General
1. What is the Dandy maneuver?
To assess for a possible cerebrospinal fluid (CSF) leak, the patient’s head is held below the waist for several minutes while in a sitting position.1
2. What is the name of the syndrome that comprises quadriplegia, anarthria, and preserved consciousness?
Locked-in syndrome. It is most commonly seen with upper brainstem infarction and can also involve the third nerve nucleus.2
3. What are the common features of normal pressure hydrocephalus?
Gait apraxia, urinary incontinence, and dementia3
4. What is the Adam triad?
The Adam triad of symptoms of normal pressure hydrocephalus includes gait apraxia, incontinence, and dementia.
5. Failure of ventriculoperitoneal shunting in patients with idiopathic normal pressure hydrocephalus may be attributed to what other causes of dementia?
Vascular dementia or Alzheimer disease
6. What are the top three causes of dementia?
Alzheimer disease, Lewy body dementia, and multiinfarct dementia4
7. What are the main differentiating factors between Alzheimer and Lewy body dementia?
In Alzheimer disease, cortical neuritic plaques and neurofibrillary tangles are seen; early impairment in short-term memory is evident. It is more prevalent in women. In Lewy body dementia, there is relatively preserved memory with impairments in executive functions; parkinsonian and autonomic features are present. It is more prevalent in men.5
8. What is pseudobulbar affect?
Pseudobulbar affect (also known as intermittent emotional expression disorder) is characterized by involuntary displays of crying or laughing, typically without any associated feelings of sadness, depression, or euphoria. Pseudobulbar affect is associated with a variety of neurologic illnesses including multiple sclerosis (MS), amyotrophic lateral sclerosis, Alzheimer disease, Huntington disease, Parkinson disease, stroke, and traumatic brain injury.6
9. How is the hearing loss from Ménière disease different from the hearing loss caused by an acoustic tumor?
The hearing loss from Ménière disease fluctuates, whereas the hearing loss from a tumor is usually progressive. Also, the brainstem auditory evoked potentials are normal in Ménière disease.7,8
10. What is the most common cause of Cushing syndrome?
Iatrogenic administration of exogenous steroids9
11. What are the classic symptoms of Parkinson disease?
Resting tremor, cogwheel rigidity, and bradykinesia10
12. What is the abduction relief sign?
The abduction relief sign is performed by the patient abducting the shoulder and placing the hand ipsilateral to the radiculopathy on the top of his head. Relief of radicular symptoms is a positive sign. A worsening of symptoms may point to thoracic outlet syndrome.11
Emergencies and Epilepsy
13. What are the features of baclofen withdrawal?
Rebound spasticity, hemodynamic lability, severe hyperthermia, altered mental status, pruritus, diaphoresis, diffuse intravascular coagulopathy, rhabdomyolysis, and multiorgan system failure12
14. What is the difference between an early and a late posttraumatic seizure?
Early implies less than or equal to 7 days, whereas late is more than 7 days.13
15. What is the definition of status epilepticus?
More than 30 minutes of continuous seizure activity or multiple seizures without full recovery of consciousness between seizures14
16. What is the most common cause of status epilepticus in an adult?
Subtherapeutic antiepileptic drug levels in a patient with known seizure disorder
17. What is the pharmacologic treatment of status epilepticus?
Intravenous (IV) glucose 50% 50 mL, IV thiamine 100 mg (folate/multivitamin)
Treatment is according to the following, in order:
• Lorazepam 1–2 mg every 5 minutes, up to 9 mg (0.1 mg/kg) or diazepam 5 mg every 5 minutes, up to 20 mg (0.2 mg/kg)
• Phenytoin—loading dose 20 mg/kg
• Phenobarbital drip—loading dose 20 mg/kg
• Pentobarbital drip 20 mg/kg if seizure does not arrest in 30 minutes15,16
18. What are some common causes of changes in mental status or coma?
The mnemonic “AEIOU TIPS” is a help in identifying common causes:
• Alcohol (drugs and toxins)
• Endocrine, exocrine, electrolyte
• Insulin
• Opiates, overdose
• Uremia
• Trauma, temperature (abnormal)
• Infection
• Psychiatric disorder
• Seizure, stroke, shock, space-occupying lesion17
19. What is sudden unexpected death in an epileptic patient typically attributed to?
Seizure-related cardiac arrhythmia18
20. What is the long-term outcome of patients who suffer from prolonged refractory status epilepticus?
Approximately 50% mortality. Of the patients surviving, only about half to two-thirds will have functional cognitive status.19
21. What are some indications for a hemispherectomy?
Intractable epilepsy in the setting of unilateral hemisphere damage, congenital hemiplegia, chronic encephalitis, hemimegalencephaly, and Sturge-Weber syndrome20,21
22. Sturge-Weber syndrome is associated with intracranial calcifications, seizures, glaucoma, and port-wine stains in the distribution of which cranial nerve?
