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Paediatrics > Ch 27 - Neurological disorders > Flashcards

Ch 27 - Neurological disorders Flashcards

1
Q

Headaches: primary (4) secondary (define)

A

Primary: Thought to be due to primary malfunction of neurones

  1. migraine
  2. tension-type
  3. cluster
  4. other primary e.g. cough/exertional headache

Secondary: symptomatic of some underlying path e.g. raised ICP

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2
Q

Tension type headache: features (3)

A

2nd most common type F - gradual onset, symmetrical, tightness/band/pressure

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3
Q

Migraine (most common headache) - without aura features (5)

A

F - 1-72hrs, bilat/unilat, PULSATILE over temoral/frontal area, +/- N&V, photophobia, phonophobia. aggravated by physical activity

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4
Q

Migraine with aura: triggers (3), features (4)

A

Triggers:

  • Food - caffeine/chocolate/OCP
  • poor sleeping patterns
  • stress

Features:

  • visual/motor/sensory aura e.g. hemianopia/scrotoma (small areas of visual loss)
  • Pulsatile headache for a few hours
  • Fortification spectra - zig zag lines
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5
Q

Give a 3 uncommon types of migraine

A
  1. Familial - Ca channel defect, autosomal dominant
  2. basilar-type - vomiting + nystagmus + cerebellar signs
  3. Periodic syndromes - often precursors of migraines:
    • cyclical vomiting
    • abdominal migraine - episodic abdo pain in bouts
    • benign paroxysmal vertigo of childhood - recurrent brief vertigo episodes which self resolve - all tests normal between episodes
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6
Q

Secondary headaches: SOLs & raised ICP: features (6)

A

Features:

  • worse lying down
  • night time waking
  • morning vomiting
  • mood/personality change
  • poor educational performance
  • visual field defects
  • CN defects e.g. facial palsy, diplopia, squin
  • Papilloedema
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7
Q

Management: when to do imaging? (1), rescue treatments for headaches (3), headache prophylaxis (3)

A

Rescue treatment:

  • Analgesia - NSAIDs/ paracetamol
  • Anti-emetics e.g. prochlorperazine + metoclopramide
  • 5-HT agnoists e.g. sumatriptan

Headache prophylaxis:

  • Pizotifen - 5HT antagonist (causes weight gain + sleepiness)
  • BBlockers - not if asthmatic
  • Sodium channel blockers e.g. valproate
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8
Q

Seizures: definition, causes: epileptic (5)

A

Definition: Sudden disturbance in neurological function as a result of abnormal/excessive neuronal discharge

Epileptic causes:

  • Idiopathic (80%) - presumed genetic
  • Secondary
    • Cerebral malformation
    • Cererbal vasc occlusion
    • cerebral damage e.g. congenital infection, hypoxia - HIE, haemorrhage
  • Cerebral tumour
  • neurodengerative disorders
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9
Q

Causes of non-epileptic seizures (5)

A

Non-epileptic:

  • Febrile seizures
  • Metabolic
    • Hypoglycemia
    • Hypo/hypernatremia
    • hypocalcemia
  • Head trauma
  • Meningitis/encephalitis
  • toxins
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10
Q

Febrile seizures: definition, features (3), risk of developing epilepsy?

A

D - A seizure accompanied by high fever in absence of IC infection due to meningitis/encephalitis

F - genetic predisposition - 10% risk if 1st degree relative had febrile seizures, seizures are brief & tonic-clonic

They do not have increased risk of developing epilepsy - unless it was a complex febrile seizure ie. prolonged, focal, recurrent

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11
Q

Paroxysmal disorder (funny turns) have broad DDx, and the diagnositc question is whether the even was that of epilepsy or a condition which mimics it.

