Neuro Flashcards

1
Q

What are febrile convulsions and at what age?

A

Febrile convulsions are seizures provoked by fever in otherwise normal children. They typically occur between the ages of 6 months and 5 years and are seen in 3% of children.

Occurs in a viral infection when the temperature is rapidly rising

Some genetic predisposition, 30% will have further seizures

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2
Q

Clinical features and types of febrile convulsions

A
  • seizures are usually brief, lasting less than 5 minutes
  • are most commonly tonic-clonic
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3
Q

Management of febrile convulsions during a seizure

A

Midazolam and diazepam or BDZPs

parents should be advised to call an ambulance if a febrile convulsion lasts >5 minutes

Ix = BLOOD GLUCOSE

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4
Q

Management of a febrile convulsions after a seizure

A

Identify and manage the cause of the fever

Immediate hospital assessment by a paediatrician if:

If no apparent focus of infection, consider urgent hospital assessment for a period of observation

Referral to paediatrician or paediatric neurologist if neurodevelopment delay and/or signs of neurocutaneous syndrome or metabolic disorder

ALL OTHER CHILDREN CAN BE MANAGED AT HOME

  • regular antipyretics have not been shown to reduce the chance of a febrile seizure occurring
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5
Q

Duchenne Muscular Dystrophy features, investigations and prognosis

A

Duchenne muscular dystrophy is an X-linked recessive inherited disorder in the dystrophin genes required for normal muscular function.

Features

  • progressive proximal muscle weakness from 5 years = majority will be wheelchair-bound by puberty → nocturnal hypoxia
  • calf pseudohypertrophy → tight achilles tendons, tiptoe walking
  • Gower’s sign: child uses arms to stand up from a squatted position
  • 30% of patients have intellectual impairment

Investigation

  • raised creatinine kinase
  • GENETIC TESTING has now replaced muscle biopsy as the way to obtain a definitive diagnosis

Management

  • is largely supportive as unfortunately there is currently no effective treatment

Prognosis

  • most children cannot walk by the age of 12 years
  • patients typically survive to around the age of 25-30 years
  • associated with dilated cardiomyopathy
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6
Q

DMD management

A

hysiotherapy helps prevent contractures

Exercise and psychological support are necessary

Tendoachilles lengthening and scoliosis surgery may be required

Glucocorticoids (e.g. prednisolone) may help delay wheelchair dependence

Ataluren is a drug that restores dystrophin synthesis and has conditional licensing for patients 5 years and over

Dietician for gastric feeding indicated in some patients. Vitamin D and calcium supplementation may be necessary to prevent and treat bone fragility.

Weakness of intercostal muscles may lead to nocturnal hypoxia

  • This presents with daytime headache, irritability and loss of appetite

Overnight CPAP may be indicated for respiratory support

If the left ventricular ejection fraction drops, cardioprotective drugs (e.g. carvedilol) and left

ventricular assist devices may be considered

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7
Q

Cerebral Palsy definition and causes

A

Cerebral palsy may be defined as a disorder of movement and posture due to a non-progressive lesion of the motor pathways in the developing brain. It affects 2 in 1,000 live births and is the most common cause of major motor impairment.

Causes

  • antenatal (80%): e.g. cerebral malformation and congenital infection (rubella, toxoplasmosis, CMV)
  • intrapartum (10%): birth asphyxia/trauma
  • postnatal (10%): intraventricular haemorrhage, meningitis, head-trauma
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8
Q

Manifestation of cerebral palsy

A

Possible manifestations include:

  • abnormal tone early infancy
  • delayed motor milestones
  • abnormal gait
  • feeding difficulties.

