CMS Neuro 3 Answers

NEURO FORM 3 by Sergio Angulo 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39

E E E C A E E E D L A C K B B F IV ANTIBIOTIC C A C G C E Maybe E, not C A B C B B C E A E E D E E B E

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C D C D G K D C A E E 1. 42 yo woman alcoholic, severe burning paresthesias both feet, decreased vibratory sens both ankles 2 months after ttmt for pulm TBC. Drugs include INH, rifampicin, vit B6, vit B9. Failure to take which drug? E. Vit B6 INH competes with vitamin B6 (pyridoxine) in its action as a cofactor in the synthesis of synaptic neurotransmitters. Resulting dose-related neurologic side effects include peripheral neuropathy, ataxia, and paresthesia. Such side effects are uncommon in the absence of risk factors. Patients at increased risk for INH-induced neurotoxicity include: Older adults Individuals with chronic liver disease Individuals who are malnourished Individuals with HIV infection Individuals with renal failure Diabetics Alcoholics Pregnant or breastfeeding women Children The risk of neurologic side effects can be reduced by concomitant administration of pyridoxine (vitamin B6). The usual dose is 10 mg/day (in patients who do not have highrisk features) and 25 to 50 mg/day (in those with one or more risk factors) [8]. Treatment of INH-induced neuropathies with pyridoxine generally requires higher doses (100 to 200 mg/day). 2. 42 yo woman, confused state for possible 3 days. Hx lithium, haloperidol, clonazepam for psych condition. 99.5F, pulse 85/min, BP 140/90. Pupils reactive 2mm. Coarse tremor upper extremities. Increased deep tendon reflexes. Responds to vigorous stim. Speech slow slurred. Appears sleepy and perplexed. WBC 14000, Urea 60mg/dl, Creat 2.5mg/dl, lithium 3.5 (normal 0.6-1.2). In add to discontinue lithium, next step? E. Begin hemodialysis Lithium is excreted almost entirely by the kidneys and is handled in a manner similar to sodium. Lithium is freely filtered but over 60 percent is then reabsorbed by the proximal

tubules. Volume depletion or renal impairment from any cause increases lithium reabsorption. Examples of such conditions include gastrointestinal losses, acute decompensated heart failure, cirrhosis, and the administration of diuretics, nonsteroidal antiinflammatory drugs, or angiotensin converting enzyme inhibitors. With lithium poisoning, it is important to ask about dehydration, medications, and coingestants. Any condition causing dehydration, such as vomiting or diarrhea, fever or heat-related illness, anorexia or infection, may contribute to lithium toxicity. Neurologic findings develop late in acute lithium poisoning because time is required for the drug to be absorbed and to penetrate the central nervous system (CNS). Potential neurologic symptoms and signs include sluggishness, ataxia, confusion or agitation, and neuromuscular excitability, which can manifest as irregular coarse tremors, fasciculations, or myoclonic jerks. Severe lithium intoxication can lead to seizures, nonconvulsive status epilepticus, and encephalopathy. The treatment for lithium poisoning includes intravenous fluids to maintain the glomerular filtration rate and replace losses, gastrointestinal decontamination in selected circumstances (Whole bowel irrigation with polyethylene glycol (PEG), and hemodialysis in cases of severe toxicity. Perform hemodialysis for the following indications: Serum lithium concentration is greater than 4 mEq/L (or mmol/L), regardless of the clinical status of the patient. Serum lithium concentration is greater than 2.5 mEq/L (or mmol/L) and the patient manifests signs of significant lithium toxicity (eg, seizures, depressed mental status), has renal insufficiency or other conditions that limit lithium excretion, or suffers from an illness that would be exacerbated by aggressive IV fluid hydration (eg, heart failure) 3. 47 yo man, alcoholic, ER for confusion. Lives alone, disoriented for 4 hours. Empty container with sweet odor in apt. Creat 1.5mEq/l, HCO3 8mEq/l. ketones (-). Urianalysis, crystals. Cause? F. Ethylene glycol

Methanol and ethylene glycol are frequently found in high concentration in automotive antifreeze and de-icing solutions, windshield wiper fluid, solvents, cleaners, fuels, and other industrial products. Few conditions other than methanol and ethylene glycol intoxication cause a profound high anion gap metabolic acidosis (serum bicarbonate less than 8 meq/L (or 8 mmol/L)), and most of these conditions present in a characteristic fashion with a high serum lactate (eg, status epilepticus, profound shock, ischemic bowel) or diabetic ketoacidosis (table 2). Ethylene glycol metabolites target the kidney and lead to reversible oliguric or anuric acute kidney injury (acute renal failure), which in turn slows elimination of ethylene glycol [7]. The renal failure is primarily due to glycolate-induced damage to tubules, although tubule obstruction from precipitated oxalate crystals may contribute [8,9]. Hypocalcemia in ethylene glycol overdose results from calcium oxalate formation. With ingestions of either parent alcohol, a profound anion gap metabolic acidosis develops, which directly correlates with the accumulation of toxic acid metabolites [2,4]. Acidemia increases the ability of the toxic metabolites to penetrate cells, further depressing CNS function and causing a rapid downward spiral of hypoxia and acidemia [10]. Urine testing — Examination of the urine for oxalate crystals and fluorescence is frequently performed in patients with possible ethylene glycol poisoning, but care should be taken not to over-interpret positive or negative results. 4. 27 yo man. Hx 2w neck and left shoulder pain, radiating through lat surface of left arm to thumb and index. Decreased biceps reflex. Strength 4/5 extension left wrist. Sens light touch decreased left thumb and index. Dx?

