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Neurology Flashcards

Cerebral palsy, epilepsy, seizures, febrile convulsions, funny turns, floppy infant, headache, ataxia, hydrocephalus, neural tube defects, muscular dystrophies, neuropathies

1
Q

What is cerebral palsy?

A

A permanent disorder of movement + posture due to a non-progressive abnormality in the developing brain

CF emerge over time due to the balance between the normal + abnormal cerebral maturation

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2
Q

What are the causes of cerebral palsy?

A
  • Antenatal (80%): brain haemorrhage, cortical migration disorders, structural maldevelopment, genetic syndromes, congenital infection
  • Perinatal (10%): HIE
  • Postnatal (10%): brain infections, head trauma, symptomatic hypoglycaemia, hydrocephalus, hyperbilirubinaemia
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3
Q

Cerebral palsy CF

A
  • Delayed motor milestones
  • Slowing head growth
  • Abnormal limb/trunk posture + tone
  • Feeding difficulties e.g. ore-motor incoordination
  • Gait abnormalities e.g. spastic diplegia
  • Hand preference before 12m old (R/L handedness)
  • Persisting primitive reflexes (inhibits normal motor development)
  • Co-morbidities like epilepsy and LD
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4
Q

How is CP classified?

A
  • Spastic type (majority): damage to UMN pathways (CSTs) causing spasticity, may have clasp life rigidity, extensor plantar responses (usually early but may initially have head hypotonia). May be a unilateral hemiplegia, a bilateral quadriplegia or a bilateral diplegia
  • Dyskinetic type: involuntary uncontrolled movements, variable tone, primitive motor reflexes persist, feeding often impaired, risk of hip deformity + scoliosis. Often p/w floppiness + delayed motor development
  • Ataxic/hypotonic type: early trunk + limb hypotonia, poor balance, delayed motor development, later may develop cerebellar signs
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5
Q

How is CP managed?

A

MDT as often a range of problems

Hypertonia-botulinum toxin injections or selective dorsal rhizotomy (cut some nerve roots to reduce spasticity), intrathecal baclofen (skeletal muscle relaxant), deep brain stimulation of the basal ganglia

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6
Q

What is epilepsy?

A

A brain disorder that predisposes to unprovoked epileptic seizures; usually a genetic ‘idiopathic’ form but also there are structural + metabolic causes

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7
Q

Outline the classification of epilepsy

A
  • Generalised: B/L synchronous discharge from both hemispheres, lose consciousness if >3s. Includes absence, myoclonic, tonic, tonic-clonic and atonic seizures
  • Focal: seizures from neurones in one hemisphere, consciousness may be lost/altered/retained, can evolve to a secondary generalised seizure, may have an aura (reflecting the site of origin)
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8
Q

Infantile spasms/West syndrome

A

Onset at 3-12 months with violent flexor spasms then arm extension, a couple of seconds but in bursts of about 20

On waking, multiple times per day

Usually an underlying neurological cause

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9
Q

Lennox-Gastaut syndrome

A

Onset 1-3y

Multiple types of seizures but mostly atonic and atypical absences

Often a/w neurodevelopmental regression + behaviour disorders

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10
Q

Childhood absence epilepsy

A

Abrupt onset + end, transient LoC, no motor signs except flickering of eyes, momentary unresponsive stare. Child has no recall

Onset 4-12y

Often precipitated by hyperventilation

Development normal but can affect school

80% go by adolescent, some evolve to juvenile absence/myoclonic

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11
Q

Benign rolandic epilepsy

A

Onset 4-10y

Tonic-clonic in sleep or simple focal seizures with abnormal sensations

Common, may not need AEDs, goes by adolescence

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12
Q

Early onset benign occipital epilepsy

A

1-5y
Autonomic features, vomiting, unresponsive staring in sleep, may convulse
Remits in childhood

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13
Q

Juvenile absence epilepsy

A

Onset 10-20y
Absence + TC seizures, photosensitivity
Learning unimpaired
Good response to AED but needs for life

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14
Q

Juvenile myoclonic epilepsy

A

Onset 10-20y

Myoclonic seizure (brief, often repetitive jerking movements of limbs/neck/trunk) + TC + absences

Usually just after waking

Learning unimpaired

Good response to AED but needs for life

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15
Q

How is epilepsy diagnosed in children?

A

ECG: rule out convulsive syncope e.g. long QT

EEG: categorise type + severity (if normal may try a sleep/sleep-deprivation EEG)

Imaging: structural (usually need MRI + CT brain for childhood epilepsies), may do functional imaging

In first 2y: metabolic investigations

Genetic tests in intractable cases

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16
Q

How is epilepsy managed?

