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Paeds Clinical Exam > Neurology > Flashcards

Neurology Flashcards

1
Q

Floppy strong causes

A
  1. Genetic: PWS, T21
  2. Structural: lissencephaly
  3. Metabolic: amino acidopathy, Zellweger, Tay-Sachs
  4. Neurocutaneous: SWS
  5. Static encephalopathy (CP)
  6. Infection: TORCH, Meningitis, encephalitis
  7. Ischaemia
  8. Trauma
  9. Endocrine (hypothyroidism, hypopituitarism)
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2
Q

Floppy weak causes

A
  1. Anterior horn: SMA
  2. Peripheral nerve: CMT, GBS
  3. NMJ: infantile botulism, MG
  4. Muscle: congenital muscular dystrophy, myotonic dystrophy, congenital myopathies
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3
Q

Investigations if floppy strong

A

History always!
Examine parents
Newborn screening results
TFTs
Serum lactate
Karyotype and microarray
Urine metabolic screen (OA, AA)
CT/MRI brain

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4
Q

Investigations if floppy weak

A

History
Newborn screening
CK
EMG and nerve conduction studies
Microarray?

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5
Q

Grading reflexes

A

0 = Absent
1= reduced (hyporeflexic)
2= normal
3= brisk
4= very brisk, elicits clonus (abnormal)

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6
Q

Which side are cerebellar signs on?

A

Cerebellar signs are IPSILATERAL to the side of the lesion

Horizontal nystagmus maximal towards side of lesion
Stagger towards side of lesion

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7
Q

Key cerebellar signs

A
  1. Dysdiadochokinesis
  2. Past pointing (dysmetria)
  3. Ataxic gait
  4. Nystagmus
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8
Q

Features of Freidreichs Ataxia

A
  1. Neuro dysfunction - cerebellar and posterior column mainly
  2. Cardiomyopathy
  3. Diabetes mellitus in 8-32%

Atrophy of spinal cord and medulla on MRI
Triplet repeat expansion of FXN gene

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9
Q

Cranial nerve pathologies that point TOWARDS side of lesion?

A

CN 5: jaw deviation
CN 12: tongue

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10
Q

Cranial nerve pathologies that point AWAY from side of lesion:

A

CN 10: uvula

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11
Q

Causes of horners syndrome (ipsilateral ptosis, miosis, anhydrous)?

A

Anything that disrupts the sympathetic nerve supply to the eye

Brainstem (lateral medullary syndrome)
- tumour, vascular insult, syringobulbia

Neck
- thyroid mass, trauma
- carotid anerysm or dissection

Lung mass
Post cardiac surgery

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12
Q

How to localise diplopia?

A

Abnormal is present at 30degrees
Cover each eye to find out which image disappears - loss of the lateral images indicates that the covered eye is responsible

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13
Q

Causes of abnormally small pupils

A
  • Horners syndrome (sympathetic chain)
  • Pontine lesions
  • Argyll robertson pupil + loss of light reflex (syphilis)
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14
Q

Causes of abnormally dilated pupils

A
  • CN3 lesion
  • Trauma
  • Surgical - lens transplant, iridectomy
  • Drugs (eg dilating)
  • Congenital
  • Adies pupil (idiopathic)
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15
Q

CN3 palsy features

A
  • Complete ptosis (partial if incomplete lesion)
  • Eye down and out
  • Dilated pupil, unreactive to light and accomodation
    +/- CN 4 lesion
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16
Q

Ddx for CN 3 or 6 lesion

A

UMN: Brainstem
- vascular, tumour, demyelination

LMN: peripheral nerve
- compression
- trauma

Raised ICP often -> CN6 palsy

17
Q

Causes of horizontal nystagmus?

A
  • Vestibular lesion (to side of lesion)
  • Cerebellar lesion (to side of lesion)
  • Toxins
18
Q

Causes of vertical nystagmus

A

Brainstem/ cerebellar
-Upbeat: lesion in floor of 4th ventricle
- Downbeat: lesion in foramen magnum

19
Q

Causes of CN7 palsy?

