Term
| What cancer is associated with myasthenia gravis |
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Definition
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| Cancer associated with Lambert Eaton syndrome |
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Definition
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Term
| cancer associated with dermatomyositis |
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Definition
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Definition
Sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder. It is one of the phakomatoses and is often associated with port-wine stains of the face, glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma. It is characterized by proliferation of arteries of the brain, resulting in multiple angiomas that occur on the same side as the physical signs described above. As a consequence, arteriovenous malformations often form. Normally, only one side of the head is affected.
Sturge-Weber is an embryonal developmental anomaly resulting from errors in mesodermal and ectodermal development. Unlike other neurocutaneous disorders (phakomatoses), Sturge-Weber occurs sporadically |
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Definition
| found in neurofibromatosis type 1, and are present in greater than 94% of patients over the age of six.[1] They are clear, yellow-brown,[3] oval to round, dome-shaped papules that project from the surface of the iris. |
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Term
single gene disorders
NF2
NF1
APC
RB
Marfan
Cystic fibrosis
Huntingtons
VHL
DCC/DCP (colon and pancreatic)
p53
cru di chat |
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Definition
NF2 - 22
NF1 - 17
APC - 5
RB - 13
Marfan - 15
Cystic fibrosis - 7
Huntingtons - 4
VHL - 3
DCC/DCP (colon and pancreatic) - 18
p53 - 17
cru di chat - 5 |
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Term
| Trinucleotide repeat expansion diseases, chromosome and repeat |
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Definition
Huntingtons chorea - Ch. 4 CAG repeats in 5' coding region
Fragile X - Ch X. CGG in 5' untranslated region.
Myotonc dystrophy Ch. 19. CTG in the 3' untranslated region. |
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Term
| Sensory nerves of the ear and what portion they supply innervation to |
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Definition
Auriculotemporal - Anterior half of the external ear canal.
Auricular branch of the Vagus - posterior half of the external ear canal.
Greater auricular nerve - lower part of the auricle.
Lesser occipital nerve - upper part of the auricle. |
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Term
| Only muscle innervated by CN IX |
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Definition
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Term
| Antidepressant associated with increased risk of seizure |
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Definition
| Bupropion - NE and Dopamine reuptake inhibitor. Contraindicated in patients with current or prior diagnosis of eating disorders. |
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Term
| migraine medication associated with hypertensive emergencies |
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Definition
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Term
| One of the few TCAs approved for treatment of OCD |
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Definition
| clomipramine. SSRI are first line. |
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Term
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Definition
■ Systolic BP > 185 or diastolic BP >110
■ Prior intracranial hemorrhage
■ Stroke or head trauma within the last three months
■ Recent MI
■ Current anticoagulation with INR > 1.7 or prolonged PTT
■ A platelet count < 100,000/mm3
■ Major surgery in the past 14 days
■ GI or urinary bleeding in the past 21 days
■ Seizures present at the onset of stroke
■ Blood glucose < 50 or > 400 mg/dL
■ Age < 18 |
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Term
| Medications used in prophylaxis for menstrual migraines |
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Definition
| naproxen sodium, mefenamic acid, frovatriptan, naratriptan, and percutaneous estradiol. |
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Term
| West Nile virus infection |
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Definition
| should be considered in every patient with symptoms of extremity weakness that begin acutely and progress over days to weeks. However, the presence of paresthesias and sensory loss on examination are not typical of West Nile virus infection. This enteroviral illness affects the anterior horn cells, causing limb weakness in the absence of sensory loss. Most cases of West Nile virus infection cause only minor symptoms that are indistinguishable from those of other viral illnesses. |
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Term
| critical illness myopathy |
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Definition
| usually recognized in the intensive care unit, either in patients who have difficulty weaning from mechanical ventilation (up to 30% of patients) or in patients who have developed severe limb weakness during or after recovery from critical illness. PT/OT, avoid steroids. Corticosteroids may play a role in the pathogenesis of critical illness myopathy. Electromgography can help exclude other causes and help confirm the diagnosis. |
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Term
A 30-year-old man is evaluated in the office for an 8-month history of intensely painful headaches, which occur up to 10 times per day and last approximately 15 minutes each. The pain is most severe around the left eye, and he has no pain between attacks. Each attack is associated with rhinorrhea, lacrimation, and conjunctival injection. The patient has a 12-pack-year smoking history. He takes a combination of acetaminophen, caffeine, and aspirin, usually taking a total of five tablets daily.