Children with cerebral palsy often have associated non-motor problems such as:

  • learning difficulties (60%)
  • epilepsy (30%)
  • squints (30%)
  • hearing impairment (20%)
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9
Q

Classification of cerebral palsy

A

spastic (70%)

  • subtypes include hemiplegia (one side), diplegia (both side) or quadriplegia (all limbs, neck down)
  • increased tone resulting from damage to upper motor neurons

dyskinetic

  • caused by damage to the basal ganglia and the substantia nigra
  • athetoid movements and oro-motor problems

ataxic

  • caused by damage to the cerebellum with typical cerebellar signs

mixed

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10
Q

Ix of cerebral palsy

signs of cerebral palsy

A

Clinical diagnosis

MRI to assess the cause, take a hx

Signs of cerebral palsy:

For children at risk: provide clinical and developmental follow-up programme by an MDT for children up to 2 years

Possible early motor features of CP

  • Unusual fidgety movements or abnormality of movement (including asymmetry or paucity of movement)
  • Abnormalities of tone (includes hypotonia, spasticity or dystonia (fluctuating tone))
  • Abnormal motor developing (including late head control, rolling and crawling)
  • Feeding difficulties

Delayed motor milestones (correct for gestational age)

  • Not sitting by 8 months
    Not walking by 18 months
  • Hand preference before 1 year

Refer all children with persistent toe walking

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11
Q

Red flags for other neurological disorders rather than CP

A

Absence of RFs

FHx of progressive neurological disorder

Loss of already attained cognitive/developmental abilities

Development of unexpected focal neurological signs

MRI findings suggestive of progressive neurological disorder

MRI findings not in keeping with CP

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12
Q

Cerebral palsy management

A

MDT = paediatrician, nurse, physio, OT, SALT, dietetics, psychology (+ orthopaedics, orthotics, visual and hearing)

Optimise nutritional status

Manage saliva control

  • consider anticholinergics (e.g. glycopyrronium bromide, transdermal hyoscine hydrobromide), consider botulinum A injection into salivary glands

Low BMD

  • calcium and vit D
  • active movement/weight bearing programme or dietetic interventions

Paracetamol for pain, discomfort, distress

Sleep disturbance

  • optimise sleep hygiene
  • consider trial of melatonin

Visual impairment

  • refer for ophthalmological/orthoptic assessment

Hearing impairment

Learning disability and behavioural difficulties

GORD

Chronic constipation → laxatives

Epilepsy → anticonvulsants

Baclofen for muscle stiffness

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13
Q

What are some cerebellar signs? DANISH

A
  • Dysdiadochokinesia/ dysmetria.
  • Ataxia.
  • Nystagmus.
  • Intention tremor.
  • Speech - slurred or scanning.
  • Hypotonia
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14
Q

What is the criteria for immediate request for CT head scan of the head in children

A
  • Loss of consciousness lasting more than 5 minutes (witnessed)
  • Amnesia (antegrade or retrograde) lasting more than 5 minutes
  • Abnormal drowsiness
  • Three or more discrete episodes of vomiting
  • Clinical suspicion of non-accidental injury
  • Post-traumatic seizure but no history of epilepsy
  • GCS less than 14, or for a baby under 1 year GCS (paediatric) less than 15, on assessment in the emergency department
  • Suspicion of open or depressed skull injury or tense fontanelle
  • Any sign of basal skull fracture (haemotympanum, panda’ eyes, cerebrospinal fluid leakage from the ear or nose, Battle’s sign)
  • Focal neurological deficit
  • If under 1 year, presence of bruise, swelling or laceration of more than 5 cm on the head
  • Dangerous mechanism of injury (high-speed road traffic accident either as pedestrian, cyclist or vehicle occupant, fall from a height of greater than 3 m, high-speed injury from a projectile or an object)
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15
Q

What part of the brain does dyskinetic CP affect?

A

basal ganglia and substantia nigra

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16
Q

What is the most common heart lesion associated with DMD?

