Neurology Flashcards
What is cerebral palsy?
Disorder of movement and posture due to non-progressive lesion of motor pathways in developing brain.
Causes of cerebral palsy?
Antenatal (80%) - congenital infection (TORCH - toxoplasmosis, rubella, CMV, herpes), cerebral malformation, metabolic disorders, teratogenic drugs, maternal seizures, pre eclampsia, maternal trauma.
Intrapartum (10%) - birth asphyxia/trauma (cord around neck, shoulder dystocia etc), group B streptococcus.
Postnatal (10%) - bleeds (IVH), meningitis and other infections, head trauma and other injuries, tumours.
Features of cerebral palsy?
Could be completely wheelchair bound, or only slight coordination and mobility problems.
Failure to meet milestones.
Increased or decreased tone generally or in specific limbs.
Problems with coordination, speech or walking.
Hand dominance earlier than 1 year (sign of other neurological conditions too).
Feeding or swallowing problems - due to lack of oromotor coordination
Learning difficulties (60%).
Associated problems in cerebral palsy?
Learning difficulties (60%) Epilepsy (30%) Squints (30%) Hearing impairment (20%).
Classification of cerebral palsy?
Spastic (70%) -
Damage to pyramidal pathways causes increased limb tone (spasticity), brisk deep tendon reflexes, extensor plantar responses .
Spastic further classified into - hemiplegia, diplegia, monoplegia, quadriplegia.
Dyskinetic -
Damage to basal ganglia or extrapyramidal pathways. Hypotonia, delayed motor development, abnormal involuntary movements.
Ataxic -
Damage to cerebellum or its pathways, causing early hypotonia with poor balance, uncoordinated movements and delayed motor development.
Mixed type.
Management of cerebral palsy?
MDT
Treat spasticity with physiotherapy, oral diazepam, oral/intrathecal baclofen, botulinum toxin A, orthopaedic surgery, dorsal rhizotomy.
Anticonvulsants and analgesia as required.
Complications of cerebral palsy?
Respiratory - aspiration, recurrent pneumonia
GI - failure to thrive, feeding difficulties, GORD, constipation
MSK - delayed motor milestones, contractures, scoliosis.
Neurological - learning difficulties, speech and language problems, hearing and visual impairment, seizures.
Features and management of childhood absence seizures?
Onset 4-8 years
Duration few-30 seconds, no warning, quick recovery, often many per day.
EEG - 3Hz generalised, symmetrical.
Sodium valproate, ethosuximide - first line.
What is juvenile myoclonic seizures and features?
Onset - teens F>M
Infrequent generalised seizures, often in morning.
Daytime absences.
Sudden, shock like myoclonic seizures.
Good response to sodium valproate.
What is west syndrome (infantile spasms) and treatment?
Brief spasms beginning in first few (4-6) months of life - M>F.
Flexion of head, trunk, limbs -> extension of arms (Salaam attack), lasts 1-2 seconds, repeat up to 50 times.
Progressive mental handicap.
EEG - hypsarrhythmia.
2nd to serious neurological abnormalities - eg encephalitis, birth asphyxia, or may be cryptogenic.
Poor prognisis
Vigabatrin/steroids to treat.
What is Lennox-Gustaut syndrome? Treatment?
Extension of infantile spasms. Onset 1-5 years Atypical absences, falls, jerks. 90% moderate-severe mental handicap. EEG - slow spike
Ketogenic diet may help.
What is benign rolandic epilepsy?
Most common in childhood, M>F.
Parasthesia (eg unilateral face), usually on waking up.
Causes of paediatric focal epilepsy?
CNS infection Head trauma Arteriovenous malformations Tumours Cortical dysgenesis Hypoxic ischaemic encephalopathy
Diagnosis of epilepsy?
Careful history
EEG
MRI brain - if focal onset seizures, seizures not responding to first-line treatment, focal neurological deficit, children <2 years with afebrile convulsions.
Management of epilepsy?
AEDs are generally started after second epileptic seizure.
1st line for tonic-clonic - carbamazepine, lamotrigine, sodium valproate.
1st line for absence seizures - ethosuximide, lamotrigine, sodium valproate.
Monotherapy should be used where possible.
What are febrile convulsions? Features?
Seizures provoked by fever in otherwise healthy children. Typically occurs between 6 months and 5 years.
Occurs early in viral infection as temp rises rapidly.
Seizures are usually brief, lasting less than 5 minutes.
Most commonly tonic-clonic.
Types of febrile convulsions?
Simple - <15 minutes generalised seizures, no recurrence in 24 hours, total recovery in 1 hour.
Complex - 15-30 minutes focal seizure, may have repeat seizures within 24 hours.
Febrile status epilepticus - >30 minutes.
Management of febrile convulsions?
Admit to paediatrics if have had first seizure or any other features of complex seizure.
Acute management:
- monitor duration of seizure
- protect child from injuries
- once seizure has stopped, check airway and put in recovery position
- observe child until they have recovered.
If seizures last >5 minutes - rectal diazepam or buccal midazolam. May repeat after 10 minutes if not stopped.
Prognosis of febrile convulsions?
1/3rd get further febrile convulsions.
Regular antipyretics do not reduce chance of febrile seizures.
Complex febrile seizures are a risk factor for developing epilepsy.
What are breath-holding attacks?
Typically occurs in toddler’s, provoked by temper, frustration or strong emotion.
Screaming toddler holds their breath, leading to cyanosis and potentially LOC.
Rapid recovery.
What are reflex anoxic seizures?
Increased vagal tone whereby there is brief paroxysmal episode of asystole triggered by pain or emotion.
More common in infants up to 2 years.
Children usually grow out of it.
Infant or toddler becomes pale and loses consciousness.
Subsequent hypoxia may cause tonic-clonic seizure.
Congenital causes of floppy infant?
Achondroplasia ASD CHARGE syndrome Down's syndrome Ehlers-Danlos Fragile X Marfans Neurofibromatosis Patau syndrome Cerebral palsy.
Acquired causes of floppy infant?
Genetic - muscular dystrophy, spinal muscular atrophy.
Infections - GB, encephalitis,s meningitis, sepsis, poliomyelitis.
Autoimmunity - MG, coeliac disease
Metabolic - Rickets
Neurological - brain injury, LMN lesions, UMN lesions.
Misc - CNS lesions, hypothyroidism, bento withdrawal.
Symptoms of floppy infant?
Reduced muscle tone and strength Hypermobile, hyperflexible joints Drooling and speech difficulties Motor skills delay Poor reflexes Reduced activity tolerance Feeding difficulties Uncontrollable head
