SANS Epilepsy Flashcards
Seorang pasien berusia 16 tahun dengan kejang partial complex yang resisten terhadap obat, dengan onset bilateral independen dirujuk untuk tindakan vagus nerve stimulation (VNS). Apakah efek samping umum dari tindakan VNS ?
A. Dyspepsia
B. Dyspnea
C. Cardiac arrhythmia
D. Suara serak
E. Infeksi luka
D. Suara serak
Hoarseness or tremulousness of the voice is the most common side effect of vagus nerve stimulation. This typically occurs during stimulation to some degree in every patient, and was reported as an adverse event in 37-66% (Salinsky et al, 1995; Handforth et al, 1998) of patients. This side effect is usually well-tolerated.
Vagus nerve stimulation (VNS) has been shown to result in 50% or greater reduction of seizures in approximately 30% of patients (Salinsky et al., 1995; Handforth et al 1998). This is often the best option for medically refractory patients who are not candidates for, or who have failed, resective surgery.
Per the manufacturer, bradycardia and/or asystole during intra-operative test stimulation was found to occur in approximately 1/875 cases over 3500 cases. Cardiac arrhythmia was assessed in the E03 trial (Salinsky et al, 1995). 24-hour Holter monitoring was performed before and during VNS in a subset of 28 patients. The frequency of ectopic beats during VNS was unchanged, and a symptomatic effect on cardiac function was not observed. Dyspnea was reported in 5.6 - 23% of patients (Salinsky et al., 1995; Handforth et al 1998). Dyspepsia occurred in 17% of patients, and was not different between the high-frequency treatment and low-frequency stimulation control groups (Handforth et al, 1998). Gastric acid monitoring was performed before and during vagal nerve stimulation in 14 patients during the E03 trial. A nonsignificant trend toward increased basal gastric acid output was observed during VNS, with no clinical correlate (Salinsky et al, 1995). Infection around the VNS device occurred in 3 of 198 patients (Handforth et al, 1998).
Seorang wanita berusia 23 tahun dengan kejang partial complex awalnya diterapi dengan phenytoin, kemudian diganti dengan carbamazepine, dan saat ini menggunakan levetiracetam setelah 2 obat pertama tersebut gagal. Setelah periode bebas kejang selama 6 bulan sejak menggunakan levetiracetam, dia sekarang mengalami rekurensi sebanyak 2-3 kejang / bulan. Langkah apakah selanjutnya yang paling tepat dalam managemen pasien tersebut ?
A. Evaluasi untuk stimulasi nervus vagus
B. Menambahkan terapi dengan oxcarbazepine
C. Merubah terapi dengan menjadi menggunakan oxcarbazepine
D. Evaluasi untuk bedah epilepsi resective
D. Evaluasi untuk bedah epilepsi resective
The patient meets the criteria for drug resistant epilepsy (Kwan et al., 2010), and is unlikely to become seizure-free with further medication trials. The most appropriate next step in her management is evaluation for resective epilepsy surgery.
In a single-institution, prospective, cohort study of 780 adolescents and adults prescribed their first AED from 1982-2001, 50.4%, 10.7%, and 2.7% percent of patients became seizure free for at least one year with the first, second and third treatment schedules, respectively (Mohanraj and Brodie 2006). Some studies show rates of seizure freedom in chronic epilepsy as high as approximately 15%, depending on trial design, how an adequate trial of AED is defined, or use of newer AEDs. However, this is still significantly lower than chances of seizure freedom if she is a candidate for resective epilepsy surgery. Therefore, monotherapy or add-on therapy with drug A is not the best next step, though either of these may be tried in parallel with surgical evaluation.
Vagus nerve stimulation (VNS) has been shown to result in 50% or greater reduction of seizures in approximately 30% of patients (Salinsky et al., 1995). However, seizure freedom is uncommon with VNS, so this is an appropriate option for patients who are not candidates for resective surgery or who have failed resective surgery.
Seorang pria berusia 24 tahun menderita kejang partial complex yang refrakter, berlokasi di lobus temporal kiri dari EEG. MRI konsisten memberikan gambaran left mesial temporal sclerosis. Langkah apakah selanjutnya yang paling tepat untuk penanganan pasien tersebut ?