Breath holding attacks - 3 features

Reflex anoxic seizures: age group, common triggers (3), features (3)

A

Breath holding attacks

  • angry/ upset toddler
  • crying, goes blue holding breath and may have LOC
  • rapid recovery with spontaenous resolution

Reflex anoxic seizures:

  • Infants- toddlers
  • Triggers e.g. head trauma, cold food, fright, fever
  • Features:
    • Goes pale + falls to floor
    • brief tonic clonic seizure with quick recovery
    • episodes are due to cardiac asystole from vagal inhibition
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12
Q

Paroxysmal disorders continued:

Neurological causes - syncope, migraine, BPV

Arrythmia

A

Neuro causes:

  • syncope - hot and stuffy environment
  • migraine
  • BPV - nystagmus, unsteadiness/falling, viral labrynthitis

Arrythmia

  • prolonged QT
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13
Q

Ix of 1st non-febrile seizure (1)

A

12 lead ECG in all children

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14
Q

Seizure classification: Generalized:

  1. absence
  2. myoclonic
  3. tonic-clonic
  4. tonic
  5. atonic
A

Generalized = discharge from both hemispheres

  1. absence - trainsient LOC, abrupt onset + termination, eyelid flickering, often preceded by hyperventilation
  2. myoclonic - brief repetitive jerking movements of trunk, limbs
  3. tonic - generalized increase in tone
  4. tonic-clonic - rhythmic muslce group contraction following tonic phase. Fall to ground, no breathing + cyanotic
  5. atonic - transient muscle tone loss - sudden fall to floor
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15
Q

Focal seizures - manifestations depend on part of cortex:

Frontal seizures - features (2)

Temporal - auditory/sensory: features (4)

Occipital seizure (1)

A

Frontal - motor/ premotor cortex

  • clonic movements which may travel proxmially
  • asymmetrical tonic seizures
  • atonic seizures

Temporal:

  • most common
  • preceded by aura e.g. lip smackign, clothes picking, smell/taste altered & if spread to pre-motor cortex then automatisms (walking in non purposeful manner), deja-vu
  • consciousness may be impaired

Occipital - visual distortion

Parietal - contralateral sensation; altered sensation or distorted body image

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16
Q

Ix epilepsy (4)

A
  • Detailed history from child + eye witness
  • EEG - indicated whenever suspected epilepsy
    • sharp waves, spike wave-complexes are evidence of neuronal hyperexcitability
    • however may be normal (or abnormal in normal child)
    • 24hr EEG
  • Imaging - not routine, indicated if focal seizures or signs between seizures, to look for tumour/vascular lesion
  • functional scans - e.g. PET used to detect areas of hypometabolism
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17
Q

Management of epileptic seizures: tonic clonic (3), absence (3), myoclonic (2), focal (lots)

A

Treatment not begun after 1 unprovoke seizure (> indicates 2nd line)

  • tonic-clonic - valproate, carbamazepine > lamotrigine, topiramate
  • absence - valproate, ethosuximide > lamotrigine
  • myoclonic - valproate > lamotrigine
  • Focal - carbamazepine, valproate > topiramate, gabapentin, vigabatrin, tiagabine
  • other treatment - ketogenic diet, vagal nerve stim, surgery
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18
Q

Epilepsy syndromes: West syndrome - features (3), Ix (1), Rx (2)

A

West syndrome - 4-6 months age group

Features:

  • violent flexor spasms of head, neck & trunk followed by extension - giving ‘salaam spasms’ look
  • often on waking
  • social interaction deteriorates - most develop LDs

Rx: Vigabatrin/ steroids

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19
Q

Epilepsy syndromes: Lennox-Gastaut (1-3 yrs): features (3), Ix (1)

A

Lennox-Gastaut

F:

  • multiple types of seizures, but mostly atonic - drop attacks
  • daily seizures + status epilepticus
  • neurodevelopmental arrest/ regression

​Ix:

  • ​EEG - slow spike waves, poor prognosis
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20
Q

Epilepsy syndromes: Childhood absence seizures (4-12 yrs)

Features (3), Ix (1)

A

Features

  • can be induced by hyperventilation
  • Child stares monentarily and stops moving +/- eyelid twitch
  • <30s, child puzzled after
  • 2/3 female & 95% remission

Ix

  • blow on piece of paper/windmill for 2 min - EEG will show generalized 3s spike and wave discharge
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21
Q

Juvenile myoclonic epilepsy: adolescence/adulthood

features (3), Ix (1)

