SCI Q & A Review Flashcards
1. Regarding epidemiology, which of the following is the most common cause of spinal cord injury (SCI)? A) Falls B) Violence C) Motor vehicle accidents D) Sports/recreation
C) Automobile accidents are the leading cause of SCI. However, there are differences among age groups. Falls are reported as the most common cause in the elderly, and violence is the leading cause in African Americans.
2. What is the leading cause of death in chronic spinal cord injury (SCI)? A) Heart disease B) Respiratory disease C) Genitourinary disease D) Suicide
B) The leading cause of death in spinal cord injury patients is respiratory diseases, with pneumonia as the most common cause.
According to the International Standards for the Neurologic Classification of Spinal Cord Injury (ISNCSCI), American Spinal Injury Association (ASIA) impairment score of B is defined as?
A) Motor preservation greater than 3 levels below neurologic level and greater than half the key muscles below the single neurologic level are less than 3
B) No motor function more than 3 levels below the motor level with sensory preservation including sacral sparing
C) Motor preservation greater than 3 levels below the neurologic level and greater than half the key muscles below the single neurologic level are graded greater than or equal to 3
D) Complete injury with no sensory or voluntary anal sphincter contraction
B) • A = Complete. No sensory or motor preservation in sacral segments, S4–5.
• B = Sensory incomplete. Sensory preservation below the neurological level with sacral sparing. No motor function more than 3 levels below the motor level.
• C = Motor incomplete. Motor preservation below the neurologic level (> 3 levels) and greater than half key muscles below the single neurologic level graded less than 3.
• D = Motor incomplete. Motor preservation below the neurologic level (> 3 levels) and greater than half muscles below the single neurologic level graded greater than or equal to 3.
• E = Sensory and motor exams are normal.
4. A spinal cord injured patient was noted to have the following on physical examination: motor preservation greater than 3 levels below the neurologic level and greater than half the key muscles below the single neurologic level were less than 3. This would be classified as an American Spinal Injury Association (ASIA) level: A) ASIA A B) ASIA B C) ASIA C D) ASIA D
- C) • A = Complete. No sensory or motor preservation in sacral segments, S4–5.
• B = Sensory incomplete. Sensory preservation below the neurological level with sacral sparing. No motor function more than 3 levels below the motor level.
• C = Motor incomplete. Motor preservation below the neurologic level (> 3 levels) and greater than half key muscles below the single neurologic level graded less than 3.
• D = Motor incomplete. Motor preservation below neurologic level (> 3 levels) and greater than half muscles below the single neurologic level graded greater than or equal to 3.
• E = Sensory and motor exams are normal.
- Which of the following are key muscles tested in the scoring of the American Spinal Injury Association (ASIA) exam?
A) C5-biceps, C7-triceps, T1-adductor digiti minimi, L5-quadriceps, S1-flexor hallucis longus
B) C5-biceps, C6-flexor carpi ulnaris, C8-flexor digitorum profundus, L3-quadriceps, L4-adductor magnus
C) C5-biceps, C8-flexor digitorum superficialis, L2-sartorius, L3-quadriceps, L5-biceps femoris
D) C5-biceps, C8-flexor digitorum profundus, T1-abductor digiti minimi, L4-tibialis anterior, S1-gastrocnemius
D) There are 10 key muscles tested, which include the following:
• C5 = biceps brachialis; elbow flexors
• C6 = extensor carpi radialis; wrist extensors
• C7 = triceps; elbow extensors
• C8 = flexor digitorum profundus; finger flexor of middle finger
• T1 = abductor digiti minimi; small finger abductor
• L2 = iliopsoas; hip flexors
• L3 = quadriceps; knee extensors
• L4 = tibialis anterior; ankle dorsiflexors
• L5 = extensor hallucis longus; long toe extensors
• S1 = gastrocnemius; ankle plantar flexors
- Which of the following is true regarding the zone of partial preservation (ZPP)?
A) Classified only in incomplete injuries
B) The most rostral segment below the assigned level that has sensory or motor function
C) The motor ZPP does not follow the sensory ZPP
D) All of the above
- C) The ZPP is defined as the most caudal dermatomes and myotomes below the sensory and motor levels that remain partially innervated. It is classified only in complete injuries. The motor ZPP does not follow the sensory ZPP. In the scoring sheet, enter the motor or sensory level if there are no segments below and N/A for incomplete injuries. To calculate the length, count the number of levels from the sensory or motor level to the ZPP level.
7. A hyperextension injury that occurs in low-velocity trauma that affects the upper (greater than the lower) extremities is called? A) Central cord syndrome B) Brown-Séquard syndrome C) Anterior cord syndrome D) Cauda equina syndrome
A) Central cord syndrome is an incomplete injury that results in lower motor neuron weakness at the level of injury and upper motor neuron spasticity below the injury. It typically occurs in the elderly who have a preexisting spondylosis. Brown-Séquard is a hemisection of the cord that results in ipsilateral motor and proprioceptive loss with contralateral loss of pain and temperature. Anterior cord syndrome occurs with a vascular injury that results in loss of motor and pain/temperature sensation with preservation of light touch and position sense. Cauda equina occurs with burst fractures or central disc herniations resulting in lower motor neuron flaccid paralysis with loss of sensation in the lower lumbar and sacral segments.
- What is the typical presentation of an individual with Brown-Séquard syndrome?
A) Ipsilateral motor loss at the level of the lesion and contralateral loss of position sense, pain and temperature below the level of the lesion
B) Contralateral proprioceptive and motor loss at the level of the lesion with ipsilateral loss of pain and temperature below the level of the lesion
C) Ipsilateral motor and proprioceptive loss at and below the level of the lesion with contralateral loss of pain and temperature below the lesion
D) Contralateral motor loss at the level of the lesion, ipsilateral proprioceptive loss at the level of the lesion, and contralateral loss of pain and temperature below the lesion
C) The neurological findings seen in Brown-Séquard are based on where various pathways travel and cross over in relation to the brainstem and spinal cord. Since it is a hemisection of the cord, the following is seen:
• Ipsilateral motor and proprioceptive loss at and below the level of lesion
• Ipsilateral sensory loss at the level of the lesion
• Contralateral loss of pain and temperature below the level of the lesion.
- Which of the following is true of the recommendations regarding prevention of upper limb pain and injury in spinal cord injury (SCI) patients?
A) Minimize frequency of repetitive upper limb tasks
B) Minimize the force used to perform upper limb tasks
C) Minimize extreme positions of the joints
D) All of the above
- D) Task frequency can be modified by limiting the number of transfers or switching to power mobility in high-risk patients. Minimizing force can be achieved by using a lighter wheelchair, improving propulsion techniques, and maintaining an optimal weight. Individuals are encouraged to avoid extreme positions of the wrist, avoid positioning above the shoulder by implementing assistive devices/technology, and avoid extreme shoulder internal rotation and abduction to prevent mechanical impingement.
- What is the recommended duration for anticoagulant prophylaxis for an uncomplicated complete spinal cord injury?
A) 6 weeks
B) 8 weeks
C) 12 weeks
D) Until discharge from rehabilitation
- B) Anticoagulant with either low-molecular-weight heparin or adjusted dose unfractionated heparin should be initiated within 72 hours of injury if there is no contraindication. It should be continued for 8 weeks in an uncomplicated case and 12 weeks or until discharge from rehabilitation for those individuals with other risk factors such as lower limb fracture, history of cancer/thrombosis, age > 70, heart failure, or obesity even in those with inferior vena cava filters.
