Paediatric Neurology Flashcards
What should be examined when investigating headaches?
Growth parameters, OFC, BP Sinuses, teeth, visual acuity Fundoscopy Visual fields (craniopharyngioma) Cranial bruit Focal neurological signs cognitive and emotional status
What are the features to examine when taking a migraine history?
Associated abdominal pain, nausea, vomiting
Focal symptoms/ signs before, during, after, attack; visual disturbance, paraesthesia, weakness
Pallor
Aggravated by bright light/ noise
Relation to fatigue/stress
Helped by sleep/rest/ dark, quiet room
Family history
What are the features that point to raised intracranial pressure?
Aggravated by activities that raise ICP (coughing, straining at school, bending)
Woken from sleep by headache
What features point to analgesic overuse headache?
Headache is back before allowed to sue another dose
Paracetamol/NSAIDs
particular problem with compound analgesics (cocodamol)
What are the indications for neuroimaging?
Features of cerebellar dysfunction Features of raised ICP New focal neurological deficit Seizure, esp focal Personality change Unexplained deterioration of school work
What is the management for migraines?
Effective pain relief, triptans (acute attack)
Pizotifen, Propanolol, amytriptyline, topiramate, valproate (preventative)
What is the management for tension type headaches?
Simple analgesia (acute attack) Amitriptyline (Prevention)
What is a convulsion?
Seizure where there is no prominent motor activity
What is an epileptic seizure?
An abnormal excessive hyper synchronous discharge from a group of neurons
What can cause non-epileptic seizures in children?
Acute symptomatic seizures (hypoxia ischaemia, hypoglycaemia, infection, trauma) Reflex anoxic seizure Syncope Parasomnias Behavioural stereotypies Psychogenic seizures
What is a febrile convulsion?
Event occurring in infancy, associated with fever but without evidence of intracranial infection or defined cause for the seizure
How are epileptic seizures chemically triggered?
Decreased inhibition (gama-amino-butyric acid, GABA) Excessive excitation (glutamate and aspartate) Excessive influx of Na and Ca ions
What are the features of a generalised epileptic seizures?
Involves both sides of cerebral hemisphere
Loss of consciousness and awareness/ gap in memory from the start
No warning
Different types (absence, myoclonic, tonic clonic)
What are the features of a focal (partial) epileptic seizure?
Involves one cortical lobe or part of it, can be preceded by aura
Simple partial: no LOC, involving one side/part of body
Complex partial: Associated with LOC or awareness
How is a diagnosis of epilepsy made in children?
History Video recording of event ECG in convulsive patients EEG MRI brain Metabolic tests
How is epilepsy managed in children?
Anti-epileptic drugs
Vagus nerve stimulation
Surgery
What anti-epileptic drugs can be used in children?
Sodium valproate
Carbamazapine
Lamotrigine
When should you suspect a neuromuscular disorder in a child?
Baby "floppy" from birth Slips from hands Paucity of limb movements Alert, but less motor activity Delayed motor milestones Able to walk but frequent falls
What are myotonias?
Delayed relaxation of skeletal muscle after voluntary contraction or electrical stimulation
What are myopathies?
Muscle weakness due to dysfunction of the muscle fibres
What are common muscular dystrophies?
Duchenne MD
Becker MD
Limb girdle MD
What are the clinical signs of Duchenne MD?
Delayed gross motor skills Symmetrical proximal weakness (waddling gait, calf hypertrophy, Gower's sign +ve) Elevated creatinine kinase levels Cardiomyopathy Respiratory involvement in teens
What are the features of myopathies?
Developmental delay
Hypotonia of variable degree
Proximal and distal muscle weakness
Creatinine kinase normal to high
What are the causes of myopathies?
Congenital Metabolic (glycogen storage, mitochondrial) Infection Drugs/toxins Autoimmune
What are the different types of myotonias?
Myotonic dystrophy (AD) Myotonia congenita (AD) Parammyotonia congenita (AD/AR)
What are the features of myotonias?
Motor developmental delay Infantile hypotonia and developmental delay Muscle stiffness and cramps Endocrinopathies Cardiac dysarrhythmias
What are the features of hereditary neuropathies?
Symmetrical distal polyneuropathy
Both motor and sensory manifestations
Clumsiness, weakness, loss of fine motor control
Distal muscle wasting and weakness, foot drop, wrist drop, absent DTR
What is the management for hereditary motor sensory neuropathy (HMSN)?
Supportive
Gabapentin