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Internal Med MKSAP Neurology
Terms in this set (56)
Diagnose normal pressure hydrocephalus.
* The triad of gait apraxia, dementia, and urinary incontinence, especially when accompanied by enlarged ventricles, is suggestive of normal pressure hydrocephalus.
This patient exhibits the classic triad of gait impairment (specifically, gait apraxia), dementia, and urinary incontinence that typifies the potentially reversible syndrome of normal pressure hydrocephalus (NPH). This triad of symptoms eventually occurs in most patients with dementia, and the diagnosis of NPH is often considered but much less often proved to be the correct diagnosis. In this patient, however, strong evidence supports a diagnosis of NPH, including the MRI evidence of ventriculomegaly. Although Alzheimer dementia (AD) is also associated with cognitive impairment and impaired gait, gait does not improve after removal of cerebrospinal fluid in AD as it does in NPH.
Treat a menstrually related migraine with an evidence-based modality.
* Evidence supports the use of mefenamic acid for perimenstrual prophylaxis of menstrually related migraines, with treatment starting 2 days prior to the onset of flow or 1 day prior to the expected onset of the headache and continuing for the duration of menstruation.
This patient should be treated with mefenamic acid. She has migraine with aura, migraine without aura, and menstrually related migraine. Her menstrually related headaches are less responsive to acute therapy than are the non-menstrually related attacks, and headache recurs daily throughout menses. The best management for this patient is, therefore, the perimenstrual use of a prophylactic agent. There is evidence that supports the use of mefenamic acid for perimenstrual prophylaxis, with treatment starting 2 days prior to the onset of flow or 1 day prior to the expected onset of the headache and continuing for the duration of menstruation. In this patient, that would mean beginning 3 days before the onset of menstrual flow and continuing throughout menstruation.
The use of combined oral contraceptive therapy (estrogen plus progestin) is contraindicated in this woman because of her history of migraine with aura. Women with migraine with aura are at a two-fold increased risk of ischemic stroke, ischemic myocardial infarction, and venous thromboembolism. The risk of stroke is increased further, up to eight-fold, in women with migraine with aura who use combined oral contraceptive pills.
Test for diabetes mellitus and impaired glucose tolerance in a patient with suspected small-fiber peripheral neuropathy.
* A history of burning or lancinating distal extremity pain and examination findings showing only sensory loss suggest a small-fiber peripheral neuropathy, which is most frequently associated with diabetes mellitus and impaired glucose tolerance.
A 2-hour glucose tolerance test to detect diabetes mellitus or impaired glucose tolerance is the most appropriate next diagnostic study for this patient. The patient's history of burning feet, in conjunction with neurologic examination findings showing only distal sensory loss (with normal reflexes and muscle strength), suggests a small-fiber peripheral neuropathy. The most common identifiable cause of small-fiber peripheral neuropathy is diabetes or impaired glucose tolerance. This patient's fasting plasma glucose level, which is just over the upper limit of normal, should prompt a 2-hour glucose tolerance test. This test is more sensitive and can detect patients with diabetes and a normal fasting plasma glucose level. Approximately 30% of patients with normal fasting plasma glucose levels will have an oral glucose tolerance test diagnostic for diabetes. A result of 140 mg/dL to 199 mg/dL (7.8 mmol/L to 11.0 mmol/L) from this test establishes a diagnosis of impaired glucose tolerance, and a result of 200 mg/dL (11.1 mg/dL) or greater is diagnostic of diabetes.
Evaluate the risk of seizure recurrence after a generalized tonic-clonic seizure.
* After a single unprovoked seizure, a greater risk of recurrence is predicted if the event was a partial seizure or if the patient has Todd paralysis, status epilepticus on presentation, an age greater than 65 years, or abnormal findings on neurologic examination.
The presence of Todd paralysis predicts a greater risk of future seizures in this patient. After a single unprovoked seizure, recurrence risk in the subsequent 2 years has been reported to be 30% to 40%. Many historical or examination findings predict a higher risk of recurrence, including presentation in status epilepticus, age greater than 65 years, known underlying neurologic disorder(s) or structural lesion(s), and Todd paralysis. Partial-onset seizures are also more likely to recur, perhaps because of the increased likelihood of there being an underlying causative structural lesion. Todd paralysis is a transient unilateral or focal weakness often seen after partial seizures, with or without secondary generalization. Todd paralysis or other focal abnormalities on the neurologic examination predict a greater risk of a future seizure. Patients with an electroencephalogram or head imaging scan showing a potentially epileptogenic lesion are also in the higher-risk category.
Diagnose intracerebral hemorrhage in a patient with hypertension.
* The classic presentation of intracerebral hemorrhage is the sudden onset of a focal neurologic deficit with subsequent symptomatic progression over minutes to hours.
This patient most likely has had an intracerebral hemorrhage. The classic presentation of an intracerebral hemorrhage is the sudden onset of a focal neurologic deficit with subsequent symptomatic progression over minutes to hours. Headache, vomiting, hypertension, and an impaired level of consciousness are its most common clinical accompaniments and help distinguish an intracerebral hemorrhage from an ischemic stroke. Intracranial hemorrhage, which accounts for 11% of stroke deaths, has symptoms similar to those of ischemic stroke at presentation; therefore, it cannot always be reliably distinguished from ischemic stroke by clinical criteria alone. The definitive diagnostic study is CT of the head or MRI of the brain. Imaging provides diagnostic and prognostic information. The volume of intracranial hemorrhage and level of consciousness are the two most powerful predictors of outcome in this setting.
A severe hemispheric ischemic stroke can cause severe focal neurologic dysfunction but generally does not progress or worsen as rapidly as occurred with this patient.
Evaluate suspected Lambert-Eaton myasthenic syndrome.
* The diagnosis of Lambert-Eaton myasthenic syndrome, a neuromuscular junction disorder that causes progressive proximal muscle weakness and areflexia, precedes the clinical recognition of cancer in up to 50% of patients.
This patient most likely has Lambert-Eaton myasthenic syndrome, as suggested by his history of proximal upper and lower limb weakness, the presence of autonomic symptoms (dry eyes/mouth, erectile dysfunction), and the finding of absent deep tendon reflexes on examination. These are characteristic signs and symptoms of the syndrome. Lambert-Eaton myasthenic syndrome is a neuromuscular junction disorder caused by disordered calcium channel function on the presynaptic nerve terminal. In most patients with this disorder, antibodies to voltage-gated P/Q-type calcium channel receptors exist. Lambert-Eaton myasthenic syndrome is typically a paraneoplastic syndrome, caused by or associated with an underlying malignancy, particularly small cell lung cancer. The diagnosis of Lambert-Eaton myasthenic syndrome precedes the clinical diagnosis of cancer in up to 50% of affected patients; therefore, in patients with newly diagnosed Lambert-Eaton myasthenic syndrome, a thorough search for an underlying cancer should be performed. If no evidence of malignancy is found, these patients should be evaluated serially for occult malignancy. In addition to elevated levels of voltage-gated P/Q-type calcium channel antibodies, the diagnosis can be confirmed through electrodiagnostic studies, particularly repetitive nerve stimulation studies, which show an increase in the muscle action potential (increment) after brief exercise.
Elevated levels of antibodies against acetylcholine receptors are present in 90% of patients with generalized myasthenia gravis. Myasthenia gravis is an autoimmune disorder that results in neuromuscular transmission failure, causing weakness of limb and cranial muscles. The diagnosis is confirmed through electrodiagnostic testing, including repetitive nerve stimulation studies and, in some patients, single-fiber electromyography. The presence of an elevated acetylcholine receptor antibody level may provide additional confirmatory evidence supporting the diagnosis of myasthenia gravis. In this patient, the absence of any bulbar signs or symptoms, such as ptosis, visual symptoms (blurred vision or diplopia), or dysphagia, in conjunction with absent deep tendon reflexes, would argue against myasthenia gravis.