C. C6 radiculopathy

5. 62 yo man. Hx 3y forgetfulness. Difficult remembering names. Progressive difficult with activities daily living. No head trauma, no drugs, no serious illness. Indifferent mood and affect. Oriented person and place. Disoriented in time. No auditory/visual halluc. Minimental 22/30. Which ttmt? a. Acetylcholinisterase inhibitors. Probable AD dementia is a syndrome of dementia defined by the following characteristics: Interference with ability to function at work or at usual activities A decline from a previous level of functioning and performing Not explained by delirium or major psychiatric disorder

Cognitive impairment established by history-taking from the patient and a knowledgeable informant; and objective bedside mental status examination or neuropsychological testing Cognitive impairment involving a minimum of two of the following domains: impaired ability to acquire and remember new information impaired reasoning and handing of complex tasks, poor judgment impaired visuospatial abilities impaired language functions changes in personality, behavior or comportment We suggest a treatment trial with a cholinesterase inhibitor for patients with mild to moderate dementia (MMSE 10-26) (Grade 2A). The choice between donepezil, rivastigmine, and galantamine can be based upon cost, individual patient tolerance, and physician experience, as efficacy appears to be similar. In patients with mild to moderate Alzheimer dementia (AD) who are interested in seeking therapy with vitamins or who are unable or unwilling to take memantine, we suggest supplementation with vitamin E (2000 IU daily) (Grade 2C). The benefits of vitamin E are likely to be modest and could be offset by combination therapy with memantine. Vitamin E is not recommended for other forms of dementia or for the prevention of AD. In patients with moderate to advanced dementia (MMSE 90 percent

Present in 10 lbs was recorded in 35 percent. 28. 32 yo woman breast CA metastasis to bone, brought to ER 12-hour hx severe pelvic pain and numbness. Medications hydrocodone. 99F, pulse 104/min, resp 18/min, BP 152/70. Decreased rectal sphincter tone. Strength 4/5 lower extremities. Decreased sens light touch in saddle distribution. Suprapubic fullness. Catheter yields to 800 mL of urine, resolves pain but no numbeness. MRI shows tumor on L4-S1 intrathecal. Next step management? B. IV administration of high dose Dexamethasone Management of patients with Neoplastic epidural spinal cord compression (ESCC) includes the immediate administration of glucocorticoids in nearly all patients, followed by surgery, external beam radiation therapy (EBRT), or stereotactic body radiotherapy (SBRT). Systemic therapy may be beneficial in patients with chemosensitive tumors. High-dose corticosteroid therapy is generally considered to be part of the standard regimen for ESCC, despite limited documented evidence of benefit and a significant risk of serious side effects [1,16,17]. Several studies have suggested that lower doses can be effective but they have not been assessed in randomized trials. For patients with ESCC and either neurologic symptoms or substantial thecal sac compression by imaging, we recommend glucocorticoids as an integral component of the initial management (Grade 1B). (See 'Glucocorticoids' above.) ●For patients with severe neurologic deficits (eg, paraparesis or paraplegia), we suggest high-dose glucocorticoid therapy (eg, dexamethasone 96 mg intravenously followed by 24 mg four times daily for three days and then

tapered over 10 days) (Grade 2C). ●For patients with minimal neurologic symptoms, we suggest moderate doses of glucocorticoids (eg, dexamethasone as a bolus of 10 mg intravenously followed by 16 mg daily orally in divided doses) (Grade 2C). ●We suggest that glucocorticoids be omitted in patients with small epidural lesions and a normal neurologic examination 29. 25 yo man, hx 4-month increasing low back most severe on awakening in the morning. Pain relieved by stretching. No trauma Hx. Occasional dysuria. Decreased forward flexion of the spine and tenderness over the lumbosacral area and at the insertion site of the achiles tendon. Dx? B. Ankylosing spondylitis Ankylosing spondylitis (AS), a form of spondyloarthritis (SpA), is a chronic inflammatory disease of the axial skeleton manifested by back pain and progressive stiffness of the spine; it can also involve the hips, shoulders, peripheral joints, entheses, and digits. Extraarticular manifestations, including uveitis, may also be seen in patients with AS and other forms of SpA. AS typically develops in young adults, with a peak age of onset between 20 and 30 years. The endpoint, which occurs in only a subset, is complete spinal fusion with hyperkyphosis. Almost all patients with AS report back pain, which frequently, but not invariably, has characteristics suggesting an inflammatory etiology. Inflammatory back pain typically exhibits at least four of the following five features: ●Age of onset 150 patients) of biopsy-confirmed GCA, TVL was the presenting symptom in just 10 to 15 percent [36,38]. It was bilateral in 27 to 35 percent of these. Common accompanying symptoms include jaw claudication and headache (each in about half of patients). However, patients with occult giant cell arteritis may have no other symptoms [37]. Erythrocyte sedimentation rate and C-reactive protein All older patients (>50 years) with transient monocular or binocular vision loss should have a sedimentation rate and C-reactive protein to exclude giant cell arteritis (GCA). If these are elevated, or if the history is very suggestive, patients should proceed to a confirmatory temporal artery biopsy. Treatment should not await pathology results. 37. 11 yo girl, ER 2-day hx increasing weakness and prickling sensation in her legs. Flu-like illness past week. 98.6F, pulse 78/min, resp 20/min, BP 115/70. Lower extremities strength 1/5, decreased sens pinprick. decreased patellar and achilles reflexes. CSF protein 80, WBC 4. Pharmacotherapy? E. Immune globulins In most cases, neurologic symptoms develop two to four weeks after having what appears to be a benign febrile respiratory or gastrointestinal infection. The predominant symptoms of GBS at presentation in children are pain and gait difficulty. Lower extremity symmetric weakness may ascend over hours to days to involve the arms and the muscles of respiration in severe cases. In those with cranial neuropathy, the facial nerve is most commonly affected, resulting in bilateral facial weakness. Autonomic dysfunction occurs in approximately one-half of children with GBS. Physical examination reveals symmetric weakness with diminished or absent reflexes. Most patients reach the nadir of their function within two to four weeks, followed by return of function occurring slowly over the course of weeks to months. With lumbar puncture, characteristic cerebrospinal fluid findings include normal pressure, fewer than 10 cells (typically mononuclear), and an elevated protein concentration (>45 mg/dL). The main modalities of therapy for GBS are intravenous immune globulin (IVIG) and plasma exchange (also called plasmapheresis). IVIG and plasma exchange for children with GBS should be reserved for those with any

