1. Amyloid plaques and neurofibrillary tangles are the hallmarks of:
- Alzheimer’s disease
- Amyotrophic lateral sclerosis
- Ataxia Telengiectasia
2. Difficulty speaking and understanding speech is termed:
3. The most common form of transient facial paralysis is:
- Alzheimer’s disease
- Transient ischemic attack
- Bell’s palsy
- Erb’s palsy
4. Gradually increasing pain and weakness and numbness in the hand or wrist that radiates up the arm suggest:
- Amyotrophic lateral sclerosis
- Carpal tunnel syndrome
- Bloch-Sulzberger Syndrome
5. All of the following may be associated with Guillain-Barre Syndrome except:
- Weakening or tingling sensation in the legs
- Weakness in the arms and upper body
- Nearly complete paralysis
- First symptom is altered mental status
6. Which of the following statement about herpes zoster is not true?
- It is caused by the varicella-zoster virus
- It causes burning, tingling pain and lesions, generally on one side of the body
- Anyone who has had chickenpox is at risk of postherpetic neuralgia
- It is a sexually transmitted disease
7. Diagnostic tests for epilepsy include all of the following except:
- Simple blood tests
- Brain scan
- Wada test
8. Treatment for epilepsy to eliminate or sharply reduce the frequency of seizures may involve all of the following except:
- Cognitive-behavioral therapy
- Narrow-spectrum and broad-spectrum antiepileptic drugs
- Vagus-nerve stimulation
9. The most common inherited neurological disorder is:
- Bloch-Sulzberger Syndrome
- Charcot-Marie-Tooth disease
- Alper’s disease
- Asperger Syndrome
10. A severe form of epilepsy that appears during the first year of life is called:
- Dandy-Walker syndrome
- Devic’s syndrome
- Dravet syndrome
- Fabry disease
11. Lack of ceramide trihexosidase, also known as alpha-galactoside-A causes:
- Fahr’s syndrome
- Fabry disease
- Fisher syndrome
- Gaucher’s disease
12. Symptoms of trigeminal neuralgia may include all of the following except:
- Extreme, intermittent facial pain in the jaw or cheek
- Tingling or numbness on one side of the face
- Pain triggered by contact with the face or facial movements
- Inability to swallow
13. All of the following are true about Tourette syndrome except:
- Drug treatment completely eliminates symptoms
- It is involuntary and may be a chronic condition
- Symptoms are generally most severe during adolescence
- Symptoms are generally detected in children
14. Huntington’s disease is a heritable disorder that involves:
- Sudden paralysis
- Chorea, loss of cognitive abilities, and emotional disturbance
- Uncontrollable swearing and repetitive actions
- Inability to recognize faces
15. All of the following statements about amyotrophic lateral sclerosis are true except:
- It causes degeneration and death of upper and lower motor neurons
- Patients lose strength and control of voluntary muscles
- It impairs cognition and senses
- It progresses rapidly and is fatal
16. Children with Angelman syndrome generally display all of the following except:
- Developmental delays and speech impairment
- Feeding problems
- Loss of hearing and sense of smell
17. All of the following are true about autism except:
- Affected persons have communication, interpersonal, and behavioral problems
- Affected persons display obsessive or repetitive behaviors and interests
- It is the direct result of immunization with thimerosal-containing vaccines
- Early intervention is associated with improved outcomes
18. Brain and spinal tumors may be treated with any or all of the following modalities except:
- Positron emission tomography
19. Symptoms of Parkinson’s disease include all of the following except:
- Tremors of the hands, arms, legs, jaw, and face
- Stiff limbs
- Bradykinesia and impaired balance
- Impaired cognition
20. Narcolepsy is a disorder characterized by:
- Narcotic abuse
- Grand mal seizures
- Reliance on soporific drugs
- Inability to regulate sleep-wake cycles
Answers and Explanations
Alzheimer’s disease (AD) is a specific type of dementia and the most common form of dementia in persons age 65 and older. It is a progressive neurological disease that produces a decline in cognitive function and memory and changes in behavior. In addition to the above-mentioned features of AD, there is a loss of connections between the neurons involved with learning and memory.
Aphasia results from damage to the portion of the brain involved in creating and interpreting language. Aphasia is not a disease. It is a symptom of a neurological disorder or injury, such as stroke, brain tumor, or head injury. Expressive aphasia is the term for problems communicating using spoken or written language. Receptive aphasia refers to problems understanding language.
Bell’s palsy is a temporary paralysis that generally affects one side of the face. It results from damage or trauma to facial nerves. Bell’s palsy has sudden onset with symptoms that may include varying degrees of weakness or paralysis, drooping eyelid or corner of the mouth, drooling, dry eye or mouth, impaired sense of taste, excessive tearing, and facial distortion.
Carpal tunnel syndrome is caused by compression of the median nerve in the wrist. As the disorder progresses, there is a loss of grip strength and manual dexterity may decrease. Resting the affected wrist, immobilization, ice packs, and nonsteroidal anti-inflammatory agents may provide relief by reducing swelling and pressure on the median nerve.
