Paed Neuro Flashcards
Core Neuro Topics
-Seizures
-Headaches
-Neuromuscular disorders
-Investigations (MRI/EEG etc)
-Cerebral palsy
-Tic disorder and co-morbidities
-Delayed development
-Genetic disorders
History taking
-Consanguinity , parents age and health, Family history
-Pregnancy (folic acid, nutrition, infection, screening, scan, foetal movement)
-Delivery and complication
-Perinatal adaptations, feeding
-Early development
-Immunisations
-Early CNS infections/Brain injury
-School progress
-Mental health
Paed Neuro Exam
-Age appropriate neurological and systemic examination, what is their baseline (neurodisability)
Hopping, walking on toes, developmental milestones
-Head circumference (if abnormal check parents OFC): growth charts
-Ophthalmological examination
-Neuro cutaneous markers (Neurofibromatosis: axillary freckling, Tuberous Sclerosis)
-Dysmorphic features/Syndromes
-Mental state and level of consciousness
-General
-Meningeal signs
-Cranial
-Motor system
-Cerebellar
-Autonomic
Paed Neuro investigations
-Neuroimaging (CT/MRI/MRS )
-CSF studies (infection, inflammation, metabolic disorders)
-Genetic studies (microarray, genetic panel, specific genes)
-Metabolic (amino acid, organic acid, MPS)
-TFT, CK
-Neurophysiological (EEG, VEP, ERG, Nerve conduction, EMG)
-Hearing test
Causes of fits in children
The commonest cause of fits in children is a ‘febrile fit’ or ‘febrile convulsion’. However other causes of fits must be excluded including metabolic causes (such as low glucose), head injury, and CNS infection. It is important to understand that in some of these instances the child will also have a high fever, but the fever was not the cause of their fit.
-Epilepsy (4/1000): primary and secondary (cerebral palsy, structural brain lesions, syndromes)
-Child abuse
-Encephalitis/ meningitis
-Poisoning
-Head injury
-Reflex anoxic seizure
-Metabolic (sodium, calcium, glucose)
Differential diagnosis of epilepsy
-Syncope/faints
-Cyanotic(blue) breath-holding Attacks
-Pallid Syncopal Attacks(Reflex Anoxic)
-Delirium
-Night Terrors
-Migraine
-Cardiac Dysrhythmias: prolonged QT
-Narcolepsy
Neurocutaneous syndromes
-Sturge-Webber
=Reddish discolouration of skin on one side of face
Epilepsy syndromes
-Seizure type
-Age of onset
=New-born: benign familial neonatal convulsions/ pyridoxine BG dependent seizures
=Infancy: Infantile spasms
=Pre-school: Lennox-Gastaut
=School age: typical absence epilepsy
=Adolescence: juvenile myoclonic epilepsy
-Seizure type
-Neurodevelopmental findings (neurodevelopmental disorder)
-Family history
-Complex febrile seizures
-EEG(ictal AND interictal)
Overview of Infantile spasms/ West’s syndrome
-Brief spasms beginning in the first few months of life
-P: flexion of head, trunk, limbs → extension of arms (Salaam attack); last 1-2 secs, repeat up to 50 times, progressive mental handicap
-I: EEG: hypsarrhythmia, usually secondary to serious neurological abnormality (e.g. tuberous sclerosis, encephalitis, birth asphyxia) or may be idiopathic
-M: possible treatments include vigabatrin and steroids, has a poor prognosis
Overview of typical absence seizures (petit mal)
-Onset 4-8 yrs
-Duration few-30 secs; no warning, quick recovery; often many per day
-EEG: 3Hz generalized, symmetrical
-Sodium valproate, ethosuximide
-Good prognosis: 90-95% become seizure free in adolescence
Overview of Lennox-Gastaut syndrome
-May be an extension of infantile spasms
-Onset 1-5 yrs
-P: atypical absences, falls, jerks, 90% moderate-severe mental handicap
-I: EEG: slow spike
-M: ketogenic diet may help
Overview of Juvenile Myoclonic Epilepsy
-Typical onset is in the teenage years, more common in girls
-P: infrequent generalized seizures, often in morning//following sleep deprivation, daytime absences, sudden, shock-like myoclonic seizure (these may develop before seizures)
-M: usually good response to sodium valproate
Overview of Benign Rolandic Epilepsy
-Benign Rolandic epilepsy is a form of childhood epilepsy that typically occurs between the age of 4 and 12 years.