of the following indications: ●Progressing weakness ●Worsening respiratory status or need for mechanical ventilation ●Significant bulbar weakness ●Inability to walk unaided We prefer IVIG to plasma exchange for the treatment of children because of its relative safety and ease of administration, although it has not been shown to have better results. Neither treatment is recommended for ambulatory children with GBS who have mild disease or for children whose symptoms have stabilized. However, children who have rapid progression followed by stabilization of symptoms within the first or second week of GBS onset may still be considered candidates for treatment. Glucocorticoids are not beneficial for GBS and have no role in its treatment. 38. 32 yo man, ER 20 min after found in bathroom bus station. Found having continuous, generalized tonic clonic seizures. Intubated, and supplemental O2. Flushed in diaphoretic. No signs trauma. 99F, pulse 110/min, resp 15/min, BP 140/70. Pupils 4mm reactive. Tonic stiffness trunk and synchronous jerking of extremities. IV dextrose started. Next step ttmt? B. Lorazepam

39. 25 yo woman, 6-month hx worsening excessive daytime sleepiness. Problem sustaining

attention, impair work productivity. Occasional muscles go limp and falls suddenly. BMI 22. Pulse 85/min, resp 12/min, BP 120/76. Initial step management. E. Moldafinil therapy. Nearly half of patients with narcolepsy report that their sleepiness and cataplexy substantially interfere with their daily lives, including school, jobs, marriages, or social lives [1,2]. The mainstays of therapy are brief daytime naps and pharmacologic therapy. There are several nonpharmacologic interventions that may benefit the patient with narcolepsy: ●Avoiding certain drugs – Some drugs should be avoided by patients with narcolepsy. Drugs that can worsen daytime sleepiness include benzodiazepines, opiates, antipsychotics, and alcohol. Other medications such as theophylline or excessive caffeine can cause insomnia, which can worsen daytime sleepiness. In addition, prazosin and other alpha-1 antagonists can worsen cataplexy. ●Napping/sleep hygiene – Behavioral interventions are often helpful for optimal control of narcolepsy. One or two well-timed 20 minute naps will often improve sleepiness for one to three hours though some patients only benefit from long naps [4]. If it can be arranged, a brief nap at work or school is often helpful. Sleep deprivation may worsen narcolepsy symptoms and therefore patients should be counseled to maintain a regular and adequate sleep schedule [5]. ●Psychosocial support – Patients with narcolepsy face various psychosocial and work-related problems throughout their lives; as a result, they may have difficulty meeting economic and social responsibilities [6]. They also have the additional burden of coping with misperceptions about the causes and the involuntary nature of their symptoms. Common misconceptions, even among medical caregivers, are that sleep attacks and cataplexy (emotionally triggered muscle paralysis resulting in partial or complete collapse) are manifestations of poor motivation, denial, or avoidance. Thus, patients often benefit from participation in support groups that focus on coping skills and identification of community resources to assist with administrative and medical issues. Many patients will benefit from these nonpharmacologic approaches, but most also require medications that reduce sleepiness and cataplexy. Modafinil has become a first line pharmacologic therapy because it provides good control of sleepiness, is generally well tolerated, and illicit use is rare. A nonamphetamine "wakefulness promoting agent," its mechanism of action is not well understood, but it may increase dopaminergic signaling. 40. 68 yo woman, 3 month hx of spinning sensation. Feeling room spinning when she tries to turn over in bed, resolves in 1 min. No vomiting, but queasy. Normal hearing. Normal ocular movements if sitting, but coarse rotatory nystagmus to one side if head is hanging from examination table. Abnormality in which site? C. Otolith Patients with benign paroxysmal positional vertigo (BPPV) present with recurrent episodes of vertigo that last one minute or less. Episodes are provoked by specific types of head movements, such as looking up while standing or sitting, lying down or getting up from bed, and rolling over in bed. The vertigo may be associated with nausea and

vomiting. Hearing loss or symptoms are typically absent [14]. Patients with BPPV typically have no other neurologic complaints. With the patient sitting, the neck is extended and turned to one side. The patient is then placed supine rapidly, so that the head hangs over the edge of the bed. The patient is kept in this position until 30 seconds has passed if no nystagmus occurs. The patient is then returned to upright, observed for another 30 seconds for nystagmus, and the maneuver is repeated with the head turned to the other side. A video demonstrating this maneuver can be viewed at http://www.neurology.org/content/70/22/2067/suppl/DC2.

