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Home , Mydriasis, Oculomotor nerve palsy, Optic disc drusen

06/05/2018

Inclusion - Exclusion • Pediatric focus Acute cranial nerve deficits • Acute < 2-3 days Eugen Boltshauser Emeritus – Department of Pediatric • Not considered – acute hearing loss Children’s Hospital Zürich EPNS Training Course May 2018 Alicante • «Acute» - sometimes a longstanding problem is only recently realized («pseudo-acute»)

Acute  «categories» ? • Trauma • – - demyelination • Haemorrhage

Cranial nerve = nucleus + nerve (lower motor neuron) •

# olfactory «nerve», optic «nerve» • Tumor • Toxic

Acute palsy

Acute IIIrd nerve palsy

1 06/05/2018

«Ophthalmoplegic migraine» (re-classified as cranial neuralgia) • Observed in (young) children • Onset usually associated with transient headache, nausea, vomiting • III rd nerve palsy, mostly not spared (variable) • Outcome – spontaneous recovery in a few weeks • Treatment – wait and see (steroids ?) • Recurrences possible • MRI – enhancement of thickened intracisternal portion of III nerve • CSF (literature) normal Enhancement of cisternal portion of oculomotor nerve

Problem with definitions…. May not be recurrent, not painful

Painful Ophthalmoplegia – Tolosa Hunt Syndrome - Cavernous sinus lesion • Not an aetiological entity

• Initial cranial nerve dysfunction variable (isolated, combined) mostly III > VI …combinations

• MRI – high priority

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Acute (isolated) Right Left • Isolated (no , normal ocular motility) • Subjective complaint – feeling of glare (no pupillary contraction) • Objective observation – parents, peers… Causes • Local (drops, patches…) • Tonic pupil syndrome (Adie) unilateral > bilateral relative mydriasis in bright illumination poor to absent reaction defective slow contraction to prolonged near-effort pupil constricts with pilocarpine (0,125%) Benign condition – often spontaneous recovery

Left side affected Acute palsy

• Most common etiologies trauma congenital

Acute palsy Acute palsy Distinguish • «Never seen» Abducens nerve palsy Abduction deficit • (Herpes zoster ophthalmicus?) Non-localizing Parainfectious, trauma, , Increased intracranial pressure incl. Chronic V nerve dysfunction (corneal lesion) in neurological syndromes Pseudotumor cerebri • Congenital indifference to pain • Pontine Tegmental Cap Dysplasia Localizing • Gomez-Lopez-Hernandez Syndrome Pons – CP angle – Clivus – middle fossa – cavernous sinus, sup orbital fissure

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Cerebellar low grade glioma – obstructing fourth ventricle  hydrocephalus  increased ICP  Acute abducens palsy and pontine glioma

• Usually NOT isolated + other cranial nerve deficits and longtract signs

T2 w MRI – flow void in aqueduct – 4th ventricle Acute benign abducens nerve palsy in infants

• Age group - usuallly infants • «Idiopathic», «postinfectious», («post vaccination») • Isolated (>< association with pontine glioma) • Spontaneous recovery in weeks • Recurrences possible Intrinsic pontine glioma • Investigations – (a matter of «temperament»)

Acute palsy Acute peripheral facial palsy • Occurrence „Idiopathic“ (Bell‘s palsy) ~ 50-70 % = „peripheral“ paresis (lower motor neuron affected) • Infectious In praxi: unilateral - Neuroborreliosis (saisonal - Europe) ~ up to 30 % isolated - Viral (herpes..) ~ 10-20 % (?) Anglo-american term • Skull base (petrous bone) - process Bell‘s palsy = acute peripheral facial paresis of unknown origin • Tumor intracranial (brainstem, cerebello-pontine angle) • Hypertension (blood pressure) Red flags  further investiations • Leukaemia (very rare) Paresis not isolated – subacute onset – bilateral • Trauma – central – Age < 2 years • Melkersson-Rosenthal Syndrome (OMIM %155900) • Varia

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Acute peripheral facial paresis Pediatr Neurol HISTORY • Suggestion for ENT process ? • Tick bite ? Erythema chron. migrans ? • Exposure to cold and wind ?

EXAMINATION • Focused neurological examination (isolated VII paresis ?) • Check ENT • Blood pressure • Optimal: grading of paresis (House Brackman) (or photo) MRI at presentation MRI 2 months later (age 2 months)

Facial nerve grading according to House, 1983, Laryngoscope Memo ad Examination - Trigeminus nerve involved ?

• Corneal reflex afferent – n. trigeminus efferent – n. facialis bilateral

In marked paresis – look for innervation on healthy side!

ADDITIONAL EXAMINATION (IMAGING ?)  not required in isolated unilateral VII paresis  wait and see – further steps depend on course Schwartz et al, Neurology 2014;82:1927-29 TREATMENT (STEROIDS ?) controversial issue - controversial publications AAO-HNSF Amercian Academy of Otolaryngology –Head and Neck Surgery limitations: often small pediatric cohorts, retrospective, # grading Foundation steroids do not impair serological tests AAN recommendation ….oral steroids should be offered

AAN guideline update 2012 [Neurology 2012;79:2209-13] „Steroids should be offered in new-onset Bell‘s palsy“ [< 72 hours] AAO-HNSF recommendation ….clinicians should prescribe oral steroids [based on 2 large controlled studies] within 72 hours of onset of symptoms

