Neurology Flashcards

Question

What is the diagnostic workup for CVA after initial and most accurate tests?

Answer 1

look for the source - embolic - ECHO - "bubble study" done to detect patent foramen ovale or cardiac defects - carotid duplex - 24 holter monitor - inherited hypercoag - EKG - in SAH you can see ischemia and inverted T waves

Answer 2

TpA should be initiated w/in 3 hr of symptom onset | Aspirin is started 24 hrs after Tpa

Answer 3

Contraindications to the use of tissue plasminogen activator include - stroke or serious head trauma within 3 months, - hemorrhage (gastrointestinal or genitourinary) within 21 days - surgery within 14 days, - history of intracranial hemorrhage, - BP >185/110 mm Hg, - current use of anticoagulants, - platelets 15 seconds)

Answer 4

if pt is allergic to aspirin or has recurrent CVA despite being on aspirin then give dipyridamole in addition to aspirin or just switch to clopidogrel alone

Answer 5

nimodipine - early (w/in days!) surgical intervention to clip off the aneurysm or embolize the vessel with catheter should be done if candidate is good for surgery.

Answer 6

recommended when an occlusion exceeds 70% of the arterial lumen and the lesion is symptomatic. - Endarterectomy may benefit those who are asymptom- atic if there is >60% stenosis in men age <60. - The benefit of endoarterectomy is less certain in women because they have a lower risk of stroke. - The more severe the disease, the greater the benefit. Carotid stenting is an alternative to endarterectomy.

Answer 7

carotid stenting

Answer 8

ymptomatic patients with >70% stenosis. | - Angioplasty and stenting should be considered only for those who cannot undergo surgical endarterectomy.

Answer 9

A seizure is a paroxysmal event due to abnormally discharging central nervous system (CNS) neurons.

Answer 10

Epilepsy is defined as a condition of recurrent seizures due to a chronic underlying process.

Answer 11

Vascular (stroke, bleed, arteriovenous malformation) Infection (meningitis, abscess, encephalitis) Trauma (especially penetrating) Autoimmune (CNS vasculitis) Metabolic (hyponatremia, hypocalcemia, hypomagnesemia, hypoglycemia, hypoxia, drug overdose/withdrawal) Idiopathic Neoplasm pSychiatric

Answer 12

A seizure is essentially a paroxysmal, involuntary event (associated with abnormal movement or change of consciousness or both). - Characteristically, seizures are sudden in onset, with or without an aura. - Patients often complain of disorientation, sleepiness, and aching muscles for minutes to hours after the event. - Patients may also experience incontinence, tongue biting, and headache as a result of the seizure. It may be difficult at times to differentiate a seizure from syncope, and it is important to obtain a complete history from any individual who witnessed the event. - Generally, patients with syncope will not complain of significant postictal symptoms. They will recover consciousness within several minutes of the event, and on physical exam will not have evidence of incontinence or tongue biting

Answer 13

Partial vs. Generalized | complex vs. simple

Answer 14

Partial seizures occur within discrete portions of the brain. The patient will often complain of involuntary jerking of a finger or hand. - When consciousness is maintained for the duration of the seizure, the seizure is termed a simple partial seizure. - When there is a change in consciousness during the seizure, the seizure is termed a complex partial seizure.

Answer 15

When a partial seizure progresses to a generalized seizure, it is called a partial seizure with secondary generalization. Typically, the seizure will begin focally and become generalized as the seizure activity involves both cerebral hemispheres.

Answer 16

Generalized seizures arise from both cerebral hemispheres spontaneously without any detectable focal onset. - Generalized tonic-clonic (grand mal) seizures are characterized by tonic contraction of muscles throughout the body followed by intermittent relaxation of various muscle groups (clonic phase). - Absence seizures (petit mal) are more common in children than adults; they are characterized by sudden, brief loss of consciousness without loss of postural tone. - Atonic seizures are characterized by sudden loss of postural tone lasting 1 to 2 seconds. - Myoclonic seizures are characterized by sudden, brief muscle contraction.

Answer 17

Status epilepticus is defined as recurrent or continuous seizures (lasting at least 5–30 min).

Answer 18

EEG is the test of choice for the diagnosis of epilepsy. The diagnosis of idiopathic seizures is made only after secondary precipitating factors have been ruled out. An abnormal EEG alone is not diagnostic of epilepsy. - Always check serum electrolytes, glucose, toxicology, and arterial blood gas to rule out hypoxia as a cause of a patient’s seizure. CT scan or MRI of the head is usually indi- cated to rule out a structural lesion as the cause of seizure

Answer 19

The treatment of seizures can be divided into the acute management of the acutely seizing patient (status epilepticus) and the chronic management of the epileptic patient. - 1st step is ABC (airway, breathing, circulation) - evaluate and treat any precipitating causes of seizure. If a reversible cause is identified, treat aggressively. If the patient continues to seize, the following strategy is appropriate. - initial drug of choice is lorazepam or diazepam, these medications work by potentiating GABA receptor function. - If the patient continues to seize, add phenytoin or fosphenytoin, which inhibits sodium-dependent action potentials. CNS side effects of phenytoin include diplopia, dizziness, and ataxia. Systemic side effects include gum hyperplasia, lymphadenopathy, hirsutism, and rash. - If the patient continues to seize add phenobarbital. Side effects include sedation, ataxia, and rash. - If, despite all of the above therapy, the patient continues to seize, add midazolam or propofol.