Diagnostic criteria employing the Dix-Hallpike maneuver have been proposed for posterior canal BPPV (figure 1) [18]: ●Nystagmus and vertigo usually appear with a latency of a few seconds and last less than 30 seconds ●It has a typical trajectory, beating upward and torsionally, with the upper poles of the eyes beating toward the ground ●After it stops and the patient sits up, the nystagmus will recur but in the opposite direction ●The patient should then have the maneuver repeated to the same side; with each repetition, the intensity and duration of nystagmus will diminish Benign paroxysmal positional vertigo (BPPV) is commonly attributed to canalithiasis, ie, calcium debris within the semicircular canal [3]. This debris likely represents loose otoconia (calcium carbonate crystals) within the utricular sac. The semicircular canals normally detect angular head accelerations. Heavy debris in the canal causes inappropriate movement of the endolymph with linear accelerations, such as gravity, and causes the erroneous sensation of spinning when the head shifts with respect to gravity. 41. 24 yo woman, ER after 1-hour generalized tonic-clonic seizure lasted 5 min. Immigrated from Central America 6 months ago. No medications. Drowsy but cooperative. 98.8F, pulse 80/min, resp 18/min, BP 150/90. Toxycology (-). CT head multiple enhancing lesions. Dx? D. Neurocysticercosis

Neurocysticercosis was observed more frequently in emergency departments of Los Angeles, Phoenix, and Albuquerque (5.7 percent), which had a higher proportion of immigrant Hispanic patients than the other hospitals. Travelers to endemic areas represent another source of cysticercosis, although such infection accounts for a minority of cases in the United States. The clinical manifestations depend upon whether the cysts are localized to the brain parenchyma, the extraparenchymal tissues, or both. In general, parenchymal cysts are associated with seizures and headache, while extraparenchymal cysts are associated with symptoms of elevated intracranial pressure (eg, headache, nausea, and vomiting) and may be accompanied by altered mental status. Other less common manifestations include mass effect, altered vision, focal neurologic signs, altered mental status, and meningitis. Fever is not typically present. Neurologic examination usually does not demonstrate focal signs in the absence of mass effect or stroke. Parenchymal cystic or enhancing lesions are the most common form of neurocysticercosis (NCC) in hospitalbased series and is present in >60 percent of these patients [3,12-15]. The onset of symptomatic NCC has been estimated to peak at three to five years after infection, but it can be delayed for >30 years [14,16,17]. Later onset may be due to calcified lesions. Ultimately, the cystic lesions either resolve or form a calcified granuloma. When present, the calcifications are associated with recurrent seizures, although the mechanism of seizures associated with calcified lesions is not completely clear. Seizures may result from inflammation, perhaps from intermittent antigen release [6,7]. Alternatively, changes in brain plasticity and scarring (eg, hippocampal sclerosis) may result in epileptogenic foci [8,9]. Studies have highlighted a potential role of matrix metalloproteinases in the pathogenesis of seizures in neurocysticercosis, especially in patients with calcified lesions [10]. 42. 19 yo woman, ER for seizure at dormitory party on campus. Drinking more water than usual, then silent and staring. Shaking for 1 minute. Unresponsive, Restless and grinds her teeth. 103.3F, pulse 90/min, resp 24/min BP 150/95. Profuse diaphoresis and piloerection. Pupils 5mm bilat. PT 16s, Na 114, Urea nitrogen 40, Creat 2, ALT 90, CK 1200. Intoxication for which substance? C. Ecstasy MDMA is a commonly abused drug, particularly among young party-goers at electronic dance music venues, including dance clubs and large music festivals. Typical effects include feelings of euphoria, wakefulness, intimacy, sexual arousal, and disinhibition. sympathomimetic amphetamine that causes release of endogenous catecholamines. MDMA differs somewhat from traditional amphetamines in that it is structurally similar to serotonin. This difference likely accounts for an increased release of serotonin and inhibition of serotonin reuptake [4,10,11]. This results in a toxicity profile for MDMA that is different from that of traditional amphetamines. Although typical findings of amphetamine toxicity (hypertension, tachycardia, hyperthermia, CNS stimulation) are often seen in MDMA toxicity, serotonergic toxicity (serotonin syndrome, SIADH) may be seen as well. MDMA (3,4-methylenedioxymethamphetamine) increases alertness, reduces fatigue, and leads to feelings of increased physical and mental powers, and euphoria. Users