Treat memory loss in a patient with Alzheimer dementia.
* First-line pharmacotherapy for mild Alzheimer dementia is an acetylcholinesterase inhibitor.
This patient should receive donepezil. The Folstein Mini-Mental State Examination (MMSE) discriminates well between the major stages of dementia used for prognosis and management purposes. The MMSE score range of 21 to 25 corresponds to mild dementia, 11 to 20 to moderate dementia, and 0 to 10 to severe dementia. This patient has Alzheimer dementia and is at a mild to moderate stage of impairment. The most appropriate medication with which to begin treatment is an acetylcholinesterase inhibitor of which there are currently three: donepezil, rivastigmine, and galantamine. Multiple large, prospective, randomized, double-blind, placebo-controlled studies have shown in patients with mild, moderate, or severe Alzheimer dementia the efficacy of donepezil (and its superiority to placebo) in the preservation of instrumental and functional activities of daily living and in the reduction of caregiver stress. Other studies have found that patients treated with donepezil have improved cognitive function compared with those treated with placebo. Donepezil was safe and well tolerated in this patient group.
Memantine is also used to treat Alzheimer dementia, but only in patients with moderate to severe impairment. There is no evidence that memantine has any effect in earlier stages of Alzheimer dementia or that it alters the course of the disease. With a score of 24/30 on the MMSE, this patient has mild dementia, which makes memantine an inappropriate treatment. In patients with severe dementia, memantine can be used alone or added to an acetylcholinesterase inhibitor.
Evaluate trigeminal neuralgia.
* Because trigeminal neuralgia usually presents after age 40 years, its diagnosis in a younger patient should prompt an evaluation for secondary causes, such as multiple sclerosis, posterior fossa tumors, and vascular or aneurysmal compression of the trigeminal nerve. Everyone should get an MRI, 10% have intracranial lesion.
This patient, whose history is most compatible with a diagnosis of trigeminal neuralgia, should have an MRI of the brain. In 90% of patients with trigeminal neuralgia, idiopathic disease presents after age 40 years. Trigeminal neuralgia in a patient this young should prompt evaluation for secondary causes, such as multiple sclerosis, posterior fossa tumors, and vascular or aneurysmal compression of the trigeminal nerve. In light of her history of transient paresthesias 3 years ago, multiple sclerosis is high among the diagnostic possibilities for this patient. An MRI can show the demyelination typical of multiple sclerosis.
Trigeminal neuralgia is a unilateral condition characterized by brief episodes of lancinating pain in the distribution of one or more divisions of the trigeminal nerve, invariably V2 and/or V3. The pain is excruciating and often described as sudden, sharp, superficial, and stabbing or burning in quality. The pain is paroxysmal and usually lasts 2 to 3 seconds and may occur in volleys of jabs or stabs of pain. The paroxysms are often punctuated by pain-free periods lasting seconds to hours. Characteristically, the painful episodes are associated with trigger zones, particularly around the mouth and nostrils, and the pain may be triggered by trivial stimuli, such as wind on the face, brushing the teeth, shaving, chewing, or even talking.
Up to 10% of patients with trigeminal neuralgia may harbor an intracranial lesion; therefore, an MRI should be obtained for every patient with trigeminal neuralgia, even those who respond to medication and whose examination findings are normal.
Treat myasthenic crisis.
* Myasthenic crisis, a potentially life-threatening neurologic emergency characterized by muscle weakness severe enough to necessitate intubation, typically requires plasma exchange therapy.
This patient has a rapidly progressive neurologic disorder with weakness of limb, bulbar, and respiratory muscles and should undergo plasma exchange. Given the absence of sensory loss and normal deep tendon reflexes, myasthenia gravis is the most likely diagnosis. Because of the rapid progression of the disease and the involvement of respiratory and bulbar muscles, the patient should be admitted to the hospital for management of a myasthenic crisis. Myasthenic crisis is a potentially life-threatening neurologic emergency characterized by weakness that is severe enough to necessitate intubation. Electromyographic studies, including repetitive stimulation of motor nerves, are indicated to establish a diagnosis of myasthenia gravis.
Plasma exchange is the treatment of choice in patients with myasthenic crisis. Myasthenia gravis is an autoimmune disorder that results from antibody-mediated attacks on the postsynaptic neuromuscular junction. This process may impair neuromuscular transmission by functional blockade of the acetylcholine receptor, by accelerating the degradation of acetylcholine receptors, and by causing damage to the postsynaptic membrane of the neuromuscular junction. Plasma exchange presumably removes these circulating antibodies.
Prednisone is an effective therapy in the treatment of patients with myasthenia gravis. However, it is not appropriate as the initial, sole therapy in patients in the midst of a myasthenic crisis. A worsening of muscular weakness is seen in some patients within the first 3 weeks of the initiation of this medication. Even if this complication does not occur, the beneficial effects from prednisone typically do not occur for 3 to 4 weeks after drug initiation. In a patient with myasthenic crisis, prednisone is often initiated after the patient has been stabilized and is improving with plasma exchange. Used in this fashion, prednisone should reach maximum efficacy by the time the beneficial effects of plasma exchange have waned (6-8 weeks).
Evaluate a suspected spinal dural arteriovenous fistula.
* Spinal dural arteriovenous fistula should be suspected in any patient with a chronic or subacute progressive myelopathy with episodes of more rapid, stepwise clinical deterioration.
The most appropriate next diagnostic test for this patient is spinal angiography for the specific purpose of confirming the presence of a spinal dura-based arteriovenous fistula. Dural arteriovenous fistula should be suspected in any patient with a chronic or subacute progressive myelopathy with episodes of more rapid, stepwise clinical deterioration.
In this patient, the diagnosis is suggested by the clinical history of stepwise spinal cord dysfunction without significant recovery and the MRI finding of a swollen spinal cord with dorsal flow voids, which is indicative of tortuous blood vessels. The spinal cord lesion represents cord congestion due to an abnormal connection between the high-pressure arterial and low-pressure venous drainage systems serving the spinal cord circulation. Patients with such spinal fistulas are often mistakenly diagnosed as having multiple sclerosis (MS), which is the most common nontraumatic disabling disease of young adults.
Diagnose carotid artery dissection.
* Dissection of the cervical arteries, although an infrequent occurrence, is a leading cause of stroke in young and otherwise healthy persons.
The most likely clinical diagnosis to explain this patient's focal neurologic symptoms is a left internal carotid artery dissection with resultant transient ischemic attacks (TIAs). Carotid dissection characteristically develops after head or neck trauma but may occur spontaneously. Manifestations of this condition include ipsilateral throbbing neck, head, or orbital pain with possible Horner syndrome. The pain from carotid dissection may occur suddenly or develop gradually. Such a dissection can cause a TIA or ischemic stroke by one of two mechanisms: either the mural hematoma expands to the point of occluding the lumen, or a thrombus forms and embolizes to cause distal arterial branch occlusion. Dissection of the cervical arteries, although an infrequent occurrence, is a leading cause of stroke in young and otherwise healthy persons. Magnetic resonance angiography of the carotid arteries and carotid duplex ultrasonography are indicated in the clinical evaluation of possible carotid dissection.
Manage asymptomatic meningioma.
