the scene of seizure is the most effective thing to diagnose epilepsy (evidence level II, hereinafter evidence level is referred to as “E”). To diagnose epilepsy, at least 2 time of spasm is necessary (Grade B). But in case if the patient, who has diagnosed as general tonic seizures at fist seizure, has anamnestic history of myoclonic-atonic seizure, absence epilepsy and simple/complex partial seizure, epilepsy can be diagnosed even with having one-time seizure (E-III). Interview contents are as follows.
For the classification of epilepsy seizure type, International League Against Epilepsy ILAE classification is used. This classification is inevitable for handling a patient, examination, selection of anti-epilepsy agent and selection of surgical treatment.
Patients taking visiting the ER office due to suddenly developed loss of conscious, 40 % of them are neutrally mediated syncope/ psychogenic non-epileptic seizure, 29% of them are epilepsy, 7% is cardiac syncope. Characteristics of syncope is that; no change in consciousness fatigue or feeling of malaise after seizure.
Febrile convulsion, Benign infantile convulsion, Minor gastroenteritis related seizure, a part of psychological seizure, acute metabolic disease, sustained cyanosis breath-holding spells
Breath-holding spells, neurally mediated syncope, a part of psychological seizure, a part of acute metabolic disease, a part of febrile convulsion
Sleep myoclonus, a part of psychological seizure
Night terror disorder/noctambulant, psychological seizure
This disease is commonly diagnosed by clinical history, symptoms, onset age and brain wave forms. But there are the cases where febrile convulsion, later stage of benign infantile convulsion and part of acute metabolic disease may indicate epileptic seizure waveform so that it is necessary to pay attention for these cases.
Examination for the diagnosis are as follows.
Sleep activation electroencephalogram (EEG) may increase appearance frequency of epilepsy discharge and to quell normal brain wave so that discharge can easily be interpreted.
CT、MRI
It is especially important to differentiate seizure type of frontal lobe epilepsy or temporal lobe epilepsy by means of polysomnography.
To diagnose Light-sensitive epilepsy where seizure is caused with light stimulation, appearance of photo paroxysmal response; PPR by light stimulation by brain wave examination is necessary. When PPR exist in brain examination, it is diagnosed as Photosensitive epilepsy；PSE. There are potential photosensitive patients who do not notice light sensitive constitution until brain examination is received. Frequency of light-sensitive epilepsy is one out of 4,000 at age of 5 to 40 years old. Hereditary component is high and tonic-clonic seizure combined with spasm.
Short time contraction of hamstrings muscle is suddenly occurred and may occasionally generalized. There is no loss of consciousness. It takes about 5 to 10 % of idiopathic generalized epilepsy. It is developed from childhood to youth period and has the following characteristics.
In principle, anti-epilepsy drug is not used at isolated seizure (first time seizure). Anti-epilepsy drug is start using from second seizure. But, in case when there are neurological abnormality, brain wave abnormality or epilepsy family history, start of treatment is considered. Treatment start from the age 65 or over, recurrence rate is getting high after first seizure (66 to 90%). Seizure appearance rate within 5 years from first seizure is about 35%. Recurrence rate within 1 year after the second seizure is 73%. Seizure rate after the start of treatment has a significant difference as shown below for the case where number of seizures before the start of treatment are over 21 times or less 20 times.
For over 21 times, recur at 37%
For less 20 times, recur at 29%.
Chemotherapy treatment should be started as early as possible from second seizure.
Alteration to generic drug is not recommended when seizure is hold. Because, there are no high-quality evidence which validate the equivalence of therapy using novel drug and generic drug. Regarding the drug alteration, it is reported that the seizure deterioration or side effect appearance may develop.
Following to Valproate
* Carbamazepine and gabapentin are not used for idiopathic generalized epilepsy because they may get diseases worse. Benzodiazepines drugs or Lennox-Gastaut Syndrome may get tonic seizure worse. They can be seen on every anti-epilepsy drugs, but Valproate is listed as E-1 for teratogenesis and newborn IQ disability.
Large-size research says that in the Progressive Myoclonus Epilepsy；PME (Unverricht-Lundborg disease), Phenytoin may hold the seizure itself but life prognosis may get worse (E-2), and cerebellar ataxia is getting worse.
