Epilepsia Flashcards

1
Q

Quais os objetivos do atendimento de um paciente com crise epiléptica?

A

When a patient presents shortly after a seizure, the first priorities are attention to vital signs, respiratory and cardiovascular support, and treatment of seizures if they resume (see “Treatment: Seizures and Epilepsy”). Lifethreatening conditions such as CNS infection, metabolic derangement, or drug toxicity must be recognized and managed appropriately.
When the patient is not acutely ill, the evaluation will initially focus on whether there is a history of earlier seizures (Fig. 26-2). If this is the first seizure, then the emphasis will be to: (1) establish whether the reported episode was a seizure rather than another paroxysmal event, (2) determine the cause of the seizure by identifying risk factors and precipitating events, and (3) decide whether anticonvulsant therapy is required in addition to treatment for any underlying illness.
In the patient with prior seizures or a known history of epilepsy, the evaluation is directed toward (1) identification of the underlying cause and precipitating factors, and (2) determination of the adequacy of the patient’s current therapy.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

O que devemos perguntar na historia de um paciente epiléptico?

A

(1) Questions should focus on the symptoms before, during, and after the episode in order to differentiate a seizure from other paroxysmal events. Seizures frequently occur outof-hospital, and the patient may be unaware of the ictal and immediate postictal phases; thus, witnesses to the event should be interviewed carefully
(2) Clues for a predisposition to seizures include a history of febrile seizures, earlier auras or brief seizures not recognized as such, and a family history of seizures. Epileptogenic factors such as prior head trauma, stroke, tumor, or infection of the nervous system should be identified.
(3) Drugs that lower the seizure threshold (Table 26-5), or alcohol or illicit drug use should also be identified.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

O que examinar em um doente epiléptico?

A

(1) The general physical examination includes a search for signs of infection or systemic illness
(2) Careful examination of the skin may reveal signs of neurocutaneous disorders such as tuberous sclerosis or neurofibromatosis, or chronic liver or renal disease
(3) All patients require a complete neurologic examination, with particular emphasis on eliciting signs of cerebral hemispheric disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Quais exames laboratoriais devem ser solicitados em doentes com epilepsia?

A

Routine blood studies are indicated to identify the more common metabolic causes of seizures such as abnormalities in electrolytes, glucose, calcium, or magnesium, and hepatic or renal disease. A screen for toxins in blood and urine should also be obtained from all patients in appropriate risk groups, especially when no clear precipitating factor has been identified. A lumbar puncture is indicated if there is any suspicion of meningitis or encephalitis, and it is mandatory in all patients infected with HIV, even in the absence of symptoms or signs suggesting infection.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Quais os dois principais exames complementares para solicitar para todo doente com crise epiléptica com screening metabólico negativo?

A

MRI scan and EEG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Quais as características que diferenciam uma crise epiléptica de uma sincope?

A

Characteristics of a seizure include the presence of an aura, cyanosis, unconsciousness, motor manifestations lasting >15 s, postictal disorientation, muscle soreness, and sleepiness.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Como diferenciar uma crise entre orgânica e psicogênica?

A

(1) They are often part of a conversion reaction precipitated by underlying psychological distress. Certain behaviors such as side-to-side turning of the head, asymmetric and large-amplitude shaking movements of the limbs, twitching of all four extremities without loss of consciousness, and pelvic thrusting are more commonly associated with psychogenic rather than epileptic seizures. Psychogenic seizures often last longer than epileptic seizures and may wax and wane over minutes to hours.
(2) Video-EEG monitoring is very useful when historic features are nondiagnostic.
(3) Measurement of serum prolactin levels may also help to distinguish between organic and psychogenic seizures, since most generalized seizures and some focal seizures are accompanied by rises in serum prolactin (during the immediate 30-min postictal period), whereas psychogenic seizures are not.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Quais os objetivos do tratamento da epilepsia?