Answer 20

In patients with first-time seizure, anticonvulsant therapy should be started only if the patient has an abnormal neurologic exam, presented with status epilepticus, has a strong family history of seizure, or has an abnormal EEG

Answer 21

``` VA phenytoin lamotrigine - decreases glutamate releases, side effects are SJS carbamazepine levetiracetam ```

Answer 22

Ethosuximide - first choice | VA

Answer 23

VA - first choice

Answer 24

Carbamazepine & phenytoin - first line | VA & Lamotrigine - alternatives or levetiracetam

Answer 25

It is very difficult to determine when to stop therapy. Therapy may be stopped if the patient has been free of seizures for 2–3 years. Sleep-deprivation EEG may be done first to determine if the patient is at low risk of a recurrence. A normal sleep- deprivation EEG means there is a lower likelihood of seizures.

Answer 26

Vertigo is defined as a false sensation of movement, i.e., the sensation of movement in the absence of actual movement.

Answer 27

Vertigo may be caused by Ménière disease, labyrinthitis, positional vertigo, traumatic vertigo, perilymphatic fistula, and cervical vertigo. - Other causes include vascular disease of the brain stem, arteriovenous malformations, brain tumor, multiple sclerosis, drug overdose, and vertebrobasilar migraine.

Answer 28

Patients who experience vertigo will describe a sensation of movement without actually moving. Commonly, patients will describe their environment spinning around them. - Once you are convinced by the history that the patient is indeed experiencing an episode of vertigo, the next diagnostic question you have to answer is whether the vertigo is secondary to peripheral or central vestibular disease. - Central vertigo - gradual onset, no tinnitus or hearing loss, vertical nystagmus, multidirectional, presence of neighborhood signs - diplopia, cortical blindness, dysarthria, ext weakness, numbness - Peripheral vertigo - sudden, tinnitus or hearing loss is present, no neighborhood signs, horizontal nystagmus

Answer 29

Ménière disease is characterized by tinnitus, hearing loss, and episodic vertigo. Each episode lasts 1 to 8 hours. The symptoms wax and wane as the endolymphatic pressure rises and falls. The two most common causes of Ménière disease are syphilis and head trauma.

Answer 30

Benign paroxysmal positional vertigo is a cause of peripheral vertigo that characteristically is exacerbated by head movement or change in head position. - Typically, episodes will occur in clusters that persist for several days. - There will be a latency of several seconds after head movement before the onset of vertigo. The vertigo usually lasts 10 to 60 seconds.

Answer 31

Labyrinthitis presents with sudden onset of severe vertigo that lasts for several days with hearing loss and tinnitus. The disease frequently follows an upper respiratory tract infection.

Answer 32

Perilymphatic fistula is a form of peripheral vertigo related temporally to head trauma (blunt trauma to the ear, e.g., a slap to the ear) or extreme barotrauma during air flight, scuba diving, or vigorous Valsalva maneuver. Explosions deafen people.

Answer 33

Central vertigo is caused by any cerebellar or brain-stem tumor, bleed, or ischemia. Drug toxicity or overdoses are important causes of central vertigo. Also, in the young patient with unexplained central vertigo, consider multiple sclerosis.

Answer 34

Symptomatic treatment for peripheral vertigo includes meclizine or, in severe cases, diazepam.

Answer 35

Ménière disease is treated with a low-salt diet and diuretics. In patients who fail medical therapy, you can consider surgical decompression.

Answer 36

Benign paroxysmal positional vertigo is treated with positional maneuvers that attempt to move the otolith out of the circular canals (e.g., Dix Hallpike and Barany maneuvers).

Answer 37

Vertigo secondary to labyrinthitis is treated symptomatically with meclizine and diazepam when the symptoms are severe. Steroids help labyrinthitis.

Answer 38

Headache is defined as pain located in the head, neck, or jaw. - divided into primary or secondary headache syndromes. - Primary headache syndromes include migraine, cluster, and tension headache. - Secondary causes of headache include intracranial hemorrhage, brain tumor, meningitis, temporal arteritis, and glaucoma. - Migrane affects 15% of the general population

Answer 39

An essential point in the history is to determine whether this is the first episode of headache that the patient has experienced. A his- tory of recurrent symptoms makes the diagnosis of a primary headache disorder more likely. - headache w fever and nuchal rigidity suggests meningitis - nuchal rigidity w/o fever suggests intracranial hemorrhage - The history of vomiting that precedes the onset of headache by a number of weeks, or a history of headache induced by coughing, lifting, or bending, is typical of posterior fossa brain tumors.