Deduction of anti-epilepsy drug is considered after the seizure disappearance in 2 to 5 years. However, in juvenile myoclonic epilepsy, recurrence rate is high.
Anti-epilepsy drug is divided into GABA actuated drug which has anti-anxiety action and manic state inhibitory effect, and glutamic acid-based inhibitory drug which has antidepressant action and anxiogenic effect.
Barbital acid, Benzodiazepines drug, Valproate, gabapentin, Topiramate,
Lamotrigine, Levetiracetam
Zonisamide has both effects.
Valproate, Carbamazepine, Lamotrigine
* Ethosuximide, Zonisamide, Primidone, High Dosage phenytoin and Topiramate may raise the acute psychological symptom. Benzodiazepines anti-epileptic drugs may cause the acute psychological symptom at abstinence. As the side effect of anti-epilepsy drugs, they are depressed state or deterioration of mental function by phenobarbital, depressed state by Ethosuximide, Carbamazepine, Clonazepam, Zonisamide and Valproate, and Hypomania State. So that having caution to them is important.
Anti-epilepsy drug is divided into GABA actuated drug which has anti-anxiety action and manic state inhibitory effect, and glutamic acid-based inhibitory drug which has antidepressant action and anxiogenic effect.
Valproate, Phenytoin, Carbamazepine, Phenobarbital, Benzodiazepines
Gabapentin, Levetiracetam
Topiramate, Lamotrigine
Valproate, Carbamazepine
Phenytoin, Carbamazepine
Phenobarbital, Zonisamide, Carbamazepine
Valproate
Phenytoin effect in hypoalbuminemia may increase.
(1) Carbamazepine, (2) Lamotrigine, (3) Levetiracetam, (4) Gabapentin
(1) Levetiracetam, (2) Lamotrigine, (3) Gabapentin
(1) Lamotrigine, (2) Valproate, (3) Levetiracetam, (4) Topiramate
Drugs which lower the epilepsy threshold are listed below.
Definition of refractory epilepsy is the status where appropriate anti-epilepsy drug, more than 2 to 3 kinds of drugs combined and with enough volume, are taking more than 2 years, but still seizure has not been inhibited for more than one year so that daily life is disturbed much.
The definition of social refractory epilepsy is the status where “seizure inhabitation cannot be achieved by over 2 years adequate chemotherapy treatment”. Inhibition rate of antiepilepsy drug under normal chemotherapy treatment are; 1st antiepilepsy drug 47%, second is 13%, third or combination case of more than 2 drugs is just 4%. Therefore, when seizure inhibition cannot be recognized with more than 2 drugs chemotherapy is differentiated to refractory epilepsy. Application reference for surgical treatment is the case where more than one-time seizure occurs in monthly average under the chemotherapy, and daily life is interfered. In the Road Traffic Act, Driving License cannot obtain unless seizure is inhibited for more than 2 years. Besides, if there is partial seizure more than 2 times a year, second grade of Health and Welfare Handbook for mentally handicapped is applicable.
In adult epilepsy, temporal lobe epilepsy with hippocampal sclerosis is the most intractable one. (case where seizure is inhibited for more than 1year is 11%), but surgical result of selective hippocampal amygdala Removal or resection of the temporal lobe hippocampal amygdala are in good treatment results with cure rate of 85%.
With the imaging examination, cerebrovascular disease, brain malformation, tumor, hippocampal sclerosis, after encephalitis/encephalopathy, and general disease are checked. Symptomatic generalized epilepsy by dentatorubro-pallidoluysial atrophy; DRPLA or epilepsy which cannot inhibit by childhood developed Lennox-Gastaut syndrome, are easy to become refractory one.
For the epilepsy where seizure cannot be inhibited for more than 1 year even though adequate 2 or 3 combined drugs with enough blood concentration has been administrated for more than 2-years, surgical operation is considered.
