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
Diagnosis of floppy infant?
CT, MRI, EEG
Genetic testing and karyotyping
Muscle or nerve biopsy
Blood tests.
Management of floppy infant?
No cure
Supportive care - SALT, OT, orthotics.
Causes of acute ataxia (<72 hours)?
Post infections Toxins Tumours Trauma Metabolic Vascular (stroke) Immune Conversion disorder
Causes of chronic ataxia?
Brain tumours
Hereditary ataxias:
- Friedrichs ataxia - most common. Wheelchair needed after 10-20 years.
Ataxia telangiectasia - rare type. Increased risk of ALL or lymphoma. Live until 19-25.
Causes of episodic/recurrent ataxia?
Toxin ingestion Basilar artery migraine Seizure disorder Metabolic Hydrocephalus Metabolic Nutritional Congenital malformation
Management of ataxia?
Treat symptoms:
- MDT
Baclofen or botulinum toxin can be used for muscle spasms.
Treat bladder problems
Eye problems
Fatigue, nerve pain, erectile dysfunction, cardiomyopathy, depression.
Features and management of intraventricular haemorrhage?
Blood within ventricular system - due to prematurity of periventricular vasculature, causing bleeding from choroid plexus.
CT head - hyper dense within dark CSF spaces within ventricles.
Increased risk of obstructive hydrocephalus -> requires surgical CSF diversion.
Neonatal - occurs in first 72 hours after birth.
Management - largely supportive.
- Intraventricular thrombolysis
- Hydrocephalus and rising ICP is indication for shunting.
Features of shaken baby syndrome?
Subdural haematoma
Retinal haemorrages
Encephalopathy.
What is spina bifida?
Failure of fusion of vertebral arches during embryonic development -> incomplete closure of spine and membranes around spinal cord.
Folic acid prevents this condition and is taken preconception and during first 12 weeks.
What are the 3 types of spina bifida? Features of each?
Spina bifida occulta:
- Dorsal vertebral arch fails to fuse
- Overlying skin abnormality, such as tuft of hair or small dermal sinus.
- Tethering of cord may cause neurological deficits with growth.
Meningocele:
- CSF filled cystic protrusion with intact overlying skin
No neurological deficit or hydrocephalus
- Surgically excised at around 3 months
Myelomeningocele:
- Open herniation of cord and meninges, often with leakage of CSF.
- Neurological deficits - motor and sensory loss of lower limbs, neuropathic bladder and bowel.
- Scoliosis and hydrocephalus due to Arnold-Chiari malformation.
- surgery prevents infection but does not restore neurological function.
Other neural tube defects that aren’t spina bifida?
Encephalocele:
- Extrusion of brain and meninges through midline skull defect.
Anencephaly:
- Cranium and brain fail to develop (detected with antenatal USS, TOP usually offered).
What is spinal muscular atrophy? Different types?
Features? Diagnosis? Treatment? Prognosis?
Progressive degenerative diseases of motor neurons they may onset as early as foetal life.
Type 1 - develops in babies <6 months, most severe.
Type 2 - babies aged 7-18 months, less severe than type 1
Type 3 - develops after 18 months, least severe type in children.
Type 4 - affects adults and usually only causes mild problems.
Hypotonia, absent or weak tendon reflexes, generalised weakness, fasciculations in tongue, deltoid, bicep.
Diagnosis - muscle biopsy and identification of genetic marker for SMN gene.
Treatment - supportive - physio, feeding tubes, braces, surgery etc.
Prognosis - severe early onset - rarely survive beyond 2 years. Intermediate forms lead to severe motor disability.
