Case Files Neurology - part 4 docx

[15.3] A 35-year-old man is diagnosed with a seizure disorder. There is no history of trauma or medical condition. What is the most common type of seizure in adults with epilepsy? A. Absence seizures B. Complex partial seizures C. Grand mal seizures D. Todd paralysis Answers [15.1] A. Lip smacking, chewing, and swallowing are common findings in complex partial seizures. [15.2] B. Absence seizures are typified by staring off episodes without con- scious awareness. [15.3] B. The most common type of seizure with epilepsy in adults is com- plex partial seizures. CLINICAL CASES 133 CLINICAL PEARLS ❖ Complex partial seizures are the most common form of seizure in adults. ❖ The differential of complex partial seizure includes absence seizures and also multiple medical disturbances, including tran- sient ischemic attacks. ❖ In approximately one-third of women with seizures, there is a rela- tionship between seizures and the menstrual cycle, and the seizure frequency can double. This is often called catamenial seizure exacerbation or catamenial epilepsy. REFERENCES Bazil CW, Morrell MJ, Pedley TA. Epilepsy. In: Rowland LP, ed. Merritt’s neurol- ogy, 11th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2005:990–1014. Murro AM. Complex partial seizures. Available at: http://www.emedicine.com/ NEURO/topic74.htm. Accessed March 20, 2007. Schacter SC. Epilepsy. In: Evans RW, ed. Saunders manual of neurologic practice. Philadelphia, PA: Saunders/Elsevier; 2003:244–265. This page intentionally left blank ❖ CASE 16 A 52-year-old healthy white male is brought to the emergency room (ER) after he has had a car accident in which he hit the dividing rail. He apparently did not suffer any significant injuries and at the time of the examination was fully awake. On further questioning, he reported driving on the highway and then without any warning hit the rail. He immediately stopped the car. His wife, who was in the car with him, stated that he suddenly stopped responding in the middle of the sentence, and the car started to go to the left. When they hit the rail, he woke up and braked the car. He denies feeling lightheaded, nausea, or warning prior to the loss of consciousness. He also denied feeling ill or dis- oriented on awakening, and he was immediately aware of his surroundings. There was no evidence of tongue biting or urinary incontinence, or convulsive jerking. In the ER, the patient had an unremarkable examination, laboratory work, and CT scan of his head. He was admitted for 24-hour observation, and a neurologist was consulted. The patient admitted to two previous syncopal episodes, both in his office, and both without provocation. On one occasion he was seated, on the second occasion he was standing and suffered a fall. In nei- ther case did he have any warning or postevent confusion. After the second episode he scheduled an appointment with his family doctor but did not have the chance to see him prior to the accident. On review of systems, the patient complained of frequent fatigue and lack of energy over the last year but attrib- uted it to work schedule and lack of adequate exercise. His detailed neurologic examination showed no abnormal findings. ◆ What is the most likely diagnosis? ◆ What is the next diagnostic step? ◆ What is the next step in therapy? ANSWERS TO CASE 16: Cardiogenic Syncope Related to Bradycardia Summary: This 64-year-old man presents with an acute and temporary loss of consciousness and history of two similar episodes in the past. These episodes were not associated with warning signs or symptoms nor followed by persist- ent confusion, weakness, or findings on examination. ◆ Most likely diagnosis: Cardiogenic syncope related to bradycardia ◆ Next diagnostic step: Cardiac evaluation and invasive electrophysiology ◆ Next step in therapy: Pacemaker placement Analysis Objectives 1. Know common causes of acute loss of consciousness or syncope. 2. Describe the workup for syncope. 