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Child health > Neurology - 1 > Flashcards

Neurology - 1 Flashcards

1
Q

What are risk factors for cerebral palsy?

A 
Maternal illness during pregnancy: 
DM
Infection - rubella, herpes, CMV, toxoplasmosis
Fever
Hypothyroidism
Seizures
Proteinuria

Rh or ABO incompability
Exposure to toxins such as methyl mercury in thermometers. Drugs, alcohol, tobacco.

Birth RF: pre-term (before 37weeks), low birth weight
Multiple pregnancy
Respiratory distress syndrome at birth or heart problems

In infancy:
Kernicterus
Postnatal meningitis, encephalitis
NAI
Symptomatic hypoglycaemia
Hyperbilirubinaemia

Trauma either during labour or after is one of the commonest causes

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2
Q

What are the types of cerebral palsy?

A

Spastic cerebral palsy

Dyskinetic cerebral palsy

Ataxic (hypotonic) cerebral palsy

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3
Q

Which factors may be indicative of cerebral palsy in the neonatal period?

A

There is usually abnormal tone, delayed development. Feeding difficulties (oromotor incoordination, slow feeding, gagging, vomiting).
Abnormal gait once able to walk
Asymmetrical hand function before 12 months

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4
Q

What are the characteristics of spastic cerebral palsy?

A

Damage to UMN (corticospinal and pyramidal tract)

Persistently increased limb tone (spasticity) which is velocity dependent (may be neonatally present)
Brisk deep tendon reflexes
Extensor plantar response

3 types:
Hemiplegia
Quadriplegia
Diplegia

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5
Q

Describe Hemiplegic spastic cerebral palsy

A

unilateral involvement of arm and leg (NOT face)
Arm > leg

At 4-12 months:
Fisting of the affected hand, flexed arm, pronated forearm.
Asymmetrical reaching/function
Then, toe-heel gait

May initially have lower tone, but then becomes hypertonic.

Sometimes caused by neonatal stroke

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6
Q

Describe quadriplegic spastic cerebral palsy

A

All limbs affected, often severely.

Extensor posturing (opisothonus)
Poor head control

Often: seizures, microcephaly, >moderate intellectual impairment

Possible history of perinatal hypoxic-ischaemic encephalopathy

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7
Q

Describe diplegic spastic CP

A

All 4 limbs affected, but legs much more than hands (almost normal - noticed with functional hand use)
Walk abnormal

associated with preterm birth due to periventricular brain damage

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8
Q

What are the characteristics of Dyskinetic CP?

A

Involuntary, uncontrolled movements. Muscle tone variable. Primitive motor reflexes pre-dominate.

Chorea - irregular, sudden, brief, non-repetitive movements

Dystonia - simultaneous contraction of agonist and antagonist muscles of the trunk and proximal muscles, giving a twitching appearance

Athetosis - slow writhing movements distally (eg. fanning of fingers)

Often unaffected mentally.
poor motor development in infancy
floppiness in infancy

involuntary movements after 1st year of life

Caused by kernicterus and hypoxic-ischaemic encephalopathy at term (most common)

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9
Q

What are the characteristics of ataxic (hypotonic CP)?

A

Most are genetic and thus symmetric. Sometimes acquired brain injury, and thus ipsilateral.

Early trunk and limb hypotonia.
Poor balance.
Delayed motor development.

Later, incoordinate movement.
Intention tremor.
Ataxic gait

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10
Q

MDT involvement in CP

A

Primary therapist does 90% of the therapy

Paediatrician
Health visitor
Social worker
Physio (movement and coordination)

Orthotis (specialized in the use of devices to correct deformities)

OT - skills and abilities

Teacher specialized in visual impairment
Orthopaedic surgeon
Neurologist

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11
Q

What are important parts of long-term management of posture and movement in CP

A

Posture and movement:
Improve symmetry, joint ranges, muscle length and power

-stretching exercises
-Orthoses: ankle foot
Wheelchair for mobility
Sleeping and standing systems
Botox to gastrocnemius
Surgery is last resort

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12
Q

Factors in long-term management of CP

A

Posture and movement
Communication (speech therapy and aids)
Independece with a tailored education program
Cognition and learning support
General medical: watch for seizures, constipation and behavioral/psychiatric disturbance