Answer 40

Patients with temporal arteritis complain of a unilateral pounding headache associated with visual changes, described as dull and boring with superimposed lancinating pain. - Patients will also complain of polymyalgia rheumatica, jaw claudication, fever, weight loss, and scalp tender- ness (difficulty combing hair or lying on a pillow). - The scalp tenderness is from pain over the temporal artery. - Temporal arteritis is a disorder of the elderly, generally presenting in patients age >50. - Temporal arteritis gives an elevated sedimentation rate and is diagnosed with biopsy of the temporal artery. - don't wait for biopsy give steriods!

Answer 41

Migraine headaches are defined as a benign and recurrent syndrome of headache, nausea/ vomiting, and other varying neurologic dysfunctions. - Patients will describe the headache as pulsatile, throbbing, unilateral, and aggravated by minor movement. - Other associated features include photophobia, phonophobia, and the time to maximal pain (4 to 72 hours). - Migraine is a likely diagnosis when a typical trigger can be identified. - Typical triggers include alcohol, certain foods (such as chocolate, various cheeses, monosodium glutamate), hunger, or irregular sleep patterns. -

Answer 42

- migraine w/o aura is migraine w/o preceding focal neuro deficit - migraine w aura (classic migraine) is w/ preceding aura consisting of motor, sensory, or visual symptoms including the pathognomonic aura for classic migraine is the scintillating scotoma. visual aura - described as stars, sparks, and flashes of light. - complicated migraine is migraine with severe neurologic deficits which persist after the resolution of pain. - basilar migraine is migraine associated with symptoms consistent with brain-stem involvement (vertigo, diplopia, ataxia, dysarthria)

Answer 43

Tension-type headaches are described as tight, band-like headaches that occur bilaterally. Patients may also describe their headache as “vise-like,” and these headaches may be associated with tightness of the posterior neck muscles. Patients will describe their pain as one that builds slowly, and the pain may persist for several days with or without fluctuations.

Answer 44

Cluster headaches, common in men, begin without warning and are typically described as excruciating, unilateral, periorbital, and peaking in intensity within 5 minutes of onset. They are rarely described as pulsatile in nature. The attacks last from 30 minutes to 3 hours and occur 1–3× day for a 4-to-8-week period. Symptoms associated with cluster headaches include rhinorrhea, reddening of the eye, lacrimation, nasal stuffiness, nausea, and sensitivity to alcohol. Horner syndrome is sometimes found. Emotion and food rarely will trigger a cluster headache.

Answer 45

Patients with severe, sudden onset of a first-time headache accompanied by strong evidence for an underlying cause on history or physical examination should have a CT scan of the head to rule out any secondary causes. - primary headaches dx via clinically

Answer 46

Pharmacologic treatment for migraine headaches can be divided into management of an acute episode and prophylaxis. - Initially, for a mild migraine—which is defined as headache in the absence of nausea or vomiting—NSAIDs may be used. - Acutely, abortive therapy consists of sumatriptan, which acts as a serotonin receptor agonist. Dihydroergotamine is the alternative to the triptans. Ergotamine can be used in combination with caffeine. - The triptans are contraindicated in patients with known cardiovascular disease, uncontrolled hypertension, or pregnancy. In addition to sumatriptan, there is almotriptan, naratriptan, zolmitriptan, and eletriptan. These medications can be given orally, intranasally, or even subcutaneously, depending on the severity of the headache. Dopamine antagonist - such as metoclopramide can be given acutely as oral formulations to aid in the absorption of other abortive medications.

Answer 47

Prophylactic treatment for migraine therapy should be initiated when patients have acute migraine headaches >3–4/month. The best prophylactic medication is a beta blocker. - Propranolol, valproic acid, and topiramate are all considered first-line therapy for migraine prophylaxis. - These medications take 2 to 6 weeks to have an effect and can be discontinued gradually over 6 months once clinical stabilization has occurred.

Answer 48

NSAIDs, aspirin, acetaminophen Triptans Ergotamine derivatitves

Answer 49