A
  • Educaiton
  • Specialist epilepsy nurse
  • Decide on AED
  • Adv about prognosis-promote confidence + independence but avoid things like swimming unsupervised
  • SUDEP: sudden unexpected death in epilepsy. If parents are asking about it emphasise how very rare it is and direct to information from groups like Epilepsy Action
  • 1/3 of children with epilepsy go to a special school because their epilepsy is part of a severe brain disorder
  • Surgery in a well-localised cause e.g. temporal lobectomy for mesial temporal sclerosis
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17
Q

What are the principles of AED therapy?

A
  • Not everyone needs it
  • Appropriate AED for the type (e.g. carbamazepine can worsen absence + myoclonic seizures)
  • Aim for monotherapy at minimum effective dose
  • Discuss potential ADRs
  • Don’t need to measure AED levels unless concerns re concordance
  • May be stopped after 2y seizure-free (but continue indefinitely in juvenile absence/juvenile myoclonic)
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18
Q

Important ADRs of valproate?

A

Weight gain, hair loss, teratogenic (don’t give to girls), rarely liver failure

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19
Q

Important ADRs of carbamazepine?

A

Rash, hyponatraemia, ataxia, liver enzyme induction - interferes with meds like COCP

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20
Q

Important ADRs of lamotrigine

A

Rash, insomnia, ataxia

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21
Q

Important ADRs of ethosuximide

A

N+V

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22
Q

Important ADRs of levetiracetam

A

Irritability (but generally quite well tolerated)

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23
Q

Important ADRs of gabapentin

A

Insomnia

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24
Q

Important ADRs of topiramate

A

Weight loss, depression, paraesthesia

25
Q

Important ADRs of vigabatrin

A

Irritability, restricted visual fields - so only used in infantile spasms + tuberous sclerosis

26
Q

What are febrile convulsions?

A

Epileptic seizure + fever, without intracranial infection

Most often 6m-6y old, Genetic predisposition, usually occurs early in a viral infection as temp rises rapidly

27
Q

What are the types of febrile convulsions?

A
  • Simple: no brain injury, child develops as normal, 1-2% chance of later getting epilepsy (similar to the chance in all children)
  • Complex: prolonged/focal/repeated in the same illness, up to 12% risk of later epilepsy
28
Q

How are febrile convulsions managed? Inc adv to parents

A

*Reassurance
*Antipyretics can be given but not shown to prevent seizures
*First aid management of seizures
*If seizure >5min give buccal midazolam for rescue therapy
*Up to 40% have another febrile seizure esp when they are younger or the seizure was
triggered at a lower temperature
*Explain that EEG not indicated
*Explain that AED not used as dont risk recurrence rate + risk of ADRs

29
Q

What exactly is a funny turn?

A

Paroxysmal disorders

30
Q

Blue breath holding spells

A

Toddlers when upset hold breath in expiration + go blue, sometimes get brief LoC

M: behaviour modification e.g. distraction

31
Q

Reflex anoxic syncope?

A

Brief cardiac systole from vagal inhibition

May have a FH of faints, triggers like pain (e.g. minor head trauma)/cold food/fright/fever – child becomes pale and falls to floor – hypoxia may induce a TC seizure

Rapid recovery

32
Q

Syncope?

A

Transient LoC with loss of postural tone + spontaneous recovery due to transient cerebral hypo perfusion, usually benign, often have clonic movements

  • Neurally mediated (usual): in response to stressors like orthostatic intolerance/sight of blood/pain/standing too long/hot room etc. Usually get a prodrome of light headedness/abnormal vision/pallor/nausea (usually maladaptive drop in BP)
  • Cardiac: arrhythmia (heart block, SVT, VT) or structural (AS, hypertrophic cardiomyopathy). Suggested if there are sx on exercise, FH of SCD, palpitations
33
Q

What are the causes of fits/faints/funny turns?

A
  • Syncope
  • Cardiac arrhythmias like long QT
  • Blue breath holding or reflex anoxic seizures
  • Migraine
  • BPPV
  • Tics, daydreaming, night terrors
  • Non-epileptic attack disorder/functional seizures
  • Pseudoseizure (child faking it), fabricated (parent faking it), induced (adult deliberate invoking it e.g. giving a child insulin to cause hypoglycaemia)
  • Paroxysmal movement disorders (no LoC)
34
Q

What is a floppy infant?

A

Abnormally limp hypotonic infant

Quite common and caused by a range of things

CF include ‘rag doll’ like, head lag, difficulty feeding, reduced movements

35
Q

How would you assess a floppy infant?