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13
Q

Aim of physio in CP

A

Encourage movement and improve capability (eg. walking aid or orthotics)

Build of strength and prevent weakening of unused muscles

Prevent muscles shortening and losing their normal range of movement (contracture)

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14
Q

Which medications are used in CP

A

Diazepam (short term for muscle pain/stiffness)

Baclofen (long-term for muscle pain and stiffness) - pump which is implanted under skin and connected to spinal cord

Botox - targets specific muscles that are stiff - lasts 3-6 months

Dyskinetic CP medications (trihexyphenidyl, Gabapentin, Clonidine)

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15
Q

Orthopaedic surgery in CP

A

Soft tissue - tendon release or muscle lengthening

Bone surgery - eg. hip dislocation

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16
Q

Generalized seizure characteristics

A

Always loss of consciousness
No warning
Symmetrical seizure
Bilaterally synchronous seizure discharge on EEG

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17
Q

What are absent seizures

A

Transient loss of consciousness
Abrupt onset and termination

No motor phenomena, apart from some flickering of the eyelids

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18
Q

What are myoclonic seizures

A

Brief, often repetitive jerking movements of the limbs, neck or trunk

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19
Q

Tonic seizures

A

Generalised increase in tone

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20
Q

Describe tonic-clonic seizures

A

Rhythmical contraction of muscle groups, following the tonic phase

In tonic (rigid) - child falls to the ground. Breathing stops - cyanosis.

Clonic. Jerking of limbs. Irregular breathing. Cyanosis persists and saliva may accumulate in the mouth.
Biting of tongue.
Incontinence of urine.

Seizure usually lasts seconds-minutes. Followed by unconscious or deep sleep for several hours.

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21
Q

Describe atonic seizures

A

Often with myoclonic jerk.

Transient loss of muscle tone with a sudden fall to the floor

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22
Q

Describe focal seizures

A

Begin in one hemisphere
Aura may be present which reflects site of origin
There may or may not be a change in consciousness

Frontal - motor phenomena
Temporal - auditory or sensory phenomena
Occipital - positive or negative visual phenomena
Parietal - contralateral altered sensation (dysasthesia)

23
Q

What is status epilepticus

A

Convulsive with tonic/clonic movements or it can be non-cpnvulsive with impairment of consciousness and often subtle twitching

Technically, a seizure lasting >30 minutes
Or repeated seizures lasting >30 minutes without recovery of consciousness

24
Q

Which syndromes are associated with epilepsy?

A 
West syndrome
Lennox-Gastaut syndrome
Childhood absence epilepsy
Benign epilepsy
Early onset benign childhood occipital epilepsy
Juvenile myoclonic epilepsy
25
Q

What is West syndrome

A

Age 4-6 months

EEG shows hypsarrhythmia (chaotic bakcground of slw wave acitivity with sharp mulfitfocal components)

Violent flexor spasms of the head, trunk and limbs followed by extension of the arms

Spasms occur for 1-2 seconds and repeat 20-30 times

26
Q

What is Lennox-Gastaut syndrome

A

Age 1-3 years

Mostly drop attacks and atypical absences

27
Q

What is childhood absence epilepsy

A

Age 4-12 years

EEG shows 3/second spike and wave discharge which is bilaterally syncrhonous

28
Q

What is benign epilepsy

A

Tonic clonic seizures in sleep or simple focal seizures with awareness of abnormal feelings

EEG shows focal sharp waves

29
Q

What is early onset benign childhood occipital epilepsy

A

Periods of unresponsiveness in young children and hallucinations/visual disturbance in older children
EEG shows occipital discharge

30
Q

What is juvenile myoclonic epilepsy

A

Myoclonic, but generalised tonic-clonic seizures or absences may also occur

characteristic EEG

31
Q

What things can be detected on EEG

A

Asymmetry or slowing - suggest underlying structural abnormality

Neuronal hyperexcitability - sharp waves or spike-wave complexes

However, children with epilepsy may have normal EEG and vice versa

Seizure needs to be captured with EEG - otherwise it is just supportive evidence

32
Q

When is structural imaging indicated in epilepsy?