typically begin to experience the desired effects of MDMA approximately one hour following oral administration [10]. Minor adverse reactions, such as agitation, nausea, bruxism (grinding teeth), ataxia, diaphoresis, blurry vision, tachycardia, and hypertension, can also occur at typical MDMA doses. These effects are usually selflimited and resolve within hours [14]. More serious effects are uncommon and described below. Vital signs — MDMA can cause hypertension, tachycardia, and hyperthermia. Cardiovascular stimulation — Life-threatening increases in heart rate and blood pressure may occur. Cardiovascular toxicity can include hypertensive emergencies, myocardial infarction, aortic dissection, and dysrhythmia [15-21]. In addition, catastrophic central neurologic hemorrhage, including intracranial hemorrhage and posterior spinal artery aneurysm, has been reported [22,23]. Hyperthermia and related effects — Hyperthermia may result from drug effects on the central nervous system (CNS), prolonged physical exertion (eg, dancing all night at a "rave"), and environmental conditions (eg, dancing in a densely populated, hot room). Both the stimulant effect of amphetamines and serotonin syndrome may contribute to severe hyperthermia in these patients [19,24]. Hyperthermia can lead to disseminated intravascular coagulation and rhabdomyolysis. (See 'Hyperthermia' below.) Manifestations of hyponatremia — MDMA use can lead to hyponatremia due to a marked increase in fluid intake and in some patients, persistent secretion of antidiuretic hormone that slows the rate of water excretion. The belief among some MDMA users that they can avoid hyperthermia by drinking large amounts of water is the reason for increased fluid intake in many cases. The mechanisms that lead to hyponatremia are described separately. (See "Causes of hyponatremia in adults", section on 'Ecstasy (MDMA) intoxication'.) Marked and often acute reductions in serum sodium can lead to serious neurologic manifestations including confusion, seizures, cerebral edema, cerebral herniation, and death. The serum sodium in such patients is usually below 120 meq/L (120 mmol/L). Young women appear to be at increased risk for both symptomatic hyponatremia and residual neurologic injury. Neurologic — Stimulation of the CNS is common and can manifest as agitation, hyperactivity, anxiety, and even delirium [24]. Seizures and status epilepticus can occur. Psychomotor agitation may be associated with hyperthermia as well as rhabdomyolysis. Hepatotoxicity — Hepatotoxicity caused by MDMA poisoning is well recognized. Even in the absence of severe hyperthermia or disseminated intravascular coagulation, hepatitis, centrilobular necrosis, and hepatic fibrosis may result from MDMA abuse [25,26]. Clinical findings are similar to other forms of toxin-induced hepatic injury and may include jaundice, abdominal pain, and vomiting. Elevations of bilirubin, aspartate transaminase (AST), and alanine transaminase (ALT) may be present. Serotonin syndrome findings — Serotonin syndrome is a potentially life-threatening condition characterized by the triad of autonomic dysfunction, abnormal neuromuscular activity, and altered mental status. MDMA use can cause serotonin syndrome, presumably via stimulation of massive serotonin release. Individuals who use MDMA in

combination with selective serotonin reuptake inhibitors (SSRIs), monoamine oxidase inhibitors (MAOIs), or other drugs that increase 5-HT1A receptor activity (such as meperidine, tryptophan, or lithium) are at greater risk of developing serotonin syndrome [27-30]. 43. 37 yo man, Er with 10-day hx increasingly severe headaches. Unremitting pain for 18h. Pain is severe in pounding. Pulse 70/min, resp 16/min, BP 142/90. Mild swelling, dusky reddish-purple skin color with venous distention over head and neck. Papilledema. No neurological local findings. Most likely cause of intracranial HTN? D. Obstruction of venous return to the heart Superior vena cava (SVC) syndrome results from any condition that leads to obstruction of blood flow through the SVC. Obstruction can be caused by invasion or external compression of the SVC by an adjacent pathologic process involving the right lung, lymph nodes, and other mediastinal structures, or by thrombosis of blood within the SVC. In some cases, both external compression and thrombosis coexist. The clinical diagnosis of superior vena cava (SVC) syndrome is made on the basis of characteristic signs and symptoms of central venous obstruction. Regardless of etiology, dyspnea is the most common symptom [8,9,30]. In addition, patients frequently complain of facial swelling or head fullness, which may be exacerbated by bending forward or lying down. Other symptoms include arm swelling, cough, chest pain, or dysphagia. Patients with cerebral edema may have headaches, confusion, or possibly coma. On physical examination, the most common findings are facial edema and distension of the veins in the neck and on the chest wall (picture 1). Arm edema, cyanosis, and facial plethora are less frequent. 44. 52 yo man episodes of loss consciousness preceded by twitching of the right thumb, then hand, wrist, elbow, and shoulder. Spread duration of 20 seconds. Find himself in floor after. Bites tongue and urinates during episodes. G. Simple partial (motor) seizure with generalization. New Classification: In the 2010 proposal, mode of onset is subdivided into generalized, focal, and unknown, the latter of which includes spasms. Generalized — Generalized seizures are conceptualized as those that originate at some point within, and rapidly engage bilaterally distributed networks, which can be subcortical or cortical structures. Generalized seizures do not need to necessarily include the entire cortex, however, and they may be asymmetric. Importantly, a generalized presentation can still arise from a focal lesion, and does not exclude the possibility of a surgical remedy. Focal — The term focal has replaced partial to describe seizures that originate in networks limited to one hemisphere [3]. Focal seizures may arise from either subcortical structures or neocortex. In addition, the terms simple partial, complex partial, and secondarily generalized have been eliminated, since they were difficult to define pragmatically and were often used incorrectly. Instead, the proposal uses various descriptors of focal seizures to describe an event more precisely. Examples of preferred descriptors include:

