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1

Clinical OF Charcot Marie Tooth

1. weakness of foot and lower muscles
2. foor deformities - pes Casus and hammertones
3. lower leg take on an inverted champagne bottle apperance due to loss of muscle bulk
4. weakness and muscle atrophy of hands - hard to do motor skills
5. scoliosis - asymmetrical forces on axial spine during growth
6. Pain ( MSK or neuropathic) / muscle cramping

2

obtaining informed consent for an intervention requires

(1) the diagnosis
(2) the nature and purpose of the proposed intervention;
(3) the probable risks and benefits associated with the intervention
(4)alternatives to the proposed intervention, including the risks and benefits associated with each option
(5) the consequencesof refusal or non-intervention.

3

Difference b/w consent and assent

consent - mental capacity - generally not paediatric population
Assent - seek to explain the childs willingness to undergo the medical intervention in a developmentally appropriate manner

4

how do you screen for deep infections?

use a blunt tipped instrument to detect sinus tracts

5

What procedure is the standard accepted for management of a non critical neuropathic and ischemic wound, prior to fitting a foot orthosis?

debridement of necrotic tissue

6

What location and type of sensation on physical examination has been found to be predictive of the ability for psychogenic vaginal lubricatio


Sensation to light touch (1 point) and pinprick (1 point) in the T11-L2 dermatomes (1 point) was found to be predictive of the ability for psychogenic vaginal lubrication

7

Cauda equina post injury ammenorhea
normal?
how long?
pregnancy?

1. Yes (1 mark)
2. Yes (1 mark) 4 -5 months post injury(1 mark)
3. Yes (1 mark)

8

Is there a difference in prevalence between the incomplete and complete tetraplegic patients developing autonomic dysreflexia


AD is 3X (1 Mark) more prevalent in complete injury (

9

symptoms of mid line shift in MCA stroke

Further deterioration of the left MCA clinical findings
• Further decrease in level of consciousness
• Incontinence (probably was already there but could be worse)
• Unequal pupillary responses from the infratentorial herniation (not seen on these images
-only subfalcine herniation is present on the image
• Any of the triad of the Cushing response (bradycardia, hypertension and irregular breathing
from raised ICP)

10

right eye does not track to the left of midline and the left eye, looking left, has nystagmus.

inter nuclear ophthalmoplegia

11

right eye does not track to the left of midline and the left eye, looking left, has nystagmus. where is lesion


Left (1 point) medial pons at the Median Longitudinal Fasciculus

12

inter nuclear ophthalmoplegia seen in

Multiple sclerosis
stroke

13

What general type of neuropathy tends to occur early in the course of HIV?

Immune mediated. (1 Mark) Any two of
AIDP
Brachial Plexopathy
Multiple mono neuropathies
Vasculitic neuropathies
Cranial neuropathies

14

2 types of neuropathy that occur late in HIV

Those associated with opportunistic infections such as CMV, syphilis and zoster, malignancy and nutritional deficiency as well as side effects of drugs used to treat HIV.

15

Three criteria used to grade severity of TBI (ex. mild, mod, severe)

1. Glasgow Coma Scale (GCS)
2. Length/duration of Loss of Consciousness (LOC)
3. Length/duration of Post-Traumatic Amnesia (PTA)

16

Define mild traumatic brain injury, according to the American Congress of Rehabilitation Medicine.

physiological disruption of brain function, with at least one of the following criteria:
- any period of LOC
- any loss of memory for events immediately before or after the accident
- any alteration of mental state at the time of accident (e.g.: feeling dazed, confused, disoriented)
- focal neurological deficits that may or not be transient

but where the severity of the injury does not exceed the following:
- LOC approximately 30 minutes or less
-after 30 minutes, an initial GCS 13-15; and
-posttraumatic amnesia not greater than 24 hours

17

With respect to diffuse axonal injury following trauma, name four locations where petechial hemorrhages commonly occur

Gray-white matter interface Midbrain
Pons
Corpus Callosum
White matter of the Cerebellum

18

THREE PREDICTORS of outcome following TBI

1. Length of coma
2. Glasgow Coma Scale
3. Post Traumatic Amnesia
4. Dysphagia
5. Immobility

19


5. Name the components of a motor unit and give an example of one pathologic condition or disease that can affect each component.