A

MRI
CT

If there are neurological signs between seizures

If seizures are focal

Can identify tumour, vascular lesion or area of sclerosis

33
Q

When are functional scans used in epilepsy?

A

Detect area of abnormal metabolism suggestive of seizure foci

PET scan
SPECT
They use isotopes and ligands taken up by metabolically active cells

Both can be used between seizures to detect areas of hypometabolism in epileptogenic lesions

34
Q

First line investigation for suspected epilepsy

A

EEG

35
Q

Role of genetic studies in epilepsy

A

Increasing
Certain epilepsy syndromes are due to genetic deletions causing abnormalities of sodium and other ion channel - eg. SCN1A mutation in severe myoclonic epilepsy

36
Q

Principles of antiepileptic drug use

A

Not all seizures require AED therapy and treatment should be based on seizure type, frequency and the social and education circumstances

Choose the appropriate drug for the seizure

Monotherapy at the minimum dose is desired

All AEDs have unwanted side effects that need discussing

Drug levels are not measured routinely

Children with prolonged seizures are given rescue therapy (usually rectal or buccal diazepam)

AED can usually be discontinued after 2 seizure free years

37
Q

First line in tonic-clonic

A

Valproate

Carbamazepine

38
Q

First line in Absence

A

valproate

Ethosuximide

39
Q

First line in myoclonic

A

Valprote

40
Q

First line in focal seizures

A

Carbamazepine
Valproate
Lamotrigine (most effective)

41
Q

Treatments in epilepsy that are not AED

A

Ketogenic diet may help some children

Vagal nerve stimulation - external programmable wire or magnet

Surgery - last resort, only if localised with useful EEG and MRI findings

42
Q

Side effects of valproate

A

Weight gain
Hair loss

Rare idiosyncratic liver failure

43
Q

Side effects of carbamazepine

A 
Rash
Neutropenia
Hyponatraemia
Ataxia
Liver enzyme induction
44
Q

Side effects of lamotrigine

A

Rash

45
Q

Side effects of Ethosuximide

A

Nausea and vomiting

46
Q

Side effects of Gabapentin

A

Insomnia

47
Q

What is SUDEP

A

Sudden unexpected death in epilepsy

Occurs in very small proportion

Cause often unknown: not due to injury, drowning or prolonged seizure causing hypoxia

48
Q

In which epilepsy is SUDEP commonest and what can be done

A

Generalised tonic-clonic
Especially in young adults

RF:
Poor seizure control
Seizures in sleep

Try to minimize through medication and surgery

49
Q

How often do febrile seizures occur? When

A

4% of children

Usually between 6 months and 6 years

Genetic predisposition: 10% risk if first-degree relative had febrile seizures

They usually occur in viral illness when the fever is rapidly rising

50
Q

Management of febrile seizure

A

Safety - move any danger away
Assistance - call for help
Treatment - if >10 min, treat for status epilepticus. Afterwards, assess for source of fever.
Meningitis? consider if stiff neck, extreme lethargy post seizure, abundant vomiting or less than 12 months old

Seizure prevention - give early anti-pyretics in early febrile illness and get expert advice if seizure lasts >10 minutes

51
Q

First aid advice in febrile seizure

A

Place in recovery position on a soft surface
Place in a safe location, away from objects that could cause injury

Stay with child
Call for help if seizure lasts >5 min

52
Q

Relationship between febrile seizures and convulsions - aka prognosis

A

Temperature may be >39°C
Seizure usually during first days of fever

Children prone to febrile convulsions are not considered to have epilepsy (up to 98% will not develop epilepsy)

25% recurrence risk in the next 12 months

Vast majority of febrile convulsions are harmless: bad prognostic factors for subsequent epilepsy are:
lengthy seizure
recur in 24 hrs
Neurological abnormalities

53
Q

Categorise febrile seizures

A

simple (typical): generalised tonic-clonic activity lasting <15 minutes without associated fever

Complex febriles seizure (atypical): 15% of cases - focal seizure activity, prolonged seizure (>15min) or multiple seizures within 1 day

Child health (51 decks)

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