A

F

  • myoclonic seizures - but can also have tonic-clonic/absence
  • shortly after waking
  • typical history - throwing drinks/cereal in the morning

Ix

  • Characteristic EEG
  • Good treatment but life long
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22
Q

Corticospinal tract (UMN) lesions - causes (3), features (5 - muslce movement)

A

C - encephalitis, HIE, stroke, tumour

F:

  • weakness + shoulder adduction + elbow flexion + forearm pronation
  • Hip adduction + internal rotation + flexion
  • Hyperreflexia
  • extensor plantars
  • loss of fine finger movement
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23
Q

Basal ganglia lesions (deep grey matter structures which store patterns of movement so that we need not put conscious effort into every movement): causes (3), features (4)

A

Causes - acquired; HIE, CO poisoning, rheumatic fever, Wilson’s, Huntingtons

F:

  • Difficulty initiating movement + fluctuating tone (largely increased dystonia)
  • Release of packets of movement information > give jerky movement chorea or writhing athetosis
24
Q

Cerebellar disorders: causes (3), features (3)

A

Causes: alcohol, post-viral (varicella), medulloblastoma, ataxia telangiectasia

F:

  • difficulty holding posture - esp with eyes closed
  • past pointing - dysmetria
  • poor alternating movements - dysdiadochokinesia
  • wide gait
25
Q

Neuromuscular disorders can occur if any part of the lower motor pathway is affected - 4 parts of this pathway, general features of neuromuscular disorders (4)

A
  1. disorders of anterior horn cell
  2. disorders of the peripheral nerve
  3. disorders of the NMJ
  4. Muscular disorders

Features

  • weakness - is key feature (progressive/static)
  • floppyness
  • delayed motor milestones
  • muscle cramps - suggests metabolic myopathy
26
Q

What is Gower’s sign? What does it indicate?

A

Gower’s sign - infant needs to turn prone to rise up to a standing from supine position

Indicates proximal muscle weakness (e.g. DMD)

27
Q

Investigations you would carry out for suspected:

  • myopathy (4)
  • neuropathy (3)
A

Myopathy

  • Serum creatine phosphokinase (high in DMD, BMD)
  • muscle biopsy
  • chromosomal testing
  • muscular USS/ MRI

Neuropathy

  • nerve conduction studies
  • DNA testing
  • nerve biopsy
  • EMG
28
Q

Anterior horn cell disorders: features (4)

  • Spinal muscular atrophy type 1: features (6)
A

General features: weakness, fasiculations, wasting, areflexia

SMA I: Due to mutations in survival motor neuron gene

  • Very severe progressive disorder; dimished foetal movement
  • arthryogryposis at birth - positional deformities of limbs + contractures
  • absent deep tendon reflexes
  • intercostal recession - weakness of intercostal muscless
  • never sit unaided - death from resp failure within 1 yr
29
Q

SMA type II & III - prognoses

A

Type II - can sit but never walk unaided

Type III - can walk and present later in life

30
Q

General features of peripheral neuropathies (3)

A

Weakness, impaired pain/temp/touch perception, reflex loss

31
Q

peripheral neuropathies:

Hereditary motor sensory neuropathies: definition, Type I (Charcot-marie-tooth aka peroneal muscular atrophy) - pathology, features (3)

A

Definition: slow progressing + symmetrical muscle wasting which is distal

CMD:

  • dominant inheritance, nerves may be hypertrophic due to repeated attempts at myelination and therefore have onion bulb appearance
  • pes cavus (high arching foot) + distal atrophy
  • dimished reflexes
  • distal sensory loss
32
Q

Guillain Barre: definition, features ( 5), rx (2)

A

D - acute post-infectious polyneuropathy

F:

  • Present 2/3 weeks post URTI or campylobacter infection
  • ascending symmetrical weakness + loss of reflexes + autonomic involvment
  • Bulbar involvment - difficulty chewing/swallowing
  • Resp depression - may require ventilation

Rx

  • supportive - full recovery within 2 yrs
  • IvIg
33
Q

Bells Palsy: definition, features (3)

what if there is CNVIII involvment? (what does it indicate)