Results of a physical examination, including a neurologic examination, are normal.
Which of the following is the most likely diagnosis? |
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Definition
| 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). |
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Term
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Definition
| one to eight attacks per day, and each attack has a mean duration of 60 minutes. |
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Term
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Definition
| Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing, usually associated with more than 30 attacks per day and a duration of 30 to 120 seconds for each attack. |
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Term
| It is important to distinguish between the trigeminal autonomic cephalalgias because they are treated differently |
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Definition
| Verapamil, indomethacin, and lamotrigine are, respectively, the treatments of first choice for cluster headache, paroxysmal hemicrania, and SUNCT syndrome. |
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Term
| Epilepsy drugs to use in liver failure patients |
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Definition
| 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. |
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Term
A 44-year-old woman is evaluated for a 4-month history of worsening gait and bilateral leg numbness. She underwent gastric bypass surgery 8 years ago but has no other relevant personal or family medical history. Her only medications are a daily multivitamin and vitamin B12.
On physical examination, temperature is normal, blood pressure is 130/80 mm Hg, pulse rate is 90/min, respiration rate is 14/min, and BMI is 29. The patient has moderate gait ataxia, lower extremity spasticity, hyperreflexia, and bilateral extensor plantar responses. Strength is normal, but vibration and proprioceptive sensation is impaired in both feet.
Results of laboratory studies show a mild normocytic anemia. Serum vitamin B12, vitamin E, methylmalonic acid, and homocysteine levels are all normal.
Nerve conduction studies and an electromyogram reveal a moderate axonal peripheral neuropathy. MRIs of the cervical spinal cord and the thoracic spinal cord show no abnormalities.
Which of the following is the most appropriate next diagnostic study? |
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Definition
| Serum copper and zinc levels should be measured in this patient. She has a progressive myeloneuropathy syndrome and mild anemia, both caused by copper deficiency. Gastric bypass surgery is a risk factor for this clinical syndrome, which can be caused by vitamin B12 deficiency, copper deficiency, or both. Given that her serum vitamin B12 level is normal, copper deficiency is the most likely cause. Overingestion of zinc can also impair copper absorption. |
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Term
| When would you stent over doing a CEA? |
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Definition
| 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. 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. |
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Term
| When would you consider an External carotid to internal carotid artery bypass surgery? |
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Definition
| was shown not to be effective for the surgical treatment of carotid artery stenosis, but this surgery may be considered for symptomatic carotid artery occlusion (that is, 100% occlusion). |
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Term
| Features of dementia with lewy bodies |
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Definition
| 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. |
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Term
| Primary lateral sclerosis |
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Definition
| A motoneuron disorder affecting the upper motoneuron, primary lateral sclerosis causes slowly progressive weakness and spasticity. In affected patients, spasticity predominates over weakness and is typically symmetric and lower limb predominant. Later in the disease course, most patients develop pseudobulbar features, with dysarthria and emotional lability. Primary lateral sclerosis is a clinical diagnosis that can be established only by excluding other diseases. Spinal cord MRIs are particularly useful in this regard. Primary lateral sclerosis is a slowly progressive neurodegenerative disorder with a much better prognosis than ALS; long-term survival is expected. There are no medications approved by the U.S. Food and Drug Administration for the treatment of primary lateral sclerosis. reflects degeneration of the posterior frontal lobe that includes the upper motoneurons. Cognitive impairment is mild but can include emotional lability and other frontal lobe features. In approximately 10% of patients with FTD and a smaller subset of those with progressive nonfluent aphasia, there is concurrent degeneration of lower motoneurons due to amyotrophic lateral sclerosis (ALS). |
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Term
| progressive supranuclear palsy. |
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Definition
| 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. |
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Term
| recommended treatment for this patient who has multiple cerebral metastases is |
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Definition
| palliative whole-brain radiation therapy and corticosteroid administration. Stereotactic radiosurgery and surgical resection are generally restricted to patients with a single metastatic lesion and with reasonably controlled systemic disease. Neurosurgery will occasionally be considered for palliation in patients with multiple metastases, but only if there is a single lesion causing immediately life-threatening or severely disabling symptoms. |
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Term
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Definition
| syndrome seen in younger children and adolescents who have seizures, usually during sleep, that begin with focal sensory and/or motor symptoms involving the face, mouth, and throat that can then secondarily generalize. |
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Term
| Frontotemporal lobar degeneration (FTLD) |
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Definition
encompasses the three main syndromes of progressive nonfluent aphasia, semantic dementia, and frontotemporal dementia (FTD). As with all degenerative diseases, symptom onset is insidious and progression gradual over the course of several years.
