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Epilepsy care often intersects with mental health care. The chapter begins with nonepileptic events.This commonly encountered diagnosis is frequently on the differential for people with new onset seizures. Prompt recognition of a nonepileptic diagnosis can lead to early evidence-based treatment with cognitive behavioral therapy. Moreover, a nonepileptic event diagnosis can avoid inappropriate treatments such as antiseizure medicines (ASMs). It is critical to understand a nonepileptic event diagnosis does not mean that the events are not real or the patient is faking. Specific diagnostic clues for nonepileptic events are thoroughly discussed. The other section of the chapter explores the management of comorbid psychiatric diagnoses in patients with epilepsy and nonepileptic events. The use of most psychiatric medications, including stimulants, can be considered without affecting an epilepsy patient’s treatment plan. An understanding of psychiatric medication and ASM interaction can guide drug selection. As psychiatric diagnoses negatively affect epilepsy patients’ quality of life, prompt recognition and compassionate care can improve your patient’s overall health care.
The use of imaging in epilepsy care is a powerful tool to show a patient why they have epilepsy. You can use it to correlate your semiology history with an imaging finding. Magnetic resonance imaging (MRI) is the most widely used modality for epilepsy care. Ordering an epilepsy protocol MRI on a 3 Tesla (3T) machine increases the likelihood of finding an epilepsy’s etiology. Common findings include stroke, tumor (i.e., ganglioglioma), hippocampal sclerosis, encephalocele, cortical dysplasia, heterotopia, polymicrogyria, or cavernoma. For MRI-negative epilepsy, additional tests to identify the epileptogenic region include PET (positron emission tomography) and SPECT (single photon emission computed tomography). Functional MRI (fMRI) can delineate brain areas critical for specific functions.Lastly, the use of CT (computed tomography) is limited to identification of acute findings like hemorrhage and tumors in new onset seizures.
An electroencephalogram (EEG) is a critical tool in epilepsy diagnosis. The three common EEG durations are 25 minutes, 1 hour, and 24 hours. One-hour EEGs are superior in showing epileptiform abnormalities, while 24-hour EEGs are used to characterize seizure and nonepileptic event semiology and guide treatment of status epilepticus. The term EEG montage refers to the way EEG electrodes are ordered for interpretation. Odd numbered electrodes are on the left, with even numbered on the right. Smaller numbers are closer to the midline, while z means the electrode is on the midline.This chapter will explore the numerous normal and variant findings like posterior dominant rhythm (PDR) and wicket spikes. Epileptiform findings like sharp waves or seizure patterns are indicative of epilepsy. Slowing or increased amplitude can indicate cerebral changes that are not epileptiform.Electroencephalogram reports should concisely accurately convey both the electrical findings and their clinical relevance to patient care. Electroencephalogram reports should indicate an epilepsy diagnosis only when clear electrical evidence exists.
Pediatric epilepsy is a broad topic with specific syndromes highlighted in this chapter. A thorough discussion for the evaluation and management of febrile seizures is first. The chapter then transitions to a discussion of high-yield epileptic and developmental encephalopathies. Examples include diagnoses like infantile spasms, Dravet syndrome, and FIRES (febrile infection-related epilepsy syndrome). The chapter than progresses toward the self-limiting epilepsy of childhoods and a discussion of clinical features, management, and long-term prognosis.The generalized epilepsies are then discussed since they commonly commence in the pediatric time period. High-yield EEG examples are provided as they so frequently confirm the diagnosis. Specific neurocutaneous syndromes are next reviewed in a concise table that defines key differences and diagnostic criteria.Lastly, the two diagnoses of Rasmussen’s encephalitis and hemimegaloencephaly that can benefit from early consideration of hemispherectomy are reviewed.
Around a third of patients have drug-resistant epilepsy (DRE). This is crucially and easily determined if a patient continues to have seizures after being on two adequately dosed and appropriately selected antiseizure medicines (ASMs). For these patients, your initial efforts to make a specific and localized diagnosis will inform next treatment decisions. If a patient presents suddenly with DRE, it is key to assess for a possible autoimmune cause, as a separate treatment pathway should be considered. Otherwise, consider epilepsy surgery as an effective treatment. These treatments include brain resections and neuromodulation. Minimally invasive techniques have recently become more common, including laser surgery as well as stereotactically placed depth electrodes. Given the prevalence of neurostimulators, consideration for obtaining MRIs in patients with these devices is addressed, as pathways exist for all of these patients to safely undergo MRI testing.
