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Flashcards in Neurology | Deck (98):

What are the 4 main groups of primary headaches?

1. Migraine
2. Tension-type headache
3. Cluster headache
4. Other e.g. primary stabbing headache


What are the characteristics of a tension-type headache (3)?

1. Symmetrical
2. Gradual onset
3. Feels like tightening, a band or pressure


What are the 2 types of migraines? How common are they?

With (10%) or without (90%) an aura


What are the characteristics of a migraine without an aura (5)?

1. Usually last 1-72 hours
2. Usually bilateral but can be unilateral
3. Characteristically pulsatile
4. Usually over the frontal or tempral area
5. Often accompanied by GI disturbance such as nausea, vomiting and abdo pain


What are the characteristics of a migraine with an aura (5)?

1. Preceded by an aura (visual, sensory or motor)
2. Absence of problems in between episodes
3. frequent presence of premonitory symptoms e.g. tiredness, difficulty concentrating etc
4. Migraines usually last for a few hours, where children like to lie down in a quiet and dark room
5. Rarely, there may be unilateral sensory or motor syndromes


What are the characteristics of the aura (2)?

Usually comprise visual disturbances
1. Negative phenomena such as hemaniopia (loss of half the visual field) or scotoma (small areas of visual loss)
2. Positive phenomena such as fortification spectra (seeing zigzag lines)


What are the characteristics of a headache secondary to a space-occupying lesion?
Main (3)
Others (3)

1. Worse when lying down
2. Morning vomiting
3. Night time waking

There may also be:
1. Change in mood
2. Personality changes
3. Change in educational performance


What symptoms are suggestive of a space-occupying lesion (3)?

1. Headaches that are worse when lying down and cause nighttime waking
2. Morning vomiting
3. Confusion, change in personality or behaviour


What physical signs are suggestive of a space-occupying lesion (10)?

1. Growth failure
2. Visual field defects
3. Squint
4. Cranial nerve abnormality
5. Torticollis
6. Abnormal coordination
7. Abnormal gait
8. Papilloedema
9. Bradycardia
10. Cranial bruits - arteriovenous malformation


What are the treatments for primary headaches in children (3)?

1. Advice on how to live with headaches
2. Analgesia/antiemetics/triptans
3. Psychosocial support


What is a seizure?

What types of seizures are there (6)?

A paroxysmal abnormality of motor, sensory, autonomic and/or cognitive function due to transient brain dysfunction

1. Epileptic
2. Syncopal (anoxic)
3. Brainstem (hydrocephalic, coning)
4. Emotional
5. Dysfunctional (psychogenic pseudo-seizures)
6. Undetermined


What makes a seizure epileptic?

Excessive and hypersynchronous electrical activity, typically in the neural networks in all or part of the cerebral cortex


What is a convulsion?

A seizure (epileptic or non epileptic) with motor components: particularly stiff (tonic), a massive jerk (myoclonic), jerking (clonic), trembling (vibratory), thrashing about (hypermotor)


What are the 2 broad classifications of epileptic seizures?
4 features of generalised?

History: Before, during and after seizure



Generalized - Discharge arises from both hemispheres
-Always LOC
-No warning
-Symmetrical seizure
-Bilaterally synchronois sizure discharge on EEG or varying asymmetry

Focal - Arise from one or part of one hemisphere
-Small area of dysfunctional neurones
-?Aura -> site of origin
- +/- LOC or decreased consciousness +/- tonic/clonic
- May be a tumour

1. Diagnosis on history/videos
2. Look for triggers
3. Any educational/psychlogical/social impairments
4. Look for skin markers of neurofibroma syndromes

1. EEG
2. MRI (structural) or PET (functional) if focal seizure
3. Metabolic investigations for specific epilepsy syndromes e.g. GLUCOSE

Management when there is seizure:
1. Start stopwatch
2. ABC
3. IV access early and bloods at the same time -> FBC, VBG, glucose
4. Drugs and fluids


What are the 5 types of seizures within the class of generalized epileptic seizures?

1. Absence
2. Myoclonic
3. Tonic
4. Tonic-clonic
5. Atonic

Can be in combination or sequence


What 4 types of focal seizures are there?

1. Frontal seizures
2. Temporal lobe seizures
3. Occipital seizures
4. Parietal lobe seizures


What are the features of absence seizures (4)?

1. Transient loss of consciousness with abrupt onset and termination
2. Usually no motor phenomena except flickering of eyelids and minor alteration in muscle tone
3. May be precipitated by hyperventilation
4. More common in children


What are the features of myoclonic seizures (3)?