Progressive nonfluent aphasia is a striking disorder that is often mistaken for a stroke because of the obvious speech impairment produced. Semantic dementia might be better considered a form of progressive anomia with less obvious clinical impairment; |
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Term
| Medical Treatment of idiopathic intracranial hypertension |
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Definition
| 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. 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. |
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Term
| Parkinsons treatment before age of 65 |
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Definition
| Pramipexole or ropinirole. Levodopa is the most effective medication used in the treatment of Parkinson disease but is associated with motor fluctuations, such as dyskinesias and a “wearing-off” effect, which refers to enhanced parkinsonian symptoms due to ineffective dopamine therapy. These motor fluctuations develop at a rate of 10% annually in patients older than 60 years but seem to develop more rapidly and are more severe in younger patients. Therefore, most clinicians will initiate therapy with a dopamine agonist in patients younger than 65 years. At some point, however, all patients will require the addition of levodopa therapy. Levodopa is the drug of choice in older patients. |
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Term
A 26-year-old woman is evaluated in the office for a change in migraine symptoms. She began having migraine attacks shortly after menarche at age 13 years, experiencing an attack approximately every 2 months. For the past 8 weeks, these attacks have been associated with visual aura. The neurologic symptoms evolve over a period of 10 minutes, last less than 60 minutes, and are followed within 30 minutes by a severe, unilateral throbbing headache associated with nausea. Sumatriptan relieves the headache within 30 minutes. The patient also has asthma. Her mother and sister have a history of migraine. Current medications are an oral contraceptive pill started 9 weeks ago, sumatriptan as needed, a daily inhaled corticosteroid, and an inhaled β-agonist as needed.
Results of physical examination, including neurologic examination, are normal.
Complete blood count results, erythrocyte sedimentation rate, serum chemistry study results, thyroid-stimulating hormone level, and anticardiolipin and antinuclear antibody levels are normal.
An MRI of the brain shows no abnormalities.
Which of the following is the most appropriate next step in management? |
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Definition
| Patients who have migraine with aura have up to an eight-fold increased risk of ischemic stroke; this risk is tripled by smoking and quadrupled by the use of oral contraception. This patient, therefore, should discontinue using oral contraception |
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Term
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Definition
| Low risk < 4, moderate 4-5, high risk > 5. Age (1 point if ≥60 years), Blood pressure (1 point if ≥140 mm Hg systolic or 90 mm Hg diastolic), Clinical presentation (2 points for the presence of unilateral weakness or 1 point for speech impairment without weakness), the Duration of symptoms (2 points for ≥60 min and 1 point for 10-59 min), and the presence of Diabetes mellitus (1 point) |
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Term
A 31-year-old woman with a history of migraine is evaluated in the office for several severe migraine attacks that are not well controlled with over-the-counter analgesics. The headaches are severe, are associated with severe nausea and vomiting, and began 2 weeks ago after she delivered a healthy baby boy. She is currently breast feeding.