Brain Surgery Committee in International Epilepsy Committee considers the surgery within 2 years of incidents, because underdeveloped may happen for children. Other than the disappearance of epilepsy seizure and its associated improvement of QOL, it is known that the psychomotor development is improved with children. Also, absolute mental retardation or existence of psychological disorder will not be the reason for the exclusion reference of surgical accommodation. Although numbers of seizure are less, surgical operation is considered for symptomatic epilepsy with intracerebral lesion, epilepsy with the fear of external injury like rollover and epilepsy which causes disadvantage on social life like work. However, symptomatic epilepsy caused by gene abnormality, such as dentatorubropallidoluysial atrophy (DRPLA) and symptomatic epilepsy caused by progressive disease are not the target of surgery operation.
MTLE-HS where hippocampal sclerosis (HS) is contained, is the best adapted for surgical treatment, and significant seizure disappearance is expected. In the Catastrophic epilepsy, there exist a partial epilepsy with broader pathological change on unilateral hemisphere. But this is developed in infancy period then paralysis or involution of psychomotor seizure is raised by drug resistance so that it is recommended to have surgical operation at early stage.
Intracranial EEG recording is the invasive examination, but it is useful to determine epileptogenic zone in neocortical epilepsy. In mesial temporal lobe epilepsy in case when hippocampal observation by MRI and brain wave observation are not contradictive each other, intracranial EEG recording can be excluded.
Brain wave recording using subdural electrode has the 10 times of space resolution accuracy. Permanent complication disease is 1.5%, and they are caused by bleeding in the brain and infection. Surgical treatment for mesial temporal lobe epilepsy (58%) is superior than medical therapy (8%) (Grade A).
Surgical treatment result for neocortical epilepsy is; temporal lobectomy (66%), Occipital and parietal lobectomy (46%), frontal lobectomy (27%), multiple subpial transection (16%), and corpus colostomy (35%). Engel classification is used for postoperative seizure outcome assessment.
In case of dominant hemisphere, it can cut maximum 4.5cm from top edge of temporal lobe, and in case of non-dominant hemisphere, able to cut up to maximum 6cm.
Inside observation of lateral cerebral ventricle: Hippocampus forms the floor of inferior horn of lateral ventricle, and amygdala forms a part of lateral ventricle inferior horn upper wall facing to hippocampus.
After suction removal of uncinate gyrus: Posterior cerebral artery, anterior choroidal artery and oculomotor nerve are observed from brain ventricle side through buffy coat wrapped around uncinate.
Cutoff of hippocampus: With cutting off anterior side of hippocampus, hippocampus can be cutoff to inferior choroidal point which is the entrance part of anterior choroidal artery into brain ventricle.
Peel anterior side of para hippocampal gyrus off: Carefully remove buffy coat on the anterior side of para hippocampal gyrus, then peel para hippocampal gyrus off under buffy coat to external side as much as possible.
Cut off hippocampal formation dorsal: Cut hippocampal formation dorsal at innominate groove between collateral eminence and hippocampal formation.
Extraction of Hippocampus / Extracted Hippocampus and hippocampal gyrus
Brain surface after hippocampus extraction: Epidemical damage on lateral cortex of frontal cortex （*）and temporal cortex(*) has not recognized.
Generalized epilepsy remarkable efficiency: 40 to 60% With cutting Left/right hemisphere off at callosum to prevent both cortical substance from generalizing to epilepsy seizure. To prevent acute withdrawal syndrome, corpus Callosotomy will commonly perform as secondary surgery so that one month later from front side 2/3 callosum cut off surgery, rear side 1/3 corpus Callosotomy is performed.
Enlargement of surgical area on corpus
lamina of translucent septum
splenium of corpus callosum cut off
Confirm Splenium of corpus callosum cut off.
(1) There are Lateral approach (Trans-ventricular hemispherotomy) and (2) vertical approach available. Lateral approach consists with four common technics; Cutting of encapsulated fibers, Resection of inside temporal lobe structure, corpus Callosotomy, Frontal lobe Horizontal Fiber cutting. (1) Resection of inside temporal lobe structure, (2) Cutting of encapsulated fibers, (3) Frontal lobe Horizontal Fiber cutting, (4) Trans ventricle callosum separation, (5) Separation of remaining falling fibers.
Surgery available at only epilepsy surgery department in the epilepsy authorized facility.