``` BB CCB TCA SSRI VA Topiramate ```

Answer 50

Treatment for tension headaches consists of relaxation. Patients should be encouraged to find activities that are relaxing for them. Initial pharmacotherapy consists of acetaminophen and NSAIDs. If the headache remains refractory to these medications, a muscle relaxant can be added to the regimen.

Answer 51

Cluster headaches are treated with a triptan or 100% oxygen. Prophylaxis of cluster headaches is best done with a calcium channel blocker. Prednisone and lithium are sometimes used.

Answer 52

- An idiopathic increase in intracranial pressure also known as benign intracranial hypertension. - The disorder is 8 to 10 times more common in women. There is an association with obesity, chronic lung disease, Addison disease, oral contraceptives, tetracycline use, and vitamin A toxicity. Often there is no identified cause and the disorder resolves spontaneously after several months.

Answer 53

Patients present with a headache, visual disturbances such as diplopia, and sixth cranial nerve (abducens) palsy. Clinical findings include diplopia, papilledema, and enlargement of the blind spot on visual field testing. - The CT and MRI are normal, and evaluation of cerebrospinal fluid is normal beyond an increase in pressure.

Answer 54

Treatment consists of weight loss, removing offending agents such as oral contraceptives, and the use of diuretics such as acetazolamide or furosemide. Steroids such as prednisone may help as well. In urgent cases, repeated lumbar punctures may help. - definitive treatment can be achieved with the placement of a surgical shunt between the ventricles and the peritoneum.

Answer 55

Also known as tic douloureux, is an idiopathic pain syndrome resulting in sudden, severe, sharp pain starting near the side of the mouth and progressing to the ear, eye, or nostril. - Attacks can be triggered by touch or movement such as talking or by eating. Trigeminal neuralgia can be so severe as to be nearly incapacitating. The pain lasts for a few seconds and disappears. Despite the pain, the sensory examination will be normal. - Generally, trigeminal neuralgia is felt to be secondary to compression of the trigeminal nerve root by a blood vessel. Occasionally it can be a manifestation of multiple sclerosis or a posterior fossa tumor. - imagining is normal except posterior fossa tumor or MS

Answer 56

Carbamazepine is the standard of care for treatment. In those not controlled with carbamazepine, phenytoin, baclofen, or gabapentin can be tried. In those not responding to any form of medical therapy, surgery or radio-frequency lesioning into the affected nerve may work.

Answer 57

- An acute, often severe polyradiculopathy whose underlying pathophysiology is an autoimmune destruction of myelin. - Evidence suggests that GBS is caused by a misdirection of the immune response, where the body’s immune system attacks self-antigens mistaken for foreign antigens (molecu- lar mimicry).

Answer 58

Most patients will present with rapidly developing weakness that typically begins in the lower extremities and moves upward. On physical examination the patient is noted to lack reflexes in the muscle groups affected - The legs are usually more affected than the arms and face. Fever, constitutional symptoms, or bladder dysfunction are rare and should raise the possibilities of alternate diagnoses. - In addition to the motor weakness, patients will typically complain of sensory disturbances that can take the form of pain or tingling dysesthesia. Sensory changes are due to loss of large sensory fibers, producing loss of reflexes and proprioception. - Autonomic instability (profuse sweating, postural hypotension, labile blood pressure, cardiac dysrhythmias) occurs in severe GBS, requiring patient treatment in an intensive care unit - Approximately 75% of patients who present with GBS will have a history of an infection 1 to 3 weeks preceding the onset of symptoms. The infection is typically in the respira- tory or gastrointestinal systems (Campylobacter jejuni), although GBS may be preceded by infections with human herpesvirus, cytomegalovirus, or the Epstein-Barr virus.

Answer 59

previous viral infection | more frequently in patients with HIV, systemic lupus erythematous, and lymphoma.

Answer 60

A lumbar puncture for protein and cell count is always the best initial test. The characteristic finding is an elevated protein without an associate rise in the cell count on CSF. These changes in the cerebral spinal fluid do not occur until 48 hours after the onset of symptoms. - The most accurate test for the diagnosis is electro- myography (EMG). EMG is used to detect evidence of demyelination of the peripheral nerves.

Answer 61

- Treatment should be initiated as quickly as possible because available therapy becomes ineffective approximately 2 weeks after the onset of symptoms. - Intravenous immunoglobulin and plasmapheresis are equally effective treatments. There is no benefit to combination therapy. - Glucocorticoids are not effective in the treatment of acute GBS. Also, it is extremely important to monitor the vital capacity in patients with GBS and initiate early respiratory support to prevent death from respiratory failure.

