Neurology Flashcards
What would you examine on a kid with a headache? [6]
Growth parameters (height, weight, OFC, BP) Sinuses & teeth Visual acuity, fields and fundoscopy Listen for cranial bruit Focal neurological signs Cognitive/emotional status
Headaches in kids fall into 4 groups:
- Migraines
- TTHs
- Raised ICP
- Analgesic Overuse
Pointers to childhood migraine
Site, character, duration
Associated sx [4]
Relieving [1]
Hemicranial, throbbing & pulsatile headache lasting 0.5-few hours
Associated abdominal pain, nausea, vomiting
Presence of an aura
Relieved by rest and quiet, often photo/phonophobic
Also useful to look for FH
How would you diagnose a tension type headache? [3]
Constant ache
Diffuse and symmetrical
Band like distribution
Management migraine
Acute attacks [2]
Preventative drugs if occurs 2 or more times a month [2]
Give them simple analgesics for acute attacks
- NSAID
- Nasal triptan in 12-17 yo
Preventative
- Propranolol
- Topiramate
TTH Mx
Principles [3]
Acute treatment, give 3 options
Prophylaxis [2]
Reassure the parent it’s nothing sinister. MDT: attention to underlying chronic physical, psychological or emotional problems. Encourage use of simple analgesia in acute attacks. Discourage chronic use of analgesics
- acute treatment: aspirin, paracetamol or an NSAID are first-line
- prophylaxis: NICE recommend ‘up to 10 sessions of acupuncture over 5-8 weeks’
- low-dose amitriptyline is widely used in the UK for prophylaxis against tension-type headache.
How would you spot a medication overuse headache [2]
High use of PCM/NSAIDs (more problematic if with compound analgesics e.g. cocodamol)
Pain returns before they can have another dose
What would be red flags of a raised ICP headache? [5]
- Wakens them from sleep
- Aggravated factors (these raise ICP) e.g. coughing, toilet straining or bending over
- Altered conscious level, memory loss, cognitive loss
- Focal neuro signs
- Papilloedema
Headaches are diagnosed clinically, when would you want to use imaging? [6]
1) Cerebellar dysfunction e.g. ataxia
2) Raised ICP
3) New focal deficit e.g. new squint
4) Seizures
5) Personality change
6) Unexplained deterioration in schoolwork
Presentation of fits/falls/funny turns - differentials? [6]
1) Epilepsy
2) Acute Symptomatic seizures e.g. febrile convulsion
3) Reflex Anoxic Seizures
4) Syncope
5) Parasomnias e.g. night terrors
6) Psychogenic Seizures
Reflex anoxic seizures Ep Define Triggers [3] Symptoms and signs [3] Ix [2] Mx
Ep: 6m/o-2y/o
Brief (15s) periods of asystole
Triggers: pain, anxiety, fear
Sy/Si:
- Pallor +/- hypotonia
- rigidity, upward eye deviation, clonic movements, urinary incontinence
- but NOT tongue biting
Ix:
- serum ferritin (ensure >50ng/mL)
- if unsure of dx can refer to specialist for vagal excitation tests under continuous ECG and EEG monitoring
Mx: reassurance benign
What is an Acute Symptomatic Seizure or non-epileptic seizure?
Name 6 and indicate which is most common
A response to an acute insult e.g. hypoglycaemia, infection or hypoxia
Reflex anoxic seizure Syncope Parasomnias Psychogenic seizures *Febrile Convulsion
What can you use to diagnose epilepsy? [7]
Mostly a clinical diagnosis!!!