- Vena cava filter placement should be considered for spinal cord injured patients with which of the following?
A) High cervical cord injury with poor cardiopulmonary reserve
B) As a substitute for prophylaxis in a complete injury
C) Older than 70 years
D) All of the above
- A) Vena cava placement is indicated in those who have failed prophylaxis, have a contradiction to anticoagulation, and high lesions with poor cardiopulmonary reserve. It is not a substitute for prophylaxis and may increase the risk of complications in the future such as cava thrombosis or filter migration.
- Which of the following is a true statement regarding women with spinal cord injury (SCI) and pregnancy?
A) The likelihood of pregnancy is reduced since amenorrhea is common
B) The risk of complications during pregnancy is equivalent to that in the able bodied population
C) A caesarian section is the preferred method of delivery
D) Autonomic dysreflexia may be the only presentation of labor in injuries above the T6 level
D) Amenorrhea may occur after injury, but most often menstruation returns 6 months after injury. Reproductive function and fertility is unaffected once menstruation returns. These women should be educated about the issues related to pregnancy in SCI, including complications such as increased risk of urinary tract infections, changes in respiratory function, and biomechanical effects of being in a wheelchair. Since uterine innervations arise from T10 to T12, these individuals may not present with the typical symptoms of labor and must be aware of autonomic dysreflexia. Although caesarian sections may be more common in this population, it is not the preferred plan for delivery.
- In the treatment of erectile dysfunction in spinal cord injured males, the physiatrist should discuss which of the following options?
A) Phosphodiesterase type 5 inhibitors
B) Intracavernosal injections
C) Vacuum devices
D) All of the above
- D) The physiatrist should begin the discussion of sexual function early and be respectful of this sensitive subject. PDE-5 inhibitors have been successful and well tolerated. Its use is contraindicated with the concurrent use of nitrates. When oral medications are ineffective, an injection of alprostadil can be given, but priapism can occur. A vacuum device requires manual dexterity and is contradicted in those on blood thinners or with a history of sickle cell. Other options include intraurethral medications (which are not as widely available) and implantable penile prostheses (which are not preferred because they can lead to corporal tissue destruction).
14. What level spinal cord injury leads one to be concerned about the risk of developing autonomic dysreflexia? A) T4 and above B) T6 and above C) T8 and above D) T10 and above
. B) Autonomic dysreflexia occurs as a result of unopposed sympathetic discharge above the major splanchnic outflow (which occurs at T6 through L2). It occurs after spinal shock, when reflexes return. The patient may present with flushing and diaphoresis above the level of the lesion, hypertension, bradycardia, piloerection, skin pallor, and/ or headache.
15. What is the most common cause of autonomic dysreflexia? A) Pressure ulcers B) Fractures or other trauma C) Restrictive clothing D) Bladder distention
D) The most common causes of autonomic dysreflexia involve the bladder and bowel. There are a number of causes, all of which involve noxious stimuli below the level of the spinal cord injury. All of the above are potential causes, as well as infection, pregnancy, sexual intercourse, diagnostic medical procedures, deep venous thrombosis, and ingrown toenails.
- A T4 American Spinal Injury Association (ASIA) C individual in your office begins to get flushed. You check the individual’s blood pressure and find that it is elevated with a decreased pulse. What should you do next?
A) Sit the person up and loosen any clothing
B) Catheterize the individual
C) Perform fecal disimpaction
D) Administer nifedipine
A) There are several causes for autonomic dysreflexia, but the first action should be to sit the person up and loosen any restrictive clothing, which will lower blood pressure by pooling blood in the lower extremities. Then, it is important to determine the stimulus causing the autonomic dysreflexia. For instance, the most common cause is bladder distention—check for kinks or flush the catheter. Pharmacologic management may be necessary if symptoms persist.
- Which of the following statements is true regarding prognosis and spinal cord injury (SCI)?
A) Preserved sacral sensation has a better prognosis for lower extremity recovery
B) Individuals older than 50 years have a better prognosis for recovery
C) A sensory incomplete injury has a less than 10% chance for ambulation
D) Muscles with antigravity strength can recover two grades in the first year
A) The recovery is faster and more favorable in incomplete injuries and in younger patients. To achieve antigravity strength, it can take an average of 2 months in a complete injury compared with 2 weeks in an incomplete injury. Muscles with a grade of 1 or 2 have a greater probability of improving one grade by 1 year compared with muscles with no activity. ASIA D has the best prognosis for ambulation. Of those persons with ASIA C, 75% will regain the ability to ambulate and 50% in ASIA B. The most significant predictor is the preservation of sacral sensation.
18. Which of the following individuals with spinal cord injury (SCI) can transfer on level surface without the use of a board? A) C4 B) C5 C) C6 D) C7
D) Expected functional outcomes are important to discuss with an acutely injured patient and his or her family. For transfers, patients with a lesion at C4/C5 usually require total assist with a board or a mechanical lift, C6 patients require minimal assist to independent with the use of a board or lift, and C7 patients are usually independent with or without the board on level surfaces (and some assist to independence on uneven terrain). An individual should gain complete independence in transfers at C8-T1, which was not listed as a choice.
19. Which of the following is a risk factor for developing depression post-spinal cord injury (SCI)? A) Male gender B) Age of onset above 40 C) Prior history of depression D) Supportive social structure
C) Screening for depression should be performed on the initial visit and subsequent follow-up appointments. Owing to limited research, the number of those suffering with depression after their injury may be underrepresented. The following are general risk factors: prior or family history of depression, age of onset below 40, chronic pain, female gender, poor support system, multiple comorbidities, substance abuse, and other major stresses. An individual with a previous depressive episode has a 50% probability of a second event. In the SCI population, the completeness and associated medical issues such as a brain injury heighten the risk of developing depression.
20. In a patient with a spinal cord injury, which of the following is not an effective evacuation technique in a bowel program? A) Push-ups B) Abdominal massage C) Supine position D) Deep breathing exercises
C) The use of certain maneuvers may aid in evacuation, including push-ups, abdominal massage, a forward leaning position, breathing exercises, and drinking warm fluids. In addition, the upright position can also stimulate motility more effectively than attempting a bowel program while in bed.
In a spinal cord injured (SCI) patient, which of the following is not true when establishing a bowel program?
A) Schedule a routine the same time of the day after a meal
B) In areflexic bowel, the goal is firm-formed stool that can be manually evacuated
C) Fiber should be uniformly started in each patient
D) A mini-enema can trigger reflexic bowel by acting as mucosal stimulus
C) The establishment of a regular and predictable bowel routine is a crucial part of postinjury management. The type of program may depend on whether the bowels are reflexic or areflexic. The bowel program should be scheduled at the same time daily—usually a half hour after a meal to stimulate the gastrocolic reflexes. For the reflexic bowel, the goal is to use a water-soluble lubricant along with suppository to create soft-formed stool that can be evacuated with digital stimulation. For areflexic bowel, the goal is firm-formed stool that can be manually evacuated. A mini-enema, which is docusate, glycerin, and polyethylene glycol, has been shown to minimize the time from medication insertion to evacuation. The decision to incorporate fiber in the diet should take into consideration the individual bowel pattern and is not recommended to be automatically started in every SCI patient.
