Unit 6: Brain, Mind and Behavior ~ Patient-Oriented Problem Solving (POPS) ~ EPILEPSY ALL CASES + ANSWER KEY (incl. pretest & posttest) Pages Learning Objectives and Reading Assignments .….…..2 Case 1: Devon Brown................................................ 3-4 Case 2: Henry Braithwaite ......................................... 7-8 Case 3: Jessica Rodriguez .................................. 12-13 Case 4: Lisa Martin ………..…………………………16 Learning Objectives: [071.01] Define the terms “seizure” and “epilepsy”. [071.02] Describe the differential diagnosis of a patient with recurrent, brief, stereotypical spells / episodes. [071.03] Explain the clinical and electrographic findings and clinical management in a specific generalized epilepsy syndrome and a specific localization-related epilepsy syndrome. [071.04] Explain the diagnostic criteria of status epilepticus, its common etiologies, and its management. Page 1 of 13 Reading Assignments: a) Required reading: Clinical Neurology 8e, David A. Greenberg, Michael J. Aminoff, Roger P. Simon; Chapter 12, “Seizures and Syncope” b) Pre-session required reading: Clinical Neurology 8e, David A. Greenberg, Michael J. Aminoff, Roger P. Simon; Chapter 12, “Seizures and Syncope”: 1. “Episodic Loss of Consciousness” 2. In the “Seizures” section, read the subsection labeled “Classification and Clinical Findings” c) Supplemental reading (not required): Harrison’s Principles of Internal Medicine, 18e, Dan L. Longo, Editor, Anthony S. Fauci, Editor, Dennis L. Kasper, Editor, Stephen L. Hauser, Editor, J. Larry Jameson, Editor, Joseph Loscalzo, Editor, Part 17, Neurologic Disorders: 1. Chapter 369, “Seizures and Epilepsy” Page 2 of 13 Case 1: Devon Brown Mrs. Brown presents with her six year old son, Devon, to Pediatric Neurology at SUNY Downstate. She demands to know if there is something wrong with him. She says that for the last six months, teachers complain that Devon daydreams in school. He saw the school psychologist who said that he has attention deficit disorder and was probably eating too many sweets. The patient’s birth history was normal, and all developmental milestones were successfully achieved on time. Devon had one febrile seizure at 14 months of age and was briefly put on phenobarbital. His 12 year old cousin has seizures. The patient started first grade this year and his teacher says that often she calls on him and he doesn’t answer, though the child isn’t aware of this. Mrs. Brown is worried he will fall behind. She says that she has noticed him staring at breakfast, but didn’t think it was important because it only lasts a few seconds. She also says that Devon is clumsier lately, dropping his cereal spoon or spilling his milk at the breakfast table. The child’s physical examination is normal. However, after 2 ½ minutes of hyperventilation in the office, Devon had an episode of staring and eye fluttering lasting 8 seconds. 1. What are these daydreaming spells? 2. What is the most likely diagnosis? 3. What would the EEG show during a seizure? 4. What is the drug of choice for this seizure type? 5. What are the side effects of this drug? 6. What other drugs might be effective? Devon does well on medication and maintains good grades in school. At age 13, medication is discontinued. At age 16, while playing outside, he fell to the ground, lost consciousness for about 1 minute, and had witnessed shaking of all four limbs with urinary incontinence. There was no head injury. After a few minutes, he was tired and confused. He fell asleep, and upon waking 1 hour later, felt better but wondered why his tongue hurt. Page 3 of 13 7. How would you characterize this episode? 8. Why did the patient’s tongue hurt? 9. What is the diagnosis now? 10. What further work up is required? 11. What is the drug of choice now? 12. What are the side effects of this drug? 13. What should you know about its metabolism? 14. If the patient is a woman, what other side effects can occur? 15. What other drugs might be considered? Page 4 of 13 Case 2: Henry Brathwaite Henry Brathwaite is a 34 year old man who is referred to a neurologist after his wife witnessed whole body shaking in his sleep. The patient denied ever having similar events, but did comment on frequent episodes of a funny sensation in his stomach, beginning about 10 years ago. On further questioning, he described the feeling as being on an elevator that is rising quickly. Sometimes he gets panicky or anxious after the rising feeling. His wife reports that with some of these episodes, he stares off into space. While he’s staring, she notices that he picks at his clothing and doesn’t answer her when she asks him what he’s doing. After this, he is tired and a little dazed. The patient couldn’t provide birth or childhood history, so he called his mother who told him that he had two febrile seizures at age 1. In addition, he reports a head trauma at age 7, when he fell off a swing and hit his head. He lost consciousness and only remembers waking up in the ER, but was discharged home after getting several stitches. Physical exam is normal, except for mild memory impairment (recall is 2/3 words) a small scar on the L parietal region. 