Neurology and Psychiatry Flashcards

Cerebral palsy, Duchenne's Muscular Dystrophy, Autism, RAD, Conduct Disorder

1
Q

What is a common cause of cerebral palsy in the antenatal period?

A

Maternal infections

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2
Q

Which of the following is NOT typically associated with the postnatal aetiology of cerebral palsy?

A) Severe neonatal jaundice
B) Meningitis
C) Birth asphyxia
D) Head injury

A

Birth asphyxia

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3
Q

Which type of cerebral palsy is characterised by slow writhing, and convoluted movements of the fingers, hands, and toes?

A

Dyskinetic

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4
Q

What is a clinical sign commonly observed in a child with cerebral palsy?

A

Reduced power with increased tone and reflexes (Spasticity)

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5
Q

What is a common management option for managing spasticity in children with cerebral palsy?

A

Oral baclofen

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6
Q

What is a potential complication of cerebral palsy?

A

Severe learning difficulties

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7
Q

Which type of cerebral palsy involves a lack of voluntary muscle control?

A

Ataxic

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8
Q

Which medication is used to manage spasticity in cerebral palsy?

A

Diazepam + Baclofen

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9
Q

A 13-month-old girl is referred to paediatrics by her GP due to concerns that she is still not attempting to ‘pull to stand’. She was born at 29 weeks by emergency cesarean section due to foetal bradycardia and weighed 1.1kg at birth.

On examination, she appears healthy and engaged. She responds to her name and has 7 meaningful words. She can drink from a cup using both hands. When put on the floor, she commando crawls to move around. Upper limb tone is normal however lower limb tone is significantly increased.

Based on this patient’s symptoms, in which part of the brain/nervous system has damage occurred?

A

Spastic cerebral palsy results from damage to upper motor neurons

= Upper motor neurons in the periventricular white matter

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10
Q

A 12-month-old child is brought to the paediatric clinic following a referral from their GP. The parents report that the child demonstrates slow, twisting movements of the hands and arms, with varying intensity that tends to increase during periods of excitement. They also observe difficulties with oral manipulation of food, as well as challenges in chewing and swallowing solid foods. The child was born at 36 weeks gestation after a prolonged and difficult labour involving multiple attempts at forceps delivery.

What area of the brain is most likely affected?

A

Dyskinetic cerebral palsy typically manifests as athetoid movements and oro-motor problems

= Basal ganglia and the substantia nigra

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11
Q

A 12-month-old baby with cerebral palsy is brought to the GP with her mother for a routine checkup. The mother reports feeding difficulties and has concerns about developmental delay. She also mentions continuous slow writhing movements of her limbs throughout the day.

Based on the type of cerebral palsy seen in this child, what area of the brain is affected?

A

Dyskinetic cerebral palsy results from damage to the basal ganglia and the substantia nigra

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12
Q

You review a 7-year-old girl who has cerebral palsy. She is having ongoing problems with spasticity in her legs which is causing pain and contractures. On speaking to her mother you ascertain that she is having regular physiotherapy, using the appropriate orthoses and has tried oral diazepam in the past. Which one of the following treatments may she be offered to try and improve her symptoms?

A

Baclofen

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13
Q

What is Duchenne’s Muscular Dystrophy?

A

X-linked recessive condition = malformation of the dystrophin gene (only boys)

Causes muscle weakness and wasting

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14
Q

Children with proximal muscle weakness use a specific technique to stand up from a lying position

This is called what?

A

Gower’s sign

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15
Q

A concerned mother brings her 4-year-old son to see the GP, worried about his walking and balance. The child learned to walk around 2 years old, much the same as his older sister. However, over the last few months, his mum has noticed that he has become reluctant to walk and often trips or falls when he does.

On examination, the child is slim built but has disproportionately large calves. When asked to walk across the room he does so on his tiptoes. Gowers test is positive.

What investigation is considered most appropriate to confirm the likely diagnosis?

A

Genetic analysis

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16
Q

A 2 1/2-year-old boy is brought to the GP because his parents are worried that he is walking strangely. They have noticed that over the last 6 months, he has started waddling when he walks.
He likes playing outside but often has tantrums when he can’t keep up with his twin sister.

It also takes him ages to climb the stairs, and when his mum picks him up she feels like he might slip through her hands. He is growing well. He learned to walk at 19 months of age; his sister walked at 13 months. Otherwise, he is generally well.
On inspection, he has large bulky calf muscles but examination is otherwise normal

What is the inheritance of this child’s condition?

A

X linked recessive

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17
Q

What are the key features of autistic spectrum disorder?

A
  1. Lack of eye contact
  2. Delay in smiling
  3. Avoids physical contact
  4. Repetitive use of words or phrases
  5. Anxiety and distress with experiences outside their normal routine
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18
Q

What is a common repetitive behaviour in children with autism?

A

Self-stimulating movements such as hand-flapping

19
Q

What is a key feature of the inhibited subtype of Reactive Attachment Disorder (RAD)?

A

Avoidance of social interactions and resistance to comfort

20
Q

What behaviours are characteristic of a child with the disinhibited subtype of RAD??