1. Anterior Horn Cell (AHC) - polio, West Nile Virus, ALS, SMA
2. Nerve root - radiculopathy due to disc herniation, spinal or foraminal stenosis, root avulsion,
tumor (schwannoma, neurofibroma, metastases)
3. Plexus - trauma, tumor invasion, radiation plexitis, birth injury (Klumpke/Erb's), etc.
4. Peripheral nerve - CTS, UNE, PIN, AIN, UNW, Radial nerve palsy, tarsal tunnel, CPN at
knee, etc.
5. Neuromuscular junction (NMJ) - Lambert-Eaton syndrome, botulism, myasthenia gravis
6. Muscle - myopathies, polymyositis, muscular dystrophies, DMD, BMD, IBM, EDMD, LGMD,

20

5 Waddell sign

Tenderness: inappropriate tenderness that is widespread and superficial
Simulation: axial pressure and movements of hip
Distraction: straight leg raise test
Regionalization: motor weakness and sensation overreaction to pin prick
Overreaction:

3/5 = positive over reaction

21

5 conditions which may feature myotonic discharges on needle EMG


· Myotonic dystrophy
· Myotonia congenital
· Paramyotonia congenita
· Acid maltase deficiency
· Polymyositis
· Myotubular myopathy
· Hyperkalemic periodic paralysis
· Chronic denervation (e.g. CMT, post-polio)

22

List 5 Characteristics of Oligoarticular Arthritis

Fewer than 5 joints
Usually knees or ankles
May lead to leg length discrepancy
Limping Contracture of affected joint
Toddler refusing to weight bear Asymptomatic uveitis

23

Grades of Whiplash using the Quebec Task Force classification

Grade 0 - No symptoms or signs
Grade 1
- Subjective complaints of neck pain, stiffness, or tenderness
- no objective signs
Grade 2
Subjective symptoms as above, plus decreased ROM and point tenderness on palpation of the neck
Grade 3
Subjective symptoms as above, plus neurological signs (depressed DTRs, weakness, sensory change)
Grade 4
Subjective symptoms as above, plus fracture or dislocation on x-rays

24

Beighton Hypermobility Score


• Palm flat on floor (0.5) while in forward flexion (0.5) of lumbar spine
• Hyperextension of 5th finger (0.5) greater than 90 degrees (0.5)
• Hyperextension of elbows (0.5) greater than 10 degrees (0.5)
• Hyperextension of knees (0.5) greater than 10 degrees (0.5)
• Passive flexion of the thumb (0.5) to touch the volar aspect of the forearm (0.5)

25

Dx of Benign Joint Hypermobility Syndrome

1. Beighton Hypermobility Score
PLUS
2. Arthralgia for longer than 3 months in 4 or more joints

26

most common MRI finding in children with spina bifida

By definition, all children with myelomeningocele have a tethered cord on MRI, but only about 20% of children require an operation to untether the spinal cord during their first decade of life, during their rapid growth spurts. Thus, the MRI scan must be placed in context of a history and examination consistent with mechanical tethering and a resultant neurologic deterioration

27

Treatment for DMD

Deflazacort

28

What is the most common ocular adverse effect of deflazacort in treated boys

Cataracts

29

Deflazacort ( treatment for DMD ) - until what age are the boys able to walk

15

30

5 ways a toddler gait differs from adult

1. Wider base
2. Decreased stride length
3. Higher cadence
4. No heel strike
5. Little knee flexion during standing
6. No reciprocal arm swing
7. External rotation of the entire leg during swing phase