Answer 62

``` Myasthenia gravis (MG) is a disease of the neuromuscular junction characterized by weakness and fatigability. - In myasthenia gravis, an autoimmune process characterized by acetylcholine-receptor antibodies leads to a decreased number of active and functional acetylcholine receptors at the postsynaptic membrane. ```

Answer 63

The major features in a patient’s history that help to diagnose myasthenia gravis are muscle weakness and fatigability. - Initially, patients will complain of diplopia, ptosis, and difficulty swallowing. Speech may have a “mushy” or nasal quality and facial weak- ness may manifest as a “snarling” appearance when smiling. - As the disease progresses, weakness may become generalized, involving proximal muscles in an asymmetric pattern. - Deep tendon reflexes are intact. Pupillary responses are normal. There are no sensory abnormalities. - Very severe disease may affect the muscles of respiration.

Answer 64

increasing muscle strength on repetitive contraction. Antibodies to Ca channels. This syndrome is seen in association with malignancy, especially small-cell carcinoma of the lung.

Answer 65

Botulism may cause a myasthenic-like illness, but the pupils are usually dilated and repetitive nerve stimulation (on EMG) shows an incremental increase in muscular fiber contraction (opposite of myasthenia gravis).

Answer 66

The best initial test for the diagnosis of myasthenia gravis is the acetylcholine-receptor antibody test. - In generalized myasthenia gravis, 80–90% of patients will have a positive test. In the presence of fatigable muscle weakness, a positive antibody test is specific and virtually diagnostic. - Antibodies are present in only 70% of those with disease limited to the eyes - The edrophonium (Tensilon) test is sensitive but not specific for the diagnosis. Additionally, patients may experience nausea, diarrhea, fasciculations, syncope (rare), or bradycardia during the test, which are cholinergic symptoms. - The most accurate test for the diagnosis of myasthenia gravis is electromyography (EMG). The characteristic finding is a decremental decrease in muscle fiber contraction on repetitive nerve stimulation.

Answer 67

Imaging studies of the chest such as x-rays and CT scan should be performed to detect a thymoma. Thymoma is found in 10–15% of patients. Thymic hyperplasia is found in 65%.

Answer 68

Anticholinesterase (usually pyridostigmine or neostigmine) medications are useful for the symptomatic treatment of MG. Pyridostigmine is longer lasting. If treatment with anticholinesterase medications is unsuccessful in providing symptomatic relief, the physician should consider immunosuppressive therapy. - There are numerous medications used for immunosuppressive therapy. These interventions primarily differ in the onset of therapeutic benefit. They are used if thymectomy is not effective. - Glucocorticoids are effective in improving weakness but take 1 to 3 months for you to observe a clinical benefit. Steroids are the initial immunosuppressive of choice.

Answer 69

If patients fail steroid therapy, azathioprine is the most widely used medication used in combination with steroids. The benefits of azathioprine therapy may take more than 3 to 6 months to peak. Cyclosporine and cyclophosphamide are alternatives to azothiaprine but are more toxi

Answer 70

Plasmapheresis and intravenous immunoglobulin are immunosuppressive therapies noted for their ability to rapidly improve weakness in myasthenia gravis. They are therefore reserved for patients in acute myasthenic crisis. These therapies are used when respiratory involvement occurs or when patients go to the operating room.

Answer 71

Thymectomy is indicated in postpubertal patients and in those younger than 60 years of age with generalized myasthenia gravis before initiation of immunosuppressive therapy. Thymectomy is performed in those not controlled with anticholinesterase medications to prevent the use of potentially toxic medication such as systemic steroids. Thymectomies are also performed when a thymoma is present to prevent the spread of malignant thymic disease.

Answer 72

Mycophenolate is a newer immunosuppressive drug with less adverse effects than steroids or cyclophosphamide.

Answer 73

aminoglycosides

Answer 74

The mean age of onset is about 57 years. etiology is unknown. - idiopathic condition due to both UMN & LMN neurons

Answer 75

muscle weakness with motor neuron loss, cranial nerve palsies, respiratory involvement, and lower motor neuron destruction, while at the same time preserving bowel, bladder sensory, cognitive, and sexual function. - The cranial nerve, or bulbar, palsies result in dysphagia, difficulty chewing, decreased gag reflex, dysarthria (difficulty in articulating words), and difficulty in handling saliva. - Since there is often respiratory muscle involvement, recurrent aspiration pneumonia is the most common cause of death. - A weak cough is also characteristic, and this only worsens the respiratory problem

Answer 76

There is no pain from abnormal sensory neuropathy because this is entirely a motor neuron disease. On the other hand, the upper motor neuron involvement gives significant spasticity that can lead to pain. Mentation, bowel, bladder, and sexual function remain intact for the same reason. In other words, a fully mentally alert patient loses nearly all motor control while still being able to think and perceive.

Answer 77

upper motor neuron manifestations are weakness with muscle wasting, atrophy, and fasciculations; this includes tongue atrophy. The combination of upper and lower motor neuron weakness is the unique presentation of ALS

Answer 78

The most accurate confirmatory test is the electromyogram which will show diffuse axonal disease. CPK levels are sometimes mildly elevated, and the cerebrospinal fluid and MRI scans are normal.

Answer 79

The only treatment that may slow down the progression of the disease is riluzole, which is thought to work by inhibiting glutamate release. Death typically results in 3–5 years. Spasticity is treated with baclofen and tizanidine

Answer 80

An autoimmune inflammatory disease of the CNS white matter characterized by a relapsing or progressive course. - The cause of multiple sclerosis (MS) is thought to be multifactorial. There is evidence that genetic susceptibility plays an important role. The disease occurs primarily in female populations of Northern European descent and of child-bearing age, respectively. This implies a role for some sort of environmental trigger (infectious, dietary, climatic). Pathologically, focal areas of demyelination are characteristic of the disease.

Answer 81

Commonly, patients will present complaining of weakness, numbness, tingling, or unsteadiness of a limb. Urinary urgency or retention, blurry vision, and double vision are all common initial manifestations of the disease. Symptoms may persist for several weeks or may resolve spontaneously over a few days. - several forms of the disease that may change the course of management and are therefore important to recognize

Answer 82

- relapsing remitting disease - progression is characterized by relapses of active disease with incomplete recovery during the periods of remisssion - secondary progressive disease - progression becomes more aggressive so that a consistent worsening of function occurs - primary progressive disease - symptoms are progressive from the onset of disease with early onset of disability (least common form)

Answer 83

Classically, the diagnosis is made clinically when a young patient (usually age <55) presents with a history of multiple neurologic complaints that cannot be explained by the presence of one CNS lesion. In other words, suspect the diagnosis when a patient presents with multiple neurologic deficits separated by time and space (anatomy).

Answer 84

A number of triggers are known to exacerbate the disease. Infections or trauma may acutely worsen the disease. Pregnancy, especially the 2 to 3 months following birth, may also exacerbate symptoms. However, there are generally fewer attacks during the pregnancy. Uncomplicated MS typically has no adverse effects on the outcome of the pregnancy.

Answer 85

MRI of the brain is the most accurate test to diagnose MS, reaching a sensitivity of 85 to 95% in symptomatic persons. Increased T2 and decreased T1 intensity represent the increased water content of demyelinated plaques in the cerebrum and spine. - Enhancement of lesions with gadolinium indicates active MS lesions that may enhance for up to 2 to 6 weeks after an exacerbation. MS is an unusual disease in that the best initial test for the diagnosis is also the most sensitive one, namely MRI of the brain and spine. - (CSF) reveals a mild pleocytosis (usually <50 cells)should be considered as evidence against the diagnosis of MS. An elevated IgG index (oligoclonal bands) is found in 70 to 90% of patients with MS. - The finding is nonspecific, and as a result, CSF for oligoclonal banding is recommended only when the MRI is nonconfirmatory but clinical suspicion for MS remains high

Answer 86