* Small, asymptomatic meningiomas in older adults should be monitored with serial imaging.
This patient should have serial MRIs of the brain. Meningiomas are benign, slow-growing tumors that are often discovered as an incidental finding when head imaging is obtained for evaluation of unrelated symptoms. Their appearance on CT scans and MRIs is diagnostic. Appropriate management depends on the age of the patient and the size and location of the tumor. For this patient, who is elderly and has a small, asymptomatic meningioma, the most appropriate management strategy is to follow up with serial MRIs of the brain. In patients with incidentally diagnosed meningiomas, most clinicians perform imaging studies every 3 to 6 months during the first year. If the tumor remains stable, yearly imaging may be performed for the next 5 to 10 years. Densely calcified meningiomas are particularly indolent.
Distinguish migraine from sinus and tension-type headaches.
* Approximately 90% of patients who present in an ambulatory care setting with a chief symptom of recurrent, moderately severe headache have a form of migraine.
This patient most likely has migraine without aura. Approximately 90% of patients in an ambulatory care setting with a chief symptom of recurrent, moderately severe headache have a form of migraine. Her 6-year history of episodic headache meets the International Classification of Headache Disorders criteria for migraine. There is no single criterion that is necessary or sufficient to make the diagnosis of migraine. In this particular example, this patient does not have unilateral or throbbing pain, and there is no associated photophobia, phonophobia, or vomiting, features that are often considered necessary for the diagnosis. Her headache is preceded by premonitory symptoms (yawning and irritability), which occur in approximately 75% of patients with migraine. In addition, her attacks are triggered by stress and changes in barometric pressure, both of which are frequent triggers for migraine. Depression is a comorbid disorder that often leads to a misdiagnosis of tension-type headache.
Chronic migraine (previously referred to as "transformed" migraine) is a very common cause of chronic daily headaches and is defined as headaches occurring on more than 15 days per month that meet the diagnosis of migraine without aura or respond to ergots or triptans (migraine-specific drugs) on at least 8 of those days. This patient does not meet the diagnostic criteria for this type of headache.
Tension-type headache is a dull, bilateral, or diffuse headache that is often described as a pressure or squeezing sensation of mild to moderate intensity. This type of headache has no accompanying migraine features (such as nausea), and its pain does not prohibit activity, characteristics that do not match this patient's symptoms. Headache triggered by stress is often mistaken as tension-type headache, even though stress is one of the most commonly reported migraine triggers. Up to 75% of patients with migraine report neck pain that precedes or occurs during the attack, and this scenario is frequently mistaken for tension-type headache.
Evaluate suspected Guillain-Barré syndrome.
* Guillain-Barré syndrome is a disorder associated with rapidly progressive extremity weakness, paresthesias, and areflexia.
This patient should undergo electromyography (EMG). She has a rapidly progressive disorder affecting the peripheral nervous system, most compatible with a clinical diagnosis of Guillain-Barré syndrome. Patients with Guillain-Barré syndrome typically develop paresthesias distally in the lower extremities that are followed by limb weakness and gait unsteadiness. In addition to sensory loss and limb weakness, deep tendon reflexes are characteristically absent or markedly reduced. The diagnosis is confirmed by EMG, which usually shows a demyelinating polyradiculoneuropathy. Cerebrospinal fluid (CSF) analysis characteristically shows albuminocytologic dissociation, whereby the spinal fluid cell count is normal but the spinal fluid protein level is elevated. CSF analysis may also yield normal results early in the course of the disease. However, a normal CSF cell count is useful in excluding other infectious conditions, such as polyradiculoneuropathies associated with HIV and cytomegalovirus infection, infection due to West Nile virus, and carcinomatous or lymphomatous nerve root infiltration. By definition, symptoms in patients with Guillain-Barré syndrome peak within 4 weeks of onset. Poor prognostic features include rapid symptom progression, respiratory failure, EMG evidence of axonal degeneration, and advanced age. Intravenous immune globulin and plasma exchange are equally efficacious in the treatment of Guillain-Barré syndrome.
Manage a thunderclap headache.
* Thunderclap headache is a potential neurologic emergency that requires urgent imaging of the cerebral vasculature with either magnetic resonance or CT angiography after a noncontrast CT scan of the head and a lumbar puncture have excluded subarachnoid hemorrhage.
* A lumbar puncture with subsequent cerebrospinal fluid analysis is necessary in any patient with thunderclap headache and normal findings on a CT scan to fully evaluate a possible subarachnoid hemorrhage.
This patient should undergo CT angiography of the head and neck. She has experienced a thunderclap headache, which is a severe and explosive headache that is maximal in intensity at or within 60 seconds of onset. Every thunderclap headache must be immediately evaluated to detect potentially catastrophic conditions, especially subarachnoid hemorrhage. Most of the other causes of thunderclap headache, such as an unruptured cerebral aneurysm, a carotid or vertebral artery dissection, cerebral venous sinus thrombosis, and reversible cerebral vasoconstriction syndrome, can be excluded by noninvasive angiography. Therefore, CT angiography of the head and neck is the most appropriate next step in management. CT angiography can detect unruptured aneurysms as small as 3 mm in diameter and thus is adequate to exclude this diagnosis. Magnetic resonance angiography (MRA) would also be appropriate in this setting. Both CT angiography and MRA can be performed with a venous phase to exclude cerebral venous sinus thrombosis. Given that most causes of thunderclap headache can be excluded by such noninvasive angiography and that prior cerebrospinal fluid analysis has shown no evidence of a subarachnoid hemorrhage in this patient, conventional cerebral angiography, in which a catheter is inserted into a large artery and advanced through the carotid artery, is unnecessary.
Treat an acute hemispheric stroke.
* A patient with acute ischemic stroke who is taking warfarin but who has a subtherapeutic INR (≤1.7) is still eligible to receive thrombolysis with intravenous recombinant tissue plasminogen activator within the recommended window of 3 hours from stroke onset.
This patient should receive intravenous recombinant tissue plasminogen activator (rtPA). He has clinical symptoms and signs and radiologic evidence of an acute left hemispheric stroke. The probable mechanism of stroke is a cardioembolism, given his history of atrial fibrillation and his subtherapeutic INR. He was brought to the emergency department within 1 hour of the witnessed onset of stroke symptoms, and his evaluation is completed 1 hour later. He does not appear to have any clinical, radiologic, or laboratory contraindication to receiving the preferred treatment of intravenous rtPA, and he can receive it within the recommended window of 3 hours from stroke onset. The standard dose is 0.9 mg/kg. Although he is on warfarin, rtPA is not contraindicated because his INR is 1.5; an INR less than or equal to 1.7 is required for rtPA administration in a patient on anticoagulation.
Aspirin is indicated for acute ischemic stroke in patients who are not eligible for rtPA, as this patient is. For patients with acute stroke who are eligible for thrombolysis, aspirin should be withheld in the emergency department and for 24 hours after rtPA administration.
Diagnose multiple system atrophy.
* Multiple system atrophy is a sporadic, heterogeneous, neurodegenerative disorder that causes impairment of multiple neurologic systems, including the autonomic nervous system, the extrapyramidal system, and the cerebellum.
Multiple system atrophy is the most likely diagnosis in this patient. He has a progressive neurologic disorder characterized by signs and symptoms that suggest impairment of multiple neurologic systems; these include the autonomic nervous system (orthostatic hypotension, erectile dysfunction, constipation), the extrapyramidal system (rigidity, impaired gait), and the cerebellum (limb ataxia). Multiple system atrophy is a progressive, ultimately fatal neurodegenerative disorder that typically causes dysautonomia, parkinsonism, and ataxia, in some combination, in affected patients. Multiple system atrophy is a clinical diagnosis that is suggested by the presence of these various features in the same patient.
