Neuro Flashcards
What are febrile convulsions and at what age?
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
Clinical features and types of febrile convulsions
- seizures are usually brief, lasting less than 5 minutes
- are most commonly tonic-clonic
Management of febrile convulsions during a seizure
Midazolam and diazepam or BDZPs
parents should be advised to call an ambulance if a febrile convulsion lasts >5 minutes
Ix = BLOOD GLUCOSE
Management of a febrile convulsions after a seizure
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
Duchenne Muscular Dystrophy features, investigations and prognosis
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
DMD management
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
Cerebral Palsy definition and causes
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
Manifestation of cerebral palsy
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%)
Classification of cerebral palsy
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
Ix of cerebral palsy
signs of cerebral palsy
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
Red flags for other neurological disorders rather than CP
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
Cerebral palsy management
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
What are some cerebellar signs? DANISH
- Dysdiadochokinesia/ dysmetria.
- Ataxia.
- Nystagmus.
- Intention tremor.
- Speech - slurred or scanning.
- Hypotonia
What is the criteria for immediate request for CT head scan of the head in children
- 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)
What part of the brain does dyskinetic CP affect?
basal ganglia and substantia nigra
What is the most common heart lesion associated with DMD?
Dilated cardiomyopathy
Headache epidemiology, which is the most common type in children
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
Criteria for paediatric migraine without aura but the International Headache Society (IHS)
Criteria for paediatric migraine without aura but the International Headache Society (IHS)
Mx of migraines in children
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
What is the second most common type of headache in children? how do we diagnose it
TTH aka tension-type headache
What is the second most common type of headache in children? how do we diagnose it
TTH aka tension-type headache
Mx for TTH
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.
What is spastic diplegia CP most commonly secondary to
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.
ADHD: DSM-V criteria
A/H persistent with an element of developmental delay
- <16 years = 6 features present
- >17 years = 5 features present
ADHD: DSM-V criteria
A/H persistent with an element of developmental delay, and psychological/social/educational impairment
- <16 years = 6 features present
- >17 years = 5 features present
ADHD epidemiology
- 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.
ADHD Mx
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