Outpatient epilepsy care can often be straightforward. This is because around 65% of patients are seizure-free on their antiseizure medicines (ASMs). Management decisions that can simplify patient care prominently include minimizing ASM dosing complexity, preferably with monotherapy and once daily dosing. Choosing ASMs with lower side effect profiles and interaction potential is also ideal. Proactively addressing comorbidities of epilepsy and its treatment can improve quality of care. Some ASMs can negatively affect bone health so routine calcium and vitamin D supplementation is useful.Psychiatric care is comprehensively discussed in Chapter 9. The relative lack of need for ASM level monitoring is discussed. The decision to stop ASM therapy and how to do it is discussed. Lastly, the chapter concludes with a concise and thorough discussion of specific management considerations for women with epilepsy. Topics include the hormone cause of the catamenial pattern as well as ASMs and contraception, pregnancy, and breast feeding.
Status epilepticus (SE) is a neurological emergency defined as a continuous seizure or cluster of seizures lasting longer than 30 minutes. Because of increased mortality risk, SE is practically defined at 5 minutes. Clinically, SE can be separated into convulsive SE (CSE) or nonconvulsive SE (NCSE). For both diagnoses, the initial treatment of choice is a benzodiazepine, most commonly lorazepam 4 mg IV. Midazolam and diazepam (to a lesser extent) are also appropriate. If the status epilepticus continues, loading doses of fosphenytoin (20 mg/kg), levetiracetam (60 mg/kg), or valproate (40 mg/kg) are the next step in management. Continuation of SE past this point is considered refractory. For CSE, patients are almost always intubated and managed with IV anesthesia. For NCSE, intubation is often not needed at this point, with additional ASMs used instead to sidestep the risk associated with intubation and IV anesthesia. A key factor in guiding SE management is identifying the etiology (i.e., antibiotics for meningitis).Lastly, post cardiac arrest is briefly discussed as it is unfortunately commonly encountered.
Antiseizure medicines (ASMs) are medicines that reduce seizure frequency. They work by influencing cellular electrical channels like sodium or neurotransmitters like GABA. They are very effective, with 65% of patients seizure-free within the first three ASMs that they take. Unfortunately, less than 1% of people are seizure-free with ASMs after failing three ASMs. These data inform the definition of drug-resistant epilepsy, which is failing two ASMs. Newer ASMs have not been found to be more effective. Lamotrigine has been shown to be better tolerated compared to several other medications. Divalproex has been specifically shown to be most effective for generalized epilepsy, but has a challenging side effect profile, particularly in women. Side effects can be a cause of poor ASM compliance. Proactively accounting for patient factors like psychiatric comorbidity or renal impairment can lead to better tolerability and thus improved compliance. Monotherapy is typically preferred. Polytherapy can be considered in specific situations as well.
First-time seizures are a common part of neurology practice. Making an accurate and specific diagnosis is achievable by taking an excellent history. Clinicians should keep in mind that seizures are only part of the differential in a patient with a first-time event, with other diagnoses like syncope common as well. This history should focus on what the seizure feels like to the patient and looks like to observers. Two classification systems, the seizure semiology and International League Against Epilepsy (ILAE), exist to make communicating complex information easier. Key semiology history includes the presence or absence of auras, altered awareness, or convulsions. In addition to history, laboratory, EEG, and imaging data can inform to the specific patient diagnosis. If you determine that the patient has had a first-time seizure without a clear epilepsy diagnosis, you can tell them seizure that the recurrence risk is 40%. If you determine the patient has epilepsy, you can tell them that 50% of people are seizure-free with the first medication used. Patients should be reassured that they can live normal lives with most jobs being obtainable and family life being a possibility if the patient so chooses.