* Multiple sclerosis begins as a relapsing-remitting disorder in 85% of patients and a primary progressive disorder in 15% of patients; those with the relapsing-remitting type have a greater than 50% risk of developing a secondary progressive disease course.
This patient experienced an episode of transient neurologic dysfunction (an attack) at disease onset and thus has relapsing-remitting multiple sclerosis (MS). Eighty-five percent of patients with MS have this type of disease onset. In relapsing-remitting MS, relapse frequency declines over time, and relapses do not become more severe with increasing disease duration; however, recovery from individual events may be slower and less complete.
Primary progressive MS, which the other 15% of patients with MS have at disease onset, is defined as gradually worsening neurologic function over more than 1 year without recovery. This patient has recovered completely from two neurologic episodes and is currently asymptomatic. Therefore, he cannot be classified as having primary progressive MS.
Secondary progressive MS is characterized by gradual, unremitting development of new symptoms over months to years in a patient who previously had a relapsing-remitting course. The lifetime risk of conversion from relapsing-remitting to secondary progressive disease is greater than 50%, but the onset and rate of the progressive phase are highly variable and not predictable for individual patients. The median time from MS onset until conversion to the secondary progressive phase typically ranges from 10 to 15 years. The establishment of a secondary progressive course is a risk factor for substantial disability, such as loss of independent ambulatory function; the median time from MS onset to the point at which unilateral gait assistance (such as a cane) is required is 15 to 25 years. This patient first had transient symptoms only 6 months ago and thus cannot be characterized as having secondary progressive disease.
Benign MS is defined loosely as no or minimal neurologic impairment 15 or more years after MS onset. This category may encompass as many as 20% of all patients with MS. The definition of benign MS is controversial because continued follow-up of such patients often uncovers late progressive disease and disability accrual. However, a small minority of patients with MS live a long and essentially unrestricted life. The factors that predict a benign course early in the disease have not yet been identified. This patient's MS diagnosis is too recent to be categorized as benign at this point.
Treat an acute dystonic reaction.
* Medications that block dopamine receptors can cause acute dystonic reactions, which can be readily treated with benztropine.
This patient is experiencing a prochlorperazine-induced dystonic reaction, which is best treated with benztropine. Acute dystonic reactions are characterized as torticollic (twisted or a turned head, neck, or face), oculogyric (deviated or rolling gaze), buccolingual (pulling sensation of the tongue), or opisthotonic (trunk or entire-body spasm). Medications, such as prochlorperazine, that block dopaminergic receptors in the extrapyramidal system can result in acute dystonic reactions. Intravenous anticholinergic agents, such as benztropine or diphenhydramine, are the treatment of choice in the acute treatment of dystonic reactions. Benzodiazepine medications can also be helpful in the acute setting. Oral anticholinergic medications are continued for 48 to 72 hours to prevent relapse.
Treat an acute carotid dissection in a patient with headache.
* Acute unilateral headache with associated Horner syndrome represents acute carotid dissection until proved otherwise.
This patient has a carotid dissection, for which he should receive heparin. Acute headache with oculosympathetic paresis (Horner syndrome) is a common presentation of carotid dissection and must be assumed to be carotid dissection until proved otherwise. Such a headache may precede the onset of cerebral ischemia (transient ischemic attack or stroke) by days to weeks. Additional manifestations of this condition may include ipsilateral throbbing neck or orbital pain. The pain from carotid dissection may occur suddenly or develop gradually. Neurologic abnormalities due to ischemia in the ipsilateral hemisphere (causing contralateral numbness or weakness) or retina (causing ipsilateral monocular visual symptoms) also may occur. Magnetic resonance angiography of the carotid arteries and carotid duplex ultrasonography are indicated in the clinical evaluation of possible carotid dissection. Although the management of carotid dissection is controversial, most experts agree on the intravenous use of heparin in the acute setting to prevent distal embolism and stroke.
Acute severe headache always requires imaging of the neck and cerebral blood vessels to rule out a vascular cause. This patient's magnetic resonance angiogram of the neck shows a long, tapered stenosis of the left internal carotid artery in the neck, which indicates dissection.
Manage juvenile myoclonic epilepsy in a young adult.
* Juvenile myoclonic epilepsy requires lifelong antiepileptic drug therapy.
This patient requires life-long treatment of her epilepsy. Appropriate recognition of specific epilepsy syndromes is essential because it will guide the clinician in selecting the appropriate therapy, making the correct prognosis, and, in some instances, providing genetic counseling. A history of myoclonic and generalized tonic-clonic seizures on awakening with onset in adolescence is highly suggestive of the syndrome of juvenile myoclonic epilepsy, which is one of the most commonly encountered forms of epilepsy, possibly affecting 5% to 10% of all patients with epilepsy.
Seizures in juvenile myoclonic epilepsy are generally well controlled with medication, but relapses can be provoked by sleep deprivation, alcohol, or exposure to flickering lights. If medication is withdrawn, seizures will recur in 75% to 100% of patients. Therefore, in this patient with a history of juvenile myoclonic epilepsy, medication withdrawal is not recommended either now, 3 months from now, or in 2 years; juvenile myoclonic epilepsy requires lifelong therapy.
Diagnose mild cognitive impairment vs. Alzheimer's
* Mild cognitive impairment denotes abnormal cognitive decline that is not severe enough to produce disability.
This patient has mild cognitive impairment (MCI), which denotes abnormal cognitive decline that is not severe enough to produce disability. His self-reported memory loss, which is confirmed by his performance on the Folstein Mini-Mental State Examination, is his only symptom; there are no other signs of dementia. Memory loss is nonspecific and is part of many dementia syndromes. However, the lack of any functional impairment in this patient makes MCI the most likely diagnosis at this time. Although there are no universally accepted criteria for MCI, the disorder has been defined as a memory abnormality corroborated by objective memory impairment on standardized tests, without general cognitive impairment or an effect on functional independence. The rate of progression to dementia is approximately 10% to 15% per year.
Alzheimer dementia is the most common cause of MCI involving memory loss. Because this patient has no functional disabilities and thus does not meet the criteria for frank dementia, Alzheimer dementia is an incorrect diagnosis at this point. He may eventually develop the disease, given that the conversion rate of MCI to dementia is roughly 10% to 15% per year and that, at autopsy, approximately 80% of patients originally diagnosed with MCI have Alzheimer dementia.
dementia with Lewy bodies
* Dementia with Lewy bodies is characterized by dream-enactment behavior, cognitive decline, parkinsonism, and visual hallucinations.
This patient has symptoms of dream-enactment behavior, cognitive decline, parkinsonism, and visual hallucinations, which together are the hallmark features of dementia with Lewy bodies. Also characteristic of this type of dementia are the physical findings of orthostatic hypotension and features of mild parkinsonism, such as a reduced degree of facial expression (hypomimia), reduced arm swing, stooped posture, and mild cogwheeling.
Treat fatigue related to multiple sclerosis.
* Adequate rest and regular physical exercise can reduce multiple sclerosis-related fatigue, but many patients require treatment with stimulants, such as amantadine.
This patient should receive a course of amantadine. Fatigue is the most common symptom of multiple sclerosis (MS) but is also highly prevalent in the general population. The fact that the patient's fatigue began with a relapse strengthens its association with her MS, but evaluation for other causes is still required. Her review of systems, physical examination, and laboratory studies do not reveal another likely cause, and she is already sleeping and exercising regularly. Therefore, pharmacologic therapy can reasonably be offered. Amantadine has been shown in several small placebo-controlled studies to reduce MS-related fatigue. The mechanism is not clearly understood but is likely related to the drug's stimulant properties.
