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MRCPCH Clinicals > Neurology > Flashcards

Neurology Flashcards

1
Q

How to examine facial nerve?

A

Open and close eyes against resistance
Puff out cheeks and whistle
Smile and show teeth
Frown

Offer to check BP
Ears for infection
Any rashes ?erythema migrans
Assess hearing - acoustic neuroma 
Any exposure keratitis
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2
Q

How do lower motor facial nerve palsies present?

A

Ipsilateral upper and lower face

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3
Q

How do upper motor facial nerve palsies present?

A

Contralateral lower face affected

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4
Q

What causes a lower motor neurone facial nerve palsy?

A 
Bell’s palsy
Chronic serous otitis media
Viral infections: EBV, mumps
Ramsay Hunt syndrome: herpes zoster vesicles in ear canal
Lyme disease
GBS - bilateral
Trauma
Tumours/leukaemia
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5
Q

What causes upper motor neurone facial nerve palsy?

A

CP
Tumours
Moebius syndrome - strabismus and immobile face

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6
Q

How is facial nerve palsy managed?

A

BP
Test for Lyme disease, varicella and leukaemia

Eye care - tape shut at night and use artificial tears

Steroids if <7 days

Should have some recovery by 3 weeks, MRI head if not recovered in 3 months

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7
Q

What is pseudo-bulbar palsy?

A

Bilateral supranuclear upper motor neurone lesions of CN IX-XII

Poor tongue and pharynx movement
Associated with spastic quadriplegic CP

Stiff, spastic tongue
Dry voice and dysarthria
Preserved gag reflexes
Exaggerated jaw jerk

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8
Q

How does a third nerve palsy present?

A

Ptosis
Downwards and lateral gaze
Pupil dilation - no reaction to light or accommodation

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9
Q

How does 4th nerve palsy present?

A

Upward deviation of eye
Head tilt to unaffected side
Unable to look out or down

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10
Q

How does 6th nerve palsy present?

A

Unable to abduct eye

Convergent paralytic squint

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11
Q

How does Horner syndrome present?

A

Partial ptosis
Pupil constriction - normal light reflexes as controlled by parasympathetic nerves
Anhydrosis
Enopthalmous

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12
Q

What causes Horner’a syndrome?

A

Can be congenital - associated with heterochromia

Central - causes anhidrosis of face, arm and trunk

  • brain tumour
  • MS
  • encephalitis

Preganglionic - facial anhidrosis
- Klumpkes paralysis

Postganglionic - no anhidrosis

  • cluster headache
  • carotid artery’s dissection
  • cavernous sinus thrombosis
  • otitis media
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13
Q

How is power graded?

A

0: no contraction
1: flicker or trace of contraction
2: active movement with gravity eliminated
3: active movement against gravity
4: active movement against gravity and resistance
5: normal power

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14
Q

What is the nerve innervation of the arm reflexes?

A

Biceps - C5/6
Supinator - C5/6
Triceps - C6/7

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15
Q

What is the innervation of the leg reflexes?

A

Knee - L3/4
Ankle - S1
Plantar - S1

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16
Q

What causes neuromuscular disease?

A

Anterior horn cells

  • SMA
  • Poliomyelitis

Nerve fibre

  • Charcot Marie Tooth
  • GBS
  • leukodystrophy
  • poison

Neuromuscular junction
- myasthenia gravis

Muscle

  • Duchenne/Becker/Facioscapulohumeral/limb girdle dystrophy
  • myotonic dystrophy
  • inflammatory: polymyositis, dermatomyositis
  • metabolic: glycogen storage disease
  • thyroid
  • steroids
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17
Q

How does SMA type 3 present?

A 
5-15yrs with waddling gait
Proximal muscle weakness
Difficultly climbing stairs
Gower’s positive
Reduced tone/power/reflexes
Tongue fasciculations
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18
Q

How does Charcot-Marie-Tooth present?

A 
Damage to peritoneal and tibial nerves
Foot drop
Distal wasting
Pes caves
Sensory involvement - proprioception and vibration

Treat with ankle orthoses

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19
Q

How does Duchenne present?

A

X-linked recessive - can be familial link but 1/3 are new mutation

Delayed walking, waddling gait, tip toe walking, falls and difficulty with stairs
Calf hypertrophy
Reduced reflexes except ankle reflexes
Associated with cognitive impairment

20
Q

How is Duchenne managed?

A

Genetics to confirm + genetic counselling for family
Resp - NIV
Cardiology - LVF and arrhythmia
Ortho - scoliosis
Gait - walking frame and then wheelchair
Neuro - cognitive impairment and speech delay

Steroids
PT/OT

21
Q

How does Becker present?

A

Less cardiac/resp involvement and present later

22
Q

How does facioscapulohumeral dystrophy present?

A

Facial and shoulder girdle muscle weakness
Face is expressionless
Winging of the scapula

23
Q

How does limb girdle muscular dystrophy present?

A

Onset in late childhood
Begins with shoulder weakness, progresses to hips and then distally
Toe walking, waddling gait and then requires wheelchair

24
Q

How does Guillain Barre Syndrome present?