A

Therapy for a patient with a seizure disorder is almost always multimodal and includes (1) treatment of underlying conditions that cause or contribute to the seizures, (2) avoidance of precipitating factors, suppression of recurrent seizures by (3) prophylactic therapy with antiepileptic medications or surgery, and addressing a variety of (4) psychological and social issues.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Qual a relação entre o tratamento da causa da crise epiléptica e o tempo de droga antiepiléptica?

A

(1) If the sole cause of a seizure is a metabolic disturbance such as an abnormality of serum electrolytes or glucose, then treatment is aimed at reversing the metabolic problem and preventing its recurrence. Therapy with antiepileptic drugs is usually unnecessary unless the metabolic disorder cannot be corrected promptly and the patient is at risk of having further seizures
(2) If the apparent cause of a seizure was a medication (e.g., theophylline) or illicit drug use (e.g., cocaine), then appropriate therapy is avoidance of the drug; there is usually no need for antiepileptic medications unless subsequent seizures occur in the absence of these precipitants.
(3) Seizures caused by a structural CNS lesion such as a brain tumor, vascular malformation, or brain abscess may not recur after appropriate treatment of the underlying lesion. However, despite removal of the structural lesion, there is a risk that the seizure focus will remain in the surrounding tissue or develop de novo as a result of gliosis and other processes induced by surgery, radiation, or other therapies. Most patients are therefore maintained on an antiepileptic medication for at least 1 year, and an attempt is made to withdraw medications only if the patient has been completely seizure free. If seizures are refractory to medication, the patient may benefit from surgical removal of the epileptic brain region.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Como orientar o paciente a evitar fatores precipitantes?

A

For example, a patient who has seizures in the setting of sleep deprivation should obviously be advised to maintain a normal sleep schedule. Many patients note an association between alcohol intake and seizures, and they should be encouraged to modify their drinking habits accordingly. There are also relatively rare cases of patients with seizures that are induced by highly specific stimuli such as a video game monitor, music, or an individual’s voice (“reflex epilepsy”). If there is an association between stress and seizures, stress reduction techniques such as physical exercise, meditation, or counseling may be helpful.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Quando iniciar a terapia antiepiléptica?

A

(A) Patients with a single seizure due to an identified lesion such as a CNS tumor, infection, or trauma, in which there is strong evidence that the lesion is epileptogenic, should be treated.
(B) The risk of seizure recurrence in a patient with an apparently unprovoked or idiopathic seizure is uncertain, with estimates ranging from 31 to 71% in the first 12 months after the initial seizure. This uncertainty arises from differences in the underlying seizure types and etiologies in various published epidemiologic studies. Generally accepted risk factors associated with recurrent seizures include the following: (1) an abnormal neurologic examination, (2) seizures presenting as status epilepticus, (3) postictal Todd’s paralysis, (4) a strong family history of seizures, or (5) an abnormal EEG.
(C) Most patients with one or more of these risk factors should be treated. Issues such as employment or driving may influence the decision whether to start medications as well.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Qual droga antiepiléptica utilizar conforme o tipo de crise?

A

(1) Carbamazepine (or a related drug, oxcarbazepine), lamotrigine, and phenytoin are currently the drugs of choice approved for the initial treatment of focal seizures, including those that evolve into generalized seizures.
(2) Valproic acid and lamotrigine are currently considered the best initial choice for the treatment of primary generalized, tonic-clonic seizures. Topiramate, zonisamide, phenytoin, and carbamazepine are suitable alternatives. Ethosuximide is a particularly effective drug for the treatment of uncomplicated absence seizures, but it is not useful for tonic-clonic or focal seizures.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Como iniciar e monitorizar a terapia antiepiléptica?