Treat migraine during pregnancy.
* Migraines during pregnancy that do not respond to simple analgesia can be safely and effectively treated with metoclopramide.
Metoclopramide has been shown to have efficacy for both the pain and nausea associated with migraine. This drug has a U.S. Food and Drug Administration (FDA) rating of pregnancy category B. Acute medications with an FDA rating of pregnancy category A or B carry the lowest risk of harm to the fetus and should be used preferentially for the treatment of migraine during pregnancy.
Amitriptyline and verapamil are used for migraine prophylaxis. Both have an FDA rating of pregnancy category C (risk to the fetus cannot be ruled out) and are not contraindicated for pregnant women. However, prophylactic migraine therapy should be avoided whenever possibly during pregnancy, especially in the first trimester. Often, migraine improves during the second and third trimesters of pregnancy, so acute therapy during the first trimester is preferred. If migraine continues to be disabling and occurs frequently during the second and third trimesters, prophylactic therapy could be considered, especially if the health of the mother and/or the fetus is compromised.
Types of seizures/epilepsy
Epilepsy is classified as partial or generalized on the basis of the areas of the brain involved at onset. Seizures that present in adulthood are usually partial in onset; partial seizures in which the patient maintains full awareness are classified as simple partial, whereas those involving an alteration of consciousness, as in this patient, are classified as partial complex. The most common site of onset for partial seizures is the temporal lobe. As described by this patient, temporal lobe events often begin with an aura of déjà vu, a rising epigastric sensation, or autonomic disturbances. Although automatisms, such as lip smacking, are occasionally reported in absence seizures, these features are atypical for that type of seizure and are more suggestive of partial complex ones. In this patient, the age of onset, duration (several minutes) of the event, presence of oral automatisms, and reported postictal confusion and speech impairment all suggest a partial complex seizure.
Absence, generalized tonic-clonic, and myoclonic seizures are all manifestations of generalized epilepsy and are correlated with generalized epileptiform discharges on an electroencephalogram. Absence seizures are characterized by brief periods of staring and unresponsiveness, typically lasting seconds, with an immediate return to normal awareness. Absence seizures usually present in childhood. The length of this patient's episodes, his confusion before returning to normal awareness, and his age argue against absence seizure being the diagnosis.
Generalized tonic-clonic (grand mal) seizures are characterized by stiffening of the trunk and extremities followed by generalized symmetric jerking. Such features were not reported in this patient.
Myoclonic seizures consist of brief, shock-like muscle jerks without loss of awareness. These features are not consistent with the history this patient provides.
Diagnose drug-induced parkinsonism.
* Drug-induced parkinsonism is a potential complication of dopamine-blocking medications, including metoclopramide.
Drug-induced parkinsonism is the most likely diagnosis in this patient. This disorder has classically been associated with neuroleptic medications but can occur with any dopamine-blocking medications, including metoclopramide. Although metoclopramide causes drug-induced parkinsonism in one third of all patients using it, the disorder is particularly underdiagnosed in such patients. Establishing a diagnosis of drug-induced parkinsonism is critical because stopping dopamine-blocking medications can reverse or improve parkinsonian features in these patients.
Diagnose early-onset familial Alzheimer dementia.
* The presenilin-1 mutation is specific for early-onset familial Alzheimer dementia.
This patient's personal and family medical history is striking for dementia at an unusually young age. Her maternal family history in particular is strongly suggestive of early-onset familial Alzheimer dementia, which typically has symptomatic onset in a person's forties or fifties. Although genetic testing is not routine in the evaluation of dementia, all known causes of early-onset familial Alzheimer dementia involve an autosomal dominant mutation. Therefore, genetic testing was appropriate for this patient and should be considered in all patients with early-onset dementia that seems to follow an autosomal dominant pattern of inheritance. The presenilin-1 mutation is specific for early-onset familial Alzheimer dementia. Although mutations of the amyloid precursor protein and presenilin-2 gene are other known autosomal dominant mutations that can cause the disease, the presenilin-1 mutation is by far the most common cause, and testing for this mutation is commercially available.
Manage in-hospital stroke care.
* In a patient with stroke, dysphagia screening should be performed before food, oral medication, or liquids are administered.
On admission to a hospital ward, a patient with stroke should be given nothing by mouth (kept NPO) until a swallowing assessment is conducted. Dysphagia screening is especially appropriate for this patient, who had difficulty swallowing when she first awoke with stroke symptoms. Dysphagia occurs in 45% of all hospitalized patients with stroke and can lead to poor outcomes, including aspiration pneumonia and death. Bedside screening of swallowing ability should thus be completed before oral intake of any medication or food; if the screening results are abnormal, a complete examination of swallowing ability is recommended. The American Heart Association/American Stroke Association recommends a water swallow test performed at the bedside by a trained observer as the best bedside predictor of aspiration. A prospective study of the water swallow test demonstrated a significantly decreased risk of aspiration pneumonia of 2.4% versus 5.4% in patients who were not screened.
Angiotensin-converting enzyme inhibitors, statins, and aspirin are appropriate treatments for secondary stroke prevention in some patients, but they should not be orally administered before ruling out the risk of aspiration.
Treat a patient with primary progressive multiple sclerosis vs. relapsing, remitting
* No therapies have convincing effects on the neurodegenerative processes that underlie progressive forms of multiple sclerosis.
This patient has primary progressive multiple sclerosis (MS); no treatments have been shown to affect the disease course. Therefore, physiatry consultation for evaluation and treatment of his spasticity and back pain are most appropriate at this time. His gradually worsening neurologic function is very likely due to a degenerative loss of axons in the central nervous system; active inflammation, the hallmark of clinical relapses, plays a much less significant role in primary progressive MS than in the relapsing-remitting type. This patient's function and pain could likely be improved substantially with a focus on symptomatic therapies, beginning with physiatry consultation for evaluation and treatment of his spasticity, impaired mobility, and musculoskeletal pain. Symptomatic therapy in patients with multiple sclerosis can have a marked beneficial effect on patient comfort, function, and quality of life, even when further disease progression cannot be effectively stopped.
Beta interferons, glatiramer acetate, and natalizumab, the currently approved MS therapies, are all most effective in altering the immunologic mechanisms that underlie relapses and thus are only appropriate for relapsing-remitting disease.
Diagnose paroxysmal hemicrania.
* The various trigeminal autonomic cephalalgias, characterized by pain referred to the first division of the trigeminal nerve and accompanying cranial autonomic symptoms, can be distinguished by the duration and frequency of each attack.
This patient has paroxysmal hemicrania, one of the trigeminal autonomic cephalalgias. These cephalalgias are characterized by pain referred to the first division of the trigeminal nerve and cranial autonomic symptoms, including lacrimation and rhinorrhea. They differ from one another in the duration and frequency of attacks. A paroxysmal hemicrania attack has an intermediate duration (mean, 15 minutes) and an intermediate episode frequency (mean, 11 per day).
Cluster headache, another trigeminal autonomic cephalalgia, closely resembles paroxysmal hemicrania except in the duration and frequency of attacks. Cluster headache is associated with one to eight attacks per day, and each attack has a mean duration of 60 minutes. Because this patient's headaches only last approximately 15 minutes each, cluster headache is unlikely.
Treat essential tremor.
* First-line medications used to treat essential tremor include propranolol, primidone, gabapentin, and topiramate.