``` The treatment of multiple sclerosis can be divided into disease-modifying therapy, treatment of complications, and treatment for symptomatic relief during an acute exacerbation. The specific agents used depend on progression of the disease at the time of diagnosis. - there are 3 disease-modifying agents IFN B1a IFN B1b Glatiramer acetate aka copolymer I ```

Answer 87

In secondary progressive disease, interferon-

Answer 88

In patients who receive mitoxantrone, dose-related cardiotoxicity is a concern; mitoxantrone should be given only to patients with a normal ejection fraction. Mitoxantrone is not a first-line agent to prevent disease progression because of its cardiotoxicity

Answer 89

Mitoxantrone, cyclophosphamide, and natalizumab are not used for a first episode of disease.

Answer 90

For patients with spasticity, baclofen is the most effective medication. Tizanidine and diaz- epam are useful for nocturnal spasticity but are limited in their use for daytime symptoms because they cause intense somnolence

Answer 91

Pain secondary to trigeminal neuralgia and dys- esthesias responds well to carbamazepine, gabapentin, phenytoin, pregabalin, or tricyclic - Bladder hyperactivity is treated with oxybutynin, whereas urinary retention is treated with bethanechol. - Fatigue may be treated with amantadine or fluoxetine. - Erectile dysfunction can be treated with sildenafil acetate. - all IFN contra in pregnancy!!

Answer 92

Fingolimod is an oral disease-modifying medication that decreases rates of MRI progression. It prevents lymphocytes from proliferating outside of lymph nodes. Cardiac toxicity can be severe.

Answer 93

Dalfampridine is an oral disease-modifying medication that increases walking speed. It is a unique potassium channel blocker for which the precise mechanism of action (for improved walking speed) is not clearly known.

Answer 94

Cognitive function is measured by various mental functions, including memory, concentration, language, praxis, visuospatial functioning, and executive functions. “Dementia” refers to loss of memory with impairment of any other cognitive function suf- ficient to interfere with social or occupational functioning

Answer 95

Among the many reversible causes of dementia, you should consider hypothyroidism, vitamin B12 deficiency, hepatic or uremic encephalopathy, CNS vasculitis, syphilis, brain abscess, brain tumor (primary or metastatic), medications (especially anticholinergics), obstructive sleep apnea, central sleep apnea, trauma, subdural hematoma, normal pressure hydrocephalus (NPH), and depression. - Irreversible causes of dementia include PML, Pick disease, vascular dementia including multi-infarct dementia and Binswanger disease, and Creutzfeldt-Jakob disease (CJD). Alzheimer disease accounts for 60 to 80% of all causes. 5% of Alz is inherited

Answer 96

Alzheimer disease is, by definition, the loss of memory as well as other cognitive disturbances, such as aphasia, agnosia (the failure to identify entities despite intact sensory function), apraxia, or the loss of the ability to make plans and execute them. There is no single diagnostic test for Alzheimer disease.

Answer 97

Patients with frontotemporal dementias such as Pick disease will typically present with personality changes early in the course of their disease, with relative sparing of their visuospatial function. Social, interpersonal, and emotional abnormalities precede memory impairment. - Frontotemporal dementia is often noted primarily by the family because the patient lacks insight into their condition. There is no proven therapy for this condition.

Answer 98

confused with delirium is characterized by fluctuating cognitive impairment

Answer 99

Dementia secondary to Parkinson disease should be accompanied by clinical findings consistent with that disease. Recurrent visual hallucinations are also characteristic.

