Neurology Flashcards
What is cerebral palsy?
Abnormality of movement + posture –> limited activity
What 5 things is cerebral palsy associated with most?
LD (60%) Epilepsy (40%) Squints (30%) Vision problems (20%) Hearing problems (20%)
Majority (80%) causes of cerebral
Antenatal
Vascular occlusion
Structural malformation
Cortical migration disorder
2 other types of causes of cerebral palsy
Hypoxic ischaemic injury
Postnatal origin
Examples of post-natal origins of cerebral palsy (6)
Preterm Meningitis Encephalitis Trauma Hypoglycaemia Hydrocephalus
Features cerebral palsy in early childhood (7)
Abnormal limb/trunk posture Delayed motor development Slow growing head Feeding difficulties --> oromotor coordination Abnormal gait Asymmetric hand fct <12m Remaining primitive reflexes
RF for developing cerebral palsy (12)
LBW Prematurity Multiple pregnancy Placental abnormalities Birth defects Meconium aspiration Emergency CSC Birth asphyxia Neonatal seizure Resp distress Hypoglycaemia Infection in neonatal period
What are the 3 types of CP
Spastic
Dyskinetic
Ataxic
What % of CP is spastic?
90%
Why does spastic CP occur?
Damage to UMN - motor cortex
Sx Spastic CP (think, its UMN)
Uni/bilateral hypertonia (velocity dependent) hyperreflexia Extensor plantar response Hypotonia of H+N
What are the 3 types of spastic CP
Hemiplegia
Quadriplegia
Diplegia
Features of hemiplegia spastic CP (4)
Unilateral involvement arm/leg
Arms worse legs
Face spared
Fisting of hand
Features of quadriplegia spastic CP (4)
All 4 limbs affected
Often severe + assoc w/ epilepsy, microcephaly + LD
Trunk involved = opisthonus
Poor head control + decr tone
Opithonus
Extensor protrusion –> arching of back
Features of diplegia CP (4)
All 4 limbs affects
Legs > arms
Walking abnormal
Hand fct remains normal
What is associated with hemiplegia spastic CP
Neonatal stroke
What is associated with quadriplegia spastic CP
HIE
What is associated with diplegia spastic CP?
Pre-term
Periventricular brain damage
What % CP is dyskinetic
6%
What structure is affected in dyskineitc CP
Basal ganglia
Most common cause dyskinetic CP
HIE
Features dyskinetic CP (4)
Variable tone
SOmetimes chorea or athetosis
Normal intellect sometimes
Presents floppy
What is chorea
Irregular, sudden + brief non-repetitive movements
What is athetosis
Slow writing movements occuring more distally
Features Ataxic CP (4)
Hypotonia of limbs + trunk
Poor balance
Delayed motor development
May develop incoordinate movements, intention tremor + ataxic gait
How is ataxic CP acquired
Genetically determined
Brain injury
2 main goals of physiotherapy in CP
Prevent weakness
Prevent mm rigidity
2 Dx used in CP
Diazepam
Baclofen
Use of orthopaedic surgery in CP (4)
Last resort - severe spasticty/fixed contractures
Repair scoliosis
Tendon lengthening
Osteotomy to realign limb
Prevalence epilepsy?
1/200
Generalised seizures featuers (4)
Discharge from both hemi-spheres
No warning
Consciouness loss
Usually symmetrical
Features absence seziures (3)
TLOC
Abrupt start + stpo
No motor phenomenon apart from eye flickering
What brings on absence seizures?
Hyperventilation
Myoclonic seizures features
Brief, repetitive jerking movements
Tonic seizures features
Generalised increase in tone
Tonic clone seizures features
Rhythmical contraction of mm groups following tonic phase
Lasts seconds/mins following by unconsciousness/deep sleep
Tonic phase of T-C seizures
Incr tone, fall to ground, cyanosis
Clonic phase of a T-C seizure
Limb jerking, irreg breathing + tongue biting
Atonic seizure features
Loss of mm tone –> sudden fall/drop of head
Features focal seziure
Can be conscious/not
May proceed into T-C
Frontal seizure features
Motor –> clonic movements
Temporal seizure features (3)
Auditory/sensory - aura w/ smell/taste
Distortions sound + shape
Lip smacking
Impaired consciousness
Features occipital seizure
Distortion of vision
Features parietal seizure
Contra-lateral altered sensation
Which focal seizure is the most common?
Temporal
What does an EEG identify? (3)
Abnormal background
Asymmetry/slowing hence structural abnormalities
Neuronal hyperexcitability
Structural imaging for epilepsy (2)
MRI
CT
Indications for structural imaging for epilepsy (2)
Neurological signs between seizures
Focal seizure
Functional imaging for epilepsy (2)
PET
SPECT
1st line Ix for epilepsy
EEG
4 Ix for epilepsy
EEG
MRI/CT
Metabolic Ix (developmental regression/feeding issue)
Genetic study (SCNA1)
Rescue therapy in prolonged therapy epilepsy (2)
Rectal diazepam
Buccal midalozam
When should anti-epileptic medication be stopped?