A

(1) The goal is to prevent seizures and minimize the side effects of therapy; determination of the optimal dose is often a matter of trial and error. This process may take months or longer if the baseline seizure frequency is low.
(2) Most anticonvulsant drugs need to be introduced relatively slowly to minimize side effects, and patients should expect that minor side effects such as mild sedation, slight changes in cognition, or imbalance will typically resolve within a few days. Subsequent increases should be made only after achieving a steady state with the previous dose (i.e., after an interval of five or more half-lives).
(3) The key determinants are the clinical measures of seizure frequency and presence of side effects, not the laboratory values. Patients may have a “subtherapeutic” drug level, but the dose should be changed only if seizures remain uncontrolled, not just to achieve a “therapeutic” level. In practice, other than during the initiation or modification of therapy, monitoring of antiepileptic drug levels is most useful for documenting compliance.
(4) If seizures continue despite gradual increases to the maximum tolerated dose and documented compliance, then it becomes necessary to switch to another antiepileptic drug. This is usually done by maintaining the patient on the first drug while a second drug is added. The dose of the second drug should be adjusted to decrease seizure frequency without causing toxicity. Once this is achieved, the first drug can be gradually withdrawn (usually over weeks unless there is significant toxicity). The dose of the second drug is then further optimized based on seizure response and side effects. Monotherapy should be the goal whenever possible.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Quando parar a terapia antiepiléptica?

A

Overall, about 70% of children and 60% of adults who have their seizures completely controlled with antiepileptic drugs can eventually discontinue therapy. The following patient profile yields the greatest chance of remaining seizure free after drug withdrawal: (1) complete medical control of seizures for 1–5 years; (2) single seizure type, either focal or generalized; (3) normal neurologic examination, including intelligence; and (4) normal EEG. The appropriate seizure-free interval is unknown and undoubtedly varies for different forms of epilepsy. However, it seems reasonable to attempt withdrawal of therapy after 2 years in a patient who meets all of the above criteria, is motivated to discontinue the medication, and clearly understands the potential risks and benefits. In most cases it is preferable to reduce the dose of the drug gradually over 2–3 months. Most recurrences occur in the first 3 months after discontinuing therapy, and patients should be advised to avoid potentially dangerous situations such as driving or swimming during this period

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Quais as monitorizações habituais de efeitos colaterais de drogas antiepilépticas?

A

(1) Almost all of the commonly used antiepileptic drugs can cause similar, dose-related side effects such as sedation, ataxia, and diplopia.
(2) Long-term use of some agents in adults, especially the elderly, can lead to osteoporosis.
(3) Most of the older drugs and some of the newer ones can also cause idiosyncratic toxicity such as rash, bone marrow suppression, or hepatotoxicity. Although rare, these side effects should be considered during drug selection, and patients must be instructed about symptoms or signs that should signal the need to alert their health care provider. For some drugs, laboratory tests (e.g., complete blood count and liver function tests) are recommended prior to the institution of therapy (to establish baseline values) and during initial dosing and titration of the agent.
(4) Importantly, recent studies have shown that Asian individuals carrying the human leukocyte antigen allele, HLA-B∗1502, are at particularly high risk of developing serious skin reactions from carbamazepine and phenytoin, so racial background and genotype are additional factors to consider in drug selection.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Quais os principais cuidados com a prescrição da carbamazepina (tegretol)?

A

For example, an advantage of carbamazepine (which is also available in an extended-release form) is that its metabolism follows first-order pharmacokinetics, and the relationship between drug dose, serum levels, and toxicity is linear. Carbamazepine can cause leukopenia, aplastic anemia, hyponatremia or hepatotoxicity and would therefore be contraindicated in patients with predispositions to these problems. Typical dose is 600–1800 mg/d (15– 35 mg/kg, child); bid/qid. Half-life: 10–17 h.

17
Q

Quais os principais cuidados com a prescrição da fenitoina (dilantin)?

A

Phenytoin has a relatively long half-life and offers the advantage of once or twice daily dosing compared to two or three times daily dosing for many of the other drugs. However, phenytoin shows properties of saturation kinetics, such that small increases in phenytoin doses above a standard maintenance dose can precipitate marked side effects. This is one of the main causes of acute phenytoin toxicity. Long-term use of phenytoin is associated with untoward cosmetic effects (e.g., hirsutism, coarsening of facial features, and gingival hypertrophy), and effects on bone metabolism, so it is often avoided in young patients who are likely to require the drug for many years. Typical dose is 300–400 mg/d (3–6 mg/kg, adult; 4–8 mg/kg, child); qdbid. Half-life: 24 h.