A

Prenatal hx: reduced fetal movements, polyhydramnios, drug exposure, maternal disease, congenital infection
Neonatal hx: preterm, birth trauma, low Apgar, breech
Developmental hx
Feeding: tiring, choking, aspirating
FH: consanguinity, prev miscarriages, NM/metabolic/genetic conditions in siblings
Examination: dysmorphic features, posture, tone in limbs + truncal, power (the 5 point scale), weak cry/use of accessory muscles can also indicate weakness, reflexes, check for myotonic dystrophy + MG in parents

36
Q

What are the causes of floppy infant?

A
  • Central (most common). Due to HIE/cerebral malformation/genetic disorders/acquired from infection or drugs/cerebral palsy/hypothyroidism. CF: abnormal consciousness, seizures, apnoeas, feeding difficulties, reflexes preserved/hyperreflexic, muscle power relatively preserved (but axial weakness), no fasciculations
  • Peripheral: due to birth trauma/spinomuscular atrophy/MG/infantile botulism/muscular dystrophies/hereditary neuropathies/IEM. CF: absent/reduced anti-gravity movements, absent postural reflexes, bone/joint deformities, fasciculations
  • Acute illnesses can also cause hypotonia
37
Q

What are the common causes of headache in childhood?

A
  • Migraine (usually without aura, last 1-72h, usually a pulsatile b/l headache over temporal/frontal area, often also GI disturbance/photophobia etc, received by sleep; sometimes get auras)
  • Tension type headaches (symmetrical, gradual onset, band/pressure, usually no other sx)
  • Uncommon primary types like familial hemiplegic migraine, cyclical vomiting (recurrent stereotyped N+V episodes with pallor + lethargy, well between episodes), abdominal migraine (idiopathic recurrent midline pain in bouts a/w vasomotor symptoms + N+V, child well in between)
  • Secondary headaches: medication overuse, H+N trauma, vascular malformations, space occupying lesions (signs of RICP, often change in mood/education, visual field defects, diplopia, CN VI palsy, torticollis, growth failure), infections, hypercapnia, acute sinusitis, emotional distress
38
Q

How would you assess headache in children?

A
  • History: aura, SQUITARS, triggers (stress, relaxation, food, menstruation), emotional/behavioural problems, head trauma, last eye test, analgesia over-use
  • Check red flags for SOL: worse lying down/draining/wakes child up in night [whereas headache on waking common in migraine], confusion, N+V, recent change in personality/school
  • Examination: visual acuity, sinus tenderness, pain on chewing (TMJ malocclusion), BP, growth, VF defects, squint, CN palsies, coordination, gait, fundi, cranial bruits
39
Q

How would you manage headaches in a child?

A
  • reassurance with a specific headache diagnosis + adv not to let control life, medicines make bad spells more bearable
  • don’t do ix unless red flags
  • rescue: analgesia (paracetamol + NSAIDs), antiemetics (prochlorperazine or cyclising), triptans (e.g. sumatriptan-can use intranasal at start of migraine), physical like cold compress
  • prophylaxis: when frequent + intrusive. topiramate (sodium channel blocker), propranolol (CI in asthma), TCAs, acupuncture
  • psychosocial support like bullying, exam stress, family illness
40
Q

What is a seizure and what are the different types?

A

Paroxysmal abnormality of motor, sensory, autonomic or cognitive function because of transient brain dysfucniton

  • Epileptic: excessive hypersynchronous electrical activity
  • Syncopal (anoxic)
  • Brainstem (hydrocephalic, coning)
  • Convulsion: a seizure (epileptic or not) with motor components
  • Acute symptomatic epileptic seizures: epileptic seizures provoked by an acute brain injury, but not an epilepsy
41
Q

What are the causes of seizures?

A
  • Epilepsies: genetic, structural or metabolic (e.g. vascular occlusion, acquired brain damage, SOL, neurocutaneous syndromes like tuberous sclerosis)
  • Acute symptomatic seizures: any acute injury like trauma, meningitis, hypoglycaemia/calcaemia/natraemia/magnesaemia, hypernatraemia, toxins
  • Febrile seizures
  • Non-epileptic seizures: convulsive syncope from heart problem/blue breath holding [expiratory apnoea]/hypovolaemic after haemorrhage/anaphylaxis, a sudden rise in ICP e.g. haemorrhage, sleep disorder (e.g. benign neonatal sleep myoclonus), functional/medically unexplained
42
Q

Early onset ataxia?

A

Usually autosomal recessive

Friedreich’s ataxia: a genetic disorder begins in teens with cerebellar ataxia + sensory neuropathy. A/w scoliosis, cardiomyopathy, optic atrophy, areflexia + DM

Other causes include ataxia telangiectasia and vitamin E deficiency

43
Q

Infantile hydrocephalus

A
  • Arnold Chiari malformation: cerebellar tonsils go into cervical canal, can develop syringomyelia
  • Stenosis of the aqueduct of Sylvius: congenital or post meningitis/haemorrhage
  • Dandy Walker syndrome: obstruction at 4th ventricle outlet
44
Q

What are some RF for neural tube defects?