If seizure free 2 years
1st line Dx TC seizures
Valporate or carbamazepine
1st line Dx absence seizures
Valporate or ethosuximide
1st line Dx myoclonic seizures
Valporate
2nd line Dx generalised seizures
Lamotrigine
1st line Dx - focal seizures
Carbamazepine, lamotrigine or valporate
SE valporate (2)
Incr W
Hair loss
Liver failure (rare)
SE carbamazepine (5)
Rash Neutropenia Decr Na Ataxia Enzyme induction
SE lamotrigrine
Rash
SE Ethosuximide
N+V
Non-Dx Tx epilepsy (3)
Ketogenic diet
Vagal nn stimulation
Surgery - focal resection/hemispherectomy
What is SUDEP?
Sudden unexpected death in epilepsy
RF SUDEP (2)
Poor control
Seizures in sleep
Preventing SUDEP (5)
Good AED adherence Seizure diary Avoid triggers Bed alarm ID jewelry or epilepsy awareness cards
What is a febrile seizure?
Seizure + fever/viral infection
What age range gets febrile seizures?
6m –> 5y/o
What type of seizure is a febrile seizure usuaully?
T-C
What % febrile seizures have recurrence?
40%
Do febrile seizures affect brain performance?
No
What % chance is there of having a febrile seizure + going on to develop epilepsy
1-2%
Mx febrile seizures (7)
Ensure case isnt > serious (rule out meningitis) Inform parents, reassure DONT - use anti-pyretics, AED or EEG Recovery position Place in safe location Stay w/ child Call for help >5mins
When does expiratory apnoea syndrome occur?
In toddlers when upset/tantrum
What happens in expiratory apnoea syndrome?
Cry –> hold breath –> cyanosed –> brief LOC + rapid recovery
Triggers of reflex anoxic seizure (4)
Pain
Head trauma
Cold food
Fright
What causes reflex anoxic seizures
Asystole from vagal inhibition
What is Ataxia?
Incoordination of mm movements
Lack of balance, staggering gate, difficulty sitting, clumsy
What is the most common cause of ataxia?
Post infection
Causes acute (<72hrs) ataxia (7)
Post infection (varicella, EBV) Toxins Tumour Trauma Metabolic Infection Vascular
Toxins that can cause acute ataxia (4)
Phenytoin
Benzos
Anti-H
Alcohol
Causes of episodic/recurrent ataxia (4)
Toxin ingestion
Basillar aa migraine
Seizure disorder
Metabolic
Causes of chronic ataxia (7)
Tumours Hydrocephalus Metabolic Congenital malformation Hereditary Ataxic cerebral palsy
Examples of hereditary causes of ataxia (2)
Freidrich’s ataxia
Ataxia telangiectasia
What is Freidrich’s ataxia?
Autosomal recessive, worsening ataxia, distal wasting in legs, absent leg reflex
What is Ataxia telangiectasia
Disorder of DNA repair, autosomal recessive, dystonia + cerebellar signs
Ix ataxia (6)
Hx CT Bloods - g, U/E, gluc Metabolic screening Genetic testing Lumbar puncture (hydrocephalus)
Symptoms of raised ICP in children (5)
Headache, worse in morning Vomiting on waking Behaviour change Visual disturbance Papilloedema
Symptoms of raised ICP in infants
Vomit Bulging fontanelle Incr head circumference Head tilt Developmental delay/regression
Sx supratentorial tumour (3)
Seizures
Hemiplegia
Focal neurology
Sx midline tumour (2)
VF loss - bitemporal hemianopia
Pit failure - growth failure, diabetes insipidus, incr W
Sx cerebellar tumour (3)
Ataxia
Co-ordination issues
Abnormal eye movements
Sx brainstem tumour (3)
CN defect
Pyrimidal tract - motor issues
Cerebellar ataxia
(No Sx incr ICP)
When does Bratten’s syndrome begin
4-10y/o
Features Bratten’s syndrome
Gradual onset:
Visual disturbance + seizures –> behaviour, speech + learning regression –> dementia –> death
What is Leukodystrophies
Disorder in WM of brain b/c incorrect myelin sheath
Features leukodystrophies
Gradual decline
Loss movement –> speech –> vision –> hearing + behaviour
What is Wilsons disease
Disorder hepatic Cu deposition
Decr Cu in serum –> incr in Liver –> liver injury
Features Wilsons (5)
Acute liver failure --> CLD Severe depression Asymmetrical tremor Difficulty speaking, MASK face, clumsy, personality change Gait change
What is SSPE
Subacute sclerosis paencephalitis
Persistent infection w/ immune resistant measles –> progressive encephalitis
Prognosis SSPE
Death within 5 days
No Tx
Progression of SSPE
1’ measles <2
6-15 years asymp
Gradual psychological + neuro deterioration
When does most of head growth occur ?