- History
- Video recordings
- ECG
- Interictal/Ictal EEG
- MRI (For malformations)
- Genetics (Familial & single gene disorders e.g. Tuberous Sclerosis)
- Metabolic tests if ass with developmental delay
Which cause and type of epilepsy is most common
Mostly it’s idiopathic
Mostly generalised
What factors are important when determining drug treatment for epilepsy? [4]
- Age
- Gender
- Seizure type
- Epilepsy type
Anti-epileptic drugs (AED) should only be considered if diagnosis is clear even if this means delaying treatment. What are the AEDs given for: Tonic clonic [2] Absence [2] Myoclonic [2] Partial
- Tonic clonic
- Sodium valproate or lamotrigine - Absence
- Sodium valproate or ethosuximide - Myoclonic
- Sodium valproate or levetiracem
Partial: carbamazepine
What are the major SEs of Anti-Epileptic Drugs (AEDs)? [4]
CNS - Drowsiness, impacted learning, cognition & behaviour
Invasive procedures for epilepsy [2]
Some forms respond to Vagus Nerve Stimulation or Epilepsy surgery
What signs would suggest a neuromuscular disorder in a walking child? [4]
Baby ‘floppy’ from birth Alert but paucity of limb movements Delayed motor milestones • Slips from hands • Able to walk but frequent falls Pes cavus (hammer) and waddling gait
What is pes cavus and hammer toes indicative of?
Charcot Marie Tooth Disease
How can you differentiate a neuropathy from a myopathy? Site of weakness Motor/sensory involvement Reflexes \+ 2 other distinguishing factors
Myopathy:
- Proximal weakness
- Purely motor
- Preserved reflexes
- Contractures
- ~Myocardial dysfunction
Neuropathies:
- Distal weakness
- ~sensory involvement
- Loss of reflexes
- ~Fasciculations
List some of the major neuromuscular disorders in kids? [4]
Muscular dystrophies e.g. Duchenne’s
Spinal Atrophy
Myasthenia Gravis
How is Duchenne’s MD inherited? What gene mutation is involved?
X-linked –> female carriers and male suffers
Xp21 - the dystrophin gene
How would you expect Duchenne’s MD to present? [6]
Specific manifestation in teens [1]
Delayed gross motor skills Symmetrical proximal weakness Waddling gait Calf hypertrophy Gowers sign ~ Cardiomyopathy Resp involvement in teens
How do you confirm Duchenne’s? [2]
Gower’s sign (required but not specific)
Raised CK (>1000)
How do we treat Duchenne’s?
Steroids
Febrile convulsions
Define [2]
Ep
Ax [3]
Define:
- Event occurring in infancy or childhood associated with fever [1] but without evidence of intracranial infection or defined cause of seizure [1]
Ep: 3m/o-5y/o
Ax: viral infection, otitis media, tonsillitis
Febrile convulsions
Describe the 2 forms that it can present with
• Simple:
- single tonic-clonic seizure lasting <15mins
- occurring as temperature rises rapidly in acute febrile illness
- brief post-ictal period of drowsiness/irritability/confusion
- full recovery within 1h
• Complex:
- partial onset or focal features (movement limited to 1 side of body or 1 limb)
- >15mins duration
- recurrence in 24h or within same febrile illness, seizure recurrence within 1h or prolonged post-ictal drowsiness or transient hemiparesis (Todd’s palsy)
Management of febrile convulsions
Immediate [4]
Examinations [4]
o Monitor duration, protect from injury by cushioning head, check airway and place in recovery position when seizure stopped
o If seizure >5mins, give IM lorazepam?
o Observe until recovered and examine and manage any injury
Ex:
- GCS, focal neuro deficit
- temp post seizure (>38oC)
- fluid status
- signs of underlying febrile illness
Febrile convulsions Ix [5]
FBC, ESR Glucose U&E Coagulation screen Blood cultures and urine MC&S if no source found
Mx when confirmed dx febrile convulsion [6]
Parental advice and education
- Not the same as epilepsy; risk child developing subsequent epilepsy
- About 1 in 3 have another febrile seizure
- Rare beyond 6 yo
- First aid advice and call ambulance if >5min
- PCM will not reduce seizure reccurrence but can make child more comfortable
Features of seizures in a history
Preceding
During seizure [5]
Post seizure [2]
o Pre-ceding events and epileptic aura (olfactory or gustatory hallucinations, visual hallucinations (flashing lights, blurred vision), déjà vu feeling, sensory disturbance (numbness, tingling, burning))
o Eyewitness account of seizure: movements, duration (>20s), lateral tongue biting, incontinence, cyanosis (arrhythmias)
o Post-seizure: post-ictal confusion or agitation, secondary injuries
Causes of epilepsy [6]
Idiopathic Genetic Brain malformation Brain damage Metabolic defects Brain tumour
Presentation of different types of epilepsy:
- Generalised
- Name 3 types - Focal - what are 3 clinical features?