What is the classification of a pressure ulcer with full thickness skin loss involving subcutaneous tissue and extending into but not through fascia? A) Stage I B) Stage II C) Stage III D) Stage IV
. C) It is vital to learn the classification of pressure ulcers. Stage I involves changes in skin temperature, tissue consistency, and sensation. The skin appears red or pigmented. Stage II is a partial thickness skin loss involving the epidermis, dermis, or both. Stage III involves full thickness tissue loss, but muscle, bone, and tendon are not affected. Stage IV is similar to Stage III, in that there is full thickness involvement but it extends to muscle, bone, tendon, and joint capsule. If eschar is present, then it cannot be staged and is labeled unstageable.
In a patient with spinal cord injury (SCI), intermittent catheterization should be considered in which of the following?
A) Small bladder capacity (
C) Intermittent catheterization is a treatment option for neurogenic bladder. It should be avoided in individuals who are unable to catheterize because of poor hand strength, a caregiver who is unwilling to assist, urethral abnormalities, small bladder capacity, high fluid intake, and the tendency to develop autonomic dysreflexia. Intermittent catheterization can lead to the development of urinary tract infections, stones, incontinence, urethral trauma, and autonomic dysreflexia. Routine urologic follow-up is crucial
In a patient with spinal cord injury (SCI), suprapubic catheterization should be considered for which of the following?
A) Sacral pressure ulcer
B) Urethral abnormalities or obstruction
C) Improved body image
D) All of the above
D) Suprapubic catheterization is an alternative to those individuals who cannot perform intermittent catheterization because of the following reasons: urethral stricture or obstruction, perineal skin breakdown, prostatitis, urethritis, or epididymo-orchitis. It can be considered to improve body image and sexual function. It is the preferred method in the acute phase in patients with urethral trauma.
25. In patients with spinal cord injury (SCI), what are the long-term complications of an indwelling catheter? A) Bladder and kidney stones B) Hydronephrosis C) Pyelonephritis D) All of the above
D) Indwelling catheters may be an option for a higher level of injury (such as complete tetraplegia). Although reports have shown that the risk of urinary tract infections is greater with an indwelling catheter compared with intermittent catheterization, there are studies that have shown that the risk may be analogous. It should be considered in individuals with elevated detrusor pressures who are at risk for upper tract complications. It is associated with an increased risk of bladder/kidney stones, epididymitis, urinary tract infections, incontinence, pyelonephritis, hydronephrosis, and cancer. Therefore, more frequent cystoscopic evaluation is warranted in these patients.
- Which statement is true regarding the use of alpha blockers in the treatment of detrusor sphincter dyssynergia?
A) Urethral resistance is increased with the use of alpha blockers
B) Phosphodiesterase inhibitors should be used with caution in patients on alpha blockers
C) Alpha blockers should be taken in the morning in the upright position
D) All of the above
B) Detrusor sphincter dyssynergia is a common bladder condition seen in spinal cord injury patients. The detruor is overactive and spastic, and the internal sphincter is also hyperactive. It results in a small bladder that is unable to empty. This increases the risk of high-voiding pressures and vesicoureteral reflux. Alpha receptors are found in the proximal urethra and bladder neck, and therefore alpha blockers can lower urethral resistance. One of the complications of using this medication is orthostatic hypotension. The medication should be taken at night while in the supine position. Patients taking alpha blockers should be cautioned to avoid phosphodiesterase inhibitors to prevent an abrupt drop in blood pressure.
The external urethral sphincter is innervated by the: A) Hypogastric nerve B) Pelvic nerve C) Vagus nerve D) Pudendal nerve
D) The external urethral sphincter is innervated by the pudendal nerve (S2-S4). The internal urethral sphincter is innervated by the hypogastric nerve (T11-L2) and allows for the storage of urine. The parasympathetic system, through the pelvic splanchnic nerve (S2-S4), promotes bladder contraction and voiding.
28. What is the most common location of heterotopic ossification (HO) in spinal cord injury (SCI) patients? A) Hip B) Knee C) Shoulder D) Elbow
. A) HO is the formation of true bone in ectopic sites that restricts range of motion. HO can present with swelling, fever, limited mobility, or pain. Ninety percentage of the time, in spinal cord injured patients, it occurs in the hips. Serum alkaline phosphatase will be elevated, but is not a specific measure and levels gradually diminish with maturation. HO may not be visible on plain films in the acute phase, but will be seen on bone scan. Treatment includes gentle ranging exercises, etidronate, and rarely radiation therapy. Surgical resection can be considered in severe cases after maturation.
29. Which of the following is a risk factor for the development of heterotopic ossification (HO) in spinal cord injury (SCI)? A) Gender B) Level of lesion C) Spasticity D) Race
C) The risk of HO is greater in complete spinal cord injuries, older individuals, in the presence of spasticity, and in patients with pressure ulcers. No relationship has been shown with gender, race, level, or cause of injury.
- Which of the following is true of calcium metabolism in spinal cord injury (SCI)?
A) Hypercalcemia occurs more commonly in females, incomplete paraplegia, and chronic injury
B) The risk of fractures is comparable to the able-bodied population
C) Passive weight-bearing (standing with the use of adaptive equipment) results in improved bone mineral density
D) All of the above
C) In SCI, there is a disparity between bone formation and resorption. This results in a greater risk of fractures from osteoporosis, hypercalcemia, and hypercalciuria. Hypercalcemia is seen most often with recent injury, male gender, complete injury, tetraplegia, dehydration, and immobilization. Chronic SCI patients may develop vitamin D deficiency, which requires supplementation. Restricting calcium intake is not recommended. Treatment includes bisphosphonates, intravenous fluids, or calcitonin. Functional electrical stimulation and weight bearing may decrease bone loss.
The most caudal end of the spinal cord is at which level? A) The 12th thoracic vertebra B) The 10th thoracic vertebra C) The 4th lumbar vertebra D) The 2nd lumbar vertebra
D) Up through the third month of fetal life, the spinal cord occupies the whole length of the vertebral canal. After the third month, the rate of lengthening of the spinal cord is slower than the lengthening of the vertebral column.
32. How many cervical nerve roots are there? A) 5 B) 6 C) 7 D) 8
- D)
33. The C6 nerve root exits: A) Above the C5 vertebra B) Above the C6 vertebra C) Below the C7 vertebra D) Above the C4 vertebra
- B) In the cervical region, nerves exit the intervertebral foramina just rostral to the vertebra of the same name with the exception of the C8 nerve root, which has no corresponding vertebral body. It resides below C7 and above T1.
34. Which of the following is the most common cause of traumatic spinal cord injury (SCI)? A) Motor vehicle crash B) Sports C) Violence D) Falls
- A) Motor vehicle crashes account for 47% of traumatic SCIs.
35. What is the most common level of spinal cord injury (SCI)? A) T10 B) T6 C) L5 D) C5
- D)
- The lateral spinothalamic tracts:
A) Control voluntary muscle activity
B) Transmit proprioception only
C) Transmit pain and temperature
D) Transmit proprioception, fine touch, and vibration
- C) Spinocerebellar tracts transmit unconscious proprioception from the ipsilateral side of the body.
Lateral corticospinal tracts control voluntary muscle activity.
Dorsal columns transmit proprioception, fine touch, and vibration sense from the ipsilateral side of the body.
- Which of the following is the most common cause of death for persons with a spinal cord injury (SCI)?
A) Heart disease
B) Disease of the respiratory system
C) Cancer
D) Stroke
- B) Twenty-two percentage of deaths for persons with SCI treated at a model systems site were due to diseases of the respiratory system. The next most common cause is heart disease (11.8%), followed by infectious diseases (10.4%).