This patient should be treated with propranolol. She has a history and examination findings consistent with the presence of essential tremor. Essential tremor primarily occurs when a patient maintains a posture, such as when the hands are outstretched. Essential tremor also may be present during movement, particularly postural adjustments. Autosomal dominant transmission occurs in approximately half of patients with this condition. Essential tremor most commonly affects the upper extremities; however, the legs, head, trunk, face, and vocal cords may be involved. Up to 15% of patients with essential tremor have major disability associated with this condition. Progression of essential tremor is typically slow, with intermittent lengthy periods of stable symptoms. Features that may be predictive of a more severe essential tremor include a positive family history of tremor, longer tremor duration, voice tremor, and unilateral tremor onset. Alcoholic beverage consumption suppresses symptoms in most patients with this condition.
Treatment options for essential tremor are often limited and frequently only partially effective. It has been estimated that 50% of patients with essential tremor have no response to medical treatment. First-line medications used to treat essential tremor include propranolol, primidone, gabapentin, and topiramate. Propranolol is typically the drug of choice in most patients with essential tremor because of its effectiveness, which has been established in multiple well-designed randomized clinical trials.
Treat epilepsy in a patient with liver disease.
* For patients who should avoid hepatically metabolized antiepileptic drugs, either because of drug interaction or underlying liver disease, levetiracetam, gabapentin, and pregabalin can be used.
Given his clinical history, MRI findings, and electroencephalographic findings, this patient is likely to have epilepsy. Treatment of epilepsy in a patient with an underlying hepatic disease requires careful selection and management of the antiepileptic medication. Levetiracetam, gabapentin, and pregabalin are the preferred choices in patients with significant liver disease because they do not undergo significant hepatic metabolism and have low protein binding. Therefore, levetiracetam is most appropriate for this patient.
For antiepileptic drugs that are hepatically metabolized or highly protein bound, alterations of hepatic enzymatic pathways and hypoalbuminemia can result in unexpected drug toxicity. For these reasons, oxcarbazepine, phenytoin, and valproic acid would be less favored options for this patient. Additionally, some antiepileptic drugs should be avoided because of their potential hepatoxicity, particularly valproic acid and felbamate.
Manage hypertension in a patient with an acute ischemic stroke.
* For uncomplicated ischemic strokes in patients without concurrent acute coronary artery disease or heart failure, antihypertensive medications should be withheld if the systolic blood pressure is less than 220 mm Hg or the diastolic blood pressure is less than 120 mm Hg, unless there are other manifestations of end-organ damage.
For uncomplicated ischemic strokes in patients without concurrent acute coronary artery disease or heart failure, consensus exists that antihypertensive medications, such as intravenous labetalol or nicardipine, should be withheld if the systolic blood pressure is less than 220 mm Hg or the diastolic blood pressure is less than 120 mm Hg, unless there are other manifestations of end-organ damage. This patient's systolic and diastolic blood pressure levels are below these limits. Many such patients have spontaneous declines in blood pressure during the first 24 hours after stroke onset.
Diagnose medication overuse headache.
* Medication overuse headache is generally defined as a headache for more than 15 days per month and the use of acute headache medication on more than 10 days per month.
Treat symptomatic severe extracranial carotid artery stenosis.
* Carotid angioplasty and stenting should be used in patients with symptomatic severe (>70%) internal carotid artery stenosis who are not eligible for surgical treatment with carotid endarterectomy.
This patient, who has had an ischemic stroke and has symptomatic severe internal carotid artery stenosis (>70% stenosis), should undergo carotid angioplasty and stenting. Although carotid endarterectomy is still considered the gold standard of surgical therapies for patients with such stenosis, it cannot be performed in those who have stenosis that is difficult to access surgically (above the C2 level), medical conditions that greatly increase the risk of surgery, or other specific conditions, such as radiation-induced stenosis or restenosis after carotid endarterectomy. For such patients, the less invasive combination of carotid angioplasty and stenting is preferable. The U.S. Food and Drug Administration has approved carotid angioplasty and stenting for patients with symptomatic severe carotid artery stenosis who are classified as high surgical risk or who have unfavorable anatomy that precludes a surgical approach. Other candidates for nonsurgical treatment of severe stenosis include patients with a history of radical neck surgery, spinal immobility, dissection, an ostial lesion below the clavicle, the presence of a tracheostomy stoma, and contralateral laryngeal nerve paralysis.
Because the hallmark features of frontotemporal dementia, such as apathy, perseveration, hoarding, disinhibition, and other personality changes, are lacking in this patient, that diagnosis is unlikely.
Diagnose amyotrophic lateral sclerosis.
* Both upper and lower motoneuron findings are typically present in amyotrophic lateral sclerosis, which helps distinguish this disorder from its mimickers, such as multifocal motor neuropathy, chronic inflammatory demyelinating polyradiculoneuropathy, and primary lateral sclerosis.
Amyotrophic lateral sclerosis (ALS) is the most likely diagnosis in this patient. Patients with ALS typically have progressive, asymmetric, painless extremity or bulbar weakness on presentation. The absence of sensory loss and the lack of bowel or bladder impairment are also suggestive of ALS. The combination of upper motoneuron findings (hyperreflexia, extensor plantar response) and lower motoneuron signs (atrophy, fasciculations) seen on neurologic examination strongly suggests ALS, which is a fatal, neurodegenerative motoneuron disease that affects both the upper and lower motoneurons. The term motoneuron disease is used to describe the heterogeneous group of disorders affecting the upper motoneuron, the lower motoneuron, or both; for example, progressive muscular atrophy is a motoneuron disease that affects the lower motoneuron, and primary lateral sclerosis is a motoneuron disease that affects the upper motoneuron. It is unclear whether different motoneuron disorders are distinct disorders or reflect different manifestations of a single disease. What is established, however, is that ALS has the worst prognosis of them all, with a mean survival of 3 to 5 years.
Treat paroxysmal hemicrania.
* Indomethacin is the treatment of choice for paroxysmal hemicrania.
This patient should receive indomethacin. He most likely has paroxysmal hemicrania, one of the trigeminal autonomic cephalalgias, which are characterized by pain referred to the first division of the trigeminal nerve and by accompanying cranial autonomic symptoms, including lacrimation and rhinorrhea. An attack of paroxysmal hemicrania has an intermediate duration (mean, 15 minutes) and an intermediate episodic frequency (mean, 11 per day). Treatment with indomethacin can immediately and completely resolve the headache. Usually, the response occurs within the first 48 hours after treatment is initiated. Indomethacin is not effective for treating any of the other trigeminal autonomic cephalalgias. Therefore, a positive response to the drug helps distinguish between paroxysmal hemicrania and the other trigeminal autonomic cephalalgias.
Diagnose progressive supranuclear palsy.
* Early falls, symmetric bradykinesia and rigidity, and lack of a resting tremor or levodopa responsiveness characterize progressive supranuclear palsy and help distinguish it from Parkinson disease.
This patient's history and examination findings are most consistent with a diagnosis of progressive supranuclear palsy. A sporadic, neurodegenerative disorder, progressive supranuclear palsy typically manifests as gait impairment and falls, slurred speech, and impaired swallowing. The presence of reduced facial expression, axial rigidity, and impairment of vertical eye movements on examination further suggests the diagnosis.
Treat an acute relapse of multiple sclerosis.
* Multiple sclerosis relapses may resolve more rapidly with intravenous methylprednisolone therapy.