Answer 100

Dementia secondary to CJD is characterized by a shorter (weeks to months), more aggressive course than Alzheimer disease. Patients with CJD will present with dementia and myoclonus. Variant CJD is bovine spongiform encephalopathy (BSE). BSE is from the ingestion of prions from affected cattle. The diagnosis of CJD is by rapidly progressive dementia, myoclonus, ataxia, and the presence of 14-3-3 protein in the CSF. EEG may also help diagnose. These criteria can eliminate the need for brain biopsy.

Answer 101

Vascular dementia is divided into multi-infarct dementia, which typically has a stepwise progression associated with discrete cerebrovascular events, and Binswanger disease, involving the subcortical white matter, which presents with a slowly progressive course.

Answer 102

Normal pressure hydrocephalus will present with prominent gait abnormalities early in the course of the disease that usually precede the onset of cognitive impairment. There will also be associated urinary incontinence.

Answer 103

All patients with cognitive impairment should be assessed with a Mini Mental Status Examination (MMSE) to identify the areas of cognitive impairment. - Initially, the workup should focus on ruling out reversible causes of the dementia. If a reversible cause is identified, it should be treated, with the hope that cognitive function can be calcium, creatinine, liver function studies, glucose, thyroid-stimulating hormone (TSH), vitamin B12, RPR, and HIV. - Brain imaging is most useful for patients who have a focal neurologic exam, seizures, gait abnormalities, and an acute or subacute onset of their symptoms. - EEG and CSF evaluation are not necessary except for NPH-opening pressure. - No CSF marker is proven beneficial with the exception of 14-3-3 protein in CJD.

Answer 104

- Treatment of dementia revolves around insuring that the family and the patient have the proper medical and emotional support to cope with the disease. - Caregivers are at an increased risk for depression and anxiety. Their concerns and frustrations should be addressed at frequent intervals.

Answer 105

Raising the level of acetylcholine in CSF benefits patients with Alzheimer disease. - Pharmacotherapy with donepezil has been shown to improve cognitive function in mild to moderate dementia. - Other anticholinesterase inhibitors (rivastigmine, galantamine) appear to have similar efficacy. - Memantine is a disease-modifying drug used in advanced disease either alone or with a cholinesterase inhibitor. Memantine seems to be neuroprotective and reduces the rate of progression of disease.

Answer 106

- Huntington disease is caused by the presence of the HD gene located on chromo- some 4p. - The gene contains a CAG trinucleotide repeat expansion that codes for a protein called huntingtin. The HD mutation leads to abnormal cleavage of the huntingtin protein, interfering with nuclear mechanisms, and causing cell death. - The disease is inherited in an autosomal dominant fashion. Successive generations tend to have the disease occurring at an earlier age. This is called anticipation.

Answer 107

The clinical hallmarks of the disease include chorea and behavioral disturbance. Onset is usually in the fourth or fifth decade and can begin with either chorea or behavioral change. - The personality changes consist of irritability, anger, paranoia, or signs of depression. Antisocial behavior may develop. The chorea may begin as fidgeting that progresses to sudden movements of the trunk or limbs. - Gait is poorly coordinated and has a choreic quality. Memory is usually preserved until late in the disease but lack of judgment, disinhibition, and inattention are early manifestations. - There is frequently an associated depression. Dementia becomes severe later in the disease.

Answer 108

Diagnosis is made by genetically testing for the presence of the CAG trinucleotide DNA repeat expansion. There is a 50% chance of passing it on to children. CT scanning shows cerebral atrophy. Atrophy of the caudate nucleus is severe later.

Answer 109

Tetrabenazine helps the movement disorder of Huntington disease but will not reverse or cure the underlying disease process. Death occurs 15–20 years after the diagnosis. Haloperidol or clozapine can be used to control behavioral changes.

Answer 110

Parkinson disease is defined as a neurologic syndrome resulting from the deficiency of the neurotransmitter dopamine as a consequence of degenerative, vascular, or inflammatory changes in the basal ganglia.

Answer 111

There are numerous causes of Parkinsonism. Many drugs can cause Parkinsonism, including neuroleptic agents (haloperidol, chlorpromazine), antiemetics (metoclopramide), alpha-methyldopa, and reserpine. - Poisoning from MPTP, carbon monoxide, cyanide, and manganese are also causes of Parkinsonism. Any structural lesion around the basal ganglia (trauma, tumor, abscess, infarct) can produce clinical Parkinson disease. - Patients who have survived an episode of encephalitis can develop postencephalitic Parkinsonism.

Answer 112

The cardinal manifestations of Parkinson disease are bradykinesia (manifested by slow movements, mask facies, reduction of automatic movements), cog- wheel rigidity, postural instability, and resting tremor.