38. In the American Spinal Injury Association (ASIA) examination, the C5 myotome correlates with what muscle group? A) Elbow extensors B) Finger abductors C) Wrist extensors D) Elbow Flexors
38. D) The Key Muscle Groups in the ASIA examination for the upper extremities are as follows: C5 = Elbow flexors C6 = Wrist extensors C7 = Elbow extensors C8 = Long finger flexors T1 = Finger abductors
39. In the American Spinal Injury Association (ASIA) examination, the C7 myotome correlates with what muscle group? A) Elbow flexors B) Long finger flexors C) Elbow extensors D) Wrist extensors
39. C) The Key Muscle Groups in the ASIA examination for the upper extremities are as follows: C5 = Elbow flexors C6 = Wrist extensors C7 = Elbow extensors C8 = Long finger flexors T1 = Finger abductors
40. In the American Spinal Injury Association (ASIA) examination, the nipple line is the key dermatome for what level? A) T4 B) T10 C) T6 D) L4
- A) The T4 dermatome includes the nipple. The T6 level is the xiphoid, L4 is the medial malleolus, and T10 is the umbilicus.
41. In the American Spinal Injury Association (ASIA) examination, the umbilicus is the key dermatome for what level? A) T6 B) T4 C) T10 D) L4
- C) The T10 dermatome includes the umbilicus. The T6 level is the xiphoid, L4 is the medial malleolus, and T4 is the nipple.
- A complete spinal cord injury is defined as:
A) A transection of the spinal cord
B) No motor or sensory function preserved in the sacral segments S4–5
C) No motor sparing, but sensory sparing below the level of injury
D) Strength less than antigravity below the level of injury
B) (ASIA A classification)
A patient sustains a spinal cord injury. He has the following motor examination: C5—5/5 bilateral C6—5/5 bilateral C7—3/5 bilateral C8—2/5 bilateral T1—2/5 bilateral L1—1/5 bilateral L2—1/5 bilateral L3—1/5 bilateral L4—0/5 bilateral L5—0/5 bilateral Sensation—Intact to pinprick and light touch to the armpit; impaired (1/2) from armpit to rectum with pinprick and light touch sparing at S4–5 and rectal tone.
What is the motor level of injury for this individual? A) C6 B) T1 C) L3 D) C7
- D) The motor level of injury is defined as the lowest key muscle that has a grade of at least 3, provided the key muscles above that level are graded as 5.
The sensory level of injury is the most caudal dermatome to have normal (score of 2) sensation for both pinprick and light touch.
The ASIA Classification System is as follows:
A = Motor and sensory complete—no sacral sparing including pin prick (PP) or light touch (LT) at any of the S4–5 dermatomes
B = Sensory incomplete
C = Motor incomplete—defined as
1) Sacral sparing of motor function (anal contraction)
2) Sacral sparing of sensation with motor function present in more than three levels below the motor level on either side (may include nonkey muscles)
Less than half of muscles 3/5 or greater below the motor level of injury
D = Motor incomplete—as ASIA C with more than half 3/5 or greater
E = Neurologically intact
A patient sustains a spinal cord injury. He has the following motor examination: C5—5/5 bilateral C6—5/5 bilateral C7—3/5 bilateral C8—2/5 bilateral T1—2/5 bilateral L1—1/5 bilateral L2—1/5 bilateral L3—1/5 bilateral L4—0/5 bilateral L5—0/5 bilateral Sensation—Intact to pinprick and light touch to the armpit; impaired (1/2) from armpit to rectum with pinprick and light touch sparing at S4–5 and rectal tone.
What is the sensory level of injury? A) T1 B) T4 C) S5 D) C7
- A) The motor level of injury is defined as the lowest key muscle that has a grade of at least 3, provided the key muscles above that level are graded as 5.
The sensory level of injury is the most caudal dermatome to have normal (score of 2) sensation for both pinprick and light touch.
The ASIA Classification System is as follows:
A = Motor and sensory complete—no sacral sparing including pin prick (PP) or light touch (LT) at any of the S4–5 dermatomes
B = Sensory incomplete
C = Motor incomplete—defined as
1) Sacral sparing of motor function (anal contraction)
2) Sacral sparing of sensation with motor function present in more than three levels below the motor level on either side (may include nonkey muscles)
Less than half of muscles 3/5 or greater below the motor level of injury
D = Motor incomplete—as ASIA C with more than half 3/5 of greater
E = Neurologically intact
A patient sustains a spinal cord injury. He has the following motor examination: C5—5/5 bilateral C6—5/5 bilateral C7—3/5 bilateral C8—2/5 bilateral T1—2/5 bilateral L1—1/5 bilateral L2—1/5 bilateral L3—1/5 bilateral L4—0/5 bilateral L5—0/5 bilateral Sensation—Intact to pinprick and light touch to the armpit; impaired (1/2) from armpit to rectum with pinprick and light touch sparing at S4–5 and rectal tone. What is the American Spinal Injury Association (ASIA) Classification for this patient? A) ASIA A B) ASIA B C) ASIA C D) ASIA D
- C) The motor level of injury is defined as the lowest key muscle that has a grade of at least 3, provided the key muscles above that level are graded as 5.
The sensory level of injury is the most caudal dermatome to have normal (score of 2) sensation for both pinprick and light touch.
The ASIA Classification Systeem is as follows:
A = Motor and sensory complete—no sacral sparing including pin prick (PP) or light touch (LT) at any of the S4–5 dermatomes
B = Sensory incomplete
C = Motor incomplete—defined as
1) Sacral sparing of motor function (anal contraction)
2) Sacral sparing of sensation with motor function present in more than three levels below the motor level on either side (may include nonkey muscles)
Less than half of muscles 3/5 or greater below the motor level of injury
D = Motor incomplete—as ASIA C with more than half 3/5 of greater
E = Neurologically intact
46. An 82-year-old man trips and falls. On presentation, he has an ecchymosis on his chin. On physical examination, bilateral upper extremities were found to have 2/5 strength with elbow flexion and wrist extension, 3/5 strength with elbow extension, finger flexion, and finger abduction, and 4/5 strength in bilateral lower extremities. He has intact sensation. What is this spinal cord injury (SCI) syndrome? A) Brown-Séquard B) Central cord C) Anterior cord D) Cauda equina
- B) Central cord syndrome is the most common of the incomplete spinal cord lesions. It produces motor weakness greater in the arms than in the legs and variable sensory loss. There is sacral sensory sparing. This syndrome is due to an injury to the central part of the cervical spinal cord.
- A 17-year-old female is stabbed in the back and presents as follows:
Loss of sensation, paralysis, and loss of vibration below T5 on the left; loss of pain and temperature below T5 on the right. What syndrome does she have?
A) Brown-Séquard
B) Central cord
C) Anterior cord
D) Cauda equina
- A) Brown-Séquard syndrome involves an injury to the transverse section of the spinal cord (relative hemisection). The resultant injury involves ipsilateral motor and proprioception loss with contralateral loss of pain and temperature.
NM 1. Which of the following motor neuron diseases typically causes both upper and lower motor neuron signs?