A

.Folate deficiency, AEDs e..g SV

45
Q

What are the types of neural tube defects?

A
  • meningoencephalocele: elements of SC+lumbosacral roots within a meningeal sac herniate through a vertebral defect; if severe both LL+sphincters paralysed but can be more minor
  • spina bifida: multiple types, failure of lumbosacral NT fusion
  • spina bifida occulta: isolated failure of vertebral arch fusion, may have a dimple/hair tuft over anomaly but otherwise usually normal
  • meningomyelocele: may occur with spina bifida, meningeal defect alone, close in first 24h of age
46
Q

DMD + BMD

A

X-linked recessive inheritance but about 1/3 spontaneous mutations – causes absent (DMD) or low (BMD) levels of dystrophin

DMD usually apparently by age 4, die in 20s, difficulty running, standing up, Gower’s sign, proximal limb weakness with calf pseudo hypertrophy, myocardium becomes affected . CK 100-200x normal, biopsy shows varying muscle fibre size/necrosis/replacement with fat + absence of dystrophin

BMD: less severe, presents as weakness in young adults

47
Q

Childhood neuropathies?

A

Charcot-Marie-Tooth disease
Group of hereditary motor + sensory neuropathies. Typically distal limb wasting + weakness that progresses slowly over many years, mostly in legs, variable sensory + reflex losses. Late stage get severe foot drop but still can usually walk, mild cases have pes cavus + toe clawing that can go unnoticed.

Hereditary motor + sensory neuropathy type III
Rare hereditary demyelinating sensory neuropathy – severe incapacity during adolescence. CSF protein very elevated. Nerve roots hypertrophied

48
Q

How does unilateral spastic CP often present?

A

Fisting of affected hand, flexed arm, pronated forearm, toe-heel gait (but arms usually worse); may initially be flaccid + hypotonic before hypertonia emerges

A/w LD + seizures

49
Q

How does bilateral spastic quadriplegic CP present?

A

Severe spasticity in 4 limbs + trunk often involved (extensor posturing, poor head control, low central tone)

A/w seizures, microcephaly, mod-severe intellectual impairment, MSK issues like scoliosis

Often a h/o HIE

50
Q

How does bilateral spastic diplegic CP present?

A

Spasticity in all limbs but legs much worse than arms - walking abnormal (as problem in periventricular area, and the leg motor fibres are closer to the ventricles), walk on toes with scissoring of legs, older child typically crushes

A/w prematurity due to periventricular brain damage

Usually no feeding/communication/cognition issues

Often a/w strabismus

51
Q

What are the movement disorders possible in dyskinetic CP?

A

o Chorea – irregular sudden brief non-repetitive movements
o Athetosis – slow writhing movements that occur distally
o Dystonia – simultaneous contraction of agonist + antagonist muscles of trunk/proximal muscles giving a twisting appearance

52
Q

What is the cause of spastic cerebral palsy?

A

Damaged UMN pathways (CST), due to antenatal causes, HIE, neonatal stroke

53
Q

What is the cause of dyskinetic cerebral palsy?

A

B/L damage to basal ganglia or extra-pyramidal tracts

Kernicterus used to be main cause, now perinatal asphyxia

54
Q

What is the cause of ataxic/hypotonic cerebral palsy?

A

Usually genetic, but can be acquired injury to the cerebellum

55
Q

What are hiccups?

A

Non-epileptic myoclonic movements of the diaphragm

56
Q

Tonic clonic seizures

A

Rigid tonic phase then clonic (rhythmical contraction of muscle groups, irregular breathing, cyanosis, biting of tongue, incontiencne of urine). usually seconds-minutes then unconsciousness/deep sleep for up to several hours

57
Q

Tonic seizures

A

Generalised increase in tone

58
Q

Atonic seizures

A

Often a myoclonic jerk then transiently lose muscle tone - sudden fall to floor/drop of head

59
Q

How might the location of a focal seizure be ascertained?

A
  • Frontal: clonic movements travelling proximally (Jacksonian march), may be asymmetrical
  • Temporal: aura feeling/smell/taste/distorted sounds or shapes, may cause lip smacking/plucking at clothing/non purposeful walking, deja-vu feelings, may impair consciousness
  • Occipital: stereotyped visual hallucinations
  • Parietal: contralateral dysaesthesias (altered sensation) or distorted body image

Paediatrics (18 decks)

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