First 2 years of life
When does posterior fontanelle close?
By 8 weeks
When does anterior fontanelle close?
by 12-18 months
If there is a rapid incr in head circumference, what must be excluded?
Incr ICP
What is macrocephaly?
Head circumference >98th centile
Causes macrocephaly (8)
Tall stature Familial Incr ICP Hydrocephalus Chronioc subdural haematoma Tumour NFM Cerebral gigantism
Ix macrocephaly if fontanelle open
USS
Ix macrocephaly if fontanelle closed
MRI or
CT
What is microcephaly?
Head circumference <2nd centile
Causes microcephaly (4)
Familial
Auto recessive
Congenital infection e.g. ZIKA
Insult to brain during development (hypoxia, hypoglycaemia, meningitis)
What is Craniosynostosis
Premature fusion of 1 or > sutures –> distortion of head shape
Which sutures are usually affected by craniosynostosis
Sagittal
Appearance of craniosynostosis - sagital suture
Long, narrow skull
Appearance of craniosynostosis - lambdoid suture
Skull asymmetry/flat
Key DDx craniosynostosis
Plagiocephaly
Causes craniosynostosis (2)
If Single suture - unknown
Multiple - genetic syndrome
S+S craniosynostosis (5)
Fused sutures
Unusual shape, headaches
Developmental delay, LD or visual problems
What is hydrocephaly
Obstruction in flow of CSF –> dilation of ventricles
What is obstructive/non-communicating hydrocephaly due to
Structure within ventricles or aqueduct blocking flow hence enlargement
Causes communicating hydrocephaly (3)
Congenital malformation
Posterior fossa neoplasm
Interventricular haemorrhage
Congenital malformations causing communicating hdyrocephaly (2)
Aqueduct stenosis
Atresia of foramine 4th ventricle
2 causes non-obstructive hydrocephaly
Incr CSF prod (choroid plexus tumour) Decr resorption (meningitis, haemorrhage)
External hydrocephaly
Benign enlargement of SAS in infancy
Self-limiting - resolves in following years :)
Features hydrocephaly (5)
Large head circumference Excessive rate of growth Bulging fontanelle Distended scalp vv Sunset eyes
Tx hydrocephaly
Insertion ventriculoperitoneal shunt
What % migraines have no aura
90%
Features migraine w/o aura: (6)
Last 4-72hrs Usually bilateral Pulsatile Over temporal/frontal area \+ N+V, abdo pain, photophobia, phonophobia Incr by exercise
Tx migraine w/o aura
Ibuprofen
Sumitriptan nasal spray
Features migraine w/ aura (3)
Preceded by; visual, sensory or motor aura
Most common = visual disturbances
Lasts few hrs
Tx migraine w/ aura
sleep
Features of tension headache (4)
Symmetrical
Gradual onset
Tight/band pressure
No other Sx
What can a 2’ headache be due to? (6)
Head/neck trauma Vascular malformation or haemorrhage Incr ICP HTN Substance use/withdrawal Meningitis/encephalitis
Red flags - headache (4)
Worse when lying down, night time waking or worse in morning
Morning vom
CHanges mood, personality, performance
Neuro defects, papilloedema
Cause of subdural haematoma (2)
Shaken baby syndrome
Falling from >3m
Sx subdural haematoma (8)
Headache Confusion Hemiparesis Lethargy Pupils asymmetrical Convulsions Incr ICP ? Retinal haemorrhages
Features of myopathies
Mm weakness (usually proximal)
Wasting
Gait
How to diagnose myopathies (3)
Creatinine phosphokinase **
Mm biopsy
Genetic testing
Sx myotonic dystrophy (6)
Myotonia - prolonged mm contraction Slurred speech Temporary locking of jaw Cataracts Cardiac arrhythmias Wasting - LL, hands, neck + face
Features of congenital mm dystrophy (2)
@ birth
Weakness, hypotonia + contractures
Proximal weakness
Features of neuropathies
Motor weakness
Sensory - impaired perception/loss reflexes
How to Ix neuropathies (3)
Nn conduction studies
DNA testing
Electromyography
What precedes Guillian-Barre
2-3 weeks prev - URTI/GE (campy)
Features GB (4)
Ascending symmetrical loss reflexes + autonomic involvement
Abnorm sensory in legs
Bulbar mm - difficulty chewing + swallowing
? resp depression
What % GB recover
95%
Mx GB
Supportive therapy
Charcot-Marie tooth cause
Hereditary (AD)
Features CMTooth (2)
Symmetrical + slowly progressive mm wasting in distal –> proximal pattern
50% - spinal issues e.g. scoliosis
Medical Mx chronic pain
Local - anaesthetic, nn blocks
Analgesia
Sedatives - midalozam, NO, GA
Neuropathic - AED or anti-D
Mild analgesia for chronic pain (2)
Paracetamol
NSAIDS
Mod analgesia for chronic pain (2)
Codeine
NSAIDS
Strong analgesia for chronic pain
Morphine