Guillain-Barre Syndrome is a rare autoimmune disease that often follows a respiratory or gastrointestinal viral illness. It also may occur following surgery or immunization. There is no cure for Guillain-Barre Syndrome; however, treatment, including plasmapheresis and high-dose immunoglobulin therapy administered intravenously, aims to reduce the severity of symptoms.
Herpes zoster, commonly known as shingles, occurs along a dermatome and produces characteristic itching, burning, blisters, and pain. It is caused by a reactivation of the varicella-zoster virus that causes chickenpox. The severity and duration of an outbreak can be reduced by prompt treatment with antiviral agents. Older adults who have had chickenpox may be given a varicella-zoster virus vaccine to significantly reduce the risk of developing shingles.
The diagnosis of epilepsy may be established using the above-mentioned tests as well as continuous video EEG monitoring, magnetic resonance imaging (MRI) and functional MRI, and single photon emission computed tomography (SPECT), which traces cerebral blood flow to detect abnormalities during and between seizures.
Because people with epilepsy, especially children and adolescents, may develop behavioral or emotional difficulties in response to stigmatization, cognitive behavioral therapy may be considered as adjunctive therapy. It is not, however, a treatment for the seizure disorder, which for the majority of affected persons is controllable with the use of antiepileptic drugs.
Charcot-Marie-Tooth disease affects an estimated 1 in 2,500 persons in the United States. It is a hereditary motor and sensory neuropathy that affects the peripheral nerves. As such, symptoms include weakness of the feet and legs and foot deformities resulting from weak muscles, which in turn may produce foot drop, tripping, and falls.
Dravet syndrome, also known as severe myoclonic epilepsy of infancy, presents with frequent fever-related seizures during the first year of life. Other types of seizures generally occur over time, and children with Dravet syndrome are often developmentally delayed in language, motor, and interpersonal skills.
Persons with Fabry disease cannot efficiently metabolize lipids and as a result, excessive lipid deposition occurs in the eyes, kidneys, cardiovascular system, and autonomic nervous system. Symptoms of this heritable disease include burning sensations in the hands, raised skin lesions, and in boys especially, corneal changes. Persons with Fabry disease are at increased risk of heart disease and stroke.
Trigeminal neuralgia is characterized by sudden onset of severe pain that lasts less than a minute. It presents most commonly in persons age 50 and older and among women. The frequency and severity of attacks recurs and remits, progressively worsening over time. While the disorder may be debilitating, it is not life threatening.
Tourette Syndrome is characterized by tics – repetitive, involuntary behaviors, vocalizations and movements such as eye blinking, grimacing, and shrugging or jerky muscle movements. Tics also may include self-harm such as repetitively punching oneself as well as coprolalia and echolalia. Persons with Tourette syndrome frequently suffer behavioral problems such as hyperactivity, impulsivity, attention disorders, and repetitive behaviors.
Huntington’s disease is a progressive brain disorder that causes the degeneration of cells in a pair of nerve clusters deep in the brain. The disease affects both the body and the mind. HD is caused by a single dominant gene and affects men and women of all races and ethnic groups. It generally begins during the third and fourth decades of life; however, there is a form of the disease that can affect children and adolescents.
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, causes muscle weakness and atrophy. As it progresses ALS patients have difficulty moving, swallowing, speaking, and ultimately, breathing on their own. Many ALS patients require mechanical ventilation and most die from respiratory failure three to five years from the time initial symptoms are detected.
Angelman syndrome is a genetic disorder that causes developmental delays and multiple neurological problems such as motor difficulties that impair functions. Children with Angelman syndrome are often hyperactive and suffer from movement, balance, and sleep disorders. They have characteristic small, flat heads and protruding tongues. Jerky movements are also characteristic of the disorder.
Although the etiology of autism remains unknown, it is thought to arise from a combination of genetic predisposition and environmental triggers. A review of the evidence conducted by the Institute of Medicine concluded that there is no causal relationship between thimerosal-containing vaccines and autism.
Brain and spinal tumors may be benign or malignant and benign tumors may be asymptomatic depending on their location. Symptoms of brain tumors include the following: headache; seizures; nausea and vomiting; vision, hearing, and motor problems; and sensory and cognitive changes. Symptoms of spinal cord tumors include pain, sensory changes, and motor problems.
Symptoms of Parkinson’s disease often begin on one side of the body and over time affect the entire body. The four characteristic symptoms of Parkinson’s disease are tremor, rigidity, slowness of movement, and postural instability. Tremor is generally most apparent at rest or when the patient is stressed and improves with deliberate movement.
Narcolepsy is a neurological disorder that causes affected individuals to experience irresistible bouts of sleep, causing them to fall asleep for periods ranging from seconds to minutes throughout the day. Along with excessive daytime sleepiness, persons with narcolepsy also suffer from cataplexy, hallucinations at the onset of sleep and/or upon awakening, and transient complete paralysis at the onset or end of sleep.