3. Be familiar with the management of syncope. Clinical Considerations In this case, the patient suffered an acute loss of consciousness that was with- out any provocation or premonitory symptoms including nausea, sweating, or abdominal discomfort. He did not become pale or ashen according to his wife. The event occurred while he was sitting in his car, and he regained conscious- ness quickly. These findings are less consistent with a vasovagal or orthostatic syncope because it was not associated with a change in position from sitting or lying down to standing or upright and was not associated with signs and symptoms suggestive of low blood pressure. His wife denied any convulsions or postictal confusion, and the patient denied any premonitory symptoms. On examination there was no evidence of tongue biting or urinary incontinence, making a good case against an epilep- tic seizure. Therefore the most likely diagnosis in this patient is cardiogenic syncope. An evaluation should be performed including a tilt-table testing. The patient should also have an MRI of the brain with and without contrast and electroencephalograph (EEG). Routine laboratory tests should be undertaken to assess for metabolic or endocrine problems; complete blood count (CBC) for evidence of anemia or infection. An electrocardiograph (ECG) and 24-hour Holter monitoring are usually obtained. After an evaluation and follow-up, the patient may have repeated bouts of syncope, which requires more extensive evaluation and therapy. This particular patient experienced repeated syncopal episodes. There was a negative workup, an invasive electrophysiological study 136 CASE FILES: NEUROLOGY was ordered, and the patient was diagnosed with “sick sinus syndrome.” The treatment was an implanted dual chamber pacemaker, and the patient was dis- charged home with resolution of syncope and fatigue. APPROACH TO CARDIOGENIC SYNCOPE Definitions Syncope: A sudden brief loss of consciousness (LOC). Orthostatic syncope: Syncope associated with a sudden change in position from supine to sitting up or sitting to standing up. Electroencephalography: The neurophysiologic measurement of the elec- trical activity of the brain by recording from electrodes placed on the scalp or, in special cases, subdurally or in the cerebral cortex. Epilepsy: Neurologic condition that makes people susceptible to seizures. A seizure is a change in sensation, awareness, or behavior brought about by a brief electrical disturbance in the brain. Tilt-table testing: Test to evaluate how the body regulates blood pressure in response to some very simple stresses while lying on a special table. It involves cardiac monitoring (ECG), blood pressure monitoring, and intravenous (IV) infusion of drugs to stress the system. Sick sinus syndrome: A type of bradycardia in which the sinoatrial (SA) or sinus node is not working as it should. Clinical Approach Syncope can result from a variety of cardiovascular and noncardiovascular causes. The most common pathophysiologic mechanism for cardiovascular syncope is decreased cerebral blood flow with resultant cerebral hypoxia, which prompts immediate and forceful rearrangement of posture to ensure an adequate flow of the blood to the central nervous system (CNS). Decreased cerebral blood flow is most commonly caused by decreased cardiac output (CO) and arrhythmias. Heart rate below 35 and above 150 beats/min can cause syncope even without the presence of cardiovascular disease. Although brady- cardia can occur at any age, it occurs most frequently in the elderly and is usu- ally caused by ischemia or fibrosis of the conduction system. Digitalis, beta-blockers, and calcium channel blockers can also cause bradycardia. However, supraventricular or ventricular tachyarrhythmias that cause syncope can be related to cardiac ischemia or electrolyte abnormalities. Among the most common non–cardiac-related mechanisms of syncope are peripheral vasodilation, decreased venous return to the heart, and hypovolemia. History is critical in making the correct diagnosis in the case of syncope. It should guide the evaluation and not the other way around. Syncope of cardiac etiology occurs suddenly and ends abruptly without warning or post-event CLINICAL CASES 137 confusion. The postural changes are often not necessary for the termination of the event. This presentation is the most common sequela of the arrhythmia and requires careful electrophysiological study as well as cardiac catheterization to rule out ischemia as the cause of the conduction defect. Exertional syncope suggests cardiac outflow obstruction, mainly caused by aortic stenosis, and therefore warrants echocardiogram as the first step in eval- uation. Cough or micturition syncope as well as syncope occurring during any natural or iatrogenic Valsalva maneuver, implicates decrease in venous return and can be present even in healthy individuals. Vasovagal syncope is not a serious or life-threatening condition but is an abnormal reflex. This results in a drop in blood pressure leading to decreased blood flow to the brain resulting in dizziness or fainting. The mechanism of vasovagal syncope is the subject of a great deal of research. It is typically pre- cipitated by unpleasant physical or emotional syncope most commonly pain, sight of blood or gastrointestinal