PANCE Exam: Neurology Review Questions & Answers

Question: What are the reversible causes of dementia?
While normal pressure hydrocephalus, brain tumors, hypothyroidism, and vitamin B12 deficiency are reversible causes of dementia, they are less commonly seen. Nevertheless, diagnostic studies should be used to rule them in or out as the underlying cause of dementia since early diagnosis will help to stop or even reverse any cognitive impairment.

Question: What is the most common cause of dementia in Western countries?
Alzheimer disease is the most common cause of dementia, followed by vascular dementia and dementia associated with Parkinson disease.

Question: What are the risk factors for Alzheimer disease?
Increasing age, Down syndrome (Trisomy 21), and family history. Familial Alzheimer disease is associated with an autosomal dominant inheritance with mutations in the gene for amyloid precursor proteins on chromosome 21.

Question: What are the characteristic pathologic features associated with Alzheimer disease?
Alzheimer disease is characterized by abnormal metabolism and deposition of β-amyloid proteins resulting in the formation of neuritic or senile plaques and neurofibrillary tangles.

Question: What is often the first sign of Alzheimer disease?
Family members often note impairment in recent memory when patients become disoriented to time and then to place.

Question: What are the manifestations of the late stages of Alzheimer disease?
As the disease progresses, patients develop psychiatric symptoms, such as delusions, hallucinations, and paranoia. Patients may also develop seizures.

Question: What are the clinical features common to subcortical dementia?
Subcortical dementias are characterized by movement disorders in conjunction with memory loss and apathy. Language and visuospatial functions are preserved.

Question: What are the causes of subcortical dementia?
Parkinson disease, hypothyroidism, normal pressure hydrocephalus, multiple sclerosis, and vascular dementia.

What type of neurologic deficits are found with cortical dementias?
Cortical dementias are associated with loss of higher cortical functions, such as language. Patients have varying degrees of aphasia, agnosia, and apraxia. Alzheimer disease is the most common type of cortical dementia.

Question: Which medications are recommended to improve the cognitive dysfunction associated with Alzheimer disease?
Acetylcholinesterase inhibitors, such as donepezil (Aricept) or rivastigmine (Exelon), address the deficits associated with degeneration of cholinergic neurons.

Question: Which medications are indicated for the management of the behavioral disturbances seen in association with Alzheimer disease?
Antipsychotic medications, such as haloperidol (Haldol) or risperidone (Risperdal), can be used to address hallucinations, delusions, or aggression. Antidepressants and anxiolytics can also be used to address symptoms of depression and anxiety, respectively.

Question: What is the underlying pathophysiology of normal-pressure hydrocephalus?
Normal-pressure hydrocephalus is called a communicating or nonobstructive form of hydrocephalus since the flow or cerebrospinal fluid (CSF) is not obstructed. Hydrocephalus develops due to impaired absorption of CSF by the arachnoid granulations along the superior sagittal sinus in the subarachnoid space.

Question: A 62-year-old man presented with the progressive gait difficulty. The patient reports unsteadiness with standing and trouble initiating walking even though he denies any muscle weakness. His wife notes that his thinking is “slower” and he is becoming more forgetful. A non-contrast CT scan of the head (see Figure 10-1) was obtained during the diagnostic evaluation. What is the most likely diagnosis?


Figure 10-1
Normal-pressure hydrocephalus (NPH). Gait difficulties are often the initial presentation of NPH. Patients describe feeling as if they are glued to the floor; once they are able to start moving, their gait is slow and shuffling. Early symptoms of NPH also include mental slowness, memory impairments, and apathy; aphasia and agnosia are uncommon.

What is the characteristic triad of normal-pressure hydrocephalus?
Dementia, incontinence, and gait ataxia (often described as a “magnetic” gait).

Question: What are the common symptoms associated with dementia with Lewy bodies?
Dementia with Lewy bodies is characterized by fluctuating cognitive impairment, visual hallucinations, and signs of parkinsonism (i.e., bradykinesia, tremor, and rigidity). Lewy bodies are intraneuronal cytoplasmic inclusions that contain similar proteins that are found in Alzheimer disease and Parkinson disease.

Question: What class of medications should be avoided in patients with Lewy body dementia?
Antipsychotic medications should be avoided since they will exacerbate the extrapyramidal symptoms.

Question: What are the risk factors associated with vascular (or multi-infarct) dementia?
Risk factors for multi-infarct dementia include a history of hypertension, diabetes, atrial fibrillation, peripheral vascular disease, or other evidence of advanced atherosclerotic disease.

Question: What is an important historical distinction between vascular dementia and Alzheimer disease?
The onset of symptoms can be helpful to distinguish between the two disorders. Alzheimer disease has a slowly progressive loss of cognitive function, whereas patients with multi-infarct dementia have acute episodes of neurologic deterioration that progress in an irregular stepwise manner with each ischemic insult.

Question: What is an associated symptom with dementia secondary to Huntington disease?
Huntington disease is an autosomal dominant inherited condition characterized by a movement disorder (choreiform movements) in addition to progressive memory loss.

Question: What is the most common neurologic complication associated with AIDS?
AIDS dementia is more common in patients who are severely immunocompromised; however, it can also be a presenting symptom.

Question: What are the associated physical examination findings with AIDS dementia?
Behavioral changes (social withdrawal) and motor symptoms can be seen. Ataxia, increased muscle tone, and hyperreflexia can all be seen during the early stage of the disease; however, the motor symptoms become much more pronounced as the disease progresses to later stages.

Question: What are the typical findings on cerebrospinal fluid analysis for a patient with AIDS dementia?
Mild to moderate elevations in protein levels (≤200 mg/dL), modest mononuclear pleocytosis (≤50 cells/μL), and the presence of oligoclonal IgG bands.

Question: What are the common, underlying characteristics associated with cerebral palsy?
Cerebral palsy refers to a chronic condition characterized by impaired muscle tone, strength, and coordination of movements. It is associated with a cerebral injury that can occur either before birth, during birth, or in the perinatal period.