Generalised
- Tonic clonic
- Absence
- Myoclonic
Focal
- Signs assoc to one hemisphere
- Reduced consciousness
- Automatisms eg lip smacking, running, rubbing face
- focal aware (previously termed ‘simple partial’)
- focal impaired awareness (previously termed ‘complex partial’)
- awareness unknown
Describe the difference in presentations of generalized seizures
- Tonic clonic
- Absence [3]
- Myoclonic [2]
- Tonic clonic: limbs stiffen and jerk forcefully
- Absence: 4-8yo, brief 10s pauses with loss of awareness, eyes may roll back
- Myoclonic: 1-4 yo, fall to floor
Brain tumour Ax [2] Sxs [3] Ix Mx [3]
Ax: brainstem astrocytoma, medullablastoma
Sy/Si: unexplained headache, nausea and vomiting
Ix: MRI brain
Mx: surgical resection, chemotherapy, RT
Brain tumour: describe presentation of raised ICP [3]
Diplopia
Papilloedema
Headache aggravated by Valsalva or wakes up
What are other more long term symptoms that can point to a brain tumour [4]
diabetes insipidus (polydipsia and polyuria), growth disturbance or failure, delayed, arrested or precocious puberty
Red flags (meningitis or encephalitis) [7]
- Irritability
- Neck stiffness
- Petechial rash
- Headache
- Photophobia
- Prolonged post-ictal period
- Focal neurological deficit >1h
Encephalitis
What is the main presenting symptom?
Aetiology can be divided into infective [11] and non-infective causes [8]
Main presenting symptom: altered consciousness
Ax:
• Infective: HSV, mumps, VZV, rabies, parvovirus B19, toxoplasmosis, TB, malaria, Lyme disease, Rikettsia, Dengue
o Immunocompromised: CMV, EBV, HHV-6
• Non-infective: hypoglycaemia, DKA, kernicterus, hepatic failure (Reye’s syndrome), lead or other poisoning, SAH, malignancy, SLE
Encephalitis
Presentation
Symptoms [3]
Signs [2]
Management
Complications [4]
Flu like prodrome
Odd behaviour
Vomiting, fever
Focal neurological deficits
Reduced GCS
Mx: antimicrobials target at causative organism eg aciclovir for HSV
Cx: Kluver-Bucy syndrome (hyper sexuality, rage, visual agnosia), auditory agnosia, autism, amnesia
Infant presents with abnormal head size and shape. What are your 2 top differentials
Hydrocephalus
Microcephaly
Hydrocephalus
Congenital causes [5]
Acquired causes [5]
Congenital:
- Arnold-Chiari malformation type 1 and 2
- Bickers-Adams syndrome,
- Dandy-Walker malformation
- agenesis of foramen of Monro, Vein of Galen aneurysm
- congenital toxoplasmosis
Acquired:
- brain tumour
- intraventricular haemorrhage
- meningitis
- increased venous sinus pressure (e.g. in achondroplasia)
- iatrogenic, idiopathic
Hydrocephalus
Classifications [2]`
Ix [2]
- Communicating: failure to reabsorb CSF e.g. post bleed/infection, Arnold-Chiari malformation
- Non-communicating: obstruction to CSF flow e.g. tumour, congenital malformation
Investigations:
- USS through anterior fontanelle in infants
- CT head
Hydrocephalus
Presentation infants: signs [5], symptoms [3], name 2 special signs
Infants: Signs - Rapid increase in OFC or OFC > 98th centile - Suture disjunction - Dilated scalp veins - Tense fontanelle - increased limb tone Symptoms - irritability - poor feeding - seizures
Setting sun sign: both globes deviated downwards, upper lids retracted and white sclera visible above iris (failure of upward gaze)
Macewan’s sign: cracked pot sound on percussing head
Hydrocephalus
Presentation in children:
- Describe an acute onset [4]