A. Lobectomy temporal
B. Intracranial EEG
C. Intracarotid amytal test
D. Stimulasi nervus vagus
E. Callosotomy corpus
C. Intracarotid amytal test
The correct answer is intracarotid amytal test (Wada test). This patient’s EEG and MRI are concordant in suggesting a left temporal focus. Temporal lobectomy will likely be the ultimate treatment. However, language dominance and memory function need to be defined preoperatively. The Wada test allows for the testing of each hemisphere individually for language and memory function and is useful for determining whether the patient’s contralateral hippocampal structures can support memory function if a temporal lobectomy were performed.
Intracranial EEG monitoring is possible in this scenario although the patient’s seizure onset seems to be localized to the left temporal lobe based on MRI and EEG. This patient has a very high chance for seizure freedom in the initial few years, as vagal nerve stimulation is reserved as a palliative procedure for refractory seizures. Corpus callosotomy is effective for drop attacks or atonic seizures which is really a different situation than secondary generalization from complex partial epilepsy.
Seorang pria 32 dengan kejang yang intractable dan MRI normal menjalani sebuah evaluasi non-invasif. Berdasarkan pada EEG kepala dan semiology kejang, dia mendapatkan elektroda subdural dan kejang dilokalisasi berada pada area motorik suplementer hemisfer dominan (kiri). Setelah reseksi bedah, pasien tersebut kemungkinan akan memperlihatkan gejala :
A. Paresis sementara pada sisi kiri
B. Quadrantopsia inferior sinistra
C. Bisu sementara
D. Anomia dan agnosia jari tangan
C. Bisu sementara
Resections in the language-dominant SMA often result in post-operative muteness, paresis (on the CONTRALATERAL side - in this case right), and neglect. The paresis may be contralateral but can affect bilateral extremities as well. The hallmark of these deficits is that they are transient. They largely resolve within 3-4 weeks of surgery. Therefore, if seizures are severe and debilitating; surgical resection may well be indicated after thoroughly counseling the patient about temporary post-operative deficits. Anomia and finger agnosia are characteristic of Gerstmann’s Syndrome, resulting from dominant parietal damage. Inferior quadrantopsia is characteristic of damage to the superior component of the optic radiations, in the parieto-occipital lobe.
Menurut study double-blind acak yang membandingkan terapi obat-obatan dengan terapi bedah untuk epilepsi, outcome dari pasien yang menjalani lobectomy temporal anterior standar dapat dijelaskan sebagai :
A. 50-60 % dari pasien bedah mengalami efek samping neurologis
B. 10-20 % dari pasien medis bebas kejang
C. 50-60 % dari pasien bedah bebas kejang
D. 10-20 % dari pasien medis mengalami efek samping neurologis
C. 50-60 % dari pasien bedah bebas kejang
In the only randomized trial for ATL, 58% of surgical patients were free of seizures impairing awareness but only 8% of medicine patients were seizure-free. 10% of surgical patients had adverse effects from the surgery but this did not count visual field defects as these were considered expected side effects of the surgery. 55% of surgical patients had visual field defects. No medicine patients developed neurological deficits during the study.
Seorang anak berusia 8 tahun dengan tuberous sclerosis mengalami kejang melumpuhkan (partial complex dengan kejang umum sekunder) yang cukup sering, yang refrakter terhadap terapi medis. EEG memperlihatkan bahwa kejang berasal dari hemisfer kanan. MRI ditunjukkan di bawah. Tindakan apakah yang paling tepat selanjutnya dalam penanganan pasien tersebut ?
A. Lesionectomy
B. Electrocorticography
C. CT kepala
D. Callosotomy
E. Hemispherectomy
B. Electrocorticography
The correct answer is electrocorticography. The patient seems to have numerous cortical tubers throughout the right hemisphere, concordant with a right-sided EEG onset. Hyperintense signal in the right hippocampus is also noted on the coronal FLAIR MR image. Electrocorticography with subdural strip electrodes is an effective way to record EEG around each of the regions of the brain and cortical tubers. It is the necessary next step to localize the onset of epilepsy in tubulous sclerosis where numerous lesions could be implicated. A cerebral CT may demonstrate calcifications but has no localizing value in the determination of seizure onset. Lesionectomy is often a very effective procedure in tubulous sclerosis, but it requires precise localization of the seizure onset and determination of the symptomatic tuber. Callosotomy effectively treats drop attacks or atonic seizures. It is unlikely to be effective with complex partial seizures where there are frequent secondary generalizations. Hemispherectomy is typically reserved for children suffering from severe cortical epilepsies due to hemimegalencephaly or Rasmussen’s encephalitis. In both these conditions, there is typically a significant neurologic deficit preoperatively that can become more involved following surgery.