●Without impairment of consciousness or awareness ●Involving subjective sensory or psychic phenomena ●With impairment of consciousness or awareness, or dyscognitive ●Evolving to a bilateral convulsive seizure Unknown — The term unknown mode of onset is used for seizure types where it remains unclear whether onset is focal, generalized, or perhaps either. A key example is epileptic spasms, concerning which there has traditionally been controversy, and current knowledge is inadequate to specify mode of onset as either focal or generalized. ILAE Classification of seizures Generalized seizures Tonic-clonic (in any combination) Absence Typical Atypical Absence with special features Myoclonic absence Eyelid myoclonia Myoclonic Myoclonic Myoclonic atonic Myoclonic tonic Clonic Tonic Atonic Focal seizures Unknown Epileptic spasms ILAE: International League Against Epilepsy.

old classification:

45. 72 yo man episodes loss of consciousness past 2 years. No warming symptons, suddenly

find on the floor. Fracture arm and bruised. During episodes becomes pale and sweaty. Jerking or twitching movements of the trunk and extremities for several seconds during episode. He recovers completely and is not confused at the end of episodes. Hx of 2 MI. K. Syncope Imitators of epilepsy: Nonepileptic paroxysmal disorders Neonates Apnea Jitteriness Benign neonatal sleep myoclonus Hyperkplexia Infants Breath-holding spells Benign myoclonus of infancy Shuddering attacks Sandifer syndrome Benign torticollis in infancy Abnormal eye movements (eg, spasmus nutans, opsoclonus-myoclonus) Rhythmic movement disorder (head banging) Children Breath-holding spells Vasovagal syncope Migraine Benign paroxysmal vertigo Staring spells Tic disorders and stereotypies Rhythmic movement disorder Parasomnias Adolescents and young adults Vasovagal syncope Narcolepsy Periodic limb movements of sleep Sleep starts Paroxysmal dyskinesia Tic disorders Hemifacial spasm Stiff person syndrome Migraine Psychogenic nonepileptic pseudoseizures Hallucinations Older adults Cardiogenic syncope Transient ischemic attack Drop attacks Transient global amnesia Delirium or toxic-metabolic encephalopathy Rapid eye movement sleep behavior disorder

Differentiation of generalized tonic-clonic seizures from pseudoseizures and syncope Generalized seizure Characteristic Pseudoseizure Syncope tonic-clonic Circumstances Usually upright; any position if Situation Awake or sleep Awake cardiogenic Sleep loss, alcohol Emotion, injury, heat, crowds; none Precipitating factors withdrawal, flashing Emotion if cardiogenic lights Presence of others Variable Usual Variable Motor phenomena Vocalization At onset, if any During course None Location of motor Proximal limb Proximal limb None component (if present) Tonic; flailing; struggling or Usually atonic; if syncope lasts Generalized motor Tonic, then clonic thrashing, or both >20 seconds: tonic, then clonic Partial flexion or Tonic posture Opisthotonic straight Head movements To one side or none Side to side Clonus/limb jerks Bilaterally synchronous Asynchronous Bilaterally synchronous Occasional, including Purposeful movements Absent Absent avoidance Biting Tongue, inside mouth Lips, arms, other people Tongue biting rare Babinski's sign Present Absent Absent Autonomic features Micturition Frequent Rare Occasional Eyes Open Closed Open Dilated or hippus during Pupils Normal Dilated attacks Colour Cyanotic or grey Rubor or normal Pale Slow if vasovagal, weak if Pulse Rapid, strong Normal vasodepressor; that of arrhythmias if cardiogenic Timing Usual duration 1 to 5 min 5 to 60 min 1 to 2 min Gradual; possibly sudden if Onset Sudden Gradual cardiogenic Sequence of symptoms Stereotyped Variable Stereotyped Spontaneous or induced by Termination Spontaneous supraorbital pressure, Rapid suggestion Sequelae Frequent, mild; scalp, Rare, but multiple bruises Injury If sudden onset face, common possible; scalp, face, rare Regains consciousness in 2 to 3 Postictal Tired, confused, sleepy Alert, emotional outburst min; alert but tired

Following the end of a seizure, there is a period of transition from the ictal state back to the individual's normal level of awareness and function. This interval is referred to as