1. What are the frequent episodes the patient has been having for 10 years? 2. What area of the brain is the most likely focus of this patient’s seizures? 3. What is the most likely epilepsy syndrome? 4. What tests would you perform to confirm your diagnosis? And what would you expect to see? 5. What are the drugs of choice for this syndrome? 6. What are other treatment options for patients with intractable temporal lobe seizures? The patient was prescribed oxcarbazepine, but didn’t tolerate it due to severe nausea. He was then started on levetiracetam and the episodes of gastric rising and panic subsided. His wife confirmed that staring spells had stopped. Three months later, his wife awoke to convulsive activity lasting 5 minutes, after which the patient didn’t rouse. She called 911, and as EMT arrived, another seizure occurred, within 15 minutes of the first. 7. What is happening? Page 5 of 13 8. What should be done? The patient is given one dosage of medication intravenously, pushed slowly, but continues to seize. He’s given another dosage of medicine, but his seizures (which have gone on for several minutes now) do not abate. 9. What medicine was the patient given? What is it’s mechanism of action? 10. What should be done now? 11. What plans should be made? The patient is given 20 mg/kg of FPHT but seizures continue. 12. What should be done now? If this next step doesn’t stop seizures, what should follow? 13. Why is intubation required? 14. If status continues, what are the next options? 15. What other therapies are available to treat status epilepticus? Over the next 24 hours, burst suppression is achieved, and over another 24 hours, the IV propofol gtt is decreased and ultimately removed. The patient is extubated and transferred to the floor on oral phenytoin. The next day, the levetiracetam level (which was drawn upon admission) returns and is <2 (therapeutic range 15-40). The patient admits that he stopped taking his pills one week ago because he thought he was “cured.” 16. What is the most common cause of status epilepticus in patients with epilepsy? 17. What could be done now? Page 6 of 13 Case 3: Jessica Rodriguez Jessica Rodriguez is a 16 year old young woman who is brought to the neurology clinic by her mother because she wants to know if her daughter had a stroke. Mrs. Rodriguez complains that her daughter has suddenly become really clumsy and is dropping things from her hands “all the time.” Jessica reports that she is only really clumsy in the morning, dropping the tube of toothpaste, her toothbrush, and sometimes even her breakfast. She also says sometimes she twitches while riding in the bus to school, especially on sunny days. Birth history and development are normal and the patient is an honor roll student. She was never told she had “staring spells” in school. Her maternal aunt used to be clumsy too, but takes some medicine for it. On exam, the patient is articulate and intelligent, and neurologically normal. 1. Why is the patient “clumsy” in the morning and dropping things from her hands? 2. What is the most likely syndromic diagnosis? 3. What are the seizure types that comprise this syndrome? 4. Why does she twitch on the ride to school, “especially on sunny days?” 5. What are the most common provoking factors for patients with this syndrome? 6. What are the EEG findings in this syndrome? 7. What would you expect to find on an MRI in this patient? 8. What are the drugs of choice for this seizure type? And what are their side effects? 9. What drugs should be avoided in patients with this syndrome? 10. What are some special considerations in this population of patients who are commonly teenagers upon diagnosis? On further questioning, Jessica denies ever having a seizure, but responds that she did wake up with a tongue bite once, two months ago. She was at her friend’s house for a sleepover and stayed up until 4 am, but was scared to tell her mother since she had choir practice the next morning and her mom didn’t want her going to the sleepover in the first place. She never bit her tongue before, and remembers being really, really tired that next morning, but figured it was just because she stayed up all night with her friend. 11. What occurred during the patient’s sleepover? 