Ramsay Hunt syndrome

A

D - isolated CNVII paresis > facial weakness. Usually due to post-infectious (HSV)

F - unilateral facial paralysis - loss of smile, unable to close eyelid - risk of conjunctivitis

VIII involvement indicates cerebellopontine angle lesion

ramsay-hunt - Bell palsy + ear vesicles (HSV) > aciclovir

34
Q

Neuromuscular junction disorders - myasthenia gravis (transient neonatal + juvenile): Give features (4), diagnosis (2), Rx (3) of juvenile

A

Binding of abs to postsynaptic nACh receptors

Features: present after 10yrs

  • opthalmoplegia
  • ptosis
  • loss of facial expression
  • diff chewing

Diagnosis:

  • edrophonium > improvement in fatigueability
  • also can test for anti-nAChR abs
35
Q

Duschenne muscular dystrophy: definition + cause, features (6), Ix (2), Rx ( 4)

A

DMD: a debilitating X-linked recessive disorder caused by dystrophin gene deletion

F:

  • Gower’s sign
  • slow running
  • clumsiness
  • waddling gait
  • calf pseudohypertrophy - muscle replaced by fat and fibrous tissue
  • scolliosis
  • die of resp failure/cardiomyopathy by 30

Ix:

  • Creatine phosphokinase, muscle biopsy

Rx:

  • palliative - exercise, passive stretching
  • splints, truncal brace
  • CPAP, steroids
36
Q

BMD: cause, features (1)

A

Some functional dystrophin is produced, so the features are similar to DMD but less severe & slower progression - survive to late 40s

37
Q

Give 3 metabolic myopathies

A
  1. Glycogen storage disorders
  2. lipid metabolism disorders e.g. MCADD
  3. Mitochondrial cytopathies e.g. MELAS
38
Q

benign acute myositis: features (3)

A

A self limiting condition caused by URTI (probs influenza) > pain in calves, raised CPK

39
Q

Inflammatory myopathies - Dermatomyositis: features (5), Ix (1), Rx (1)

A

A systemic illness probably due to an angiopathy, is usually chronic

Features:

  • insidious with fever + misery > eventually symmetrical prox. muscle weakness
  • heliotrope rash to eye lids
  • perio-orbital oedema

Ix: Muscle biopsy - Inflammatory cells infiltrate + atrophy

Rx: steroids

40
Q

The ‘floppy infant’: define, causes can be cortical, genetic, metabolic or neuromuscular - give example of each. Features of floppy infant (3), how to differntiate cause

A

D - persistent hypotonia

Causes:

  • Cortical - HIE
  • Genetic - Down/ Prader willi
  • Metabolic - hypothyroid/ hypocalcaemia
  • neuromuscular - SMA, myasthenia

Features: infant slios through fingers, hangs like rag doll, head lag when lifted

Differentiation:

  • Central - poor truncal tone + preserved limb tone
  • genetic - dysmophia
  • LMN lesion - areflexia
41
Q

Friedreich ataxia: cause (1), faetures (5)

A

Cause - Trinucleotide repeat - fraxtin mutation

Features:

  • ataxia, distal muscle weakness
  • lower limb areflexia
  • pes cavus babinski +ve
  • impaired proprioception + vibraiton sense (in contrast to HMND)
42
Q

Ataxia telangectasia: cause, Features (4), Complications (2)

A

Cause - AR condition with mutation in ATM gene - disorder of DNA repair

F -

  • motor developmental delay
  • oculomotor issues - incoordination + delay in pursuit (oculomotor dyspraxia)
  • Telangiectasia in conjunctiva

​Complications:

  • increased risk of infections - due to IgA surface Ab defect
  • ALL, raised AFP
43
Q

Extradural haemorrhage: pathology (2), features (4), Rx (3)

A

Usually due to direct head trauma: middle meningeal artery tear - passes through foramen spinosum.