Question: What are the most common causes of cerebral palsy?
Intrauterine hypoxia, intrauterine bleeding, infections, toxins, congenital malformations, neonatal infections, neonatal hypoglycemia, and kernicterus; the underlying etiology is never identified in about 25% of cases.

What is the most common clinical manifestation seen in cerebral palsy?
The most common manifestation of cerebral palsy is spasticity of the limbs, which can involve one or more limbs. Spasticity can present as a monoplegia, hemiplegia, paraplegia, or quadriplegia.

Question: What associated movement disorder can be seen in patients with cerebral palsy?
Choreoathetosis, or twisting and jerking involuntary movements, can be seen in a small percentage of patients.

Question: What type of neurologic deficits can be associated with cerebral palsy?
Associated neurologic deficits can include seizures, which can be seen in about 50% of patients; varying levels of mental retardation can also be seen, ranging from mild to severe. Patients can have various language, speech, hearing, and vision problems in isolation or in combination with other deficits.

Question: What physical examination findings can be seen with cerebral palsy?
Spasticity, hyperreflexia, choreoathetosis, and ataxia.

Question: In addition to findings consistent with cerebral palsy, which additional physical examination findings are associated with a congenital infection with cytomegalovirus (CMV) or rubella?
The presence of congenital cataracts, retinopathy, and congenital heart defects suggest an infection with CMV or rubella.

Question: What is the cause of Bell palsy?
Bell palsy is an idiopathic lower motor neuron type of facial weakness.

Question: What are the signs and symptoms commonly associated with Bell palsy?
Additional signs and symptoms include impaired taste, lacrimation, or hyperacusis; the clinical presentation varies depending on the location of the lesion. For example, a lesion distal to the sternomastoid foramen will produce purely a motor deficit, whereas a lesion in the petrous temporal bone will produce an ipsilateral facial weakness with impaired taste, lacrimation, and hyperacusis.

Question: What is Ramsay Hunt syndrome?
Ramsay Hunt syndrome is a facial nerve palsy associated with a herpes zoster infection of the geniculate ganglion.
Patients have an eruption of an erythematous vesicular rash involving the ear, palate, pharynx, and neck.

Question: What is synkinesia?
Synkinesia is a complication associated with Bell palsy in which there are abnormal facial movements attributed to problems with regeneration of the nerve. Active movement of one part of the face is associated with involuntary movement of another part of the face. For example, the patient’s eye might close when he smiles.

Question: How can you tell the difference between a central facial palsy and a peripheral facial palsy?
A peripheral facial palsy is a lower motor neuron lesion affecting the entire side of the face ipsilateral to the lesion. A central facial palsy is an upper motor neuron lesion affecting the lower part of the face contralateral to the lesion. Central facial palsies are often associated with motor weakness of the upper extremity on the same side as the facial palsy.

What are the predisposing factors associated with Guillain-Barré syndrome?
Minor illnesses, gastrointestinal infections with Campylobacter jejuni, recent immunizations, and surgical procedures.

Question: What are the signs and symptoms associated with Guillain-Barré syndrome?
Guillain-Barré syndrome is characterized by an ascending symmetrical lower extremity weakness. Bulbar muscles can also be involved, resulting in facial weakness and dysphagia. If the diaphragm is involved, patients will present with respiratory insufficiency. Deep tendon reflexes will be depressed or absent. Patients may also complain of sensory loss, which is in a “glove-and-stocking” distribution. Sensory losses are generally less severe than muscle weakness.

Question: What clinical findings would help to rule out Guillain-Barré syndrome as a diagnosis for a patient with the new onset of weakness?
Pertinent negatives would include a markedly asymmetrical pattern of weakness, bowel or bladder incontinence at the onset of symptoms, a well-defined sensory level, and/or a CSF white blood cell count >50.

Question: What is a characteristic finding on cerebrospinal fluid (CSF) analysis for patients with Guillain-Barré syndrome?
The CSF will show an elevation in protein levels without an elevation in the white cell count.

Question: Does early treatment with intravenous immunoglobulin (IVIG) or plasmapheresis accelerate the recovery for patients diagnosed with Guillain-Barré syndrome?
Yes, IVIG or plasmapheresis will shorten the recovery time as well as decrease the likelihood of long-term neurologic disability.

Question: What is the prognosis for patients with Guillain-Barré syndrome?
Approximately 70% to 75% of patients have a full recovery; 25% of patients have mild, residual neurologic deficits, and 5% of patients will die. Advanced age, respiratory insufficiency requiring ventilator support, and rapid progression of symptoms are all associated with a poorer prognosis.

Question: What are the clinical features of chronic inflammatory demyelinating polyneuropathy?
The clinical presentation is the same as Guillain-Barré syndrome; however, the course is progressive and characterized by relapses. Predominant features include muscle weakness affecting the upper and lower extremities, distal greater than proximal, decreased or absent deep tendon reflexes, and distal sensory deficits (glove-and-stocking distribution).

Question: What role do corticosteroids have in the treatment of Guillain-Barré syndrome?
Corticosteroids are associated with adverse outcomes and may actually delay recovery and are therefore contraindicated in the treatment of Guillain-Barré syndrome (an acute inflammatory polyneuropathy); chronic inflammatory demyelinating polyneuropathy, however, is responsive to corticosteroids. Accordingly, treatment with prednisone for 2 to 4 weeks, followed by a tapered dosage is indicated.

Question: What medical conditions can be associated with myasthenia gravis?
Thyrotoxicosis, disseminated lupus erythematous, tumors of thymus, and rheumatoid arthritis.

What is the hallmark of myasthenia gravis?
Fluctuating weakness and fatigability of voluntary muscle activity, with involvement of ocular, bulbar, limb, or respiratory muscles. Bulbar involvement presents as dysarthria and dysphagia.

Question: What is the underlying pathophysiology of myasthenia gravis?
Myasthenia is characterized by auto-antibodies to acetylcholine at neuromuscular junction. Accordingly, there is a block in neuromuscular transmission as well as a decrease in the number of functioning receptors, which account for the fatigable weakness.