A) Spinal muscle atrophy II
B) Primary lateral sclerosis (PLS)
C) Amyotrophic lateral sclerosis (ALS)
D) Poliomyelitis
C) Weakness may be due to upper or lower motor neuron loss. Upper motor neuron (UMN) signs include weakness, spasticity, hyperreflexia, and upgoing plantar response. Lower motor neuron (LMN) signs include weakness, atrophy, flaccidity, hyporeflexia, and fasciculations. ALS patients with UMN pathology will often have a loss of dexterity or feeling of stiffness in their limbs. Spasticity may further exacerbate weakness and loss of function. This is due to the involvement of the vestibulospinal and reticulospinal tracts. LMN symptoms in the ALS population include muscle weakness, with some muscle fasciculations, atrophy, and muscle cramping. Cramping of abdominal or other trunk muscles should prompt a clinician to strongly consider ALS as a possible diagnosis. Primary lateral sclerosis (PLS) is classified as an UMN lesion. Spinal muscle atrophy II (SMA II) and poliomyelitis are classified as LMN lesions.
NM 2. A 60-year-old man without any significant past medical history presents to your outpatient office with asymmetric atrophy, weakness, and fasciculations. He also complains of some difficulty swallowing his meals and complains of a strained and strangled quality in his speech. He describes normal bowel and bladder function. Which of the following is most likely his diagnosis?
A) Amyotrophic lateral sclerosis (ALS)
B) Spinal muscle atrophy III
C) Primary lateral sclerosis (PLS)
D) Poliomyelitis
A) ALS most commonly affects people in the age group of 40 to 60 years, and the mean age of onset is around 60 years. Onset is usually insidious and painless. Asymmetric weakness is the most common presentation. Dysphagia (oral, pharyngeal), dysarthria, drooling, and aspiration can occur and are signs and symptoms representing bulbar muscular weakness. Also, strained, strangled quality of speech, reduced rate, and low pitch indicate a spastic dysarthria. Bowel and bladder function is typically spared in ALS
NM 3. Which of the following is considered a poor prognostic factor in patients with amyotrophic lateral sclerosis (ALS)?
A) Predominance of upper motor neuron (UMN) findings at diagnosis
B) Long period from symptom onset to diagnosis
C) Younger age of onset
D) Pulmonary dysfunction early in the clinical course
D) Poor prognostic factors include predominance of lower motor neuron (LMN) findings at diagnosis, short period from symptom onset to diagnosis, and older age at time of onset. Bulbar and pulmonary dysfunction early in the disease course is also a poor prognostic factor. Women typically present with bulbar symptoms, as compared with men. It is important to note that electrodiagnostic indicators of poor prognostic indicators include profuse spontaneous fibrillations, positive sharp waves, and low-amplitude compound muscle action potential.
NM 4. What is the prognosis for patients with amyotrophic lateral sclerosis? A) 50% die within 3 years B) 100% die within 3 years C) 50% live up to 7 years D) 50% live up to 10 years
- A) The overall median 50% survival rate is 2.5 years after diagnosis. Survival rate is largely dependent on a patient’s decision to use mechanical ventilation and/or a feeding tube, but the 5-year survival rate is between 4% and 30%. Around 10% will live for 10 years.
NM 5. Which of the following pharmacologic agents has been approved for patients with amyotrophic lateral sclerosis (ALS) to slow the progression and improve survival? A) Levodopa B) Riluzole C) Baclofen D) Rebif
B) This is an antiglutamate agent that may be effective in slowing the disease, prolonging ventilator time, and may improve survival in patients with bulbar onset disease. However, side effects can include asthenia, and the medication is expensive. Nonpharmacological management of ALS includes rehabilitation, preventing contractures, submaximal exercise, tracheostomy, and respiratory therapy. Rebif is a beta interferon that is used to modify the course in multiple sclerosis patients. Baclofen is a derivative of gamma-aminobutyric acid (GABA) and is primarily used to treat spasticity. Levodopa is a medicine used to control symptoms of Parkinson’s disease. Levodopa does not slow the disease process, but it improves muscle movement and delays severe disability.
NM 6. Which of the following motor neuron diseases typically causes lower motor neuron signs and has the earliest disease onset?
A) Kugelberg–Welander disease
B) Primary lateral sclerosis (PLS)
C) Amyotrophic lateral sclerosis (ALS)
D) Werdnig–Hoffmann disease
D) Upper motor neuron signs include weakness, spasticity, hyperreflexia, and upgoing plantar response. Lower motor neuron signs include atrophy, flaccidity, hyporeflexia, and fasciculations. Werdnig–Hoffmann disease is also known as spinal muscular atrophy type I, or acute infantile-onset SMA. This severe disorder often results in death by the age of 2, and disease onset is 3 to 6 months. Recent studies have shown that there is a reported increase in longevity, most likely a result of better overall medical management. However, SMA type I carries the worst prognosis of all the forms of SMA, as patients may never be able to reach the childhood milestone of sitting independently. The disease course is rapid and fatal secondary to respiratory failure. SMA II and III have later onset, and progression is generally slower. Kugelberg–Welander disease is also called SMA type III. All forms of spinal muscle atrophy are classified as lower motor diseases. PLS is an upper motor neuron disease, and ALS is classified as both upper and lower lesion motor disease.
NM 7. A 16-year-old male presents to your office with concerns that recently he uses his hands and arms to “walk” up his own body from a squatting position. He states that he was otherwise independent with standing and walking and has been doing well as a student and plans on attending college. Which lower motor neuron disease does he most likely have?
A) Spinal muscular atrophy (SMA) type I
B) SMA type II
C) SMA type III
D) Amyotrophic lateral sclerosis (ALS)
C) SMA type III (spinal muscular atrophy) is also known as Kugelberg–Welander disease. The disease onset is later than that of type I and II, occurring between 2 and 15 years of age. Patients typically live a normal life expectancy. The progression is slower than the other two variants. Patients usually achieve independent standing/walking. This patient presents with Gower’s sign, which can be seen in SMA type III patients because proximal weakness is greater than distal weakness. These patients typically have normal intelligence. Complications in SMA III are less frequent, but may include hand tremor, tongue fasciculations (late onset), and areflexia. All forms of spinal muscle atrophy are classified as lower motor diseases, and ALS is classified as both upper and lower lesion motor disease.
NM 8. A patient with spinal muscular atrophy (SMA) type II (chronic Werdnig–Hoffmann) can usually achieve which of the childhood milestone below? A) Assisted sitting B) Independent sitting C) Independent standing D) Independent ambulatio
B) SMA type II is also known as chronic Werdnig–Hoffmann. The disease onset is between 2 and 12 months, and death (often by respiratory failure) occurs by 10 years of age. These patients can usually achieve milestones including independent sitting. They may be able to stand or walk with an assistive device. Answer choice D is therefore incorrect and refers to someone with SMA type III. SMA type I patients never attain the ability to sit independently, and this severe disorder usually results in death by the age of 2.
NM 9. Which of the following motor neuron diseases has the best prognosis?
A) Kugelberg–Welander disease
B) Chronic Werdnig–Hoffmann
C) Werdnig–Hoffmann disease
D) Both B and C, as their clinical course is similar
- A) Spinal muscular atrophy (SMA) type I is also known as Werdnig–Hoffmann disease. Death usually occurs by 2 to 3 years of age. The progression is rapid and fatal. Patients with SMA type II, known as chronic Werdnig–Hoffmann, usually die by about 10 years of age. Again, the progression is fatal. Patients with SMA type III, known as Kugelberg–Welander disease, have a normal life expectancy with a slow progression.