12. What are the drugs of choice now? Page 7 of 13 13. What is the prognosis for patients with this syndrome? Page 8 of 13 Case 4: Lisa Martin A 29 year old woman, Lisa Martin, comes to your office with her husband, Chris, who complains that she’s having nightmares every night. Chris says that for the last 4 years he can’t sleep because Lisa’s screaming in her sleep every night. The patient doesn’t remember these and denies that she’s having bad dreams. Her husband says that her episodes are now frequent, sometimes many per night. He awakens to her screaming, sees her looking anxious, and then complains that she kicks him as she thrashes about, before falling back to sleep. He has a job as a crossing guard and is having trouble staying awake during the day, so he wants you to stop her nightmares. Birth history and development are normal. The patient saw a psychiatrist since her husband thinks she’s having panic attacks, and was prescribed Zoloft 50 mg two months ago, but she’s still screaming every night. Neurologic exam is normal. 1. What is the differential diagnosis of this patient’s events? 2. What is the work up? The patient was referred for video EEG monitoring and three typical events were captured overnight. Clinically, there was sudden screaming from sleep lasting 2-3 seconds, followed by a facial expression of panic and fear, followed by pelvic thrusting and bicycling leg movements. The whole event lasted 20-25 seconds, and was immediately followed by a return to sleep. In the morning, after the events, the patient had no recall of any of them, and felt she slept soundly. The EEG showed muscle and movement activity during the events, but no clear seizure activity was present. 3. What is the diagnosis of these events? 4. What are the EEG findings in patients with seizures from this area? 5. What are other manifestations of seizures from this area? 6. What are some common etiologies of epilepsy syndromes from this area? 7. What is the treatment of this patient’s epilepsy? 8. If MRI reveals a focal cortical dysplasia in the right frontal lobe, what other treatment options could be considered? Page 9 of 13 Unit 6: Epilepsy POPS Pretest 1. A 37 year old woman was brought to the Emergency Department after her friend saw her “pass out and shake” on the subway. Differential diagnosis includes: a. Seizure b. Cardiac arrhythmia c. Non-epileptic seizure d. Syncope e. A and C only f. A-D 2. The patient described in #1 is found to be confused by ED physicians. This feature is most indicative of: a. Seizure b. Cardiac arrhythmia c. Syncope d. A and C only e. A, B, and C 3. The following are all examples of primary generalized seizures except: a. b. c. d. Drop attacks in a 3 year old boy with Lennox-Gastaut who wears a helmet. Staring spells in a 9 year old girl who is an A student. Whole body shaking preceded by a feeling of fear in a 23 year old man. Whole body shaking preceded by upper body jerks in a 17 year old girl. 4. A 35 year old woman presents after having her first generalized tonic-clonic seizure. Her only history includes brief episodes of fear and panic for the last 20 years which have not responded to Zoloft or Prozac. What do these episodes of fear and panic most likely represent: a. Generalized Anxiety Disorder b. Panic disorder c. Simple partial seizures d. Complex partial seizures 5. A 47 year old man with PMH of DM and HTN is brought to the ER after being found confused in the park. On neurologic exam, he is found to have right arm weakness and a stroke code is called. What is the differential: a. Infarct in the L MCA territory b. Intracerebral hemorrhage c. Partial seizure d. A and B e. A, B and C Page 10 of 13 Unit 6: Epilepsy POPS Pretest ANSWER KEY 1. A 37 year old woman was brought to the Emergency Department after her friend saw her “pass out and shake” on the subway. Differential diagnosis includes: a. Seizure b. Cardiac arrhythmia c. Non-epileptic seizure d. Syncope e. A and C only f. A-D ANSWER: (f) Generalized tonic clonic (GTC) seizures are nearly always associated with loss of consciousness and bilateral limb convulsive activity. (In rare cases, patients with convulsive seizures of frontal lobe origin, specifically the supplementary motor area, may remain awake.) Cardiac arrhythmias may cause a patient to lose consciousness. Depending on the degree of hypoxia, myoclonus or even convulsive movements of the limbs may ensue. This is called convulsive syncope and can be very difficult to differentiate from GTC seizure activity. The presence of classic provoking features (venipuncture, strong emotions) and the rapid return to normal mentation point toward convulsive syncope, while the lack of electrical seizure activity on video EEG recording (with EKG recording demonstrating the rhythm abnormality) is diagnostic. Finally, in non-epileptic seizures, patients are frequently witnessed to pass out and shake by observers. While historical details can raise the suspicion of NES, video EEG recording of an event is often diagnostic. 