Question: What are the two subtypes of myasthenia gravis?
Ocular and generalized. The ocular type is characterized by diplopia and ptosis, which are two of the most common presenting symptoms of myasthenia. Patients with ocular symptoms often progress to the generalized subtype. Generalized myasthenia presents with weakness of the upper and/or lower extremities and overall fatigability.

Question: What type of conditions or medications are associated with exacerbations of myasthenia gravis?
Minor infections, pre-menses, pregnancy, stress, and hot weather can all lead to an exacerbation; medications that are associated with exacerbations include quinine, quinidine, procainamide, propranolol, phenytoin, lithium, tetracycline, and aminoglycoside antibiotics.

Question: A 32-year-old female patient presents with diplopia and ptosis, which are both worse by the end of the day. She also notes dysarthria if she has been talking a lot. What diagnostic test is indicated for the evaluation of this patient?
The most commonly used test is the edrophonium or Tensilon test. Up to 10 mg can be given as an intravenous dose to look for improvement in muscle strength. The effects of the Tensilon test are transient.

Question: What additional diagnostic studies can be used for a patient with suspected myasthenia gravis?
Acetylcholine receptor antibodies are elevated in up to 90% of patients; repetitive nerve stimulation will show a decrement in response amplitude. A single fiber EMG, which is a specialized EMG that analyzes individual muscle fiber’s firing pattern, is the most sensitive test.

Question: What is the treatment of choice for myasthenia gravis?
The treatment of choice is pyridostigmine (Mestinon).

Question: What are additional treatment options for the management of myasthenia gravis?
Thymectomy will provide symptomatic relief or remission. Corticosteroids for patients who are nonresponders to anticholinesterase medications. For patients with progressive disease who do not respond to thymectomy, anticholinesterases, corticosteroids and azathioprine can be used.

Question: What is myasthenic crisis?
Patients in myasthenic crisis have significant impairment in respiratory function, requiring intubation and ventilator support. Plasmapheresis is also indicated.

Question: What underlying conditions are associated with Lambert-Eaton syndrome?
Lambert-Eaton is associated with malignancies, especially oat cell carcinoma. It can also be associated with pernicious anemia.

How does the clinical presentation of Lambert-Eaton syndrome differ from that of myasthenia gravis?
Although both conditions are associated with muscle weakness, extraocular muscle function is preserved in Lambert-Eaton syndrome.

Question: What are the different types of diabetic neuropathy?
Mixed (sensory, motor, and autonomic) in 70%; primary sensory in 30%.

Question: What is the classic presentation of a diabetic sensory neuropathy?
The classic presentation is a symmetric stocking-glove sensory loss distribution. The lower extremities are generally affected first, followed by the upper extremities. Patients complain of numbness, tingling, and other paresthesias. Patterns of loss can be assessed with monofilament testing.

Question: What is the clinical presentation of mononeuropathy simplex and multiplex?
Mononeuritis simplex involves a superficial nerve, whereas mononeuritis multiplex presents with asymmetric involvement of multiple superficial nerves. The lower extremities are more commonly affected; acute onset of pain associated with cramping. Pain is typically worse at night.

Question: What symptoms are associated with autonomic dysfunction secondary to diabetes mellitus?
Postural hypotension, impaired temperature regulation, gastroparesis, alternating diarrhea and constipation, urinary hesitancy, overflow incontinence, and impotency.

Question: What is the treatment for diabetic polyneuropathy?
Maintaining optimal glucose control is key; painful paresthesias can be treated with gabapentin, carbamazepine, clomipramine, or topical capsaicin ointment.

Question: What complications are associated with a diabetic sensory polyneuropathy?
Complications include foot ulcerations; due to sensory neuropathy patients are unaware of minor traumas to the feet which can progress to ulcerations.

Question: What complication of diabetes is characterized by degenerative changes in the tarsal and tarsometatarsal joints of the foots?
Charcot joint, a neuropathic joint disease.

Question: What are the common causes of acute or new onset headaches?
Headaches associated with an acute onset include subarachnoid hemorrhage, meningitis, or encephalitis, and ophthalmologic conditions such as acute-angle closure glaucoma or acute iritis.

Question: What are the common causes of subacute headaches that generally develop over weeks to months?
Causes of headache associated with a subacute onset include temporal (or giant cell) arteritis, intracranial mass lesions (primary or metastatic tumors, abscesses), and neuralgia (trigeminal neuralgia (or tic douloureux)), glossopharyngeal neuralgia, or postherpetic neuralgia.

What are the common causes of chronic headaches that can occur intermittently over years?
Migraines, cluster headaches, and tension headaches. Chronic headaches can also be associated with degenerative disease in the cervical spine.

Question: What are the pain-sensitive structures associated with headache?
Intracranial pain-sensitive structures are arteries, venous sinuses, meninges, and cranial nerves. Extracranially, the periosteum of the skull, skin and subcutaneous tissues, muscles, eyes, ears, paranasal sinuses, and oropharynx are all pain-sensitive structures.

Question: What are the common precipitating factors for migraine headaches?
Common precipitating factors include meats with nitrite preservatives (i.e., hot dogs or bacon), tyramine-containing cheeses, chocolate, monosodium glutamate, fasting, menses, medications (oral contraceptives), and bright lights.

Question: A 64-year-old female patient presents with a bilateral headache associated with scalp tenderness and stiffness in her jaw with eating. What is the most likely diagnosis?
Giant cell (temporal) arteritis. Other associated symptoms include malaise, myalgias, arthralgias, and fever; symptoms are consistent with polymyalgia rheumatica.

Question: What diagnostic studies are used in the evaluation of temporal arteritis?
The erythrocyte sedimentation rate (ESR) will be elevated, often as high as 100 mm/h. A temporal artery biopsy will be diagnostic for the presence of giant cells.

Question: What is the treatment of giant cell arteritis?
Prednisone, 40 to 60 mg/day for 102 months, and then the dose can be tapered gradually over 1 to 2 years.

Question: What complication is associated with temporal arteritis?
Blindness can develop in half of the patients with temporal arteritis if left untreated.