18
Q

Quais os principais cuidados com a prescrição da lamotrigina (lamictal)?

A

Lamotrigine tends to be well tolerated in terms of side effects. However, patients need to be particularly vigilant about the possibility of a skin rash during the initiation of therapy. This can be extremely severe and lead to StevensJohnson syndrome if unrecognized and if the medication is not discontinued immediately. This risk can be reduced by slow introduction and titration. Lamotrigine must be started slowly when used as add-on therapy with valproic acid, since valproic acid inhibits lamotrigine metabolism, thereby substantially prolonging its half-life.
Typical dose is 150–500 mg/d; bid mg/d (20– 60 mg/kg); bid-qid. Half-life: 25 h 14 h (with enzymeinducers) 59 h (with valproic acid).

19
Q

Quais os cuidados com a prescrição do valproato?

A

Gastrointestinal side effects are fewer when using the valproate semisodium formulation (Depakote). Valproic acid also rarely causes reversible bone marrow suppression and hepatotoxicity, and laboratory testing is required to monitor toxicity. This drug should generally be avoided in patients with preexisting bone marrow or liver disease. Furthermore, this drug can cause transient alopecia and hyperammonemia.
Irreversible, fatal hepatic failure appearing as an idiosyncratic rather than dose-related side effect is a relatively rare complication; its risk is highest in children <2 years old, especially those taking other antiepileptic drugs or with inborn errors of metabolismo. Typical dose is 750–2000 mg/d (20– 60 mg/kg); bid-qid. Half-life: 15 h.

20
Q

Como utilizar a politerapia das drogas antiepilépticas?

A

Approximately one-third of patients with epilepsy do not respond to treatment with a single antiepileptic drug, and it becomes necessary to try a combination of drugs to control seizures. Patients who have focal epilepsy related to an underlying structural lesion or those with multiple seizure types and developmental delay are particularly likely to require multiple drugs. There are currently no clear guidelines for rational polypharmacy, although in theory a combination of drugs with different mechanisms of action may be most useful. In most cases the initial combination therapy combines first-line drugs (i.e., carbamazepine, oxcarbazepine, lamotrigine, valproic acid, and phenytoin). If these drugs are unsuccessful, then the addition of a newer drug such as levetiracetam, topiramate, and zonisamide is indicated. Patients with myoclonic seizures resistant to valproic acid may benefit from the addition of clonazepam, and those with absence seizures may respond to a combination of valproic acid and ethosuximide. The same principles concerning the monitoring of therapeutic response, toxicity, and serum levels for monotherapy apply to polypharmacy, and potential drug interactions need to be recognized. If there is no improvement, a third drug can be added while the first two are maintained. If there is a response, the less effective or less well tolerated of the first two drugs should be gradually withdrawn.

21
Q

Quais doentes podem ter beneficio do manejo cirúrgico da epilepsia?

A

Approximately 20–30% of patients with epilepsy continue to have seizures despite efforts to find an effective combination of antiepileptic drugs. For some, surgery can be extremely effective in substantially reducing seizure frequency and even providing complete seizure control. The most common surgical procedure for patients with temporal lobe epilepsy involves resection of the anteromedial temporal lobe (temporal lobectomy) or a more limited removal of the underlying hippocampus and amygdala (amygdalohippocampectomy). Focal seizures arising from extratemporal regions may be abolished by a focal neocortical resection with precise removal of an identified lesion (lesionectomy).

22
Q

Qual a avaliação pré-operatória para o manejo neurocirúrgico da epilepsia?

A

Presurgical evaluation is designed to identify the functional and structural basis of the patient’s seizure disorder.