the "postictal period" and signifies the recovery period for the brain. Manifestations typically include confusion and suppressed alertness; focal neurologic deficits may also be present. The postictal state may last from seconds to minutes to hours, depending upon several factors including which part(s) of the brain were affected by the seizure, the length of the seizure, whether the individual was on antiseizure drugs, and age. As an example, young adults with focal seizures of frontal lobe origin may have postictal states that last only several seconds, while elderly patients with secondarily generalized seizures may have postictal confusion and sleepiness that persists for as long as several days to a week, particularly if there is underlying brain dysfunction [27]. If a person had a focal seizure with impairment of consciousness or a convulsion, his or her level of awareness gradually improves during the postictal period, much like a person waking up from anesthesia after an operation. 46. 25 yo woman, growths in her lips and tongue for years. Nodules in lips, buccal mucosa, tongue. Café au lait spot in trunk, limbs. Scattered nodules on skin. Greatest risk for? D. optic nerve glioma The typical order of appearance of clinical manifestations is café-au-lait macules, axillary and/or inguinal freckling, Lisch nodules (iris hamartomas), and neurofibromas [32]. Osseous lesions, if present, usually appear during the patient's first year after birth, and symptomatic optic pathway glioma (OPG) usually occurs by the time the patient is three years of age (table 1). Other tumors and neurologic complications typically begin to appear after the first year of life. Hypertension may occur in childhood. Malignant transformation of tumors may also occur in childhood, but more often occurs in adolescence and adulthood. OPGs occur in 15 percent of children younger than six years of age with NF1 [52]. They rarely occur in older children and adults [53,54]. OPGs are typically low-grade pilocytic astrocytomas [55]. They can arise anywhere along the anterior visual pathway to the optic radiations and involve the optic nerves, chiasm, and postchiasmal optic tracts. 47. 12 yo girl with SLE in ER b/c difficulty awakening this morning. Need constant stimulation to stay awake. Past 2-day, flu-like symptoms and vomiting. Unable to take medication corticosteroids and antihypertensive. 98.6F, pulse 60/min, resp 40/min, BP 180/130. Obtunded but moves extremities to painfull stimuli. Bilat papilledema with flame hemorrhages. Tendon reflexes brisk symmetric. ESR 12. Dx? C. Hypertensive encephalopathy Hypertensive emergencies in children usually manifest as hypertensive encephalopathy: severe BP elevation with cerebral edema and neurological symptoms of lethargy, coma, and/or seizures. This pathology is caused by cerebrovascular endothelium breakdown secondary to failure of cerebral autoregulation. In addition to hypertensive encephalopathy, visual changes may also result from retinal hemorrhage or exudates that involve the macula (central vision) or cause noticeable defects in peripheral vision. Funduscopic examination is of particular importance since papilledema and retinal hemorrhage or exudates may be the only sign of a hypertensive emergency. 48. 48 yo woman, hx 6-month gradual hearing loss left ear. Difficulty maintaining her balance past 3 months. 6 months ago episode of nephrolithiasis complicated with UTI and require antibiotic. Decreased left nasolabial fold and slight perioral droop when

smiles. Forehead normal. Gait normal, but difficulty with tamden walking. High frequency hearing loss left with impaired speech discrimination. Dx? A. acoustic neuroma (vestibular schwannoma) The median age at diagnosis is approximately 50 years [2]. The tumors are unilateral in more than 90 percent of cases [5], affecting the right and left sides with equal frequency. Bilateral vestibular schwannomas are primarily limited to patients with neurofibromatosis type 2. Childhood exposure to low-dose radiation for benign conditions of the head and neck has been associated with an increased risk of vestibular schwannoma. The clinical presentations of these tumors are illustrated by a series of 1000 vestibular schwannomas treated at a single institution [18]. Clinical manifestations in this series included the following: ●Cochlear nerve — Symptomatic cochlear nerve involvement occurred in 95 percent of patients [18]. The two major symptoms were hearing loss and tinnitus. Hearing loss was present in 95 percent but only two-thirds of these patients were aware of this limitation. The hearing loss was usually chronic, with an average duration of about four years. Occasionally, vestibular schwannomas can present with sudden sensorineural hearing loss. Tinnitus was present in 63 percent with an average duration of three years [18]. The incidence of tinnitus was higher in hearing than in deaf patients but was also present in 46 percent of deaf patients. ●Vestibular nerve — Involvement of the vestibular nerve occurred in 61 percent of patients [18]. Affected patients frequently acknowledged having unsteadiness while walking, which was typically mild to moderate in nature and frequently fluctuated in severity. True spinning vertigo was uncommon because these slow growing tumors cause gradual rather than acute asymmetries in vestibular function. In this setting, the central vestibular system can often compensate for the gradual loss of input from one side. ●Trigeminal nerve — Trigeminal nerve disturbances occurred in 17 percent of patients [18]. The most common symptoms were facial numbness (paresthesia), hypesthesia, and pain. The average duration of symptoms was 1.3 years; the symptoms usually occurred after hearing loss had been present for more than two years and vestibular symptoms for more than one year. ●Facial nerve — The facial nerve was involved in 6 percent of patients [18]. The primary symptoms were facial paresis and, less often, taste disturbances. ●Tumor progression — Other presenting signs can be the result of tumor progression, leading to pressure on adjacent posterior fossa structures. Very large tumors can press on the cerebellum or brainstem and result in ataxia. Brainstem compression, cerebellar tonsil herniation, hydrocephalus and death can occur in untreated cases. The functions of the lower cranial nerves can also become impaired, leading to dysarthria, dysphagia, aspiration, and hoarseness. 49. 62 yo man, 5 days after hemicolectomy in ICU, present with 105F, lethargy, diaphoresis, labile blood pressure for 3 hours. Post-op is complicated with fever, nocturnal agitation since day 2. Haloperidol nightly (2mg) Hx of Bipolar. Medication morphine, lithium,