A

Dilated cardiomyopathy

17
Q

Headache epidemiology, which is the most common type in children

A

Epidemiology

  • up to 50 per cent of 7-year-olds and up to 80 per cent of 15-year-old have experienced at least one headache
  • equally as common in boys/girls until puberty then strong (3:1) female preponderance

MIGRAINES without aura is the most common cause of primary headache in children

18
Q

Criteria for paediatric migraine without aura but the International Headache Society (IHS)

A
18
Q

Criteria for paediatric migraine without aura but the International Headache Society (IHS)

A
19
Q

Mx of migraines in children

A

assess severity and frequency of attack and impact on QoL

headache diary = minimum 8 weeks to identify triggers

Acute mx (12-17 year olds):

Step 1: Simple analgesia (ibuprofen > paracetamol)

  • only consider aspirin if >16 (Risk of Reye’s syndrome)

Step 2: nasal sumatriptan

  • oral NOT for <18

Step 3: combination therapy with nasal triptan + NSAID/paracetamol

  • consider adding anti-emetic e.g. metoclopramide or prochlorperazine

Prophylactic tx

Offer topiramate or propranolol = specialist referral required

  • NOTE: topiramate has a risk of foetal malformations
20
Q

What is the second most common type of headache in children? how do we diagnose it

A

TTH aka tension-type headache

20
Q

What is the second most common type of headache in children? how do we diagnose it

A

TTH aka tension-type headache

21
Q

Mx for TTH

A

Reassure that this is not a concerning cause of headaches

Offer simple analgesia (paracetamol, ibuprofen, aspirin) for acute treatment

  • Do NOT offer aspirin to <16-year-olds due to risk of Reye’s syndrome
  • Do NOT offer opioids

Consider course of up to 10 sessions acupuncture over 5-8 weeks for prophylactic treatment of chronic tension type headache in over 12-year-olds.

22
Q

What is spastic diplegia CP most commonly secondary to

A

periventricular leukomalacia, a condition occurring more frequently in premature infants. Necrosis of the white matter in the fragile area surrounding the lateral ventricles causes upper motor neuron lesions resulting in the spasticity seen in this patient’s lower limbs.

23
Q

ADHD: DSM-V criteria

A

A/H persistent with an element of developmental delay

  • <16 years = 6 features present
  • >17 years = 5 features present
23
Q

ADHD: DSM-V criteria

A

A/H persistent with an element of developmental delay, and psychological/social/educational impairment

  • <16 years = 6 features present
  • >17 years = 5 features present
24
Q

ADHD epidemiology

A
  • ADHD has a UK prevalence of 2.4%, about twice that of autism, and is more common in boys than in girls (M:F 4:1);
  • Most children are diagnosed between the ages of 3 and 7;
  • There is a possible genetic component.
25
Q

ADHD Mx

A

MDT: paediatrician, psychiatrist, ADHD nurses, MH and LD trusts, CAMHS, parent groups, social care, school/college

  • watchful-waiting 10 weeks
  • group/indivualised-based ADHD-focused support for parents = education, parenting strategies, school liaison with consent, parents and carers
  • refer to specialist (secondary care aka developmental paediatrician or CAMHS) if problems severe/persist

Children <5 years

  • ADHD-focused group parent-training programmes (10-16 meetings, groups of 10-12)
  • if fails → seek advice from specialist ADHD service (do NOT offer medication unless under instruction from this service)

Children >5 years (medication if persist/significant impairment)

  • Baseline physical state and ECG before starting medication
  • Yearly off-medication trials recommended
  • 1st line = methylphenidate 6wk
  • 2nd line = lisdexamphetamine (s/e: abdominal pain, nausea, dyspepsia) MONITOR height + weight every 6 months
  • 3rd line = if responding but not tolerating → dexamphetamine
  • 4th line = atomoxetine/guanfacine
  • Other medications = clonidine for sleep/rages/tics, antipsychotics for aggression and irritability

CBT for children/teens if significant impairment in = social skills, problem solving, self-control, active listening, dealing with expressing feelings

Monitoring medication:

  • sx rating scales (e.g. Conner’s)
  • height/6 months, weight/3 months (if affected significant → tx holiday over school holidays)
  • HR + BP every 6 months
  • monitor tic development after taking stimulants
  • monitor for sexual dysfunction, seizures, sleep disturbance, worsening behaviour

Diet:

  • Balanced diet and regular exercise
  • food diary (explore foods that influence behaviour)
  • consider dietician referral