Results of physical examination, including neurologic examination, are normal.
Which of the following is the most appropriate treatment for this patient? |
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Definition
| Frovatriptan and sumatriptan are both considered compatible with breast feeding by the American Academy of Pediatrics. The patient has not responded to first-line therapy with simple analgesics and is reporting severe migraine attacks, for which a triptan is indicated. |
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Term
A 68-year-old man is evaluated in the office for a 4-week history of continuous left leg pain and weakness. The pain is severe, burning, and in the distribution of the anterior thigh. He has had a 4.5-kg (10-lb) weight loss in the last 3 weeks. The patient had prostate cancer 1 year ago and underwent radiation therapy to the pelvis. He also has hypertension treated with lisinopril.
General physical examination findings are normal, except for a BMI of 31. Neurologic examination shows normal speech, language, and cranial nerve function. Strength and deep tendon reflexes are normal in the upper extremities and in the right leg. There is diffuse weakness, greatest in the quadriceps, hip adductor, and iliopsoas muscles, and absent knee and ankle jerks in the left leg; plantar response is flexor. Sensory loss is evident in the distribution of the saphenous sensory nerve on the left.
Laboratory studies show an elevated fasting plasma glucose level of 132 mg/dL (7.3 mmol/L). All other laboratory results, including erythrocyte sedimentation rate; serum electrolyte, creatinine, and thyroid-stimulating hormone levels; and liver chemistries, are normal.
Which of the following is the most likely diagnosis? |
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Definition
| 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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Term
A 69-year-old woman is evaluated in the emergency department for the sudden onset of headache, nausea, vomiting, and imbalance. The patient has hypertension for which she takes lisinopril. She has no other relevant personal or family medical history.
On physical examination, blood pressure is 160/90 mm Hg, pulse rate is 80/min, and respiration rate is 18/min. Her Glasgow Coma Scale score is 12 (of a possible 15), which indicates moderate brain injury. Right finger-to-nose and heel-to-shin testing reveals dysmetria.
Results of laboratory studies, including a complete blood count, a metabolic profile, and coagulation tests, are normal.
A CT scan of the head shows an acute right cerebellar hemorrhage (>4 cm in diameter), perihematoma edema, compression of the right pons, and effacement but no compression of the fourth ventricle.
Which of the following is the most appropriate next step in management? |
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Definition
| This patient has signs of neurologic deterioration, given her score on the Glasgow Coma Scale. She has an acute cerebellar hemispheric hemorrhage that is greater than 3 cm in diameter and thus should be considered for neurosurgical intervention. The priority in such cases is to decompress the posterior fossa by surgically evacuating the hematoma. Patients with posterior fossa hematomas are at risk for life-threatening complications, including herniation and hydrocephalus, without such intervention. |
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Term
| Treatment of myasthenia crisis |
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Definition
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Term
| Progressive supranuclear palsy |
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Definition
| parkinsonian signs and early falls due to marked postural instability. However, significant dysautonomia and ataxia are not expected. Marked impairment in vertical gaze is a hallmark of progressive supranuclear palsy. |
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Term
| Dementia with Lewy bodies |
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Definition
| cognitive impairment, parkinsonian signs and symptoms, and possible evidence of dysautonomia. However, gait or limb ataxia is not expected, and the degree of dysautonomia typically is not as severe as that seen in multiple system atrophy. |
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Term
| Standard treatment of primary CNS lymphoma |
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Definition
| chemotherapy (usually high-dose methotrexate) followed by whole-brain radiation therapy if the patient’s tumor is refractory to chemotherapeutic agents. However, neither of these treatments is appropriate before CSF analysis or a biopsy establishes a tissue diagnosis. |
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Term
| Diabetic lumbosacral radiculoplexus neuropathy, or diabetic amyotrophy |
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Definition
| 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 |
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