(1) Inpatient video-EEG monitoring is used to define the anatomic location of the seizure focus and to correlate the abnormal electrophysiologic activity with behavioral manifestations of the seizure. Routine scalp or scalp-sphenoidal recordings are usually sufficient for localization, and advances in neuroimaging have made the use of invasive electrophysiologic monitoring such as implanted depth electrodes or subdural electrodes less common.
(2) A high-resolution MRI scan is routinely used to identify structural lesions, and this is sometimes augmented with MEG. Functional imaging studies such as SPECT and PET are adjunctive tests that may help verify the localization of an apparent epileptogenic region.
(3) Once the presumed location of the seizure onset is identified, additional studies, including neuropsychological testing and the intracarotid amobarbital test (Wada test) may be used to assess language and memory localization and to determine the possible functional consequences of surgical removal of the epileptogenic region. In some cases, the exact extent of the resection to be undertaken is determined by performing cortical mapping at the time of the surgical procedure, allowing for a tailored resection. This involves electrocorticographic recordings made with electrodes on the surface of the brain to identify the extent of epileptiform disturbances. If the region to be resected is within or near brain regions suspected of having sensorimotor or language function, electrical cortical stimulation mapping is performed on the awake patient to determine the function of cortical regions in question in order to avoid resection of so-called eloquent cortex and thereby minimize postsurgical deficits.

23
Q

Quais os resultados do tratamento cirúrgico para crises epilépticas?

A

Clinically significant complications of surgery are <5%, and the use of functional mapping procedures has markedly reduced the neurologic sequelae due to removal or sectioning of brain tissue. For example, about 70% of patients treated with temporal lobectomy will become seizure free, and another 15–25% will have at least a 90% reduction in seizure frequency. Marked improvement is also usually seen in patients treated with hemispherectomy for catastrophic seizure disorders due to large hemispheric abnormalities. Postoperatively, patients generally need to remain on antiepileptic drug therapy, but the marked reduction of seizures following resective surgery can have a very beneficial effect on quality of life.

24
Q

Quando indicar estimulação de nervo vago na epilepsia refrataria?

A

Not all medically refractory patients are suitable candidates for resective surgery. For example, some patients have seizures arising from more than one site, making the risk of ongoing seizures or potential harm from the surgery unacceptably high. Vagus nerve stimulation (VNS) may be useful in some of these cases, although the benefit for most patients seems to be very limited (i.e., the efficacy of VNS appears to be no greater than trying another drug, which rarely works if a patient has proved to be refractory to the first two to three drugs). The precise mechanism of action of VNS is unknown, although experimental studies have shown that stimulation of vagal nuclei leads to widespread activation of cortical and subcortical pathways and an associated increased seizure threshold. Adverse effects of the surgery are rare, and stimulation-induced side effects, including transient hoarseness, cough, and dyspnea, are usually mild.

25
Q

Qual o tratamento inicial do estado mal epiléptico?

A

The first steps in the management of a patient in GCSE are to attend to any (1) acute cardiorespiratory problems or hyperthermia, (2) perform a brief medical and neurologic examination, (3) establish venous access, and send samples for laboratory studies to identify metabolic abnormalities. (4) Anticonvulsant therapy should then begin without delay.
A. Lorazepam 0.1–0.15 mg/kg IV over 1–2 min (repeat x 1 if no response after 5 min)
B. Fosphenytoin 20 mg/kg PE IV 150 mg/min or phenytoin 20 mg/kg IV 50 mg/min OR Consider valproate 25 mg/kg IV in pts. normally taking valproate and who may be subtherapeutic
C. IV anesthesia with propofol or midazolam or pentobarbital OR Phenobarbital 20 mg/kg IV 60 mg/min if no immediate access to ICU.

26
Q

Como fazer o manejo das questões psicossociais da epilepsia?

A

(1) Many patients with epilepsy are completely normal between seizures and able to live highly successful and productive lives. Many patients with epilepsy harbor fears such as the fear of becoming mentally retarded or dying during a seizure. The Epilepsy Foundation of America (www .epilepsyfoundation.org) is a patient advocacy organization and a useful source of educational material, as is the Web site www.epilepsy.com.
(2) Depression occurs in ∼20% of patients, and the incidence of suicide is higher in epileptic patients than in the general population. Depression should be treated through counseling or medication.
(3) However, a significant number of patients die from accidents, status epilepticus, and a syndrome known as sudden unexpected death in epileptic patients (SUDEP), which usually affects young people with convulsive seizures and tends to occur at night
(4) Federal and state legislation is designed to prevent employers from discriminating against patients with epilepsy, and patients should be encouraged to understand and claim their legal rights.
(5) Loss of driving privileges is one of the most disruptive social consequences of epilepsy.In general, most states allow patients to drive after a seizure-free interval (on or off medications) of between 3 months and 2 years.
(6) Thus, depending on the type and frequency of seizures, many patients need to be instructed to avoid working at heights or with machinery, or to have someone close by for activities such as bathing and swimming.