discomfort. It usually occurs in the upright position, and the patient describes a sensation of lightheadedness, dimmed vision and hearing, depersonalization, sweating, nausea, and increased heart rate. The patient usually wakes up immediately after the event but if prevented from obtaining a supine position, usually by well-wishing observers, syncope can be prolonged and accompanied by brief convulsions (so-called convulsive syncope). This almost always precipitates neurologic consult for the new onset seizures. The picture is often complicated by the spontaneous micturition, which is widely believed to be a sign of epileptic activity. Contrary to popular belief, incontinence can be the result of any syncopal episode if the patient happens to have a full bladder prior to the event. Most often, if clearly elucidated, a pure vasovagal episode in the patient without any risk factors for cardiovascu- lar disease and a normal post-event physical examination does not require any further evaluation. Syncope caused by epileptic seizure is abrupt in onset and most of the time associated with focal or generalized tonic or clonic muscular activity, clearly described by the witnesses. Tongue biting and urinary incontinence are common but by no means required for the diagnosis. Most of the time the patient experiences at least brief postictal confusion, making it the single most important sign for the differentiation from other causes of syncope. In patients with known epilepsy, defined as recurrent seizures, between which there is complete recovery, evaluation should be centered over the antiepileptic medications. Blood levels should be checked for the current medications and if low, the cause or causes need to be elucidated. The most common causes are noncompliance or introduction of the new medication that interferes with the absorption or metabolism of the current antiepileptic drug or drugs. Frequently, however, recurrent seizures happen despite adequate blood level of antiepileptic medication. It can be a result of concurrent acute illness, behavioral changes (staying up all night, skipping meals, or drinking alcohol) or simply inadequate seizure control. 138 CASE FILES: NEUROLOGY Orthostatic syncope has different etiology in elderly (e.g., over 50 years) and young patients. When occurring in the young it is most often confused with epilepsy because of age and absence of cardiovascular risk factors. Orthostatic syncope almost always happens with the sudden change of posture from lying or sitting to standing or after prolonged standing without moving. The classic example is a young soldier fainting during the military parade on a hot summer afternoon. When this happens in young and otherwise healthy individuals, it always requires a table-tilt test, because sequential measure- ments of orthostatic blood pressure changes in the clinic may not be enough. In the elderly, however, orthostatic syncope is often caused by hypov- olemia, or increased venous pooling as seen after prolonged bed rest. The other contributing factor in this population is polypharmacy that often includes combination of beta-blocker, loop diuretic, and nitrate; combining dehydra- tion, vasodilatation and delayed cardiac response to cause sudden orthostatic changes in blood pressure without adequate compensatory response. The other possibility for the orthostatic hypotension leading to the syncope is autonomic nervous system abnormality. By far the most common cause of dysau- tonomia is diabetic neuropathy, where interruption of the sympathetic reflex arc inhibits adequate adrenergic response to standing. The other less frequent causes of autonomic failure are amyloidosis, syphilis, spinal cord injury or syringomyelia, alcoholic neuropathy, or acute inflammatory demyelinating polyradiculoneuropa- thy (AIDP) also known as Guillain-Barré syndrome, which can all affect the peripheral or central autonomic pathways. Orthostatic hypotension is also one of the cardinal features of multiple system atrophy, an atypical Parkinsonian syn- drome, which consists of a variable combination of parkinsonism, cerebellar dys- function, dysautonomia, and pyramidal symptoms. However, orthostatic hypotension can be present later and often to a milder degree in idiopathic Parkinson disease, and often aggravated by the use of dopaminergic agents. Evaluation Patients suspected of cardiogenic syncope or any nonepileptic syncope should undergo an extensive evaluation including 12-lead ECG, two-dimensional- echocardiography, 24-hour Holter monitoring for arrhythmias, and possibly cardiac catheterization. Patients should have serial orthostatic blood