Question: A 56-year-old man presents with a mild to moderate dull headache (HA) that has steadily progressed over the past month. The HA is worse when he gets up in the morning or with coughing and sneezing. He also notes the recent onset of nausea and vomiting. What is the most likely diagnosis?
An intracranial tumor. Patients with brain tumors do not always have a headache as an initial symptom. However, when patients do develop HA, the pain is dull and aggravated by position changes or coughing and sneezing, which increase intracranial pressure. Any patient presenting with a HA associated with a focal neurologic deficit (i.e., weakness, visual field deficit) needs to undergo a neuroimaging study.

Question: A 67-year-old woman complains of intermittent episodes of pain in her cheek and jaw that feel like a
“lightning bolt hit her face.” The pain is triggered by eating or touching her face. Her physical examination is unremarkable. What is the most likely diagnosis?
Trigeminal neuralgia, or tic douloureux, is associated with severe facial pain in the distribution of the trigeminal nerve; most commonly affecting V2 or V3. Episodes of pain may be triggered by any sort of sensory stimulus to the face, i.e., touching, chewing, talking, shaving, cold weather, or wind.

Question: What is the first-line treatment for the patient described in the previous question?
First-line treatment would be carbamazepine with dosing between 400 and 1200 mg/day.

A 26-year-old woman complains of a dull, throbbing right-sided headache (HA) that lasts for hours but is relieved by sleep. The HA is associated with nausea, vomiting, and a sensitivity to light and sound. She gets similar HA at least once a month and says that prior to the HA, there are “holes in her vision” along with “zigzag lines.” What is the most likely diagnosis?
Classic migraine headache. While classic migraine HA are less common, they are associated with an aura that is usually a visual disturbance (i.e., scintillating scotomata).

Question: What is the clinical presentation of a common migraine headache?
Common migraines, now referred to as migraine without aura, occur more frequently than classic migraines. About 70% of all migraine-type headaches are common migraines or migraine without aura. Common migraines are associated with unilateral pulsatile pain with nausea, vomiting, phonophobia, and photophobia. There is usually a strong family history of migraine headaches.

Question: What is the abortive treatment for migraine headaches?
Mild analgesics, such as nonsteroidal anti-inflammatory medications or acetaminophen, are effective in relieving headache pain. Other first-line options are the 5-HT agonists or triptans, i.e., sumatriptan. For analgesics and triptans to be effective, they must be administered at the onset of the headache. Antiemetics are helpful as well.

Question: What are the contraindications for the use of 5-HT agonists or triptans?
Poorly controlled hypertension, coronary artery, or peripheral vascular disease.

Question: What are the indications for the use of prophylactic medications in the management of migraine headaches?
Frequent headaches (more than one per week), headaches that are difficult to control, or for patients who cannot tolerate triptans or ergot alkaloids.

Question: What classes of medications are effective for prophylactic management of migraines?
Beta-blockers, tricyclic antidepressants, anticonvulsants, calcium channel blockers, and ergot alkaloids.

Question: What is the recommended treatment for migraines during pregnancy?
Migraines during pregnancy should be treated with opiates, i.e., meperidine. All other migraine treatments have risks of teratogenicity or complications with the pregnancy.

Question: What signs and symptoms are seen in conjunction with a basilar migraine?
Basilar migraines are associated with visual disturbances (scintillating scotomata, visual acuity loss, and visual field deficits), nausea/vomiting, impaired consciousness (syncope, confusion, stupor, coma), bilateral paresthesias, vertigo, dysarthria, and ataxia.

Question: A 39-year-old man presents with intermittent episodes of severe headaches around the left eye and temple. The headaches awaken him nightly for several days in a row then he is headache-free for a couple of months before another “round” begins. What is the most likely diagnosis?
Cluster headaches present with severe nonthrobbing headaches that typically occur at the same location and the same time each day. Headaches generally last for a few minutes up to a couple of hours. The headaches occur daily for weeks to months before they spontaneously resolve; patients may be pain-free for months to years.

What are the associated signs and symptoms seen with cluster headaches?
Conjunctival injection, lacrimation, and nasal congestion on the same side as the headache; Horner syndrome (anhidrosis, miosis, and ptosis) can also be seen on the affected side.

Question: What are the abortive therapy options to treat cluster headaches?
100% oxygen, sumatriptan, or dihydroergotamine.

Question: What is the role of corticosteroids in the treatment of cluster headaches?
Prednisone can be initiated at the beginning of a cluster cycle to effectively eliminate the patient’s symptoms; most patients will be pain-free within 2 days.

Question: What are the presenting signs and symptoms associated with tension headaches?
Patients describe a nonthrobbing, bilateral headache (“tight band”) without the associated visual disturbances, nausea, and vomiting seen in conjunction with migraine headaches.

Question: What types of medications are effective in the treatment of tension headaches?
Acetaminophen, nonsteroidal anti-inflammatory drugs, and ergotamine can be helpful for acute headaches; tricyclic antidepressants and beta-blockers can be used for prophylaxis.

Question: What is the differential diagnosis for fever, headache, and nuchal rigidity?
Bacterial meningitis, aseptic meningitis, encephalitis, and brain abscess.

Question: What are the most common pathogens associated with bacterial meningitis in a neonate?
Group B Streptococcus, E. coli, Listeria.

Question: What are the most common pathogens associated with bacterial meningitis in adults 20 to 50 years of age?
Streptococcus pneumoniae, Neisseria meningitidis, and Hemophilus influenzae.

Question: What are the most common bacterial pathogens for adults who are older than 50 years of age or immunocompromised?
S. pneumoniae, Listeria, and gram-negative bacilli.

Question: What risk factors are associated with bacterial meningitis?
Exposure during delivery (E. coli, group B Streptococcus); colonization from respiratory tract, sinusitis, otitis (S. pneumoniae); crowded conditions (military, college (N. meningitidis)); head trauma (Staphylococcus), and neurosurgical procedures (Staphylococcus, gram-negative organisms).

Question: What are the presenting signs and symptoms associated with bacterial meningitis?
Fever, nuchal rigidity, headache, altered mental status, photophobia, nausea, and vomiting; cranial nerve palsies and seizures can be seen in 40% of patients.