The trigeminal nerve. The intracranial findings in Sturge-Weber syndrome are only seen in patients in whom the port-wine lesions involve the first division of the trigeminal nerve.
23. What does the electroencephalogram (EEG) show in an absence seizure?
Spike and wave pattern at exactly three per second22
24. What is the most common type of seizure?
Febrile convulsions23
25. Which type of seizure is associated with West syndrome?
Infantile spasms, also known as epileptic spasms. West syndrome produces seizures in early childhood and consists of the classic triad of mental retardation, infantile spasms, and hypsarrhythmia on EEG (completely chaotic and disorganized background pattern).24
26. What is the treatment of infantile spasms?
Adrenocorticotropic hormone (ACTH) rather than antiepileptics25
27. What is Aicardi syndrome?
A triad of callosal agenesis, ocular abnormalities, and infantile spasm26
28. What is the most common cause of complex partial seizures?
Mesial temporal lobe epilepsy is the most common cause of complex partial seizures. About 70 to 80% of these seizures arise from the temporal lobe, and more than 65% of these originate in the mesial temporal lobe structures, especially the hippocampus, amygdala, and parahippocampal gyrus.
29. Which region of the hippocampus is spared in mesial temporal lobe epilepsy?
The CA2 subregion and the dentate granule cells are relatively spared as cell loss is more pronounced in the CA1, CA3, and CA4 subregions.
Infectious and Inflammatory
Infectious Diseases
30. What are the most common agents that cause transplacental infections?
Use the mnemonic “TORCHES”:
• Toxoplasma
• Other agents such as human immunodeficiency virus (HIV)
• Rubella
• Cytomegalovirus
• Herpes virus
• Syphilis27
31. Discuss the CSF profile in the different types of meningitis.
Refer to Table 8.1 for details.
32. What is the most common cause of viral meningitis?
Enterovirus in ~80% of cases28
33. Which histologic stain is used to diagnose cryptococcosis?
An India ink stain is utilized to identify cryptococcosis, which is shown as single budding yeasts with a thick capsule.29
34. What is the most common central nervous system (CNS) fungal infection?
Candidiasis, which is caused by Candida albicans
35. What is the causative agent of neurocysticercosis?
The larval stage of the pork tapeworm Taenia solium. It is the most common parasitic infection involving the CNS.30
36. What antihelmintics are used to treat neurocysticercosis?
Praziquantel and albendazole. Steroids are also given concurrently to reduce the edema that tends to occur initially during treatment with antihelmintics.31
Infectious Agent | Opening Pressure (mm H2O) | Glucose (mg/dL) | Protein (mg/dL) | White Blood Cell (Type) |
Bacteria | High (< 200) | Low (< 40) | High (> 100) | Elevated (Neutrophilic) |
Viral/Aseptic | Normal/Mildly elevated (< 200) | Normal (50–70) | Normal/Mildly elevated (15–40) | Elevated (Lymphocytic) |
Tuberculosis | Normal/Mildly elevated (180–300) | Very Low (< 40) | High (> 100) | Elevated (Pleocytosis) |
Fungal | Normal/Mildly elevated (180–300) | Low (< 40) | Mildly elevated (50–200) | Elevated (Lymphocytic) |
37. What is Gradenigo syndrome?
Petrous apex osteomyelitis with sixth cranial nerve palsy and retroorbital pain. It occurs in children from extension of severe otitis.32
38. What are Negri bodies?
Intracytoplasmic eosinophilic collections in neurons seen with rabies.33
39. How does the rabies virus reach the CNS?
The virus travels in a retrograde fashion through the peripheral nerves to reach the CNS.34
40. What is the treatment of rabies?
Passive immunization for 10 to 20 days with rabies immunoglobulin.35
41. What is the causative agent of Lyme disease?
Borrelia burgdoferi36
42. What antibiotic is most commonly used to treat Lyme disease?
Ceftriaxone37
43. Which organism is associated with Sydenham chorea?
Group A β-hemolytic streptococcus. Sydenham chorea is one of the main criteria of rheumatic fever. Other features include arthritis, erythema marginatum, and endocarditis.38
44. What are the major symptoms and signs of tabes dorsalis?
The major symptoms are ataxia, lightning pains, and urinary incontinence; the chief signs are absent tendon reflexes at the knee and ankle, impaired vibratory and position sense in the legs and feet, and a positive Romberg sign. An Argyll-Robertson pupil is found in 90% of cases.39
45. What is Hutchinson triad (congenital syphilis)?
Notched teeth, deafness, and interstitial keratitis40
46. The presence of an “owl’s eye” intranuclear inclusion is suggestive of what kind of infection?
Cytomegalovirus (CMV) encephalitis. Cowdry type A intranuclear inclusions are characteristic of CMV, but these are also found with other infections.