NM 10. Which of the following pharmacologic agents is a first-line treatment for spasticity in patients with amyotrophic lateral sclerosis (ALS)? A) Tizanidine B) Dantrolene C) Benzodiazepine D) Baclofen
- D) Baclofen is a GABA analogue used to facilitate motor neuron inhibition at spinal levels and is the first-line treatment. Dosing can be started at 5 to 10 mg two to three times per day. It can be titrated up to 20 mg four times per day. Potential side effects include weakness, fatigue, and sedation. Patients must be informed that abrupt discontinuation of baclofen may cause withdrawal seizures. Tizanidine is an alpha-2 agonist. Benzodiazepine can be helpful, but can cause respiratory depression and somnolence. Dantrolene blocks calcium release in the sarcoplasmic reticulum and is ineffective at reducing muscle tone, but can cause generalized muscle weakness.
NM 11. Which of the following is the most common presenting form of motor neuron disease in adults?
A) Amyotrophic lateral sclerosis (ALS)
B) Poliomyelitis
C) Spinal muscular atrophy (SMA)
D) Primary lateral sclerosis (PLS)
- A) ALS is also called Lou Gehrig’s disease, as it was named after the New York Yankees’ first baseman who passed away from this disorder. This is unfortunately still the most widely known motor neuron disease and is the most common presenting form. The incidence of ALS is approximately 1.6 to 2.4 cases per 100,000 population.
NM 12. Which of the following is not included in the Halstead and Rossi (1987) criteria in defining postpolio syndrome?
A) History of previous diagnosis of polio
B) Stability for approximately 5 years
C) Recovery of function
D) No other medical problem to explain new symptoms of weakness/atrophy
- B) The answer choice would be correct if it was stability for approximately 15 years. Postpolio syndrome is a diagnosis based on exclusion. It has been well defined by the Halstead and Rossi criteria (1987):
- Confirmed history of poliomyelitis
- Partial to fairly complete neurologic and functional recovery
- A period of neurologic and functional stability of at least 15 years in duration
- Onset of two or more of the following health problems since achieving a period of stability: unaccustomed fatigue, muscle and/or joint pain, new weakness in muscles previously affected and/or unaffected, functional loss, cold intolerance, new atrophy
- No other medical diagnosis to explain these health issues.
NM 13. Which virus has been implicated in the development of poliomyelitis? A) Herpes virus B) Papillovirus C) Picornavirus D) Poxvirus
- C) Acute poliomyelitis is a disease that causes degeneration of the anterior horn cell, and is caused by the polio virus. The polio virus is a small RNA virus belonging to the enterovirus group of the picornavirus family. Picornavirus orally enters the body and spreads via lymphoid system leading to orphaned muscle fibers and potential central nervous system involvement. All other answer choices are DNA viruses.
NM 43. Which of the following is false regarding multiple sclerosis (MS) and pregnancy?
A) Relapses decrease during pregnancy
B) Higher relapse rate in the first 3 months postpartum
C) Women should not worry about pregnancy worsening their disease process
D) Increased incidence of MS by over 50% among their offspring
- D) The incidence of MS in the offspring of patients with MS is only slightly increased (3% for girls and 1% for boys). The net effect of pregnancy on the course of MS is neutral. This is due to the fact that there is a decrease in relapse during pregnancy, but higher than normal relapse during the first 3 months postpartum. Therefore, women should not fear that pregnancy can worsen their MS.
NM 44. Which of the following is true of multiple sclerosis (MS) and pregnancy?
A) Interferon beta is a safe treatment option during pregnancy
B) Glatiramer acetate is a safe treatment option during pregnancy
C) Breastfeeding increases relapse rate in the first 6 months postpartum
D) Restarting disease-modifying agents during breastfeeding is recommended
- B) Interferon beta is FDA category C for pregnancy and is therefore stopped before a woman decides to try to have a child. Interferon beta has been shown to increase the rate of miscarriage. The risk for relapse is lower overall during pregnancy, so the drug is discontinued before conceiving. Glatiramer acetate is FDA category B and may be a better option for those who want to continue medications during pregnancy. Breastfeeding is known to decrease relapse rate in the first few months postpartum, and it is recommended to restart medications when breastfeeding is stopped.
NM 45. The etiology of which disease is thought to be an autoimmune response causing demyelination, axonal damage, and brain atrophy? A) Parkinson’s disease B) Huntington’s disease C) Multiple sclerosis (MS) D) Guillain–Barré syndrome
- C) MS is considered an autoimmune disease. The disease affects the central nervous system most likely by causing demyelination, leading to plaque formation. The plaque causes oligodendrocyte destruction, astrocyte proliferation, and glial scarring, making the propagation of action potential down a nerve impossible. Remission may occur during this disease process as remyelination occurs.
NM 46. Which of the following is false regarding multiple sclerosis?
A) Affects males more frequently than females
B) Affects Caucasians more frequently than African Americans
C) Increased incidence in higher socioeconomic class
D) There is no change in long-term relapses in pregnancy
- A) Multiple sclerosis actually affects females more than males (2:1 female to male ratio). Answer choices B, C, and D are all true. The net effect of pregnancy on the course of MS is neutral. This is due to the fact that there is a decrease in relapse during pregnancy, but higher than normal relapse during the first 3 months postpartum. Therefore, women should not fear that pregnancy can worsen their MS.
NM 47. Of the several patterns of multiple sclerosis, which is most common? A) Secondary progressive B) Progressive-relapsing C) Relapsing-remitting D) Primary progressive
- C) There are six different subtypes of multiple sclerosis (MS). Eighty-five percentage of MS cases are the relapsing-remitting form of MS. This form is characterized by an acute exacerbation followed by a remission period. During the remission, patients can return to their baseline function or may have some form of disability after an exacerbation. The six subtypes of MS are as follows:
- Relapsing-remitting
- Secondary progressive
- Benign
- Progressive-relapsing
- Primary progressive
- Malignant
NM 48. The following are all considered good prognostic factors in multiple sclerosis (MS) except:
A) Age of onset greater than 35 years
B) Optic neuritis at onset
C) Monosynaptic symptoms
D) Ataxia and tremor
- D) Ataxia and tremor have a poorer prognosis for MS patients. All others choices are good prognostic factors. Optic neuritis is inflammation of the optic nerve. It may cause sudden, reduced vision in the affected eye and is considered a good prognostic factor when presenting at onset in MS patients.
NM 49. The following are common symptoms in patients with multiple sclerosis (MS), except: A) Bowel/bladder dysfunction B) Decreased IQ C) Pain D) Fatigue
- B) The classic symptoms of MS include bowel/bladder dysfunction, fatigue (which is central in nature), and pain. Other symptoms that patients may report are balance problems, weakness/paralysis, numbness/tingling, spasticity, cognitive problems, depression, emotional lability, and tremor. MS produces a wide variety of problems depending on the location of the lesion in the central nervous system. However, IQ is not usually affected.
NM 50. Of the following choices, which is not a major problem affecting activities of daily living (ADLs) reported by multiple sclerosis (MS) patients? A) Sensory disturbance B) Fatigue C) Balance difficulties D) Weakness
- A) Fatigue is more problematic in patients in the afternoon and can be experienced in 77% of patients with MS. Energy conservation techniques can be used to treat fatigue, and medications such as methylphenidate and amantadine can also be used. Balance difficulties and weakness can contribute to falls in this patient population.
NM 51. Which of the following can be seen in patients with multiple sclerosis (MS)?