clonazepam. Lying in pool of sweat and drooling. 105F, pulse 140/min, BP 180/min, Wet skin and dry mucosa. Herpetic lesion lower lip. Neck supple and IV catherer insertion site is non-tender, no erythema. Bowel sounds hyperactive. Wound clean and dry, no erythema. Neurological shows masked facies and intention tremor. Reflexes hyperactive and symmetric. Babinski (-). Labs Hb 15, WBC 20000 , Neutro 80%, bands 5%, Lymphs 10%, mono 5%. Urea nitrogen 38, Creat 1.6, CK 1000. Urianalysis and CXR normal. Dx? E. Neuroleptic malignant syndrome. NMS is most often seen with the "typical" high potency neuroleptic agents (eg, haloperidol, fluphenazine) [8-10]. However, every class of neuroleptic drug has been implicated, including the low potency (eg, chlorpromazine) and the newer "atypical" antipsychotic drugs (eg, clozapine, risperidone, olanzapine) as well as antiemetic drugs (eg, metoclopramide, promethazine) [4,11]. NMS is defined by its association with a class of medications that block dopamine transmission and a tetrad of distinctive clinical features: fever, rigidity, mental status changes, and autonomic instability [3,34]. Typical symptoms — The tetrad of NMS symptoms typically evolves over one to three days. Each feature is present in 97 to 100 percent of patients: Mental status change is the initial symptom in 82 percent of patients [35]. It is not surprising, given the usual psychiatric comorbidity of the typical patient, that its significance is often underappreciated. This often takes the form of an agitated delirium with confusion rather than psychosis. Catatonic signs and mutism can be prominent. Evolution to profound encephalopathy with stupor and eventual coma is typical [15]. Muscular rigidity is generalized and is often extreme. The increased tone can be demonstrated by moving the extremities and is characterized by "lead pipe rigidity" or stable resistance through all ranges of movement. Superimposed tremor may lead to a ratcheting quality or a cogwheel phenomenon. Other motor abnormalities include tremor (seen in 45 to 92 percent), and less commonly, dystonia, opisthotonus, trismus, chorea, and other dyskinesias [2,4]. Patients can also have prominent sialorrhea, dysarthria, and dysphagia. Hyperthermia is a defining symptom according to many diagnostic criteria. Temperatures of more than 38ºC are typical (87 percent), but even higher temperatures, greater than 40ºC, are common (40 percent) [4]. Autonomic instability typically takes the form of tachycardia (in 88 percent), labile or high blood pressure (in 61 to 77 percent), and tachypnea (in 73 percent) [2,19]. Dysrhythmias may occur. Diaphoresis is often profuse. In an analysis of 340 cases, 70 percent of patients followed a typical course of mental status changes appearing first, followed by rigidity, then hyperthermia, and autonomic dysfunction [35]. Some case reports document delay in the appearance of fever of more than 24 hours, leading to initial diagnostic confusion [2]. There is substantial variability in the presentation of NMS, and other reports do not necessarily substantiate a typical course. Drugs that can cause neuroleptic malignant syndrome Neuroleptic agents Antiemetic agents Aripiprazole Domperidone

Chlorpromazine Clozapine Fluphenazine Haloperidol Olanzapine Paliperidone Perphenazine Quetiapine Risperidone Thioridazine Ziprasidone

Droperidol Metoclopromide Prochlorperazine Promethazine

50. 62 yo woman, week hx intermittent drooping of his left eyelid and double vision. Bilateral ptosis, more prominent on left. Weaknees on left eye abduction/adduction and right abduction. Pupillary reflexes normal. Greatest risk for? E. Thymoma In adults, thymomas and thymic carcinomas are the most common neoplasms arising in the thymus. Thymomas account for about 20 percent of mediastinal neoplasms (table 1). Most thymoma patients are between 40 and 60 years of age, and there is a similar incidence in men and women [1]. There are no known risk factors, although there is a strong association with myasthenia gravis and other paraneoplastic syndromes. The most common paraneoplastic syndrome associated with thymoma is myasthenia gravis. Myasthenia gravis is an autoimmune disorder caused by interference with acetylcholine receptors of voluntary muscle at the neuromuscular junction. Common symptoms include diplopia, ptosis, dysphagia, weakness, and fatigue. Paraneoplastic syndromes associated with thymic neoplasms Myasthenia gravis, polymyositis, sensory neuropathy, stiff person syndrome, neuromyelitis Neurologic and optica, Isaac's syndrome (neuromyotonia), Eaton Lambert syndrome, hemichorea neuromuscular Pure red cell aplasia, agranulocytosis, hemolytic anemia, pernicious anemia Hematologic Alopecia areata, pemphigus, scleroderma, oral lichen planus, vitiligo Dermatologic Addison's disease, Cushing syndrome, panhypopituitarism, thyroiditis Endocrine Acquired hypogammaglobulinemia, myocarditis, nephrotic syndrome, rheumatoid arthritis, Miscellaneous sarcoidosis, hepatitis, gastrointestinal pseudoobstruction, ulcerative colitis

The clinical features of Lambert-Eaton myasthenic syndrome LEMS were well defined by Lambert and Eaton in Minnesota [29] and by O'Neill and colleagues in England [18]. Most patients present with complaints of slowly progressive proximal muscle weakness, and this feature is present in almost all patients at some point in the illness. Occasional patients have a subacute or acute presentation. Patients typically describe an alteration in gait or difficulty arising from a chair or managing stairs. Many describe aching or stiff muscles. Muscle fatigability or cramping is common, particularly after protracted exercise. There tends to be relative preservation of distal muscle function. Symmetrical muscle involvement is the rule. Upper extremity complaints are usually modest and typically involve proximal muscle function. Patients with LEMS and small cell lung cancer typically develop a more rapidly progressive course of proximal and

then distal arm muscle weakness than patients who have non-tumor LEMS Autonomic dysfunction is often present and can be an important clue to the diagnosis. Dry mouth from reduced salivation is the most common autonomic symptom and occasionally is the presenting complaint, while erectile dysfunction is common in men with LEMS. In the largest study of autonomic dysfunction in LEMS involving 30 patients, dry mouth was identified in 77 percent, and impotence was present in 45 percent of all men [31]. Other symptoms may include blurred vision and constipation.