- Describe a chronic onset [4]
Mx
Complications [4]
Acute onset:
- headache and vomiting
- papilloedema
- impaired upward gaze
- seizures
Chronic onset:
- unsteady gait (leg spasticity)
- large head (although sutures closed
- skull still enlarges due to chronic increased ICP)
- unilateral or bilateral VI palsy due to increased ICP
Mx: ventriculopertitoneal shunt insertion
Cx: LD and epilepsy if untreated; shunt infection; CSF over-drainage
Causes of microcephaly [7]
Definition
OFC <2nd centile
- Normal variation (small child w/ small head)
- Familial (parents w/ small head)
- Congenital infection: toxoplasmosis, rubella, cytomegalovirus
- Fetal alcohol syndrome
- Hypoxic ischaemic encephalopathy (association w/ cerebral palsy)
- Patau syndrome
- Craniosyntosis
Cerebral palsy
Definition [3]
Prenatal causes [4]
Def:
- Permanent non-progressive (lesion static, clinical picture not) motor disorder
- due to brain damage
- before birth or before 2y/o
Prenatal:
- Placental insufficiency
- Toxaemia
- Smoking, alcohol, drugs
- TORCH2 infection
Cerebral palsy
Perinatal causes [4]
Postnatal causes [2]
Perinatal:
- Prematurity
- Anoxia
- Kernicterus
- Erythoblastosis fetalis
Postnatal:
- CMV or rubella infection
- Head trauma
Classification of CP [4]
State the affected part of the nervous system
- Spastic (pyramidal system and motor cortex)
- Athetoid/dyskinetic (extrapyramidal system (basal ganglia)
- Ataxia (cerebellum and brainstem)
- Other: rigid (basal ganglia and motor cortex), hemi ballistic, mixed
Describe the gross motor function classification score (GMFCS)
- I: walks w/out limitations
- II: walks w/ limitations
- III: walks using hand held mobility device
- IV: self-mobility w/ limitations, may use powered wheelchair
- V: transported in manual wheelchair
Types of spastic CP [3]
Spastic CP:
- Hemiplegia
- Diplegia
- Quadriplegia
Presentation of:
hemiplegia spastic CP [3]
diplegia spastic CP [1]
quadriplegia [2]
o Hemiplegia: delayed walking, hand preference <1y/o, increased deep reflexes of affected limb
o Diplegia: scissor legs on picking up
o Quadriplegia: associated with seizures and reduced IQ
Presentation of athetoid CP [4]
Presentation of ataxia CP
Athetoid:
- unwanted movements and poor posture control
- spasticity and hypotonia
- reduced hearing, dysarthria
- kernicterus association
Ataxia CP: hyper or hypotonia
General clinical features of CP [4]
Investigations [3]
- Spasticity, contractures
- Lack of voluntary control, poor co-ordination
- Delayed walking
- Sensory impairment
XR, MRI, gait analysis
Types of contractures [2] and the difference between them
Dynamic
- Increased muscle tone
- Hyperreflexia
- No fixed deformity
Fixed muscle contracture
- Persistent spasticity
- Shortened tendon muscle units cannot be overcome
- Fixed contractures with joint subluxation
- Secondary bone changes
Management of CP
- MDT [3]
- For spasticity [3]
- Ortho review [2]
MDT: Physiotherapy and OT, ortho scoliosis correction, posture management of hips and spine
Spasticity:
- Medication: Baclofen or diazepam
- Botulinum toxin injections
- Surgery: soft tissue release of adductors or hamstrings, bony realignment, osteotomy
Ortho review:
- Annual exam
- Hip XR
Management of CP
Prognosis
persistence of >2 primitive reflexes suggests child will be non-ambulatory (moro startle reflex, parachute reflex, tonic neck reflex, neck righting reflex, extensor thrust)