A) Pill rolling tremor
B) Lower motor neuron signs
C) Adson’s sign
D) Weakness and/or Lhermitte’s sign
- D) Patients commonly present with upper motor neuron (UMN) signs. MS is an autoimmune disease of the central nervous system. UMN signs include hyperreflexia, Hoffmann’s and Babinski’s sign, and spasticity. Weakness and decreased sensation can also be noted. Lhermitte’s sign is a classic finding in MS. Patients complain of an electrical sensation that runs down the back and into the limbs (shoulders). It is elicited by bending the head forward (neck flexion); this is caused by involvement of the posterior columns. There is increased sensitivity of the myelin to stretch or traction. A pill-rolling tremor is frequently seen in Parkinson’s disease.
NM 52. The pathognomonic test for multiple sclerosis (MS) includes:
A) Increased cerebrospinal fluid (CSF) protein, oligoclonal IgG bands
B) Multifocal “bright” areas of hyperintensity on T2-weighted images
C) Both of the above
D) None of the above
- D) Although both answer choices A and B may be seen in patients with MS, there is no pathognomonic test for MS. These tests are nonspecific and have to be interpreted with the entire clinical picture.
NM 53. Corticosteroid use in multiple sclerosis (MS) is considered:
A) Effective in long-term use
B) Most responsive in cerebellar and sensory symptoms
C) To speed recovery
D) To prevent further attacks
- C) Corticosteroids (methylprednisolone) are used in acute attacks in MS patients. Corticosteroids have anti-inflammatory and antiedema properties. The dose that is given is 500 to 1,000 mg/day IV for 3 to 5 days and can be given with or without an oral taper. Long-term use is not recommended, as hyperglycemia, hypertension, osteoporosis, and cataracts can occur with prolonged use. Cerebellar and sensory symptoms are least responsive to corticosteroids. Steroids can hasten recovery, but do not prevent further attacks, or alter progression.
NM 54. Which first-line treatment choice can reduce relapse in multiple sclerosis (MS) patients? A) Corticosteroids B) Immunomodulator agents C) Intravenous immunoglobulin D) Immunosuppressive agents
- B) Immunomodulator agents include interferon beta-1a (Avonex and Rebif), interferon beta-1b (Betaseron), and glatiramer acetate (Copaxone). These have been shown to reduce relapse rate in MS patients. Immunosuppressive agents include cyclosporin, azathioprine, methotrexate, and mitoxantrone and can reduce relapse rate, but have a greater side-effect profile and are therefore used as a second-line agent. Intravenous immunoglobulin is also an immunosuppressive agent and is still being studied for use in this patient population. Steroids can hasten recovery, but do not prevent further attacks, or alter progression.
NM 59. Neuroplasticity is a concept that refers to all of the following except:
A) The potential ability of the central nervous system (CNS) to modify its structural and functional organization
B) Partial recovery is possible long after sustaining a brain injury
C) The brain remains capable of changing in response to experience and injury
D) Insult or injury to the CNS is permanent and functional ability cannot be altered with any type of intervention
- D) Insult or injury to the CNS is permanent and functional ability cannot be altered with any type of intervention. Neuroplasticity refers to the dynamic nature of the brain and CNS and its ability to change in response to experience and injury.
NM 60. Which of the following statements regarding constraint-induced movement therapy (CIMT) is true?
A) It requires constraint of the affected extremity
B) It is based on principles of repeated practice and intense activity
C) It utilizes a passive nonintensive approach
D) It aims to increase the use of the unaffected extremity
- B) CIMT is an intervention directed at improving the function of the affected upper extremity after a brain injury. It involves intensive motor training of up to 6 hours daily and motor restriction (constraint) of the unaffected extremity. CIMT is based on research findings that the affected limb is negatively impacted by learned nonuse because of increased dependence on the intact limb.
NM 61. Neural strategies of functional improvement after central nervous system injury include all of the following except: A) Restoration B) Redacting C) Recruitment D) Retraining
- B) Restoration focuses on reengaging residual brain areas that are initially dysfunctional after an injury. Recruitment involves engaging new residual areas of the brain. Retraining involves training the residual brain to perform new functions. Redacting refers to editing or drafting a document for publication
NM 62. Benefits of partial body weight supported gait training include:
A) Addresses ambulation issues for patients who have sufficient strength and balance
B) Enhances development of compensatory gait strategies
C) Provides earlier weight-bearing to increase strength and increase spasticity
D) Allows for the simulation of task-specific walking movements
D) Partial body weight supported gait training allows the simulation of task-specific walking movements and enables therapists to assist patients in the components of gait rather than bearing body weight. Gait training is necessary for the restoration of self-ambulation after brain injury. The patient works at improving coordination of movement and gradually increasing muscle strength. The lack of sufficient strength and balance to maintain an erect posture typically prevents gait training. Partial body weight support gait training is postulated to result in earlier weight-bearing, increased strength, and reduced spasticity. It helps to prevent development of compensatory strategies for ambulation that may develop while using a cane or walker and creates undesirable motor habits. It also reduces the demands of muscles.
NM 63. Neuroplasticity is not positively influenced by: A) Environment and stimulation B) Repetition of tasks C) Motivation D) Compensation
- D) Environmental manipulation might influence both morphological change and the functional outcome after a cortical injury. Research studies revealed that animals reared or housed as adults in complex environments led to enhanced dendritic growth that was correlated with enhanced behavioral abilities. Patient motivation is an important factor in the success of his or her rehabilitation program. An important component of the rehabilitation process is to assist the patient in avoiding development of negative compensatory strategies.
NM 66. Which of the following features is considered a good prognosis indicator in patients with multiple sclerosis?
A) Age at onset greater than 35 years
B) Rapidly progressive onset
C) Sensory findings/optic neuritis at onset
D) Male sex
- C) All of the above features portend a poor prognosis except for C, sensory findings/optic neuritis at onset. Other good prognostic indicators include a sudden onset with a long remission, retained ability to ambulate, and low current disability.
NM 67. All of the following symptoms are seen in multiple sclerosis (MS) except:
A) Impairment of deep sensation, proprioception
B) Scanning speech
C) Impaired convergence
D) Bowel and bladder incontinence
- C) All the above are symptoms of MS except for choice C. Choice A is due to demyelination of the dorsal column tracts. Choice B is seen in “Charcot’s triad” or advance stages of the disease where scanning speech, intention tremor, and nystagmus can manifest themselves. Scanning speech is characterized as being explosive speech that is broken into syllables. Bowel and bladder incontinence are symptoms seen due to demyelination of the spinal cord. Convergence is one of the optical features not seen in MS, whereas nystagmus, optic neuritis, diplopia, and internuclear ophthalmoplegia (a disorder of lateral conjugate gaze in which the affected eye has impaired adduction, causing diplopia) may be noted. However, convergence is generally intac
NM 68. Lhermitte’s sign is produced by:
A) Passive neck flexion causing electrical shock-like sensation radiating to the spine, shoulders, and other areas
B) Axial load placed by pressing downward force on top of the patient’s head causing reproducible numbness to one or both limbs
C) Rotating head to side with neck extended with ipsilateral shoulder abducted at 45 degrees and elbow extended with patient inhaling and holding inspiration
D) None of the above
- A) Lhermitte’s sign is commonly seen in multiple sclerosis (MS), but is not universally seen in all cases. This test assesses for increased sensitivity of the myelin to traction and is similar to an upper motor neuron sign. Choice B refers to Spurling’s test, which is done to test for radiculopathies. Choice C is Adson’s maneuver, which is done to assess for neurogenic thoracic outlet syndrome by causing scalene compression of the brachial plexus.