2. The patient described in #1 is found to be confused by ED physicians. This feature is most indicative of: a. Seizure b. Cardiac arrhythmia c. Syncope d. A and C only e. A, B, and C ANSWER: (a) Post event confusion is a powerful tool differentiating seizures from syncope. With syncope (or cardiac arrhythmias leading to syncope), patients return to full lucidity rapidly, within 20-30 sec. Patients do not report feeling confused though they may report feeling “dazed” but this should resolve rapidly, within 30 sec. Following seizures, though, patients can have brief or more prolonged confusion, depending on the extent of the seizure. 3. The following are all examples of generalized seizures except: a. Drop attacks in a 3 year old boy with Lennox-Gastaut who wears a helmet. b. Staring spells in a 9 year old girl who is an A student. c. Whole body shaking preceded by a feeling of fear in a 23 year old man. Page 11 of 13 d. Whole body shaking preceded by upper body jerks in a 17 year old girl. ANSWER: (c) The feeling of fear preceding whole body shaking is an example of an aura. Auras are simple partial seizures and vary depending on the site of seizure onset. Some common temporal lobe auras are gastric rising and fear. Generalized seizures are not preceded by an aura, though rare patients may report a vague feeling of dizziness prior to their seizure. Drop attacks in a child with Lennox Gastaut represent either tonic or atonic seizures in this symptomatic generalized epilepsy syndrome. Staring spells in the 12 year old child most likely represent typical absence seizures. Upper body jerks progressing to whole body shaking are myoclonic seizures progressing to a generalized tonic clonic seizure in a 17 yo girl with Juvenile Myoclonic Epilepsy. 4. A 35 year old woman presents after having her first generalized tonic-clonic seizure. Her only history includes brief episodes of fear and panic for the last 20 years which have not responded to Zoloft or Prozac. What do these episodes of fear and panic most likely represent: a. Generalized Anxiety Disorder b. Panic disorder c. Simple partial seizures d. Complex partial seizures ANSWER: (c) Simple partial seizures (SPS) vary in symptomatology depending on the site of onset. SPS of occipital origin may be characterized by visual hallucinations, while those of parietal onset may consist of contralateral arm and face paresthesias. As above, classic temporal lobe auras include fear or panic, and may be misdiagnosed as anxiety if they are the only seizure type present. However, the SPS would not respond to common anxiety medicines (except benzodiazepines) and would not be described as brief episodes rather than more prolonged feelings. Finally, the presence of the GTC seizure should raise suspicion that the fear/panic is not of psychiatric cause. Generalized anxiety disorder is characterized by excessive anxiety and worry which is difficult to control. Panic disorder consists of episodes of sudden onset of fear or discomfort, associated with palpitations, shortness of breath, an urge to flee or escape and feelings of impending doom. Finally, complex partial seizures are associated with alteration of awareness during the events. 5. A 47 year old man with PMH of DM and HTN is brought to the ER after being found confused in the park. On neurologic exam, he is found to have right arm weakness and a stroke code is called. What is the differential: a. Infarct in the L MCA territory b. Intracerebral hemorrhage c. Partial seizure d. A and B e. A, B and C ANSWER: (e) Certainly MCA infarcts and intracerebral hemorrhages affecting the area of left motor cortex responsible for right arm motor function could both cause right arm Page 12 of 13 weakness. Depending on the size of the hemorrhage, there may also be confusion, though confusion would not be typical of a left MCA infarct. Focal epilepsy arising from the same area could be associated with this presentation. On occasion, patients may present with transient focal weakness following a seizure, or Todd’s paralysis, which occurs because of excessive inhibition in the area surrounding the seizure focus. Though coined “Todd’s paralysis”, patients with epilepsy may manifest other “Todd’s” symptoms that are not motor depending on the seizure onset zone, e.g. aphasia if language centers are involved or visual field deficits if the occipital lobe is involved. Page 13 of 13