A seizure is defined as abnormally coordinated electrical activity that is clinically noted as abnormal sensations, movements, or behaviors. Epilepsy is defined as the tendency to have seizures, practically as two unprovoked seizures at least 24 hours apart or one seizure with a 60% chance of additional seizures. The two overall epilepsy types are generalized and focal. Focal epilepsy most commonly indicates a focal brain injury like traumatic brain injury or tumor. In contrast, generalized epilepsy is due to a genetic etiology, not necessarily inherited. An epilepsy diagnosis is clinically made and can be supported by typical EEG and MRI findings. Nearly 4% of people develop epilepsy, with the largest peak during later years and the second peak during childhood. Nearly 70% of people are seizure-free after five years, whether via control with medications or, less commonly, the epilepsy itself remits. Life expectancy for epilepsy patients is lower. A key contributor to epilepsy mortality is sudden unexpected death in epilepsy (SUDEP). Uncontrolled generalized tonic clonic seizures are a key risk factor for SUDEP.
A common care location for seizure and epilepsy patients is the emergency department and inpatient setting. A thorough history to discern a specific diagnosis and localization guides testing and treatment decisions. These decisions include the need for additional imaging/laboratory testing and whether to start an antiseizure medicine (ASM). Electroencephalograms (EEGs) are often required with time length depending on clinical question. If an epilepsy diagnosis is certain without clear localization, one hour of testing is preferred. If the question (or diagnosis) is status epilepticus, 24-hour EEG is indicated. For patients with known epilepsy, ASM alteration can occur for seizures with appropriate compliance. If there is noncompliance, an ASM increase may not be required. The perioperative period requires ASM continuation, although the administration route may change. Patients with renal or hepatic impairment often necessitate dose adjustment. Specific situations like alcohol withdrawal seizures have accepted paradigms to follow and are discussed. Lastly, patients in epilepsy monitoring units (EMUs) are hospitalized for diagnostic reasons with management needs different than other epilepsy patients.
Epilepsy is a frequently encountered disease, affecting 1-2% of the population, but one that is often mistreated due to misunderstandings of specific diagnoses and treatments. This practical manual provides a succinct and clinically relevant reference of routine clinical epilepsy care. The book is designed around the four main aspects of an epilepsy patient's care: first seizure, inpatient epilepsy care, outpatient epilepsy care, and diagnostic modalities. These four aspects are carefully delineated to illustrate the key differences in best practice management. Rational use of EEG and imaging testing is thoroughly covered to guide improved utility of these results. Caring for the mental health of epilepsy patients is also covered, as this is pertinent at every stage and location of epilepsy patient care. An excellent resource for neurology trainees at the resident, fellow, and medical student level as well as advanced practice providers.
The insula is known to play a central role in the regulation of autonomic function, although the precise nature and localization of such functions have not been conclusively identified. Autonomic and particularly cardiac disturbances are strongly implicated in sudden unexpected death in epilepsy (SUDEP). Herein, we discuss the contribution of the insula to seizure-related cardiac dysfunction and SUDEP. We also present two illustrative cases of intractable epilepsy patients with structural damage to the insula who succumbed to SUDEP. Both had progressive changes in heart rate variability (HRV), a standard measure of cardiac autonomic function, which noninvasively reflects sympathetic and parasympathetic balance using EKG recordings. In addition, further evidence of insular resection leading to autonomic changes is suggested in patients with intractable epilepsy and radiological evidence of insular damage after epilepsy surgery. The presence of intrinsic insular lesions or acquired insular damage in patients with refractory epilepsy may predispose to cardiac dysfunction and contribute to SUDEP pathomechanisms; however, a definitive association to SUDEP is still undetermined.
There is significant mortality and morbidity associated with medication-resistant epilepsy, with attendant major impact on physical, psychological, emotional, socioeconomic and other aspects of the lives of persons with epilepsy and their families. Emphasis on these issues is an imperative that is often underappreciated or overlooked, with subsequent far-reaching consequences that may be avoidable. Young adults with active epilepsy from low socioeconomic groups are more likely to die prematurely, and important contributors to mortality include comorbid disorders, such as cardiovascular diseases, cancer and unintentional injuries [1]. Notably, deaths from direct epilepsy-related causes occur in about 10% of cases, but are likely to be underestimated [2]. In this chapter, we examine the risks, types, impact and governing factors of mortality and morbidity in the population of persons with intractable epilepsy. We also discuss the commonest category of death in the intractable population – sudden unexpected death in epilepsy (SUDEP).
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