Question: Which pathogen associated with bacterial meningitis presents with a petechial and purpuric rash?
N. meningitidis.

What are the signs of meningeal irritation?
Kernig and Brudzinski signs are used to assess for meningeal irritation. Kernig is performed by having the patient supine with the hip and knee flexed; pain and resistance to extension of the knee is considered a positive sign. Brudzinski is performed with the patient supine; flexion of the hips and knees with forward flexion of the neck is considered a positive sign. These signs may not be present in very young or very old patients or in patients who have severe impairment in consciousness.

Question: What is the key to establishing the diagnosis of bacterial meningitis?
Cerebrospinal fluid analysis is the key; gram stain will identify the organism in 60% to 90%. Perform LP with CSF analysis first unless contraindicated.

Question: What are the contraindications to performing a lumbar puncture for a patient with suspect bacterial meningitis?
Papilledema, focal deficits, or altered mental status are consistent with elevations in intracranial pressure. Obtain blood cultures, then begin empiric antibiotic therapy depending upon the patient’s age and the most likely organisms before the CT scan.

Question: What are the cerebrospinal fluid (CSF) findings in bacterial meningitis?
Elevated WBC (>1000 cells/mm3), decreased CSF glucose (<45 mg/dL), and elevated CSF protein levels (>500 mg/dL). Serum glucose levels should be checked at the time of lumbar puncture to correlate the CSF and serum glucose levels. The CSF-to-blood glucose ratio is normally 0.6; in bacterial meningitis, this ratio is less than 0.4.

Question: What is the recommended antibiotic treatment for a newborn (<1 month old) with bacterial meningitis?
Ampicillin and cefotaxime.

Question: What is the recommended antibiotic treatment for an 18-year-old patient with bacterial meningitis?
Cefotaxime (or ceftriaxone) and vancomycin.

Question: What is the recommended antibiotic treatment for a 60-year-old adult with bacterial meningitis?
Ampicillin, cefotaxime (or ceftriaxone), and vancomycin

Question: What role do corticosteroids play in bacterial meningitis?
Dexamethasone has been shown to decrease the morbidity and mortality, in particular sensorineural hearing loss.

Question: What complications are associated with bacterial meningitis?
Impaired mental status/cognition, cerebral edema, seizures, focal neurologic deficits, sensorineural hearing loss, and brain abscess.

Question: What type of prophylaxis is indicated for meningitis secondary to N. meningitides
or H. influenzae?
Prophylaxis with rifampin (600 mg BID × 2 days) is indicated for any close contacts of patients with meningococcal meningitis or meningitis due to H. influenzae.

What type of vaccination is required for the prevention of bacterial meningitis?
Meningococcal vaccination is indicated for the military personnel and travelers to endemic areas (i.e., the “meningitis belt” in Africa); it is not routinely given in the United States.

Question: What is the most common cause of aseptic meningitis?
The most common of aseptic meningitis are enteroviruses (90%); herpes simplex virus (HSV), varicella zoster virus (VZV), mumps, HIV, EBV, and West Nile virus can also cause aseptic meningitis.

Question: What are the uncommon causes of aseptic meningitis?
Uncommon causes include malignancy due to direct invasion of the meninges from leukemia, lymphomas, and breast, pulmonary, or gastrointestinal malignancies. Medication reactions are another uncommon cause; may be associated with NSAIDs, antibiotics, or IVIG. Considered a diagnosis of exclusion once other causes are ruled out; symptoms will resolve with discontinuation of medication.

Question: What is the current gold standard in the diagnosis of aseptic meningitis?
Viral cultures are the current gold standard; however, they can take from 4 to 8 weeks to obtain results. Reverse transcriptase polymerase chain reaction assays (RT-PCR) are a more sensitive and specific test. The turnaround time for results is approximately 34 hours, but RT-PCRs are not routinely available at present, but they have the potential to become new gold standard.

Question: What are the cerebrospinal fluid (CSF) findings in aseptic meningitis?
The WBC is modestly elevated with a predominance of lymphocytes (100–1000 cells/mm3); CSF protein is usually normal but can be slightly elevated (less than 80–100 mg/dL); CSF glucose to blood glucose ratio is normal.

Question: What is the most common cause of encephalitis?
Herpes simplex virus type 1 is the most common; arboviruses (West Nile Virus. St. Louis, eastern equine, western equine) can also cause this; uncommon causes include varicella zoster virus, Epstein-Barr virus, and HIV.

Question: What are the presenting signs and symptoms consistent with encephalitis?
Headache, progressive alterations in mental status, and focal neurological deficits (cranial nerve palsies, hemiparesis); seizures are common.

Question: What will CSF studies show in patients with encephalitis?
CSF findings will be similar to findings seen with aseptic meningitis. There will be increased WBC (less than 250/mm3) of predominantly lymphocytes; CSF glucose will be normal.

Question: What is the recommended treatment for encephalitis?
Acyclovir 10 mg/kg q8h IV × 14 days along with seizure prophylaxis.

Question: What is the prognosis for patients diagnosed with encephalitis?
14% mortality at 1 year; HSV encephalitis can be fatal if untreated; poor neurologic recovery associated with diffuse cerebral edema or intractable seizures at time of presentation.

What is the most common cause of meningitis in patients with AIDS?
Cryptococcus neoformans.

Question: What is the most common involuntary movement disorder?
Essential tremor.

Question: What is the etiology of essential tremor?
The underlying etiology is unknown; about 50% of patients have a positive family history that is consistent with an autosomal dominant pattern of inheritance.

Question: What factors can cause an exacerbation of a physiologic postural tremor?
Anxiety, sleep deprivation, alcohol withdrawal, drug intoxication (i.e., bronchodilators, tricyclic antidepressants), carbon monoxide poisoning, and thyrotoxicosis.

Question: What are the clinical features of essential tremor?
Bilateral involvement of the upper extremities with sparing of the legs; tremor is usually symmetric although one side can be affected more than the other; can also see voice tremors and head tremors (yes–yes or no–no).

Question: What are the recommended treatments for essential tremor?
Propranolol and primidone.