A

10-14 days post viral illness

ASCENDING flaccid paralysis
MOTOR > sensory
Reduced power/reflexes/tone - plantar reflexes —> flexion

Involvement of cranial nerves - ptosis and facial nerve palsy (bilateral)

25
How is Guillain Barre Syndrome diagnosed?
MRI brain and spine - normal LP - high protein Nerve conduction studies - abnormal
26
How is Guillain Barre treated?
Monitor with daily spirometry - may require ventilation Can have CV autonomic involvement IVIG/plasma exchange PT and rehab
27
Causes of a floppy infant?
Central - encephalopathy - CP - IVH - neurometabolic Spinal cord - spina bifida - cord transection - tumour/haematoma Anterior horn cell - SMA/polio Nerve fibre - GBS Neuromuscular - transient myasthenia gravis, congenital myasthenia Muscle - congenital myopathy - congenital myotonic dystrophy - congenital musclar dystrophy - Pompes Other - Downs/Prader Willi - hypothyroid/hypercalcaemia
28
How does SMA 1/2 present?
Present within first year Normal face Frog like posture Bell shaped chest - see saw respiration Reduced tone/power/reflexes Fasciculations Never sit - die of resp failure 12-18 months Never stand - may require NIV
29
How does congenital myotonic dystrophy present?
``` Reduced fetal movements Floppy infant Facial diplegia with triangular fancies Resp problems Associated with talipes and hip problems High mortality ```
30
How does myotonic dystrophy present in the older infant?
``` Myotonic, expressionless, immobile face Hypotonia Poor muscle bulk Weakness Normal/reduced reflexes ``` AVOID GENERAL ANAESTHETIC
31
What causes ataxia?
Acute - infectious: chickenpox, measles, mycoplasma - structural: tumour, hydrocephalus - drugs: phenytoin - metabolic - vascular: basilar artery thrombosis Intermittent - migraine - epilepsy Chronic - perinatal e.g. encephalopathy - cerebellar: Dandy Walker - spinocerebellar degeneration: Friedrichs ataxia - ataxia telangectasia - metabolic: leukodystrophy, Wilson’s, abetalipoproteinaemia
32
How does Friedrichs ataxia present?
``` Progressive, presents before 15th birthday Ataxia and dysarthria Lower limb weakness, absent reflexes Up going planters Pes cactus Nystagmus Romberg positive ``` Cardiac: hypertrophic cardiomyopathy
33
How does ataxia telangiectasia present?
Cerebellar ataxia Telangiectasia of conjunctiva and behind ears Malignancy Low IgA and IgG
34
What is spina bifida associated with?
``` Associated with hydrocephalus - regular head circumference and imaging if indicated Learning difficulties Scoliosis Urine and faecal incontinence Foot ulcers ```
35
What clinical features are seen in NF1?
``` Visual fields, lisch nodules, ptosis and pro ptosis Macrocephaly Scoliosis Irregularly shaped, brown macules Axillary freckling Blood pressure ```
36
How is NF1 diagnosed?
2 of: ``` First degree relative Axillary/inguinal freckling Gliomas - brain or optic Lisch nodules >1 Osseous lesions - kyphoscoliosis, tibial bowing, sphenoid dysplasia Neurofibromas >6 cafe au lait >5mm / >15mm ```
37
What is the prognosis of NF1?
Learning disabilities Monitor tumours as can become malignant Risk of developing phaeochromocytoma, Wilms, thyroid cancer
38
How is NF2 diagnosed?
Bilateral acoustic neuromas ``` 1st degree relative Unilateral acoustic neuroma Schwannoma Neurofibromas Meningitis Glioma ```
39
Which professionals are involved in NF?
Paediatrician - monitor growth, BP, scoliosis Paeds surgeons - excision of neurofibromas Ortho - scoliosis ENT and audiologist - acoustic neuromas Plastic surgeons Ophthalmologist SLT Geneticist Psychology
40
How does tuberous sclerosis present?
Skin - ash leaf lesions (need Woods lamp) - adenoma sebaceous (warty lesions along nasal folds) - periungal fibromas - shagreen patches - cafe au lait Teeth - teeth pits Eyes - hamartomas GI - rectal polyps CNS - Astrocytomas - tubers - subependymal nodules Kidney - PCK - lipomas Cardiac - rhabdomyoma Resp - interstitial lung disease
41
How is tuberous sclerosis investigated?
BP Renal ultrasounds Echocardiogram ECG EEG and MRI head
42
How does Sturge Weber present?
Facial naevus Can have glaucoma CNS involvement - intracranial calcification, ipsilateral angiomas, contralateral hemiparesis, LD and GDD
43
What is incontinentia pigmenti ?
``` Girls Hyperpigmented, whirled lesions across body Seizures, LD, spasticity Ocular Peg shaped teeth ```
44
How does hypomelanosis of Ito present?
Whirled, hypopigmentation | Seizures, LD, hemiplegia
45
What is posterior fossa syndrome?
Secondary to medulloblastoma treatment Cerebellar mutism Hemiparesis Mutism Supranuclear cranial nerve palsy

MRCPCH Clinicals (5 decks)

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