NM 69. Although a multitude of tests can be done to help diagnose multiple sclerosis (MS), which of the following is not suggestive of this diagnosis?
A) Multifocal bright T2-weighted periventricular images
B) Increased latency seen in somatosensory evoked potentials (SSEP)
C) Increased cerebrospinal fluid (CSF) protein with oligoclonal bands
D) Decreased amplitudes of sensory nerve action potentials (SNAPs) and compound motor action potentials (CMAPs) in nerve conduction studies (NCSs)
- D) All the following are suggestive findings of MS except for choice D. Often, clinical evidence alone is diagnostic of MS if there is an apparent history of repeated attacks and signs of more than two lesions. However, in other cases, magnetic resonance imaging (MRI) findings and spinal tap can help assist in the diagnosis. MRI has the greatest sensitivity, as lesions of white matter are seen in approximately 85% of the cases involving the optic nerves, spinal cord, and brain. Enhancement with gadolinium is a sensitive indicator of disease activity. CSF tap will show increased protein because of the lost myelin and increased amount of IgG. SSEPs are frequently delayed, since MS is a central process and generally involves the cord. Since NCSs test peripheral nerves, they are generally not affected.
NM 70. Which of the following is not used in the treatment of multiple sclerosis (MS)? A) Corticosteroids B) Interferon beta C) Glatiramer acetate D) Rituximab
- D) All the above are used in the treatment of MS except for choice D, which is used in Devic’s disease or neuromyelitis optica. Corticosteroids are often used in acute attacks and are used in high doses and tapered down. Interferon beta and glatiramer acetate are immunomodulating agents that help in decreasing the progression of the disease. Rituximab is a monoclonal antibiotic against B-cell CD20 antigen used in hematological diseases (lymphomas) and many autoimmune diseases, but not used in MS.
NM 71. What is Uhthoff’s phenomenon?
A) Passive neck flexion causing shock-like symptoms radiating to the spine and shoulders
B) Inability to adduct the left eye on right lateral gaze and inability to adduct the right eye on left lateral gaze with intact convergence
C) Worsening of neurological symptoms including visual problems seen with increased body temperature
D) None of the above
- C) Choice A refers to Lhermitte’s sign, and choice B refers to internuclear ophthalmoplegia (INO) all seen in multiple sclerosis. Choice C describes Uhthoff’s phenomenon. This effect is thought to reflect areas of impaired but still functioning myelin that breaks down in transmitting electrical impulses when surrounding fluid is heated.
NM 72. What are the typical features of amyotrophic lateral sclerosis (ALS) associated with lower motor neuron disease? A) Babinski sign B) Increased tone C) Spasticity D) Fasciculations
- D) Increased tone, Babinski sign, and spasticity are all signs of an upper motor neuron (UMN) syndrome, whereas fasciculations are a sign of lower motor neuron syndrome. ALS is a disease with progressive injury and death to both pyramidal (upper motor) neurons and anterior horn (lower motor) neurons.
NM 75. Which of the following medications used for spasticity has the least amount of sedation and cognitive impairment? A) Diazepam B) Dantrolene sodium C) Baclofen D) Clonidine
- B) Although seldom used for spasticity, dantrolene has the least amount of sedation and cognitive impairment of all the antispasticity medications. This is primarily because its mechanism of action takes place peripherally in the sarcoplasmic reticulum, decreasing calcium release. It is an agent that is considered in the treatment of spasticity for patients suffering from brain injury.
NM 77. Arnold–Chiari malformation type II (the downward displacement of the medulla and brainstem through the foramen magnum causing kinking of the brainstem) is associated with which condition? A) Spina bifida occulta B) Meningocele C) Myelomeningocele D) All the above
- C) In spina bifida, a congenital malformation of the vertebral columns and spinal cord exists. Only in myelomeningocele, where the protruding sac contains meninges, spinal cord, and spinal fluid, is there an associated finding of Arnold–Chiari malformation, which presents in approximately 80% to 90% of patients.
NM 78. Which of the following is not true regarding spina bifida?
A) Complicated by hydrocephalus in 90% of cases
B) Of those with Arnold–Chiari malformation, more than 80% of children will require ventriculoperitoneal shunting
C) Three-fold increase in the incidence of patients suffering from lower IQ than the normal population
D) Most common cause of death is cardiovascular complications
- D) All of the above statements are correct except for D. The number one cause of death in spina bifida is central respiratory dysfunction.
NM 86. In the Miller Fischer variant (MFV) of Guillain–Barré syndrome (GBS), what are the classical findings?
A) Ophthalmoplegia, ataxia, and areflexia
B) Dysrhythmias, impaired diaphoresis, photophobia
C) Dysphagia, nausea, diarrhea
D) All of the above
- A) The classic findings in the MFV of GBS are ophthalmoplegia, ataxia, and areflexia. MFV represents only 5% of GBS cases and presents in descending fashion as opposed to the conventional ascending paralysis seen in conventional GBS forms. Thus, the eye is usually affected first. Gait and trunk muscles are often affected with general sparing of the limb muscles. The other choices B and C are seen in another variant of GBS, acute pan-autonomic neuropathy (which is the most rare form and quite fatal).
NM 100. Amyotrophic lateral sclerosis (ALS) is a lesion of: A) Upper motor neurons B) Lower motor neurons C) Upper and lower motor neuron D) None of the above
- C) In ALS, there is weakness and muscle atrophy caused by degeneration of upper and lower motor neurons.
NM 101. Lower motor neuron (LMN) signs include all of the following except: A) Spasticity B) Atrophy C) Hyporeflexia D) Fasciculations
- A) LMN lesions can present with flaccidity, atrophy, and fasciculations. Spasticity is a sign of an upper motor neuron lesion.
NM 102. In a patient with multiple sclerosis (MS), pregnancy usually results in: A) Death B) Symptom relapse C) Complete recovery D) Decreased relapses
- D) Pregnancy in MS decreases relapses. However, relapses may increase after the delivery.
NM 103. Lhermitte’s sign is classically seen, but is not pathognomonic, for this disease:
A) Multiple sclerosis (MS)
B) Amyotrophic lateral sclerosis (ALS)
C) Duchenne muscular dystrophy
D) Poliomyelitis
- A) Lhermitte’s sign is most commonly seen in MS. The patient may experience an electric shock–like sensation radiating to the spine and extremities when the neck is flexed. This is thought to be due to increased myelin sensitivity to traction.
NM 104. Fatigue in a patient with multiple sclerosis (MS) will increase with: A) Heating B) Cooling C) Wetness D) Dryness
- A) Heat can worsen symptoms in patients with MS. They should be encouraged to use air conditioning. If pool therapy is used, the water should be below 84°F (29°C). When exercising, these patients must avoid raising their body core temperature.
NM 107. A baby with spinal muscular atrophy (SMA) type I (Werdnig–Hoffmann disease) may present with: A) Weak cry B) Dysphagia C) Weak suck D) All of the above
- D) SMA is an autosomal recessive disorder of infancy that presents within the first 2 months of life with hypotonia and symmetric weakness of the lower extremities more than the upper extremities. This is due to degeneration of the anterior horn cell of the spinal cord and brainstem. All of the above are presenting symptoms of SMA type I, which is the most severe. Death usually occurs by age 3.