1. Brief
2. often repetitive
3. jerking movements of the limbs, neck or trunk


What are the features of tonic seizures?

Generalised increase in tone


What are the features of tonic-clonic seizures (4)?

1. Rhythmic contraction of the muscle groups following the tonic phase
2. In rigid tonic phase, children may fall, they do not breathe and become cyanosed.
3. Followed by the clonic phase with jerking of the limbs. Breathing is irregular with cyanosis, there may be biting of the tongue and incontinence of urine.
4. Usually lasts from a few seconds to minutes, followed by unconsciousness or deep sleep for up to several hours (post-ictal phase)


What are the features of atonic seizures (2)?

1. Often combined with a myoclonic jerk
2. followed by a transient loss of muscle tone causing a sudden fall to the floor or drop of the head


What are the features of frontal seizures (3)?

1. Involve motor or pre-motor cortex, may lead to clonic movements, or tonic seizures with both arms raised high for several seconds
2. Asymmetrical tonic seizures can be seen
3. Jacksonian March - from distal to prox


What are the features of temporal lobe seizures (5)?

1. Aura with smell and taste abnormalities and distortions of sound and shape
2. Lip-smacking, plucking at one's clothing and walking in a non-purposeful manner may be seen
3. Consciousness can be impaired
4. Seizures usually longer than absence seizures
5. Deja-vu


What are the features of occipital lobe seizures?

Cause stereotyped visual hallucinations


What are the features of parietal lobe seizures?

Cause contralateral dysaesthesias (altered sensation) or distorted body image


When is an inter-ictal EEG indicated in epilepsy? why?

Whenever epilepsy is diagnosed. It helps categorize the epilepsy type and severity. It can make the diagnosis if seizures are frequent.


when are MRI scans used in epilepsy?

Required routinely for childhood epilepsies for structural brain imaging


What investigations are done for epilepsy (3)?

1. ECG - in all children with seizures
2. EEG - whenever epilepsy is diagnosed
3. Brain imaging - MRI/CT routinely


What is the management of epilepsy (3)?

1. Ensure child's safety until certain of diagnosis
-O2, ABC, iv fluids, benzo after 5 mins (buccal midazolam), then continually assess.
-Time 15 mins after onset of seizure -> 2nd dose of midazolam (max 2 doses of benzo as resp depression)
-Time 25 mins: alert PICU/anaethetics, start phenytoin and rectal paraldehyde
-Time 45 mins: anaethetic agents, intubate and ventilate

2. Once diagnosed, a clear explanation and advice to help adjust, support groups

3. Long-term: antiepileptic drug therapy (valproate is 1st line for most types)


What is the aim of management of epilepsy?

To maximise the child's educational potential and support their social development


What are the common first-line drugs for tonic-clonic seizures (3)? What are their side effects?

1. Valproate = Weight gain, hair loss, teratogenic, rare idiosyncratic liver failure
2. Carbamazepine = Rash, hyponatraemia, ataxia, liver enzyme induction, can interfere with metabolism of other drugs
3. Lamotrigine = Rash, insomnia, ataxia


What are the common first-line drugs for absence seizures (3)? What are their side effects?

1. Valproate = Weight gain, hair loss, teratogenic, rare idiosyncratic liver failure
2. Ethosuximide = Nausea and vomiting
3. Lamotrigine = Rash, insomnia, ataxia


What are the common first-line drugs for myoclonic seizures (3)? What are their side effects?

1. Valproate = Weight gain, hair loss, teratogenic, rare idiosyncratic liver failure
2. Levetiracitam = Irritability/sedation
3. Topiramate = Weight loss, depression, parasthesia


What are the common first-line drugs for focal seizures (4)? What are their side effects?

1. Carbamazepine = Rash, hyponatraemia, ataxia, liver enzyme induction, can interfere with metabolism of other drugs
2. Valproate = Weight gain, hair loss, teratogenic, rare idiosyncratic liver failure
3. Levetiracetam = Irritability/sedation
4. Lamotrigine = Rash, insomnia, ataxia


What is SUDEP in epilepsy? How common is it?

Where can family get info on it?

Sudden unexpected death in epilepsy. Very rare in childhood
Info available from self-help groups and organisations such as 'Epilepsy Action'


How would you explain to a parent what febrile convulsions are, how they occur and how often?

An epileptic seizure + fever in the absence of intercranial infection.
They are usually a brief generalised tonic-clonic seizure.