Question: How can essential tremor be differentiated from Parkinson disease?
Resting tremor, bradykinesia, rigidity, and micrographia are all absent.

Question: What type of hyperkinetic movement disorder is characterized by rapid, nonpatterned, dancelike movements?
Chorea.

Question: What is the most common cause of Parkinson disease?
Idiopathic degeneration of dopamine-producing neurons in the substantia nigra.

Question: What is the peak age of onset of Parkinson disease?
The peak is in the early 60s; onset ranges from 35 to 85 years of age.

Question: What are the risk factors for Parkinson disease?
Male gender, history of prior head injury, exposure to pesticides, and positive family history.

Question: What factors are associated with a decreased incidence of Parkinson disease?
Smoking, coffee consumption, use of NSAIDs, and estrogen replacement in women who are postmenopausal.

Question: What medications are associated with parkinsonism symptoms?
Conventional antipsychotics; haloperidol and perphenazine have the highest risk.

What signs and symptoms are associated with Parkinson disease?
Rigidity, bradykinesia, shuffling gait, and resting tremor.

Question: What is cogwheel rigidity?
Brief interruptions in muscle resistance during assessment of passive movement.

Question: What is a festinating gait?
The patient appears to accelerate in an attempt to catch up with the center of gravity due to flexed posture and loss of postural reflexes; classic sign of parkinsonism.

Question: What type of nonmotor symptoms are seen in association with Parkinson disease?
Depression, sleep disturbances, cognitive impairments, and autonomic dysfunction.

Question: What are the signs of autonomic dysfunction in Parkinson disease?
Orthostatic hypotension, urinary urgency, constipation, and excessive sweating.

Question: What is the first-line treatment of Parkinson disease?
Levodopa-carbidopa or a dopamine agonist.

Question: What complications are associated with levodopa-carbidopa treatment?
Motor fluctuations (“on–off” phenomena) and dyskinesias.

Question: Which class of drugs is indicated in the management of a patient with Parkinson disease who has a debilitating tremor as the predominant symptom?
Anticholinergics.

Question: What are the indications for the surgical treatment of Parkinson disease?
Intractable tremor and drug-induced motor fluctuations or dyskinesias.

Question: What is the preferred surgical procedure for Parkinson disease?
Deep brain stimulation.

Question: What is the pattern of inheritance for Huntington disease?
Autosomal dominant; due to a mutation on chromosome 4p.

Question: What is inheritance with anticipation?
The trend for earlier age of diagnosis for subsequent generations; most pronounced if disease inherited from the father.

Question: What is the usual age of onset for Huntington disease?
Fourth or fifth decade of life.

What is the average lifespan for a patient with Huntington disease once there is the onset of symptoms?
15 years.

Question: What are the primary symptoms associated with Huntington disease?
Chorea, dementia, dysarthria, and gait disturbances.

Question: How do clinical features change as Huntington disease progresses to an advanced stage?
Choreiform movements become less prominent; dystonia, rigidity, spasticity, bradykinesia, and myoclonus may begin.

Question: What type of behavioral issues are common with Huntington disease?
Depression, suicidal ideations, aggressiveness, psychosis, and dementia.

Question: What is the treatment for the choreiform movements associated with Huntington disease?
Dopamine-blocking agents, such as haloperidol or chlorpromazine, can be used to treat chorea. However, they are not routinely used because they may actually aggravate motor symptoms.

Question: What is the age of onset for multiple sclerosis?
Usually between 20 and 40 years of age.

Question: What are the diagnostic criteria for multiple sclerosis?
At least two or more documented episodes of neurologic symptoms and examination findings affecting distinct areas of the central nervous system; symptoms must last at least 24 hours and the interval between episodes needs to be separated by at least 1 month.

Question: What is the underlying etiology for multiple sclerosis?
The cause is unknown; however, several viral autoimmune and genetic factors have been implicated.

Question: What is the most common clinical type of multiple sclerosis?
Relapsing remitting.

Question: What are the common presenting symptoms associated with multiple sclerosis?
Paresthesias, gait disorders (unsteadiness, disequilibrium), lower extremity weakness, and incoordination.

Question: What common physical examination findings are seen with multiple sclerosis?
Spasticity, hyperreflexia, ataxia, extensor response (positive Babinski), impaired rapid alternating movements, impaired vibration, and proprioception.

Question: What is dysdiadochokinesia?
The inability to perform rapid alternating movements.

What is internuclear ophthalmoplegia?
Impairment of adduction with eye movements; bilateral involvement is a pathognomic sign of multiple sclerosis.

Question: A 28-year-old female patient presents with a 1-week history of diplopia and vertigo. Three months earlier, she had an episode of blurred vision that resolved spontaneously after 2 days. Physical examination was remarkable for nystagmus and bilateral horizontal gaze palsy on adduction. The remainder of her neurologic examination was normal. An MRI was obtained (see Figure 10-2). What is the most likely diagnosis?

Figure 10-2 (Reproduced, with permission, from Simon RP, Greenberg DA, Aminoff MJ. Clinical Neurology. 6th ed. New York, NY: McGraw-Hill; 2009, Fig. 5-2B.)
Multiple sclerosis; the initial presentation was consistent with optic neuritis. Note the signal abnormalities in the white matter, which are consistent with multiple sclerosis.

Question: A patient with multiple sclerosis describes an electric shocklike sensation that runs down her back to her legs when she flexes her neck forward. What is this phenomenon called?
Lhermitte sign.

Question: What are the findings on examination of the cerebrospinal fluid for a patient with multiple sclerosis?
Mild pleocytosis and the presence of oligoclonal bands.

Question: What neurophysiologic tests are indicated in the evaluation of a patient with suspected multiple sclerosis?
Evoked potentials will show slowed conduction due to demyelination. Visual evoked potentials will evaluate the integrity of the optic nerve, brainstem evoked responses (BAERs) evaluate the integrity of the auditory nerve, and somatosensory evoked potentials (SSEPs) evaluate the integrity of peripheral sensory pathways.