Evidence from placebo-controlled trials involving multiple sclerosis (MS) and optic neuritis supports the intravenous use of methylprednisolone to speed recovery from acute MS relapses. The long-term outcome of an individual MS attack, however, is not affected by the therapy chosen. Therefore, in light of the potential adverse effects of such drugs, intravenous administration of corticosteroids should be offered only for relapses that result in substantial discomfort or reduced function. This patient's 3-day history of worsening weakness and numbness of the right arm and leg qualifies him for corticosteroid therapy.
It is necessary to rule out infection as a cause of neurologic worsening in patients with MS because infections can cause a "pseudoexacerbation"; this patient, however, is asymptomatic except for his neurologic symptoms and is afebrile. Patients with moderate or severe MS-related disability are more susceptible to the effects of mild infections, especially urinary tract infections, and investigation for such infections is warranted, even in the absence of systemic symptoms. However, empiric antibiotic therapy to treat a possible occult infection is not warranted in the absence of evidence or a high clinical suspicion of infection.
Diagnose juvenile myoclonic epilepsy.
* Juvenile myoclonic epilepsy is characterized by myoclonic and generalized tonic-clonic seizures on awakening that are often provoked by sleep deprivation or alcohol.
This patient most likely has juvenile myoclonic epilepsy. Recognizing the specific epilepsy syndrome affecting a patient is crucial in selecting the appropriate therapy, making the correct prognosis, and, in some cases, providing genetic counseling. A history of myoclonic (rapid, unprovoked jerks) and generalized tonic-clonic seizures on awakening with onset in adolescence strongly suggests a diagnosis of juvenile myoclonic epilepsy. One of the most commonly encountered forms of epilepsy, juvenile myoclonic epilepsy may affect 5% to 10% of all patients with epilepsy. Seizures are often provoked by sleep deprivation, alcohol, or exposure to flickering lights.
Manage an unruptured intracranial aneurysm.
* Annual magnetic resonance or CT angiography to monitor aneurysmal growth is appropriate as management of a low-risk unruptured intracranial aneurysm.
This patient should have an annual magnetic resonance angiogram (MRA) or CT angiogram to monitor aneurysmal growth. For patients without a prior subarachnoid hemorrhage, the lowest-risk aneurysms are those in the anterior circulation and less than 7 mm in diameter. The annual risk of rupture for an aneurysm of the size of this patient's is 0.05% annually. The risk of neurologic disability associated with intervention exceeds the potential benefit. After 3 successive years of annual monitoring, an MRA or CT angiogram obtained once every 3 years is sufficient.
The second report from the International Study of Unruptured Intracranial Aneurysms was a prospective observational study of patients who either underwent open or endovascular repair of asymptomatic intracranial aneurysms. Open surgery was associated with surgery-related death or poor neurologic outcome of nearly 13% at 1 year, compared with approximately 10% for endovascular repair. Complication rates increased with increasing age (30% at age 70 years or older), aneurysm size, and location of the aneurysm in the posterior circulation. Because the complication rate of intervention is likely to exceed the complication rate of observation alone, neither clipping nor coiling is indicated.
Diagnose frontotemporal lobar degeneration on the basis of progressive nonfluent aphasia.
* In frontotemporal lobar degeneration, which encompasses the syndromes of progressive nonfluent aphasia, semantic dementia, and frontotemporal dementia, symptom onset is insidious and progression gradual over the course of several years.
This patient has progressive nonfluent aphasia that is most likely due to frontotemporal lobar degeneration. Progressive nonfluent aphasia, semantic dementia, and frontotemporal dementia comprise the three main syndromes of frontotemporal lobar degeneration. Symptom onset is insidious and progression is gradual over the course of several years. Her early decline in social interpersonal conduct is typical of this disorder, as is her aspontaneity and economy of speech. Approximately 10% of patients with frontotemporal lobar degeneration, especially frontotemporal dementia, have concurrent motoneuron disease.
Treat idiopathic intracranial hypertension.
* Acetazolamide is the option of first choice for the medical treatment of idiopathic intracranial hypertension.
This patient should be treated with acetazolamide. She has idiopathic intracranial hypertension, a disease that primarily affects young obese women. She has a progressive daily headache associated with pulsatile tinnitus and transient visual obscurations, the most common symptoms of this disease. She also has papilledema and impaired lateral gaze to the left on presentation. These signs and symptoms suggest the possibility of a left sixth cranial nerve palsy. Abducens nerve palsies are sometimes seen in patients with idiopathic intracranial hypertension because of compression of the sixth cranial nerve as a result of elevated intracranial pressure. Definitive diagnosis is established by an elevated cerebrospinal fluid (CSF) pressure and normal results on CSF analysis. Because there is not yet evidence of visual field or visual acuity impairment, urgent surgical intervention is not necessary and medical therapy is appropriate. However, all patients with suspected idiopathic intracranial hypertension must undergo a thorough ophthalmologic evaluation, including formal visual perimetry testing, to detect enlargement of the blind spots or visual field defects that are not detected by confrontation visual field testing at the bedside.
Acetazolamide is the medical option of first choice for the treatment of idiopathic intracranial hypertension. Although its exact mechanism of action is unclear, acetazolamide is a carbonic anhydrase inhibitor that decreases the production of CSF and relieves intracranial hypertension.
Diagnose potential adverse effects of dopamine agonist medications.
* A potential adverse effect of dopamine agonist therapy is the development of compulsive behaviors, such as pathologic gambling, shopping, and hypersexuality.
This patient has developed an excessive gambling behavior after receiving treatment with the dopamine agonist ropinirole for Parkinson disease. Patients who are initiated and maintained on dopamine agonist medications to control Parkinson disease should be warned about the potential for developing abnormal, compulsive behaviors, such as excessive gambling, excessive shopping, and hypersexuality. These adverse effects, which can also develop in patients taking such medications for restless legs syndrome, are likely due to effects on the dopaminergic reward centers in the brain. Factors that can increase the risk of these behaviors include a young age at diagnosis in men and a history of mood disorders, alcohol abuse, or obsessive-compulsive behaviors. Other potential adverse effects of dopamine agonist therapy include orthostatic hypotension, nausea, vomiting, hallucinations, and sleep attacks.
Manage the withdrawal of antiepileptic drugs
* Patients on antiepileptic medication who have been seizure free for 2 years should be considered for medication withdrawal.
Phenytoin therapy should be discontinued in this patient in a tapered fashion. Although lifelong antiepileptic drug therapy is required for some patients and for some types of epilepsy, this is by no means always the case. As a general rule, discontinuation of antiepileptic drugs should be considered for patients who have been seizure free for 2 or more years. Medications should not be withdrawn in patients with epilepsy syndromes known to be lifelong, with underlying structural brain lesions, with symptomatic neurologic disorders, or (in most cases) with a history of medically refractory seizures. The risk of recurrent seizure when the patient is no longer taking the medication must always be balanced against the risks associated with continued antiepileptic drug treatment. Unfortunately, too many patients are treated unnecessarily for years because of the common misconception that antiepileptic drug therapy can never be safely discontinued. This patient has been seizure free for more than 40 years; in fact, the decision to initiate therapy was questionable because she only had a single event. Now she has osteoporosis, a condition which can be worsened by continued exposure to phenytoin. Therefore, the most appropriate next step in management is to gradually withdraw the medication.
Treat hypertension in a patient with ischemic stroke who has received recombinant tissue plasminogen activator, intravenously.
* In a patient with ischemic stroke treated with recombinant tissue plasminogen activator (rtPA), systolic blood pressure should be kept below 180 mm Hg and diastolic below 105 mm Hg for 24 hours after rtPA treatment; intravenous labetalol or nicardipine can best achieve this goal.
