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-MSK Diseases > 14. Neuromuscular Conditions > Flashcards

14. Neuromuscular Conditions Flashcards

1
Q

What are Neuromuscular Conditions?

A

Conditions affecting control of Voluntary Muscles:

  1. Cerebral Palsy (Problem in the Brain)
  2. Spina Bifida (Problem in the Spinal Cord)
  3. Muscular Dystrophy (Problem in the Skeletal Muscle)
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2
Q

What are the Range of Needs for someone with a Neuromuscular Condition?

A

1 .Outpatient Care

  1. Inpatient Care
  2. Complex Needs
  3. Complex Exceptional Needs
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3
Q

When is a Child with Multiple (Complex) Disabilities regarded to have Complex Needs?

A

Whe it has at least 2 different types of severe / profound impairment such that no one professional, agency, or discipline has a monopoly in the assessment and management

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4
Q

What is included in Complex Exceptional Needs?

A 
Severe impairment 4+ categories (or 2+ with ventilation):
1. Learning and Mental Function
2. Communication
3. Motor Skills
4. Self Care
5. Hearing
6. Vision
Note - Also the case if impairments are sustained for 6 months +
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5
Q

What is Cerebral Palsy?

A
  1. A permanent (non-progressive) motor disorder, due to the brain being damaged before the child reaches 2
  2. The lesion is static but the clinical picture is not
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6
Q

What can cause Cerebral Palsy?

A
  1. Prenatal:
  2. a) Placental insufficiency
  3. b) Smoking / Alcohol / Drugs
  4. c) Infection (Toxoplasmosis, Rubella, CMV, Herpes) and Toxaemia
  5. Perinatal: Prematurity, Anoxic Injuries, Infections
  6. Postnatal: Infection (CMV, Rubella) / Head Trauma
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7
Q

What are the different Physiological Classifications of Cerebral Palsy?

A
  1. Spastic (Pyramidal System, Motor Cortex)
  2. Athetoid (Extrapyramidal System, Basal Ganglia)
  3. Ataxia (Cerebellum, Brainstem)
  4. Rigid (Basal Ganglia, Motor Cortex)
  5. Hemiballistic
  6. Mixed (Combination of Spasticity and Atheosis)
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8
Q

What are the Different Anatomical Classifications of Cerebral Palsy?

A
  1. Monoplegia (One Limb Involved)
  2. Hemoplegia (One Side of the Body)
  3. Deiplegia (Lower Limbs)
  4. Quadriplegia / Total Body Involvement
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9
Q

How might Cerebral Palsy present?

A
  1. Spasticity
  2. Lack of Voluntary Control / Poor Coordination
  3. Weakness / Sensory Impairment
  4. Persistence of 2+ Primitive Reflexes
  5. Dynamic Contractures (Increased Muscle Tone and Hyperreflexia; No Fixed-Deformity of Joints)
  6. Fixed Muscle Contractures (Persistent Spasticity and Contractures; Shortened Muscle Tendon Units)
  7. Fixed Contractures with Joint Subluxation / Dislocation and Secondary Bone Changes
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10
Q

What are the Orthropaedic Priorities in Cerebral Palsy?

A
  1. Spine
  2. Hip
  3. Feet
  4. Torsional Lower Limb Problems
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11
Q

What are the System Goals of Orthopaedics in Cerebral Palsy?

A
  1. Sitting Balance
  2. Standing Posture
  3. Gait
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12
Q

How is Pathology of Cerebral Palsy assessed?

A
  1. History - Functional Problems
  2. Examination - Forces of Concern; Anatomical Issues
  3. Investigation - Gait Analysis; Radiographs; MRI
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13
Q

How is Gait Analysed in Cerebral Palsy?

Note - This is done in a compliant patient > 5 years old

A
  1. Observation (Antalgic, Trendelenburg)
  2. Video
  3. 3D instrumented Analysis
  4. EMG, Energy Expenditure
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14
Q

What is analysed in Gait in Cerebral Palsy?

A

Kinematics:

  1. Cadence - Steps per Minute
  2. Step Length - Right Initial Contact to Left Initial Contact
  3. Stride Length - Right Initial Contact to Right Initial Contact
  4. Velocity - Distance / Time
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15
Q

What Spine Complication is common in Cerebral Palsy?