There is a genetic predisposition and usually occurs early in a viral infection when the temperature is rising rapidly

Occur in 3% of children between the age of 6 months to 6 years. About 30-40% will have further febrile seizures.
Rare past age of 5/6

There is a 1-2% chance of developing epilepsy, similar to the risk in all children.


What investigation/management advice should be given to family regarding febrile convulsions (2)?

Investigations if unsure:
Blood culture, urine culture, ?LP
EEG if unusual, or focal

1. Tell parents to give paracetemol but doesn't really help
2. In a history of prolonged seizures (>5mins), rescue therapy with buccal midazolam can be given. Give it after 5 mins, but call ambulance as soon as you give it
3. They should be taught 1st aid


What is a paroxysmal disorder?

A sudden recurrence or intensification of symptoms, such as a spasm or seizure. Occurs in epilepsy, breath-holding spells. reflex asystolic syncope etc


What are the typical features in the history of a child with blue breath holding spells i.e. expiratory apnoea syncope (4)

1. Toddler is upset
2. They cry, hold their breath in expiration and go blue
3. Child may briefly lose consciousness but rapidly recover fully
4. Attacks resolve spontaneously


How do reflex anoxic seizures present (3)? What are their triggers (4)?

Triggered by: pain or discomfort, particularly by:
1. minor head trauma
2. cold food
3. fright
4. fever.

1. the child becomes pale and falls to the floor.
2. The hypoxia may cause tonic-clonic seizures.
3. The seizure is brief and the child rapidly recovers


What are the cause of reflex anoxic seizures?

Episodes are due to cardiac asystole from vagal inhibition


How does syncope (transient loss of consciousness) present and what are their triggers (3)?

Children may faint in a:
1. hot and stuffy environment
2. on standing for long periods
3. from fear.

Clonic movements lasting a few seconds are common


What are the possible causes of convulsive syncope (transient loss of consciousness) (4)?

1. Cardiac syncope
2. Reflex mediated syncope (neutrally mediated syncope)
3. Blue breath-holding spells
4. Hypovolaemic syncope e.g. with haemorrhage, dehydration or anaphylaxis


What could be a cause of cardiac syncope?

Prolonged QT interval


What are the causes of cerebral palsy?
Antenatal (5)
During delivery (1)
Postnatal (4)

Antenatal (80%)
1. Cerebrovascular haemorrhage or ischaemia
2. cortical migration disorders
3. structural maldevelopment of the brain during gestation
4. Genetic
5. Congenital infection

During delivery (10%)
1. hypoxic-ischaemic injury

1. meningitis/encephalitis/encephalopathy
2. head trauma
3. symptomatic hypoglycaemia
4. hydrocephalus


What is the main risk factor for cerebral palsy?


vulnerable to brain damage from periventricular leukomalacia secondary to ischaemia and/or severe intraventricular haemorrhage and venous infarction


What are the different categories of CP (4)?

1. Spastic: unilateral, bilateral, not otherwise specified (90%) - UMN lesion
2. Dyskinetic (6%)
3. Ataxic (4%)
4. Other


What are the features of CP (5)?

1. Abnormal limb and/or trunk posture and tone in infancy with delayed motor milestones
2. May have slowing of head growth
3. Feeding difficulties
4. Abnormal gait once walking is achieve
5. Asymetric hand function before 12 months of age


What are the features of spastic CP (3)?

1. Limb tone is increased (spastic)
2. Associated brisk deep tendon reflexes and extensor plantar response.
3.Limb involvement may be unilateral, or bilateral


What are the features of dyskinetic CP (3)?

1. Involuntary, uncontrolled movements, often more evident with active movement or stress.
2. Muscle tone is variable
3. Primitive motor reflex patterns predominate


What are the features of ataxic CP (6)?

1. There is early trunk and limb hypotonia
2. poor balance
3. delayed motor development
4. Incoordinate movements
5. intention tremor may be evident later
6. Ataxic gait may be evident later


Why is a multidisciplinary approach important for CP patients?

They have a wide range of associated medical, psychological and social problems


What are the various therapies for CP?
Medical: non-pharmacological (3)
Medical: Pharmacological (2)
Surgery (1)

Medical: non-pharmacological
2. physiotherapy
3. CBT/behaviour management

Medical: Pharmacological
1. Baclofen pumps: a skeletal muscle relaxant
2. Antispasmodic drugs

Orthopaedic surgery: for hip subluxation/dislocation, painful muscle spasm, spinal deformity, osteoporosis/fractures etc


What is ataxia?

A neurological sign (usually of the cerebellum)consisting of lack of voluntary coordination of muscle movements that includes gait abnormality


What are the causes of ataxia (10)?