pressure measurements to document a decrease in blood pressure or increase in heart rate with postural changes, which is associated with orthostatic syncope. Discussion Bradycardias are caused by two problems: disease of the sinus node or disease of the conduction system. The sinus node is the pacemaker of the heart. The electrical impulse that generates the heart beat arises in the sinus node. Disease of the sinus node, therefore, can result in the lack of sufficient electrical impulses (and thus a lack of sufficient heart beats) to maintain the body’s CLINICAL CASES 139 needs. Sinus node disease that leads to symptoms caused by a slow heart rate is called sick sinus syndrome. Most sinus node disease is related to simple deterioration in sinus node function caused by aging. Likewise, tachyarrhyth- mias caused by Wolff-Parkinson-White syndrome or prolonged-QT syndrome can also lead to insufficient cardiac output and syncope. Treatment In the case of syncope, diagnosis is the most difficult part. The treatment is only as effective as the diagnosis is correct. In the case of vasovagal syncope, treatment often is not required. Orthostatic hypotension can be treated by avoiding hypovolemia, electrolyte imbalance, and excess alcohol intake. If this is not enough increased salt intake and fludrocortisone can be recommended. If orthostasis is related to venous pool- ing in the legs, fitted elastic hose can enhance the venous return and cardiac output. Obviously, if seizures were found to be the cause of the syncope, they have to be treated with appropriate antiepileptic medications, and the patient needs to be referred to as epileptologist for further evaluation. Tachyarrhythmias are treated with variety of antiarrhythmic drugs, the discussion of which is beyond this case. Sick sinus syndrome, if symptomatic, is often treated with permanent pacing, to avoid an onset of fatal arrhythmia or sinus arrest. Comprehension Questions [16.1] A 22-year-old nursing student passes out when observing a woman giving birth. A. Seizure B. Vasovagal syncope C. Orthostatic hypotension D. Cardiogenic syncope [16.2] A 17-year-high school football player passes out on the field while run- ning practice sprints. A. Vasovagal syncope B. Exertional syncope C. Seizure D. Orthostatic hypotension [16.3] A 43-year-old woman with history of previous brain trauma is found unconsciousness in her home by a visiting neighbor. She has urinated on herself, and there is a small amount of blood and saliva coming from the side of her mouth. A. Cardiogenic syncope B. Vasovagal syncope C. Seizure D. Orthostatic hypotension 140 CASE FILES: NEUROLOGY Answers [16.1] B. This is most likely caused by a vasovagal reflex (drop in blood pres- sure) in response to painful or emotionally charged stimulus and is usually non–life threatening. [16.2] B. Exertional syncope is caused by insufficient cardiac output neces- sary to meet exertional demands. This is usually caused by cardiac out- flow obstruction often associated with aortic or subaortic stenosis and requires an echocardiogram. [16.3] C. Seizure is the most likely diagnosis given a prior history of brain trauma, which can predispose to a seizure focus. Although clinical signs such as incontinence and tongue laceration are not specific for seizure, in the context of a possible cerebral focus, seizure is the most appropriate answer. CLINICAL CASES 141 CLINICAL PEARLS ❖ It is prudent to ask for clarification when the patient complains of “dizziness.” ❖ Vertigo and lightheadedness should be differentiated because their evaluations are very different. ❖ Lightheadedness often includes dimmed vision, nausea, palpita- tions, and diaphoresis before syncope. ❖ Tongue biting and urinary incontinence is not pathognomonic for seizure activity, nor does the absence of those signs exclude epileptic seizures. REFERENCES Armour A, Ardell J. Basic and clinical neurocardiology. Oxford: Oxford University Press; 2004. Kosinski DJ, Wolfe DA, Grubb BP. Neurocardiogenic syncope: a review of patho- physiology, diagnosis and treatment. Cardiovasc Rev Rep 1993;14:22–29. Linzer M, Yang EH, Estes NA 3rd, et al. Diagnosing syncope. Part 1: value of history, physical examination, and electrocardiography. Clinical Efficacy Assessment Project of the American College of Physicians. Ann Intern Med 1997 Jun 15; 126(12):989–996. Linzer M, Yang EH, Estes NA 3rd, et al. Diagnosing syncope. Part 2: unexplained syncope. Clinical Efficacy Assessment Project of the American College of Physicians. Ann Intern Med 1997 Jul 1;127(1):76–86. This page intentionally left blank [...]