A

Excessive familiarity with strangers and lack of selectivity in attachment

21
Q

Children with RAD may demonstrate what during interactions with caregivers?

A

Lack of distress in situations where distress would normally be expected

22
Q

What is a key difference between Conduct Disorder and Reactive Attachment Disorder (RAD)?

A

Children with Conduct Disorder can form relationships with peers and adults, while those with RAD struggle to do so

23
Q

What risk factor is most commonly associated with the development of Conduct Disorder?

A

Emotional neglect or abuse

24
Q

Wha condition is often co-morbid with Conduct Disorder?

A

Attention Deficit Hyperactivity Disorder (ADHD)

25
How can Conduct Disorder affect the child's long-term development?
It can result in difficulties with relationships and an increased risk of criminal behaviour
26
Define spastic CP
stiff, tight muscles (↑ tone), jerky movements. Most common
27
Define Dyskinetic CP
involuntary, writhing or jerky movements. Tone varies
28
Define Ataxic CP
shaky, uncoordinated movements, poor balance. Rare
29
A 10-month-old baby is brought to clinic because they are not yet sitting independently. On exam, they have increased muscle tone and persistent Moro reflex. The mother also reports frequent drooling and difficulty with feeding. What condition is most likely?
Cerebral palsy — likely spastic type, given the delayed milestone
30
You're reviewing a 9-month-old baby in a developmental clinic. Which combination of signs would raise concern for cerebral palsy?
1. Delayed sitting and walking 2. Increased tone 3. Persistent Moro reflex 4. Abnormal movements 5. Feeding difficulties 6. Drooling
31
What brain region is affected in ataxic cerebral palsy, and what is a hallmark sign?
The cerebellum Hallmark sign = poor balance and uncoordinated, shaky movements. Trouble with voluntary control (shaky when trying to do things)
32
Dyskinetic cerebral palsy is caused by damage to which brain area, and how does it present?
The basal ganglia Presents with involuntary movements (writhing or jerky), and variable tone = unwanted movements you can’t stop (e.g. twisting arms or face movements)
33
Which part of the brain is affected in spastic cerebral palsy, and what is a key clinical feature?
The motor cortex or corticospinal tracts Key feature = stiff, tight muscles (↑ tone) and jerky movements = kids might struggle to walk, move arms easily, or perform coordinated actions because their muscles are constantly tight.
34
Which infants are at increased risk of hypoxic-ischaemic encephalopathy (HIE)?
Infants born >42 weeks gestation who experience prolonged or obstructed labour.
35
What is a common antenatal cause of cerebral palsy?
Hypoxic ischaemic encephalopathy
36
What are typical early clinical features of cerebral palsy in infants?
Delayed achievement of motor milestones and hypertonia
37
What perinatal complication increases the risk of cerebral palsy?
Prolonged or obstructed labour leading to HIE
38
A term baby born after a difficult delivery presents with poor tone and seizures shortly after birth. What is the most likely diagnosis?
Hypoxic ischaemic encephalopathy
39
A 2-year-old girl is referred to the paediatrician with delayed motor milestones. She is otherwise well, and there is no family history of note. She is crawling but has not yet been cruising or walking. She was born at 30 weeks by normal vaginal delivery and weighed 1.2kg On physical examination there is spastic hypertonia in the lower limbs, weakness of lower limbs flexor muscles, knee/ankle hyperreflexia and upgoing plantar The most likely underlying lesion is located at which anatomical location?
spastic = Pyramidal tracts specifically the corticospinal tract
40
A baby born at 38 weeks by normal vaginal delivery is still not breathing 1 min after birth. The heart rate is 110 bpm, but the baby is floppy and has cyanosed extremities. There is no response to stimulation. On inspection of the mouth, there is no visible meconium What is the most appropriate next step in management?
5 breaths via a 250 ml bag-valve mask
41
A 17-year-old boy presents after he hurt his arm while falling off his bike. He appears withdrawn and does not make eye contact as you ask him what happened. As his mother tells you he loves railways, you try to engage the boy by pointing at the train on his t-shirt, but he just looks at your finger. When he speaks, his tone is monotonous, and he refers to himself and his mother as 'you'. When you try to examine him with his mother's permission, he screams and has a tantrum What is a core feature of this patient's condition?
Repetitive behaviours = Another core feature of autism is repetitive behaviours, like rocking backwards and forwards or repetitive hand movements
42
An anxious mother presents to the GP with her nine-month-old boy. She reports he had a difficult birth, was born two weeks premature, and required a short stay in the NICU. The mother is worried he is not developing quickly enough. On examination, the infant has no interest in sitting, has a rounded back, and has poor head control. The legs are pointed, stiff and scissor-like, and when held up, the child does not attempt to weight bare. What is the clinical picture suggestive of?
Cerebral palsy
43
'Commando crawling' is a sign of what
using arms more because legs are stiff therefore spastic cerebral palsy
44
Where is periventricular leukomalacia (PVL) seen instead of hypoxic-ischaemic encephalopathy?
Spastic diplegia