1. Friedreich ataxia
2. Ataxia telangiectasia
3. Cerebellar agenesis/dysgenesis
4. Postinfectious cerebellitis - varicella
5. Posterior fossa tumours
6. Other hereditary cerebellar ataxias
7. Miller Fisher syndrome
8. Mitochondrial disease
9. Drugs e.g. carbamazepine, lamotrigine
10. Toxins e.g. ethanol


What investigations could be done for ataxia (7)?

1. Genetic
2. Brain scans
3. Lumbar puncture (infection)
4. Nerve conduction studies and electromyography (EMG)
5. ECG
6. Echocardiogram
7. Videofluoroscopy


During which years does head growth mostly occur in?

First 2 years of life


At what age does the posterior and anterior fontanelle close?

Posterior: 8 weeks
Anterior: 12-18 months


What should be checked for in rapidly increasing head circumference?

Raised intracranial pressure


Define microcephaly

A head circumference below the 2nd centile


What are the causes of microcephaly (4)?

1. Familial - when present from birth and development normal
2. An autosomal recessive condition - associated with developmental delay
3. Caused by congenital infection
4. Acquired after an insult to the developing brain e.g. perinatal hypoxia


Define macrocephaly

A head circumference above the 98th centile


What are the causes of macrocephaly (5)?

1. Tall stature
2. Familial macrocephaly
3. Raised ICP
-chronic subdural haematoma
-brain tumour
4. Cerebral gigantism (Sotos syndrome)
5. CNS storage disorders e.g. Hurler syndrome


What is craniosynostosis?

The sutures of the skull bones start to fuse during infancy but do not finally fuse until late childhood


What is hydrocephalus?

Accumulation of cerebrospinal fluid in the brain


What are the different categories of causes of hydrocephalus (30?

1. Obstruction in the ventricular system
2. Communicating (failure to reabsorb CSF)
3. External (an accumulation of fluid in subdural spaces)


What are the causes of non-communicating/obstructive types of hydrocephalus (3)?

Congenital malformation:
1. Aqueduct stenosis
2. Atresia of the outflow foramina of the 4th ventricle
3. Chiari malformation (cerebellar tonsils herniation through foramen magnum)


Posterior fossa neoplasm or vascular malformation


Intraventricular haemorrhage in preterm infant


What are the causes of communicating types of hydrocephalus (2)?

Failure to reabsorb CSF:
1. Subarachnoid haemorrhage
2. Meningitis e.g. pneumococcal, tuberculous


How do brain tumours present specifically in Infants (3)?

1. Developmental delay/regression
2. Progressive increase in head circumference, separation of sutures, bulging fontanelle
3. Lethargy


How do brain tumours present specifically in children/adolescents (5)?

1. Persistent/recurrent headaches
2. Blurred or double vision
3. Lethargy
4. Deteriorating school performance
5. Delayed or arrested puberty, slow growth


How do brain tumours present generally in any age (6)?

1. Persistent or recurrent vomiting
2. Problems with balance, coordination or walking
3. Behavioural change
4. Abnormal eye movements
5. Seizures (without fever)
6. Abnormal head position - wry neck, head tilt or persistent stiff neck


What are some causes of developmental regression in children (5)?

1. Battens disease
2. Rett syndrome
3. Leukodystrophies
4. Wilson's
5. SSPE (Subacute sclerosing panencephalitis)


How does Battens disease present (5)?

After 4 to 6 years of normal development, children with this condition develop:
1. vision impairment
2. intellectual disability
3. movement problems
4. speech difficulties
5. seizures

which worsen over time


How does Rett syndrome present (4)?

1. Occurs in females.
2. After birth, girls with classic Rett syndrome have 6 to 18 months of apparently normal development
3. Then they developing severe problems with:
a. language and communication
b. learning
c. coordination etc
4. Early in childhood, affected girls lose purposeful use of their hands and begin making repeated hand wringing, washing, or clapping motions


How does Leukodystrophy present?

Damages myelin.
1. Usually appears in the second year of life.
2. Children lose any speech they have developed
3. become weak
4. Develop problems with walking (gait disturbance).
5. As the disorder worsens, muscle tone generally first decreases, and then increases to the point of rigidity.


What is Wilson disease?

Wilson disease is an inherited disorder in which excessive amounts of copper accumulate in the body, particularly in the liver, brain, and eyes.


What is SSPE? How does SSPE present (3)?