A

Scoliosis

Note - Severity parallels Neurological Involvement

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16
Q

What Hip Complication is common in Cerebral Palsy?

A

Hip Displacement

Note - Likelihood of Displacement is related to the General Motor Function

17
Q

What is assessed in a Musculoskeletal Examination of the Hip, for someone with Cerebral Palsy?

A
  1. Hip Range of Movements - Pain
  2. Hamstring Tightness
  3. Pelvic Obliquity
    Note - This is unreliable
18
Q

What are the different Levels of General Motor Function Classification (GMFCs), in relation to Cerebral Palsy?

A

Level 1 - Walks without Limitation
Level 2 - Walks with Limitations
Level 3 - Walks using a Hand-Held Mobility Device
Level 4 - Self-Mobility with Limitations (May use Powered Mobility)
Level 5 - Transported in a Manual Wheelchair
Note - The Higher the Level, the higher the risk of dislocation

19
Q

What are the different Interventions available for Cerebral Palsy treatment?

A
  1. Posture Management - Physiotherapy
  2. Spasitcity Management:
  3. a) General - Baclofen, Diazepam
  4. b) Specific - Botulinum Toxin (BoTox), Surgery
  5. Deformity Management:
  6. a) Soft-Tissue Release - Adductors, Hamstring
  7. b) Bony Realignment - Varus Derotation / Pelvic Osteotomy
20
Q

In relation to the decision for Surgical treatment of Cerebral Palsy, what are the

  1. Pro’s?
  2. Con’s?
A
  1. a) Reduced Risk of Dislocation
  2. b) Reduced Risk of Pain
  3. c) Better Seating
  4. a) Not all would have done on to dislocate
  5. b) Big Surgery
21
Q

What are the aims for Surgical Treatment of the Spine, for Cerebral Palsy?

A
  1. a) Maintain Seating
  2. b) Maintain Respiratory Function
  3. c) Avoid Rib / Pelvic Impingement
22
Q

What is Spina Bifida?

A

Failure to close the Neural Tube

23
Q

What is protective against Spina Bifida?

A

Folic Acid - 400mcg/day

24
Q

What is the Spectrum of Spina Bifida?

A
  1. Occulta - Benign
  2. Meningocele (Rare)
  3. Myelomeningocele
  4. Encephalocele
  5. Anecephaly
25
Q

What is the management of Meningocele Spina Bifida?

A

Cyst Removed

Note - Usually Neurologically Intact

26
Q

What are the complications of Myelomeningocele Spina Bifida?

A

The Nerve Roots are in the Cyst, so:

  1. Hydrocephaly
  2. Chairi 2 Malformation
  3. Tethered Cord
  4. Urinary Tract Problems
  5. Locomotor Limitation
  6. Learning Disability
  7. Latex allergy
27
Q

What is Muscular Dystrophy?

A

Progressive Muscle Weakness

28
Q

Who is more likely to get Muscular Dystrophy?

A

Male

29
Q

How is Muscular Dystrophy diagnosed?

A
  1. Muscle Biopsy
  2. DNA
  3. Blood Enzymes
  4. Electromyogram (EMG)
30
Q

What are the concern of Muscular Dystrophy?

A
  1. Cardiomyopathy

2. Respiratory Failure

31
Q

What are 2 main types of Muscular Dystrophy?

A
  1. Duchenne Muscular Dystrophy

2. Becker Muscular Dystrophy

32
Q

What is the difference between:
1. Duchenne’s Muscular Dystrophy
and
2. Becker’s Muscular Dystrophy?

A
  1. Failure to make Dystrophin, Wheelchair by 12, Surgery Indicated
  2. Limited but poor Dystrophin, Milder, Later onset
33
Q

What is the presentation of Muscular Dystrophy?

A
  1. Shoulers / Back held back awkwardly when walking
  2. Sway Back / Weak Butt Muscles
  3. Belly Sticks out due to weak Abdominal Muscles
  4. Weak Thighs / Knees Bend back to take weight
  5. Poor Balance
  6. Thick lower leg muscles, Tight Heel Cord
  7. Weak Muscles in from of the Leg - Foot Drop

-MSK Diseases (43 decks)

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