Nervous system Flashcards

1
Q

What is the most common form of cerebral palsy and what are its features?

A

Spastic (~70%).
Affects limbs, increased tone (clasp-knife), brisk reflexes, extensor plantar responses.
Can be hemiplegic (one side affected), diplegic (one half e.g. lower body affected), quadriplegic or monoplegic

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2
Q

What is cerebral palsy?

A

The permanent disorder of motor or posture development caused by non-progressive insult to the developing brain. (the insult can occur, antenatally/intrapartum/postnatally).

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3
Q

What is the name of the classification system for severity of gross motor impairment?

A
Gross Motor Function Classification System (GMFCS)
Class 1 (Mildest) to class 5 (most pronounced)
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4
Q

What are the types of cerebral palsy?

A

Spastic - most common
Athetoid - hyperkinesia, “stormy movement”
Ataxic - loss of orderly muscle co-ordination
Mixed

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5
Q

How is CP managed?

A

MDT approach. Splints, mobility aids.
Medical: Baclofen can help with the spasticity (beware withdrawal Sx from baclofen). Botox - paralyse muscles.
Orthopaedic surgery: Selective dorsal rhizotomy

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6
Q

Between what ages do febrile fits most commonly occur?

A

6 months - 6 years

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7
Q

Roughly what percentage of children get febrile fits?

A

3%

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8
Q

What features of a febrile fit make it atypical?

A

Longer than 15 mins
More than one in the same illness
Focal neurology

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9
Q

What is the recurrence rate of simple febrile convulsions?

A

~33%

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10
Q

What are some risk factors for developing epilepsy?

A

Atypical febrile seizure, abnormal/focal neurology or neurodevelopment, positive family history

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11
Q

What is the risk of developing epilepsy following a simple febrile convulsion with no risk factors?

A

1% (same as background risk)

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12
Q

What is the risk of developing epilepsy following a febrile convulsion with one risk factors?

A

2%

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13
Q

What is the risk of developing epilepsy following a febrile convulsion with two or more risk factors?

A

10%

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14
Q

What can trigger a typical absence seizure?

A

Hyperventilation

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15
Q

What are the features of juvenile myoclonic epilepsy?

A

Onset 8-26 years. More common in females. Get tonic-clonic seizures provoked by sleep deprivation, absence seizures, early morning myoclonic jerks of upper limbs. Flashing lights triggers attack in 40%.

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16
Q

What is the treatment of juvenile myoclonic epilepsy?

A

Lifelong anti-epileptic medication.

17
Q

What are the features of benign epilepsy with centro-temporal spikes aka Benign Rolandic epilepsy?

A

Onset 3-12 years. Peak 9 years. Get unilateral facial parasthesia with ipsilateral facial motor seizure. No LOC but often unable to speak and salivation. Last 1-2 mins. Seizures often at night.
Usually self-resolves by 13

18
Q

What triad is seen in West Syndrome?

A

Seizures (often infantile spasms), motor regression, Typical EEG pattern (hypsarrhythmia).

19
Q

What is the treatment for West syndrome?

A

Steroids, vigabatrin, ACTH

20
Q

What percentage of children with West syndrome have developmental delay and persistent seizures?

21
Q

What age group tends to be affected by infantile spasms seen in West syndrome?

A

1-6 month olds

22
Q

What are the features of tension-type headache?

A

Tight-band like pain, symmetrical. Gradual onset. Related to stress. May feel tense in muscles around back of head and neck

23
Q

What are the features of migraine without aura?

A

Lasts 1-72 hours. Commonly bilateral but may be unilateral. Pulsatile. GI Sx may co-occur. May be aggravated by physical activity and may be photo- and phonophobia

24
Q

What are the features of migraine with aura?

A

Frequent presence of premonitory symptoms. Usually lasts a few hours.

25
What are some red flag features of a headache?
Worse when lying down or with coughing/straining. Headache wakes the child. Associated confusion or persistent N+V. Change in personality/behaviour/educational performance
26
What is plagiocephaly?
Parallelogram shaped head due to pressure on the skull. Improves with age and mobility
27
What is the inheritance pattern on Duchenne's Muscular Dystrophy?
X-linked recessive disorder. | Abnormal/absent dystrophin protein
28
What condition is Gower's sign seen in?
Duchenne's Muscular Dystrophy
29
By what age do boys with DMD tend to no longer be ambulant due to progressive muscular atrophy?
10-14
30
What are the forms of spina bifida?
Spina bifida occulta - overlying skin lesion (tuft of hair/lipoma/birthmark/small dermal sinus) in lumbar region, there may be underlying cord tethering which with growth may cause bladder dysfunction Meningomyelocele (most common and severe form) - paralysed legs with sensory loss below level of lesion. Hip dislocation and leg deformities common. Hydrocephalus. Neuropathic bladder and bowel Meningocele - Rare and less serious. Swelling over back is covered by skin and contains no neural tissue