In patients who have received recombinant tissue plasminogen activator as treatment of stroke, systolic blood pressure should be maintained below 180 mm Hg and diastolic blood pressure below 105 mm Hg for at least the first 24 hours after thrombolysis treatment. According to current clinical guidelines, intravenous administration of labetalol or nicardipine can best achieve this goal (class II recommendation). Therefore, of the options listed, intravenous administration of labetalol is most appropriate for this patient whose systolic blood pressure is 190 mm Hg and whose diastolic blood pressure is 105 mm Hg.
Diagnose Bell palsy.
* Any cause of a complete facial neuropathy will impair the entire hemiface, including the forehead muscles.
This patient's physical examination findings most strongly suggest right facial nerve (Bell) palsy. The precise cause of Bell palsy is not known, and it is still considered an idiopathic disorder. Research strongly suggests it may be the result of herpes simplex virus infection of the facial nerve. Bell palsy is not considered contagious. The seventh cranial nerve innervates all muscles of facial expression (the mimetic muscles). Any cause of a complete facial neuropathy will therefore impair the entire hemiface, including the forehead corrugators typically spared by cerebral lesions. Bell phenomenon describes the reflexive rolling upwards of the globe during eye closure. When a patient is asked to close the eyes, forced eyelid opening will reveal this phenomenon, as will the selective paralysis of the orbicularis oculi due to a facial neuropathy. Facial neuropathies will otherwise spare the extraocular muscles that govern globe movement. Because Bell palsy is a diagnosis of exclusion, clinicians need to make every effort to exclude other identifiable causes of facial paralysis, such as Lyme disease, acute and chronic otitis media, cholesteatoma, and multiple sclerosis. Other common causes of acute peripheral facial paralysis will often have findings on history or physical examination that suggest the correct diagnosis.
Treat Parkinson disease.
* Dopamine agonist medications are used as first-line treatment of Parkinson disease in patients younger than 65 years, whereas levodopa is used in patients age 65 years or older.
This patient should be treated with pramipexole. He has classic signs of Parkinson disease, including tremor, rigidity, and bradykinesia. There are no effective neuroprotective agents to treat this disorder. Dopamine agonist medications, either pramipexole or ropinirole, are indicated for the initial treatment of the parkinsonian symptoms in this young patient with apparent Parkinson disease. Motor complications, such as dyskinesias (abnormal involuntary movements), an end-of-dose "wearing-off" effect, and fluctuations, may be less frequent and less severe with dopamine agonist medications than with levodopa.
Levodopa, a precursor of dopamine, is the most efficacious medication used to treat the symptoms of Parkinson disease but is typically initiated only in patients older than 65 years. The associated development of motor fluctuations occurs at a rate of 10% annually in these patients but may develop more rapidly and be more severe in younger patients taking levodopa as an initial medication. Carbidopa is administered in conjunction with levodopa to prevent the peripheral conversion of levodopa to dopamine.
Manage a transient ischemic attack.
* Patients with a diagnosis of a recent transient ischemic attack are at an appreciably high short-term risk of stroke and should be evaluated in a hospital in an expedited and emergent fashion.
This patient should be admitted to the hospital. Given his clinical history, he most likely has had a recent transient ischemic attack (TIA). His ABCD2 score (based on Age, Blood pressure, Clinical features, the Duration of symptoms, and the presence of Diabetes) is 5: one point is for his age (>60 years), one point for his hypertension, one point for a symptom duration of greater than 10 minutes, and two points for the focal weakness he described. This score is moderately high and carries an estimated stroke risk of 5% over the next 2 days, 7% over the next week, 10% over the next 30 days, and 12% over the next 3 months. Therefore, the most appropriate response is for this patient to undergo urgent evaluation within the next 24 hours at an emergency department, at a hospital during a brief admission, or at an organized urgent TIA clinic.
Evaluate suspected neuromyelitis optica in a patient with transverse myelitis and paraparesis.
* Neuromyelitis optica (NMO) is a severe demyelinating disease of the central nervous system that is distinct from multiple sclerosis and associated with the autoantibody marker NMO-IgG (anti-aquaporin-4).
This patient very likely has neuromyelitis optica (NMO), a severe demyelinating disease of the central nervous system that is distinct from multiple sclerosis (MS). She should be tested for the autoantibody marker NMO-IgG (anti-aquaporin-4). NMO occurs more commonly in nonwhite persons, is often associated with serum autoantibodies or other autoimmune diseases, and has a predilection for the optic nerves and spinal cord with relative sparing of the brain. This patient's spinal cord lesion is also characteristic of NMO because it extends over more than three vertebral segments; cord lesions in typical MS are usually less than two segments in length. The finding of the NMO-IgG autoantibody marker is approximately 75% sensitive and more than 90% specific for NMO. Differentiating between NMO and MS as early in the disease course as possible is important because the prognosis and treatment of the two diseases are different. NMO is a more severe disease treated with immunosuppressive drugs, whereas MS is initially treated with immunomodulatory therapies, such as β-interferon and glatiramer acetate.
Treat a patient prophylactically for migraine.
* Prophylactic medication should be initiated in patients with two or more migraine attacks per week.
Prophylactic treatment should generally be initiated in patients with two or more migraine attacks per week. There is level 1 evidence to support the use of topiramate for the prevention of migraine, and the U.S. Food and Drug Administration has approved the drug for this purpose. This patient is obese (BMI of 34) and has type 2 diabetes mellitus. Any medication with the potential for weight gain must, therefore, be used with caution, given the morbidity associated with obesity and the potential to worsen her underlying hyperglycemia. Topiramate is associated with weight loss.
Gabapentin is a second-tier drug because of the lower level of evidence supporting its use. It is also not approved by the FDA for the preventive treatment of migraine.
There is no evidence of nortriptyline's efficacy in the preventive treatment of migraine. Moreover, it is also associated with weight gain.
Propranolol, a nonselective β-blocker, is contraindicated in patients with persistent asthma and has a relative contraindication in patients with diabetes mellitus.
Valproic acid, although also supported by level 1 evidence and FDA approval for migraine prevention, is associated with weight gain and is not the best treatment for this patient. Additionally, in light of the potential teratogenicity associated with this drug, it is often avoided in women of childbearing potential.
* Chorea can occur in patients with anti-phospholipid antibody syndrome or systemic lupus erythematosus.
Diagnose diabetic lumbosacral radiculoplexus neuropathy.
* Diabetic lumbosacral radiculoplexus neuropathy (diabetic amyotrophy) is characterized by severe, initially unilateral lower limb pain and weakness.
Diabetic lumbosacral radiculoplexus neuropathy, or diabetic amyotrophy, is the most likely diagnosis in this patient. This neuropathy is a subacute, progressive disorder that causes asymmetric leg pain, sensory loss, and weakness. Weight loss of 4.5 kg (10 lb) or more occurs in most affected patients. Many patients with this disorder are unaware that they have diabetes mellitus before the development of diabetic lumbosacral radiculoplexus neuropathy, and in most patients, glycemic control is not severely compromised. This disorder usually begins with unilateral leg pain followed by weakness and sensory loss, which spread to involve the contralateral leg nearly all the time. Weakness is often greatest proximally initially, but over time, diffuse weakness involving proximal and distal muscles ensues. Electromyographic studies characteristically show dysfunction at the level of multiple peripheral nerves, the lumbosacral plexus, and multiple nerve roots. MRI studies of the lumbosacral plexus are typically normal in this disorder but are most helpful in excluding an infiltrative neoplastic process, which can present similarly.
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