A

UMNL (forehead sparing)
- pons tumour or vascular lesion

LMNL
- vascular, tumour, demyelination
Posterior fossa: acoustic neuroma, meningioma
Petrous temporal bone: Ramsay Hunt, #, otitis media
- Bell’s palsy
- Parotid tumour or sarcoid

20
Q

Causes of SNHL

A
  • Weber localises to good ear and AC>BC

Unilateral
- tumour eg acoustic neuroma
- trauma eg # of temporal bone

Bilateral
- toxicity eg gent, frusemide
- congenital rubella
- Meniere’s

21
Q

Causes of conductive hearing loss

A
  • Wax
  • OM/effusion
  • Otosclerosis
  • Bony disease eg Pagets
22
Q

What is a pseudo bulbar palsy?

A
  • Bilateral UMNL of corticobulbar tract, affecting CN 9, 10, 12
  • dysasthria
  • dysphagia
  • facial and tongue weakness
  • increased gag reflex
  • emotional lability
23
Q

What is a bulbar palsy?

A

Bilateral impairment of the LMN of CN 9, 10, 12

  • reduced gag reflex
  • wasted tongue with fasciculations
  • nasal speech
  • normal emotions

Caused by brainstem strokes and tumours, GBS and some genetic conditions

24
Q

Features of myotonic dystrophy

A

Facial: V shaped upper lip, narrow head, thin cheeks, high arched palate
Weakness and wasting distal >proximal
Weak speech and swallow
Myotonia from >5yrs
External ophthalmoplegia and cataracts
Endocrine anomalies incl dm, hypothyroidism, adrenal insufficiency
Low IgG

25
Q

Features of Dandy Walker syndrome?

A

Congenital condition where the cerebellum doesn’t develop properly

  • Cerebellar signs
  • Hydrocephalus (incr HC, sunsetting)
  • Hypotonia
  • Hearing/vision problems
26
Q

Features of limb girdle muscular dystrophy

A
  • Weakness of shoulder/pelvic girdle
    (minimal facial/EOM weakness)
  • Nasal speech, dysarthria
  • Elbow contractures
  • Cardiac conduction defectsF
27
Q

Features of facioscapulohumeral muscular dystrophy

A
  • Facial weakness
  • Weakness of shoulder, hips, wrist drop
  • Scapular winging
  • Atrophy of biceps, triceps
  • Epilepsy and SNHL with infant form

Infant and classic form (2nd-3rd decade onset)

28
Q

Features of myotonic dystrophy

A

Weakness of facial muscles, V shaped lip
Weakness of SCM, forearm, hand, ankle -> foot drop
Mytonia (slowed relaxation): percussion over thenar eminence, grip hypotonia
Cardiac conduction defects
Cataracts
Hypogammaglobulinemia, insulin resistance

29
Q

Features of CIDP

A

Symmetric sensorimotor polyneuropathy
Weakness > sensory loss
Distal = proximal weakness
(Sensory involvement often worse distally and more posterior column than spinothalamic)
Reduced/absent reflexes
Symptoms present for >8 weeks

NCS: conduction velocity slowing
CSF protein elevated, with normal WCC (albuminocytologic dissociation)
Would do MRI spine with GAD

30
Q

Clinical features of transverse myelitis

A
  • back pain
  • paraparesis
  • initial flaccidity then spasticity, preferentially affecting flexors of legs and extensors of arms
  • reduced reflexes initially -> Hyperreflexia lateral
  • sensory level with pain and paraesthesias below that
  • autonomic symptoms incl urgency, retention, constipation
31
Q

Investigations for transverse myelitis

A
  • MRI brain and spine with GAD - evidence of inflammation (hyperintense T2 signal), exclude compression
  • CSF: mild elevation in protein, normal or mildly incr WCC, oligoclonal bands (can be seen with MS)
  • Serology for MOD and AQ-4 (NMOSD)
  • Serology for campylobacter
32
Q

Causes of cerebellar ataxia?

A

Genetic
- Freidrichs, A-T, Joubert, Dandy Walker syndrome, congenital cerebellar hypoplasia
Vascular
- AVM/stroke
Infective
- meningitis, encephalitis, cerebellitis
Structural
- Chiari II
Tumour
- Medulloblastoma, astrocytoma
Metabolic
- LSDs, leukodystrophies, Wilsons disease

33
Q
A

Paeds Clinical Exam (4 decks)

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