27
Q

Quais os cuidados especiais com epilepsia nas mulheres?

A

(1) Drugs such as carbamazepine, phenytoin, phenobarbital, and topiramate can significantly decrease the efficacy of oral contraceptives via enzyme induction and other mechanisms. Patients should be advised to consider alternative forms of contraception, or their contraceptive medications should be modified to offset the effects of the antiepileptic medications
(2) Since the potential harm of uncontrolled convulsive seizures on the mother and fetus is considered greater than the teratogenic effects of antiepileptic drugs, it is currently recommended that pregnant women be maintained on effective drug therapy.
(3) Patients should also take folate (1–4 mg/d), since the antifolate effects of anticonvulsants are thought to play a role in the development of neural tube defects, although the benefits of this treatment remain unproved in this setting.
(4) The overall incidence of fetal abnormalities in children born to mothers with epilepsy is 5–6%, compared to 2–3% in healthy women. Part of the higher incidence is due to teratogenic effects of antiepileptic drugs, and the risk increases with the number of medications used (e.g., 10–20% risk of malformations with three drugs) and possibly with higher doses. A recent metaanalysis of published pregnancy registries and cohorts found that the most common malformations were defects in the cardiovascular and musculoskeletal system (1.4–1.8%). Valproic acid is strongly associated with an increased risk of adverse fetal outcomes (7–20%).
(5) Enzyme-inducing drugs such as phenytoin, carbamazepine, oxcarbazepine, topiramate, phenobarbital, and primidone cause a transient and reversible deficiency of vitamin K–dependent clotting factors in ∼50% of newborn infants. Although neonatal hemorrhage is uncommon, the mother should be treated with oral vitamin K (20 mg/d, phylloquinone) in the last 2 weeks of pregnancy, and the infant should receive intramuscular vitamin K (1 mg) at birth.
(6) Given the overall benefits of breast-feeding and the lack of evidence for longterm harm to the infant by being exposed to antiepileptic drugs, mothers with epilepsy can be encouraged to breast-feed. This should be reconsidered, however, if there is any evidence of drug effects on the infant such as lethargy or poor feeding.

28
Q

Qual a tríade da síndrome de Lennox-Gastaut?

A

(1) Multiple seizure types (usually including generalized tonic-clonic, atonic, and atypical absence seizures); (2) an EEG showing slow (<3 Hz) spike-and-wave discharges and a variety of other abnormalities; and (3) impaired cognitive function in most but not all cases

29
Q

Qual a tríade da síndrome de West?

A

(1) Spams; (2) an EEG showing hypsarrythmia; and (3) developmental delay

30
Q

Quais os mecanismos de inicio e propagação das crises epilépticas?

A

The initiation phase is characterized by two concurrent events in an aggregate of neurons: (1) high-frequency bursts of action potentials and (2) hypersynchronization
With sufficient activation there is a recruitment of surrounding neurons via a number of synaptic and nonsynaptic mechanisms, including: (1) an increase in extracellular K+, which blunts hyperpolarization and depolarizes neighboring neurons; (2) accumulation of Ca2+ in presynaptic terminals, leading to enhanced neurotransmitter release; and (3) depolarization-induced activation of the N-methyl-D-aspartate (NMDA) subtype of the excitatory amino acid receptor, which causes additional Ca2+ influx and neuronal activation; and (4) ephaptic interactions related to changes in tissue osmolarity and cell swelling. The recruitment of a sufficient number of neurons leads to the propagation of seizure activity into contiguous areas via local cortical connections, and to more distant areas via long commissural pathways such as the corpus callosum