F:

  • lucid interval after trauma
  • then LOC > seizures (as haematoma gets bigger)
  • focal neurology - pupil dilation, contralateral paresis, 6th nerve paresis

Rx: confirm with CT > correct hypovolaemia > haematoma evacuation

44
Q

Subdural haemorrhage features (3)

A

NAI - shaking/direct trauma

Retinal haemorrhage often present

45
Q

SAH: features

A

Usually caused by aneurysm or AVM; much more common in adults

head pain + neck stiffness

Seizures + coma may develop

CT identifies blood in CSF, avoid LP due to raised ICP

46
Q

Stroke causes in children (6)

A
  1. cardiac - congeintal cyanotic HD e.g. ToF, IE
  2. Inflammatory e.g. SLE
  3. post-infectious e.g. varicella
  4. congeintal vascular malformation e.g. moyamoya disease ‘puff of smoke’ - becaue of that appearance on angiography
  5. metabolic/genetic e.g. MELAS, homocystinuria
  6. Haematological e.g. SCD, or anti-thrombotic factor def e.g. Protein S
47
Q

Neural tube defects - failure of normal fusion of neural plate to form neural tube: anencephaly, encephlocele

A

Anencephaly - failure of development of most of cranium + brain > stillborn > detected on USS and TOP performed

Encephalocele - Extrusion of brain + meninges via midline skull defect > surgical repair but underlying associated malformations

48
Q

Neural tube defects; Spina bifida occulta; what is it? Features (2)

A

Failure of fusion of verterbal arch

there is usually and overlying skin lesion e.g. skin tuft or lipoma

may get cord tethering to cause neuro symp

49
Q

Neural tube defects: meningocele, myelomeningocele- 2 features

A

Meningocele - jst a protrusion of meninges via vertebral arch

Myelomeningocele - protrusion of both meninges + spinal cord - so can get leg paralysis, sensory loss, neuropathic bladder/ bowel

50
Q

Hydrocephalus: defintion, Noncommunicating vs communicating, features (4), Rx (1)

A

D: obstruction to CSF flow > dilation of ventricular system proximal to site of obstruction

Types:

Noncommunicating - if obstruction within ventricular system or aqueduct: e.g. Aqueduct stenosis, chiari malformation, posterior fossa neoplasm

Communicating - if obstruction at site of CSF reabsorption e.g. due to SAH or meningitis

Features: enlarged head, suture separation, bulging fontanelle, downward gaze aka ‘sunset eyes’ (late sign), raised ICP signs

Rx: Ventriculoperitoneal shunt

51
Q

Neurocutaneous syndromes: why do they occur?

A

Embryologically nervous system & skin have common ectodermal origin - so embryological disruption causes syndromes involving abnormalities to both systems

52
Q

Neurocutaneous syndromes: NF type 1: criteria for diagnosis (5)

A

AD condition, in order to diagnose need 2 or more of following:

  • Cafe au lait spots > 5mm pre puberty & > 15 mm after
  • > 1 neurofibroma - unsightly firm nodular overgrowth of any nerve
  • axillary freckles
  • optic glioma - may cause visual impairment
  • Lisch nodule
  • 1st degree relative with NF1
53
Q

Neurocutaneous syndromes: NF2 features (3)

A

Less common than type I and presents in adolescence

Bilateral acoustic neuromata present with deafness +/- cerebellopontine angle syndrome with VII paresis + cerebellar ataxia

54
Q

Neurocutaneous syndromes: Tuberous sclerosis

A

Dominant inheritance

Cutanaeous features

  • De-pigmented ‘ash leaf’ patches- flouresce under Wood’s ligh
  • Shagreen patches - rough patch over lumbar spine
  • Adenoma sebaceum - angioibromata in butterfly distribution over face

Neuro features:

  • developmental delays
  • epilepsy
  • LDs

Other features

  • Subungual fibromata - beneath nails

Diagnosis - MRI - calcified subependymal nodules/ tubers

55
Q

Sturge weber syndrome: features (3), Ix (1)

A

Haemangiomatous facial lesion (port wine stain) in distribution of trigeminal nerve

opthalmic division of CN V always involved

epilepsy + LD

Ix - Skull xray - calcification of gyri - rail road track

Paediatrics (22 decks)

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