... His friends took him to the emergency room ◆ What is the most likely diagnosis? ◆ What is the next diagnostic step? ◆ What is the most likely useful consultation? 144 CASE FILES: NEUROLOGY ANSWERS TO CASE 17: Pseudoseizure Summary: A 23-year-old man suddenly “seized” in all four extremities, remained conscious, complaining about pain and querying his surroundings ◆ ◆ Most likely diagnosis: Pseudoseizure... alopecia and tremor Beta-Blockers Beta-blockers have been used in the prophylactic treatment of migraine since 1972 The most commonly prescribed beta-blocker is propranolol (Inderal) 158 CASE FILES: NEUROLOGY The long-acting (LA) form is often prescribed, and the dose is usually increased until the blood pressure drops to 100/60 mmHg, and the pulse drops to 60 beats/min Once a beta-blocker has been instituted,... Gardner WN, Toone BK Pseudoseizures and asthma J Neurol Neurosurg Psychiatry 2003 May; 74( 5):639– 641 University of Michigan Adult health advisor: seizures Available at: http://www med.umich.edu/11ibr/aha/aha_seizure_crs.htm Updated 2005 This page intentionally left blank ❖ CASE 18 A 2 4- year-old white female has a 12-year history of headaches These headaches started in grade school, and the patient remembers... aura Migraine without aura [18.1] A 38-year-old man presents with right-sided neck pain and left-sided numbness of face, arm, and leg after a chiropractic manipulation of his neck [18.2] A 37-year-old woman presents with nasal congestion and post-nasal drip, complaining of bilateral pain above and around her eyes [18.3] A 21-year-old college student studying for final examinations complains of recurrent... or sensory changes ◆ What is the most likely diagnosis? ◆ What is the most likely next diagnostic step? ◆ What is the likely next step in therapy? 162 CASE FILES: NEUROLOGY ANSWERS TO CASE 19: Chronic Headache Summary: A 38-year-old white female has a 10-year history of daily headaches, located in the temples and often radiating to the neck The patient also reports a different headache approximately... daily or almost daily headache greater than 15 days a month The patient suffers from headaches at least 4 hours a day, and there is usually a history of episodic 1 64 CASE FILES: NEUROLOGY migraine during this chronic phase This transformation to a more chronic picture usually takes place over a 3- to 6-month period of time Chronic Tension Type Headache Patients with a history of episodic tension headaches... symptoms can be eliminated Comprehension Questions [17.1] A 35-year-old man is suspected to have pseudoseizure Which of the following is the best method to confirm the diagnosis? A B C D Resting EEG monitoring Initiation of anti-epileptic therapy and observation Psychiatric evaluation Video EEG monitoring [17.2] A 23-year-old man is noted to have tonic-clonic activity while yelling and screaming for a fire... ANSWERS TO CASE 18: Migraine Headache Summary: A 2 4- year-old white female has a 12-year history of monthly headaches that started in grade school The headache starts over the right eye and is preceded by flashing lights and zigzag lines Once the headache occurs, there is extreme nausea and vomiting, and the patient goes into a dark room to help with her head pain Generally, the headache lasts 4 to 6 hours,... [19.2] A 33-year-old woman is noted to have daily severe headaches Her physician prescribed botulinum toxin injections, which have been highly effective Which of the following types of headaches is most likely to be present? A B C D Migraine vascular headache Cluster vascular headache Cervical muscle spasm Tension headache [19.3] A 40 -year-old woman comes into the physician’s office with a 20-year history... brain imaging In difficult cases, ongoing EEG monitoring might be necessary, to assess brain physiological function and processing during the episode Psychiatric assessment is also very important A good rule is that bilateral seizure activity without confusion or unconscious (i.e., able to talk coherently to the examiner while their arms and legs are shaking), 146 CASE FILES: NEUROLOGY is rarely organic . step? ◆ What is the next step in therapy? 150 CASE FILES: NEUROLOGY ANSWERS TO CASE 18: Migraine Headache Summary: A 2 4- year-old white female has a 12-year history of monthly headaches that started. 17-year-high school football player passes out on the field while run- ning practice sprints. A. Vasovagal syncope B. Exertional syncope C. Seizure D. Orthostatic hypotension [16.3] A 43 -year-old. nonepileptic syncope should undergo an extensive evaluation including 12-lead ECG, two-dimensional- echocardiography, 2 4- hour Holter monitoring for arrhythmias, and possibly cardiac catheterization.