Di dalam sindrome epilepsi pada anak-anak, pasien dengan sindrom Lennox-Gastaut paling sering menunjukkan gambaran EEG apakah ?
A. Multifocal slow spike and wave
B. Normal or discontinous
C. Bilateral hypsarrhthmia
D. Triphasic spike and wave
A. Multifocal slow spike and wave
Lennox-Gastaut syndrome typically affects children 1 to 7 yr old and is characterized by multifocal seizures and a background EEG consisting of slow spike wave activity. There is no gender predominance. Triphasic spike and wave activity is characteristic of CJD. Hypsarythmia is characterisitic of infantile spasms (West Syndrome). Normal or discontinuous EEG patterns may be seen in Benign Childhood Epilepsy.
Gejala klinis yang paling umum dihasilkan dari kejang lobus temporal medial adalah :
A. Visual aura
B. Ictal oral automatisms
C. Ictal bicycling movements
D. Ipsilateral dystonic posturing
B. Ictal oral automatisms
Oral automatisms are a very common ictal behavior during medial temporal lobe seizures and may consist of lip smacking, chewing, swallowing or tooth-grinding. Bicycling movement of the lower extremities are more commonly seen in complex partial seizures that originate in the frontal lobe, particularly supplementary motor area. Gustatory or olfactory aurus, not visual auras, are commonly seen in medial temporal lobe seizures. Dystonic posturing, while common in mesial temporal seizures, is reliably contralateral. Mesial temporal seizures may be historically related to febrile convulsions most commonly found in early childhood in upt to 67% of people with documented medial temporal lobe epilepsy. Epigastric sensations are frequently reported as auras as well.
Sindrom West’s memiliki karakteristik klinis kejang yang bagaimanakah ?
A. Kejang umum primer dan sekunder
B. Kejang umum tonic clonic
C. Kejang partial complex
D. Spasm infantile
E. Atonic
D. Spasm infantile
West’s Syndrome produces seizures in early childhood and consists of infantile spasms, mental retardation, and hypsarrhythmia (complete disorganization) on EEG. With maturity however, these patients can subsequently develop other seizure types. Clasically, West’s syndrome is treated with ACTH rather than antiepileptics. Vigatrabine is one AED with improved control of infantile spasps - results compared to ACTH are mixed.
Ketika menangani status epileptikus pada pasien dewasa, pengobatan intravena manakah yang sebaiknya dibarengi dengan benzodiazepines ?
A. Phenytoin
B. Propofol
C. Phenobarbital
D. Succinycholine
E. Nitroprusside
A. Phenytoin
Intravenous phenytoin 18 mg/kg should be administered simultaneously with intravenous benzodiazepines to provide longer term seizure control. Benzodiazepines are first-line treatment for status epilepticus, preferably lorazepam (Ativan) 1-4 mg IV slow push. Diazepam can be used instead of lorazepam but has a longer half-life and may therefore cause greater respiratory depression.
Phenobarbital may be employed but is generally reserved for cases in which the status epilepticus is refractory to both Ativan and Dilantin. Likewise, propofol (and other general anesthetics) may also be used but only if the previous measures fail to control the seizures.
Succinylcholine, a depolarizing paralytic agent, is used for emergent intubation, which is often necessary in the aggressive management of status epilepticus. It should not however be used as a primary means of stopping convulsions. Neuromuscular blockade may mask persistent epileptic brain activity leading to further injury. Furthermore, this agent may lead to higher serum concentrations of potassium which may already be elevated by seizure driven muscle contraction.
Nitroprusside is an effective hypotensive agent but is not used in the regular management of status epilepticus. Nitroprusside may be be particularly dangerous in the management of status epilepticus because it is known to raise intracranial pressure.