Rare and chronic form of progressive brain inflammation caused by a persistent infection with measles virus

Characterized by:
1. a history of primary measles infection usually before the age of 2 years
2. followed by several asymptomatic years (6–15 on average)
3. then gradual, progressive psychoneurological deterioration, consisting of:
a. personality change
b. seizures
c. myoclonus
d. ataxia
e. photosensitivity
f. ocular abnormalities
g. spasticity,
h. coma


What are the main causes of a subdural haematoma?

A tearing of the bridging veins as they cross the subdural space, usually caused by non-accidental injury that is shaking or direct trauma in infants and toddlers.


What are the symptoms of a subdural haematoma (6)?

1. Worsening headache
2. Nausea and vomiting
3. confusion
4. personality changes, such as being unusually aggressive or having rapid mood swings
5. Drowsiness
6. Loss of consciousness


What is the management approach to chronic pain?

Recognising, responding and reassessing


How do you recognise pain in a child?

Observation, parental impression, self-assessment tools


What are the treatment options for pain (3)?

1. Reassurance
2. Psychosocial support: distraction, psychological etc
3. Medical:
a. Local anaesthetic
b. Analgesia
c. Sedatives and other anaesthetic agents
d. Antiepileptic and antidepressant drugs for neuropathic pain


What is Charcot-Marie-Tooth disease?


A neuropathy caused by mutations in the myelin genes, leading to symmetrical, slowly progressive distal muscular wasting


How does Charcot-Marie-Tooth disease present (3)?


1. Children may present at preschool with tripping from bilateral foot drop
2. Loss of ankle reflexes, progressing to loss of knee reflexes
3. May have pes cavus


What is Guillain-Barre syndrome?


An acute post-infectious polyneuropathy that occurs at any age, and usually presents 2-3 weeks after an upper respiratory tract infection or campylobacter gastroenteritis, with an ascending, progressive, symmetrical weakness over a few days to 2 weeks.


How does Guillain-Barre syndrome present (5)?


1. Loss of tendon reflexes
2. Sensory symptoms usually in distal limbs or trunk
3. Bilateral facial weakness
4. Involvement of bulbar muscles - difficulty chewing and swallowing, with risk of aspiration
5. Dysautonomia occurs in 70% - tachycardia, bradycardia, arrythmias, hypertension etc


What is myotonic dystrophy? What are the different types (2)?


Myotonic dystrophy is an autosomal-dominant disorder which affects muscle function.
Type 1 (DM1) due to mutations in the DMPK gene and type 2 (DM2) due to mutations in the CNBP gene


How does myotonic dystrophy DM1 present in neonates (5)?


1. Hypotonia
2. Feeding and respiratory difficulties due to muscle weakness
3. Thin ribs
4. Talipes at birth
5. Oligohydramnios and reduced fetal movements during pregnancy


How does myotonic dystrophy DM1 present in older children (3)?


1. Myopathic facial appearance
2. Learning difficulties
3. Myotonia


How does myotonic dystrophy DM1 present in older adults (2)?


1. Cataracts
2. Males get baldness, testicular atrophy and T2DM


How does Duchenne muscular dystrophy present in children (4)?

1. Wadding gait and/or language delay
2. Mounting stairs 1 by 1 and run slowly compared to peers
3. Gower's sign
4. Pseudohypertrophy of the calves


How does Duchenne muscular dystrophy present in early school children (3)?

1. Affected boys are slower and clumsier than their peers
2. The progressive muscle atrophy and weakness means that they are no longer ambulant by the age of 10-14 years
3. 1/3 have learning difficulties


What is the management of Duchenne muscular dystrophy (3)?

1. Physiotherapy with splints to prevent contractures
2. Overnight CPAP
3. Corticosteroids to preserve mobility and prevent scoliosis


What are congenital muscular dystrophies? How does it present?


An autosomal recessive disorder mostly present at birth or in early infancy with weakness, hypotonia or contractures.
1. The proximal weakness is slowly progressive with a tendancy to contracture when the ability to walk is lost
2. Feeding difficulties may occur
3. Breathing difficulties may occur.


DDx of epilepsy?

1. Reflex seizure
2. Syncope
3. Breath-holding
4. Infantile spasm


What is West syndrome?

Epilepsy syndrome
4-6 months, "spasms", assc with developmental delay
Treat with Vigabatrin +/- steroids

Hypsarrythmia EEG (looks like spaghetti on EEG)


Childhood absence epilepsy EEG?

EEG - 3hz spike and wave


MAnagement of CP

1. MDT
2. Posture and movement - physio, orthotics, botox, surgery
3. Communication - SALT
4. Medical
-seizure, constipation etc
5. Ortho/neurosurgical
-ops for scoliosis, tendon lengthening