Answer 113

Bradykinesia Rigidity (cogwheel) Instability (postural) Tremor (resting)

Answer 114

Parkinsonism + vertical gaze palsy = supranuclear palsy Parkinsonism + prominent ataxia = olivopontocerebellar atrophy Parkinsonism + prominent orthostatic hypotension = Shy-Drager syndrome (now called multiple-system atrophy)

Answer 115

- Severe depression can cause a paucity of spontaneous movement that can mimic Parkinsonism. - Essential tremor can be mistaken for the tremor of Parkinson disease, but the lack of other neurologic symptoms and a positive family history of tremor and its amelioration with alcohol distinguish the two entities.

Answer 116

The diagnosis of Parkinson disease is a clinical one. It is important to identify any secondary causes of a patient’s Parkinsonism that are potentially reversible. There is no diagnostic test of choice that can identify patients with Parkinson disease.

Answer 117

There are many medications available for the treatment of Parkinson disease. The underlying pathophysiology that causes Parkinson disease is the imbalance of dopaminergic (too little) and cholinergic (too much) tone on the basal ganglia. Thus, medical treatment revolves around increasing dopaminergic tone or decreasing cholinergic tone on the basal ganglia. - he medications available for the medical treatment of Parkinson disease directly stimulate dopamine receptors (carbidopa/levodopa, dopamine agonists), indirectly increase the amount of dopamine available (COMT inhibitors, selegiline, amantadine), or block acetylcholine stimulation of the basal ganglia (benztropine, trihexyphenidyl).

Answer 118

Direct-acting dopamine agonists such as pramipexole or ropinirole can be used alone as initial therapy or in combination with small doses of levodopa/carbidopa. - Two other dopamine agonists are bromocriptine and cabergoline. All of them are less efficacious than levodopa. Dopamine agonists do, however, have less dyskinetic side effects. - Bromocriptine and pergolide are ergot derivatives and can cause cardiac toxicity.

Answer 119

The first step when considering what medication to start with is evaluating the patient’s functional status. Patients with an intact functional status are managed differently from patients with a compromised functional status. - Patients with intact functional status (less bradykinesia) are not generally given carbidopa/ levodopa as initial therapy. Such patients are started on anticholinergic medication when they are age 60, the treatment of choice is amantadine. The reason why anticholinergics are relatively contraindicated in elderly patients is because the side effects (dry mouth, urinary retention, constipation, confusion/hallucinations) occur more frequently and severely.

Answer 120

Anticholinergics such as benztropine and trihexyphenidyl are used mostly to relieve tremor and rigidity. Avoid with BPH and glaucoma.

Answer 121

For patients with compromised functional status (more significant bradykinesia), the best initial therapy is carbidopa/levodopa. Carbidopa inhibits extracerebral dopa-decarboxylase, allowing more of the levodopa to reach the central nervous system, where it is needed. Levodopa is the precursor to dopamine. Carbidopa protects the levodopa from breakdown in the periphery, ensuring its secure delivery to the central nervous system.

Answer 122

There are several late complications to carbidopa/levodopa therapy: Dyskinesia (abnormal movements), akathisia (restlessness), and “on-off” phenomena are all disconcerting to the patient. All of these late side effects are termed “response fluctuations” and can be managed by using a sustained release form of carbidopa/levodopa, adding a dopamine agonist, selegiline, or a COMT inhibitor, or restricting the main protein meal to the night.

Answer 123

COMT inhibitors are tolcapone and entacapone. They are always used in conjunction with levodopa to help reduce the dose or modify response fluctuations. COMT inhibitors have no effect alone; they decrease the metabolism of the levodopa. They are an adjunct to the use of levodopa to reduce adverse effects.

Answer 124

Selegiline was once thought to slow the progression of the disease. Selegiline can be used in those with a declining or fluctuating response to levodopa. Selegiline offers mild symptomatic benefit in early disease. - Rasagiline is a newer version.

Answer 125

This is an idiopathic disorder consisting of an isolated tremor of the hands, head, or both. The lower extremities tend to be spared. - Essential tremor can be worsened by the use of caffeine or beta agonists. - Examination reveals no other abnormalities. Although the level of disability tends to be limited, there can be interference with manual skills such as the ability to write. - It is characteristic of this disorder that there is an improvement with the use of alcohol. The patient will describe shaky hands, which improve with 2–3 drinks.

Answer 126

There is no specific diagnostic test for this disorder. Treatment is propranolol. If propranolol is ineffective, alternate medications are primidone, alprazolam, and clozapine. If no medical therapy is effective, thalamotomy is indicated.

Answer 127

RLS is idiopathic condition resulting in sensation of creeping and crawling dysesthesia within the legs, leading to involuntary movements during sleep. - Often the condition is brought to attention because of multiple bruises sustained by the sleep partner. - The condition can be familial and is exacerbated by sleep deprivation, caffeine, and pregnancy. - There is also an association with uremia, iron deficiency, and peripheral neuropathy.

Answer 128

There is no specific diagnostic test for this disorder. Treatment is a dopamine agonist such as pramipexole or ropinirole, although some patients may need levodopa/carbidopa. Other therapies are narcotics and benzodiazepines.

Neurology Flashcards

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