A 34-year-old woman develops blurred vision associated with unilateral retro-orbital pain that is worse with eye movement. On physical examination, her visual acuity is decreased but extraocular eye movements are intact. Funduscopic examination reveals mild papillitis but no exudates or hemorrhages. What is the most likely diagnosis?
Optic neuritis.

Question: What is the treatment of choice for an acute exacerbation of multiple sclerosis?
Corticosteroids; intravenous methylprednisone, followed by a prednisone taper.

Question: Which pharmacologic intervention is used as a disease-modifying agent in the treatment of multiple sclerosis?
Interferon has been shown to decrease the number of lesions seen on MRI as well as the number of exacerbations experienced by the patient.

Question: What is the treatment for the spasticity associated with multiple sclerosis?
Lioresal (Baclofen).

Question: What is the most common cause of seizures in infants and children?
Febrile seizures are most common between the ages of 3 months and 5 years of age.

Question: What is the most common cause of seizures in adolescents and adults?
Head trauma.

Question: What is the most common cause of seizures in adults older than 65 years?
Cerebrovascular disease (ischemic strokes more than hemorrhagic strokes).

Question: What is the most common epilepsy syndrome associated with complex partial seizures?
Mesial temporal lobe epilepsy syndrome (MTLE), which is characterized by sclerosis of the hippocampus. MTLE is usually refractory to anticonvulsants but responds well to surgical resection.

Question: Which epilepsy syndrome, seen in children, is characterized by cognitive impairments and multiple seizure types?
Lennox-Gastaut syndrome.

Question: What type of seizure disorder is characterized by a sudden loss of postural muscle tone?
Atonic seizures; may present with a sudden head drop or complete collapse associated with falling.

Question: What type of symptoms can be seen with simple partial seizures?
Motor, sensory, autonomic, or psychic.

Question: A patient initially experiences clonic movements of the hand, which then spread to involve the forearm and upper arm. What is this phenomenon called?
Jacksonian march; reflective of seizure involvement spreading to neighboring areas of the motor cortex.

What is Todd paralysis?
Following a motor seizure of an extremity, there is a paresis of the muscles that were involved in the seizure that can last from several minutes up to several hours.

Question: What happens to the level of consciousness during a simple partial seizure?
Consciousness is preserved.

Question: What type of autonomic symptoms can be seen with a simple partial seizure?
Pupillary dilation, pallor, flushing, sweating, vomiting, and urinary incontinence.

Question: What type of psychic symptoms can be seen in association with a simple partial seizure?
Memory distortions (i.e., déjà vu), fear, detachment, depersonalization, illusions (objects may appear to grow larger or smaller).

Question: Which type of seizure disorder is characterized by focal seizure activity accompanied by impaired consciousness?
Complex partial seizures.

Question: What are automatisms?
Automatisms are involuntary muscle activity seen in association with complex partial seizures; typical automatisms include lip smacking and chewing and picking or fumbling with clothing.

Question: A 6-year-old child is brought to your office by her mother. The mother states that for the past 6 months she and the girl’s teacher have frequently noticed the child staring into space. Each episode lasted about 15 seconds and was associated with eye blinking. What is the most likely diagnosis?
Absence seizures.

Question: What is the recommended first-line treatment for the patient described in the previous question?
Ethosuximide or valproic acid are recommended as first-line medications for absence seizures.

Question: A 22-year-old female patient presents with recurrent episodes of altered consciousness during which time she would smack her lips and fumble with her clothing, according to witnesses. She was not responsive during these episodes and afterwards she would remain confused for up to an hour. What is the most likely diagnosis?
Complex partial seizure.

Question: What medications can be used as first-line treatment for the patient described in the previous question?
Carbamazepine, phenytoin, lamotrigine, and valproic acid are all first-line treatment options for complex partial seizures.

A 25-year-old male patient is brought to the emergency department by his brother. They were watching a football game when he suddenly lost consciousness and fell to the floor. He was initially rigid before beginning to “shaking all over” for about a minute or two. He was sleepy and disoriented afterwards. What is the most likely diagnosis?
Generalized tonic–clonic seizure.

Question: What is the recommended first-line treatment for the patient described in the previous question?
Valproic acid, lamotrigine, and topiramate.

Question: What percentage of epilepsy patients are refractory to treatment with anticonvulsants?
Approximately 20% to 30%; these patients should be referred to neurosurgery for consideration of a possible surgical resection.

Question: What type of seizure disorder is characterized by 30 minutes of continuous seizures or frequent seizures that occur without full restoration of consciousness?
Status epilepticus.

Question: In addition to the “ABCs,” what pharmacologic intervention is recommended for the first-line treatment of status epilepticus?
Lorazepam.

Question: What is the definition of a transient ischemic attack?
TIAs have been redefined as the acute onset of a neurologic deficit that resolves within an hour without a residual deficit.

Question: A patient presents with the loss of vision in the left eye, which he describes as “someone pulling a shade down over his eye.” The episode resolved spontaneously within 30 minutes. What is this neurologic symptom called?
Amaurosis fugax.

Question: Amaurosis fugax is consistent with pathology involving which blood vessel?
Ipsilateral carotid artery; debris from an atherosclerotic plaque in the internal carotid can embolize to the central retinal artery.

Question: What is the risk of stroke following a transient ischemic attack?
10% to 15% during the first 3 months; however, most strokes occur within the first 2 days following a TIA.

Question: What are the types of stroke?
Ischemic (more common) and hemorrhagic.

Question: What is the most common cause of ischemic stroke?
Atherosclerosis.

What is the most important risk factor for stroke secondary to atherosclerosis?
Hypertension, systolic or diastolic; increased blood pressure can triple the risk of stroke.

Question: What cardiac conditions are associated with increased stroke risk?
Mural thrombus postinfarction, atrial fibrillation, endocarditis, and paradoxical emboli via a patent foramen ovale.

Question: What hematologic conditions are associated with increased risk for stroke?
Thrombocytosis, polycythemia, and sickle cell disease; hypercoagulopathy is also associated, but it is uncommon.

Question: What is a lacunar infarct?
Lacunar infarcts are strokes that are secondary to small vessel disease.

Question: What are common locations for lacunar infarcts?
Basal ganglia, thalamus, and pons.