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Rationale for steroid treatment Practical management – Zürich children’s hospital Recovery in severe palsy often incomplete Additional investigations Synkinesias when smiling - always: blood film, serum sample for later serological tests Smile  ptosis - if neuroborreliosis possible – discuss CSF examination Equal HBS grade 3 - no neuroimaging Treatment - steroids for 7 days (preferably within 48 -72 h after onset) Inflammation  swelling of facial nerve at entry - «oculoprotection» (eye drops, «artificial tears») to internal auditory canal / facial canal Information to child/parents (shown by MRI) Arrange short-term follow-up  compression within bony structures

J Child Neurol 2014;29:NP96 Authors from UK Birmingham

Conclusion – all children recovered, with or without steroids …further studies needed…. Retrospective study, n=56 CAVE Retrospective study – some treated, some not Pediatric Neurol 2014 No grading ! Newer large controlled trials in adults not even cited

Acute IX / X glossopharyngeal / palsy • Exceptional ! • «Idiopathic-parainfectios» (post vaccination) (historic – diphtheria) • Paresis of palate - if unilateral – may be asymptomatic - nasal voice (rhinolalia) - fluid regurgitation into nasal cavity - dysphagia • Examination -failure to elevate palate - unilateral  deviation towards unaffected side

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Acute palsy XII Paresis Tongue deviation towards the affected side • Very rare! • Problem – onset may not be realised (seen patient with XII nerve palsy as an «incidental» finding)

Reported • Complication of bacterial meningits

• Following dental treatment (controversial) This situation allows normal speech, swallowing

«Cranial polyneuropathy» - bilateral

• Very exceptional • Consider - within GBS -spectrum

Recovery, 5 months later IMAGE IN CLINICAL Rockholt M, NEJM 2014;371

Acute visual loss in children Visual loss - Examination

Children Adults • (both sides separately) • • Retinal (detachment, ischemia) • Visual fields • Functional • Optic neuropathies • Pupillary reactions (? Relative Afferent Pupillary Defect) • …. ischemic • Ocular motility inflammatory (limitation ? movements painful ?) ….. Optic neuritis • Fundus - Papillitis • Reduced acuities – reduced - Retrobulbar neuritis

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Visual acuity testing – separate for each eye Swinging flash-light test

If severe reduction of vision • Test for Relative Afferent Pupillary Defect (RAPD)  Check finger counting • Prerequisite: unilateral lesion, no efferent defect, no • Normal reaction: initial constriction of pupil • RAPD: initial dilatation of pupil • RAPD: typical for (not retinal)

Optic neuritis Optic neuritis Children versus Adults • Retroorbital pain: may precede visual loss / 1-2 weeks duration • (Headache) • Often postinfectious • Blurring – fogging of vision • Usually associated with disc swelling (papillitis) • Rapid - subacute significant visual loss (hours 7 – 10 days) • Reduced contrast acuities (reduced color vision, dyschromatopsia) • Commonly bilateral • Variable visual field defects (central , peripheral..) • Fundus - retrobulbar neuritis  normal - papillitis  swollen (blurred) disc (mild peripapillary hemorrhages possible) • RAPD (Relative Afferent Pupillary Defect)

CLINICAL DIAGNOSIS

Optic disc blurred – normal visual acuity blurred - swollen («papilloedema») - simplified! MRI normal, CSF (opening pressure) normal • Increased intracranial pressure („symptomatic - idiopathic“) Vision normal • Inflammation (»Papillitis») Vision markedly reduced

Pseudopapilloedema • Optic disc Vision normal • Other variants Vision normal • Varia (myelinated retinal fibres, hypermetropia…)

8 06/05/2018

Optic neuritis MOG antibodies reported in -- Postinfectious – days or weeks [viral, EBV, bacterial, toxoplasmosis…] • ADEM -- «idiopathic» • Multiphasic DEM (MDEM) • ADEM followed by ON -- ON as first Acquired Demyelinating Syndrome (ADS) • Isolated ON • Isolated ON • Recurrent ON • ON with anti-MOG antibodies • NMOSD without AQP4-ab • ON with AQP4-antibodies (NMO spectrum) • ON in ADEM (monophasic, recurrent) • Transverse myelitis • ON   • # MS • …..

MOG antibodies (in children) • MOG ab prevalent (> 1/3) in ADS Clinical diagnosis of optic neuritis – and now? • MOG ab at presentation ~ 50% relapses • MOG ab at onset  non-multiple sclerosis course • [Adult ON, Optic Neuritis Treatment Trial (n=177) 1,7% +MOG] Additional investigations to consider Treatment • Neuroimaging • Steroids • Disease phenotypes depent on age MRI - iv younger children – ADEM (MRI spine) - oral older children – ON, TM • CSF examination

Duignan et al DMCN 2018 (n=237 ADS) • Antibodies • (referral to ophthalmologist) • 64 % +MOG in ADEM (45/70) anti-MOG • 96 % +MOG in relapsing DEM anti-Aquaporin-4 (AQP4) [NMO] • 43% +MOG in ON (28/65) … • 06% +MOG in TN (3/50)

Adults (> 18 years) 55 randomized patients 3 days treatment: - iv 1000 mg methylpredisolone Toosy AT et al Lancet Neurol 2014 - oral 1250 mg prednisone No taper

Imaging of – Optic nerve – of limited value (personal view) No significant group differences in recovery / outcome

Conclusion: bioequivalent doses of oral corticosteroids may be used as an alternative to IV corticosteroids

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