47. What does Aspergillus look like with silver stain?
It has branching septate hyphae.
48. What does Mucor look like histologically?
It has nonseptate right-angle branching hyphae.
49. What patients are at risk of suffering from mucormycosis?
Diabetic patients. This disease is fatal within a few days unless treated aggressively.41
50. What is the most common pathogen that causes brain abscess?
Streptococcus. These abscesses occur most commonly at the gray–white matter junction and are multiple in ~30% of cases.
51. How is a brain abscess acquired?
An abscess can form from local spread of an adjacent ear or sinus infection, hematogenous spread from another infected site (most commonly lung), from trauma, or it can be iatrogenic.42
52. In what U.S. states can histoplasmosis be found?
In Ohio and Mississippi
53. Where is blastomycosis found in the United States?
In the Eastern United States
54. Where is coccidioidomycosis found in the United States?
In the Southwest, especially in California and Arizona
55. What is the intermediate host in hydatid disease?
Sheep. Hydatid disease is caused by Echinococcus granulose, a dog tapeworm. Cysts form in the liver, lung, and brain.43
56. What is the intermediated host in schistosomiasis?
The snail. Schistosomiasis is caused by Schinia masoni, S. haematobium, and S. japonicum. These trematodes live in blood vessels.
57. What are the cells of origin that are found in primary CNS lymphoma in a patient with HIV?
B cell origin44
58. What is the most common cause of myelopathy in HIV?
Vacuolar myelopathy. It is prudent to obtain an MRI to exclude a mass lesion before giving this diagnosis because vacuolar myelopathy is a diagnosis of exclusion.45
59. Which parts of the spinal cord does acquired immunodeficiency syndrome-(AIDS) associated vacuolar myelopathy involve?
It mainly involves the posterior and lateral columns of the thoracic spinal cord.46
60. Subacute sclerosing panencephalitis is a postinfectious process of which viral infection?
Measles. It occurs several years after a measles infection, usually before 2 years of age. Death occurs within 1 to 3 years.47
61. What is the causative agent of cat-scratch disease?
Bartonella henselae. Most people acquire this bacterial infection following a scratch or a bite by a cat. Local inflammation and regional adenitis develop. In immunocompromised people, this disease can progress to produce virulent encephalitis associated with status epilepticus. A brain magnetic resonance imaging (MRI) scan may show a characteristic hyperintensity in the pulvinar region.48
62. What types of treatments are given for ADEM (acute disseminated encephalomyelitis)?
High-dose steroids, plasmapheresis, and IV immunoglobulin49
63. What viruses have been associated with ADEM?
Paramyxovirus, varicella, rubella, and Epstein-Barr virus50
64. What do CSF studies typically show with ADEM?
Protein slightly elevated with a lymphocytic pleocytosis51
65. What is the clinical triad of neurologic manifestations in Lyme disease?52
1. Cranial neuritis (mimicking Bell palsy)
2. Meningitis
3. Radiculopathy
66. What is the causative agent of PML (progressive multifocal leukoencephalopathy)?
PML is the result of infection of the brain with the JC virus (a papovavirus). PML is usually associated with impaired immunity, specifically altered cell-mediated immunity.53
67. What is the drug of choice for the treatment of herpes simplex encephalitis?
Acyclovir. Because it is a relatively safe drug, it is acceptable to start this drug empirically in presumptive cases of herpes encephalitis.54
68. What is the most common organism found in hematogenous pyogenic vertebral osteomyelitis?
Staphylococcus aureus55
69. What are the most common ganglia involved in herpes zoster?
The trigeminal and thoracic ganglia are the most common neuronal sites in herpes zoster; these dermatomes are also the most common sites of cutaneous eruption.56
Inflammatory
70. What is the most common human prion disease?
Creutzfeldt-Jakob disease accounts for ~85% of all human prion disease.57
71. What type of inflammatory response is seen in Creutzfeldt-Jakob disease histologically?
None
72. Positive immunoassay for what protein in the CSF supports the diagnosis of Creutzfeldt–Jakob disease?
14–3-3-brain protein58
73. What is the most frequent chronic neurologic disease of young adults?
Multiple sclerosis59
74. Describe the revised McDonald criteria for diagnosis of multiple sclerosis.
Revised McDonald criteria were initiated in 2005. They are as follows:60,61
• Two or more acute attacks with clinical evidence of two or more lesions or
• Two or more attacks with clinical evidence of at least one lesion and two or more lesions found on MRI, or CSF findings suggestive of MS or
• One or more attacks with clinical evidence of two or more lesions with dissemination in time (DIT) evidence or
• One attack and clinical evidence of a lesion with either dissemination in space (DIS) with more than two lesions found on MRI with CSF findings or at least DIT by another attack
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