-P: seizures characteristically occur at night, seizures are typically partial (e.g. paraesthesia affecting the face) but secondary generalisation may occur (i.e. parents may only report tonic-clonic movements), the child is otherwise normal
-I: EEG characteristically shows centrotemporal spikes
The prognosis is excellent, with seizures stopping by adolescence
Overview of febrile convulsions
-Usually in toddlers, not usual for first after 3 years (3 months to 5 years): 3% children
-Usually a family history
=Simple 70%: <15 minutes, generalised, typically no recurrence within 24 hours, should be complete recovery within hour
=Complex 30%: 15-30 minutes, focal seizure, may have repeat seizures within 24 hours
-P: Usually stop before 5 minutes, usually generalised tonic-clonic, usually only occur early in a viral infection as temp rises rapidly
-I: ABCDE, glucose
-M:
=First seizure or complex: admitted to paediatrics
=If a child has a febrile convulsion you will need to find where the fever is coming from, and treat that illness. If it is fever due to a viral illness the treatment will be supportive.
=Treat a fit with anticonvulsant medication if it continues at 5 minutes; parents phone ambulance
=Regular antipyretics not shown to reduce chance o febrile seizure occurring
=If recurrent convulsions occur then benzodiazepine rescue medication may be considered (specialist advice, rectal diazepam/ buccal midazolam)
-Prognosis: 1/3 risk of further convulsion depending on age of onset <18 months, fever <39, shorter duration of fever before seizure, family history
-Complications
=Brain damage after 30 minutes (status epilepticus)
=Injuries/ death
=Educational/ psychosocial/ treatment related
History of fits
-What were they doing when the fit started?
-How did the fit start?
-Was there loss of consciousness?
-Were bladder/bowels opened?
-What the movements were like, and in which parts of the body?
-Was there eye rolling or staring, eyes open or closed, eyelid flutter, jerking or deviation?
-Was there tongue biting/ facial twitching/ body stiffness/ chaotic or rhythmic jerking of limbs?
-What was the tone?
-What was the colour (pallor or cyanosis)?
-How long did it last?
-How did it stop? (was it self resolving?) Duration of recovery
-Were they sleepy afterwards (how long was this post-ictal phase?)
-Was there headache afterwards?
-Was there any injury sustained?
-After a description of the fit you need to ask about what happened before the fit:
=Was there a fever?
=Has the child been unwell recently?
=What is the past medical history?
=What is the birth history?
=Has the child been developing normally?
=Did the child complain of anything prior to the fit?
=Were they exercising? (cardiac syncope may come suddenly at rest, or during exercise, Wolff Parkinson White)
=Any warning signs? Awareness of surroundings, aura
-With fever
=Do not assume a ‘febrile convulsion’. Remember that CNS infections (meningitis and encephalitis) also cause fever and fits. Check for signs of meningism / bulging fontanelle. Check for personality or behaviour change.
=Even in the case of a febrile convulsion, remember to make sure that you find the cause of the fever and assess the child for serious bacterial infection. This includes a thorough examination and urine check at the least. Blood tests may be required.
=Children who have epilepsy may also have their fit threshold lowered by fevers. We do not call these febrile convulsions.
Established epilepsy: Fit pattern and fit nature
Examples of non-seizure events
-Faints
-Cardiac syncope: rapid recovery once flat, not younger children
Fit examination
-Generalised fits
=Tonic-clonic fits are the most common and involve whole body jerks (the tonic phase is the initial stiffening, the clonic phase is the jerking)
=The eyes are usually rolled upwards, the jaw is clenched and the child is unresponsive, salivation, sweating, blue: grand mal
=Petit mal (absence seizure)
-Focal fits
=The child is awake and the fit only affects a part of the body, partial seizure if aware
=Neuroimaging may be required for a first focal fit (CT scan)
=Sometimes a generalised fit starts as a focal fit.
-After fit
=Check Airway (obstructed by unconsciousness?), Breathing depressed from medication?), Circulation (Dehydration, sweating, sepsis) and Disability (watch for airway problems and slow breathing post-ictally)
=As part of D (disability) check the AVPU score (P/U is common initially post-ictally) and the blood glucose. Full neuro exam when awake
=Children may have a headache or be agitated or drowsy: paracetamol
=Examine for complications of the fit and the cause of the fit
=Check for urine infections after febrile fits
Fit Investigation
-CT or MRI?
-1-1.5% of children with epilepsy have brain tumour (brain tumours RARELY present with seizures)
-Imaging indicated in :
=Focal seizures
=Neuro deficit or not responding to treatment
=Early onset