Question: What are the signs of an anterior cerebral artery stroke?
Contralateral sensorimotor deficit affecting the leg and urinary incontinence.

Question: What are the signs of a middle cerebral artery stroke?
Contralateral sensorimotor deficit affecting the arm, central VII palsy, visual field deficit, and language disturbances.

Question: What type of language deficit is seen with a middle cerebral artery stroke affecting Broca area in the dominant hemisphere?
Expressive (nonfluent) aphasia; presentation may vary from word-finding difficulty to total mutism.

Question: What type of language deficit is seen with a middle cerebral artery stroke affecting Wernicke area in the dominant hemisphere?
Receptive aphasia. Patients will not be able to follow commands since their ability to understand language will be lost; speech will be fluent but nonsensical.

Question: What type of visual field deficit is seen with a middle cerebral stroke?
Contralateral homonymous hemianopsia.

Question: What are the signs of a posterior cerebral artery stroke?
Contralateral visual field deficit (homonymous hemianopsia with or without macular sparing), and visual agnosia; cortical blindness can be seen with bilateral involvement.

Question: What is prosopagnosia?
The inability to recognize familiar faces; it is seen with a posterior cerebral artery stroke.

Question: What are the signs of vertebrobasilar insufficiency?
Cranial nerve palsies (diplopia, dysarthria), hemiplegia or quadriplegia, and altered mental status.

A patient presents with the acute onset of quadriplegia. He is able to communicate only by blinking or moving his eyes up and down. What is the most likely diagnosis?
Locked-in syndrome; consistent with a pontine stroke.

Question: What is the key diagnostic study in the evaluation of an acute stroke?
Noncontrast CT scan of the head to distinguish an ischemic from hemorrhagic stroke.

Question: What vascular territory is involved in the patient described in the previous question?
Middle cerebral artery (MCA). The wedge-shaped density changes on CT are consistent with the vascular territory for the middle cerebral artery; symptoms are also consistent with a MCA stroke.

Question: What is the recommended treatment of an asymptomatic carotid bruit?
Aspirin.

Question: What is the recommended long-term treatment for transient ischemic attacks secondary to cardiac emboli?
Warfarin.

What is the time window to administer recombinant t-PA to treat an acute stroke if contraindications have been ruled out?
3 hours from the onset of symptoms.

Question: What are contraindications for the use of thrombolytics in the management of an acute stroke?
Poorly controlled hypertension (systolic BP > 185 mm Hg; diastolic BP > 110 mm Hg); major surgery within past 2 weeks; prior intracranial hemorrhage; other intracranial disease (history of trauma); history of bleeding from either the gastrointestinal or genitourinary tracts within past 3 weeks; abnormal coagulation profile.

Question: What is the most common cause of intracerebral hemorrhage?
Hypertension.

Question: What are the most common locations for intracerebral hemorrhages?
Basal ganglia (most common), thalamus, pons, and cerebellum.

Question: When is surgical decompression indicated in the management of an intracerebral hemorrhage?
Cerebellar hemorrhages require decompression so as to avoid brainstem compression; surgery is not indicated for bleeds in the basal ganglia, thalamus, or pons.

In addition to hypertension, what are other causes for intracerebral hemorrhages?
Trauma, arteriovenous malformations, amyloid angiopathy, recreational drug use (amphetamines or cocaine), anticoagulation therapy, and coagulopathies.

Question: What is the most common cause of a nontraumatic subarachnoid hemorrhage?
Congenital berry aneurysms.

Question: What additional congenital abnormalities may be seen with berry aneurysms?
Polycystic kidney disease and coarctation of the aorta.

Question: What are the most frequent locations for intracranial aneurysms?
Middle cerebral artery (29%), internal carotid artery (16%), anterior communicating artery (15%), basilar artery (14%), and anterior cerebral artery (9%).

Question: A 44-year-old patient presented with the “worst headache of her life.” The headache came on suddenly and was associated with neck stiffness and vomiting. There was nuchal rigidity on examination, but no focal motor or sensory deficits. What is the most likely diagnosis?
Subarachnoid hemorrhage.

Question: What is the first-line diagnostic study indicated in the evaluation of a possible subarachnoid hemorrhage?
Noncontrast CT scan of the head; will be diagnostic in at least 90% of patients.

Question: For patients who have a suspected subarachnoid hemorrhage but a nondiagnostic CT scan, which diagnostic study is indicated?
Lumbar puncture with cerebrospinal fluid analysis.

Question: What are the typical findings on analysis of cerebrospinal fluid (CSF) for a patient with a subarachnoid hemorrhage?
Grossly bloody CSF and an elevated opening pressure; xanthochromia will be seen with centrifuged CSF.

Question: What is the definitive diagnostic study used in the evaluation of a subarachnoid hemorrhage?
Four-vessel angiography.

Question: What is the recommended treatment of an intracranial aneurysm?
Surgical clipping or endovascular placement of a coil into the aneurysm.

Question: What complications are associated with a subarachnoid hemorrhage?
Vasospasm, hydrocephalus, recurrent hemorrhage, and seizures.

Question: What pharmacologic intervention is recommended to minimize the effects of vasospasm?
Nimodipine, a calcium channel blocker, can help to minimize the ischemic effects associated with vasospasm.


REFERENCES
Aminoff MJ, Greenberg DA, Simon RP. Clinical Neurology. 6th ed. New York, NY: McGraw-Hill; 2005.
Fauci AS, Kasper DL, Longo DL, et al. Harrison’s Principles of Internal Medicine. 17th ed. New York, NY: McGraw-Hill; 2008.
Fiebach NH, Kern DE, Thomas PA, Ziegelstein RC. Barker’s, Burton, and Zieve’s Principles of Ambulatory Medicine. 7th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007.
Hay WW, Levin MJ, Sondheimer JM, Deterding RR. Current Diagnosis and Treatment in Pediatrics. 18th ed. New York, NY: McGraw-Hill; 2007.
Mumenthaler M, Mattle H, Taub E. Fundamentals of Neurology: An Illustrated Guide. New York, NY: Thieme; 2006.