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A Wobbly Child5/8/2015 Morning Report

Laura Williams, M M!", !#$%

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"!&2% mo o/' healthy male

(omiting )1 day, de* !+, nml +!

-ha.ing, *linging to Mom )2 days remors 'ith moements started t'o days ago

'hile *amping 'ith amily

o abnormal moements 'hen he is still

3rustrated 'hen trying to moeReuses to 'al., een sha.y 'hen *ra'ling

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"istory *ont4d!M" term, 'eighed 6 lbs, 7 o9 eliery at o 'ithout

*ompli*ation9 o ma:or or *hroni* illnesses9 o surgeries9

eelopment WL per parents

Meds o**asional +C meds or seasonal allergies, nonere*ently;

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=)am  %>95, "R 118, ?! 1>5/@>, RR 2>, -p+2 7> RA9

Weight 55ile, Length 65ile, +3C 70ile

#= ussy, sitting in Mom4s lap, *onsolable, but not*ooperatie 'ith e)am

"== WL

=C

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euro =)amM- ussy but *onsolable, tired but appropriate

C =+M&9 !=RRL9 (isual Belds seem ull to *onrontation9 3a*esymmetri*9 o nystagmus9 ongue midline9

Motor mus*le tone and bul. nml9 ormal strength demonstrated

'hen resisting e)am9

-ensation inta*t to light tou*h throughout9

Ree)es 1D and symmetri* in upper and lo'er e)tremities9 oesdo'nEgoing9 o *lonus9

Coordination otably abnormal 'ith trun*al titubation FtremorG andappendi*ular ata)ia9 yssynergy on rea*hing or ob:e*ts9

#ait Ata)i*, 'ide based gait9 Cannot remain upright 'ithout support9

https//'''9youtube9*om/'at*hHIo)%Jo)8C2

https://www.youtube.com/watch?v=Dox3_ox8C2Uhttps://www.youtube.com/watch?v=Dox3_ox8C2Uhttps://www.youtube.com/watch?v=Dox3_ox8C2Uhttps://www.youtube.com/watch?v=Dox3_ox8C2Uhttps://www.youtube.com/watch?v=Dox3_ox8C2Uhttps://www.youtube.com/watch?v=Dox3_ox8C2U

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) o *hroni* ata)iaCongenital anomaly o posterior ossa

andyEWal.er

Chiari

=n*ephalo*oele

Cerebellar aplasia/dysplasia/hypoplasia

"ereditary ata)ia

Ata)iaEtelangie*tasia

3riedrei*h ata)ia

egeneratie C- disease

-pino*erebellar ata)ia

iemannE!i*. disease

Multiple -*lerosis

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Wor.EupLabs rom =

C?C 'b* 596 F21, >%LG, hgb 1@9@, h*t @295, plt @%%

CM! WL

(R! neg

CR! K095, =-R 5

rine to) Dbenos Fgien ersed in = or CG

-erum to) neg

C brain normal

C angio normal

MR& brain normal

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Approa*h to a*ute ata)iaA*ute ata)ia generally due to a*ute ine*tion,

postEine*tious inammatory pro*ess, to)in,tumor, or trauma

80 o pts eal or ata)ia and subseuentlyadmitted 'ere d/*4d home 'ith d) o a*ute*erebellar ata)ia, to)i* ingestion, or #uillanE?arre

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Approa*h to a*ute ata)iaLieEthreatening

 umor

@5>0 o *hildhood brain tumors arise rom brainstem orposterior ossa

-lo'ly progressie ata)ia, s)s o in*reased &C!

&ntraE*ranial hemorrhage trauma 9 as*ular malormation

-tro.e

-i*.le *ell

"yper*oagulable state Fas in -L=G

&ne*tionCerebellar abs*ess oten rom *ontiguous spread

?rainstem en*ephalitis C abnormalities, AM-, seiure

A=M

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Approa*h to a*ute ata)iaA*ute *erebellar ata)ia

Most *ommon; a**ounts or %050 o *hildhood ata)ia; ages 25 yo

3reuently postEine*tious

(ari*ella inoled in oer 25 o *ases

Can also o**ur ater a**ines

-ymptoms other than ata)ia *an in*lude nystagmus F50G, slurredspee*h, omiting, heada*he

o + hae eer, meningismus, seiure

+nset 'ithin hours to 1E2 days

Wor.Eup to) s*reen

?rain imaging only i asymmetri* or o*al e)am, AM-, atypi*al disease

*ourse, s/s o in*reased &C!, potential trauma

!rognosis usually resoles 'ithout seuelae in 2E% 'ee.s

& s)s not improing in seeral 'ee.s, should *onsider alternate diagnosis

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Approa*h to a*ute ata)iaCommon *onditions

#uillanE?arre

!redominantly aNe*ts motor neres, but up to 15 may deelopsensory ata)ia

MillerE3isher ariant triad o ata)ia, aree)ia, ophthalmoplegia

Labyrinthitis"earing loss, omiting, ertigo

Can be diO*ult to distinguish rom a*ute *erebellar ata)ia in a toddler

 o)i* e)posure

Responsible or up to %0 o a*ute ata)ia in .ids

#enerally 'ith AM- as 'ell

Migraine syndromes

#enerally episodi*, but *an be diO*ult to d) at Brst presentation

 rauma

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"ospital *ourseWor.ing diagnosis o a*ute *erebellar ata)ia

Continued admission or poor !+, !/+

" 2, deeloped urinary retention that resoled by " %E@,un*lear etiology, behaioralH

" 5E> urinary retention improed, *onsidering transer toinpatient rehab

" >, opso*lonus noted on e)amhttps//'''9youtube9*om/'at*hHICiA8$A0i$

+pso*lonus Myo*lonus Ata)ia

https://www.youtube.com/watch?v=UCiAz8YA0iYhttps://www.youtube.com/watch?v=UCiAz8YA0iY

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+pso*lonus Myo*lonus Ata)iaRare 0918 *ases/million ppl/year in

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+pso*lonus Myo*lonus Ata)ia reatment

Remoal o neuroblastoma doesn4t improe symptoms

o *ontrolled studies

&mmunologi* treatment

-teroids, AC", plasma e)*hange, &(, immuran, *y*lophosphamide, *ell*ept

!rognosis#uarded motor symptoms improe or resole in >0 o *ases 'ith initial

treatment, but *an relapse

>080 o patients hae some residual behaioral abnormalities orpsy*homotor slo'ing

+ur patient &( )% days, AC" P+, 'ee.ly ritu)imab, monthly &( ollo'ed by

de)amethasone

Repeat imaging has been negatie or neuroblastoma

-igniB*ant regression ater diagnosis, regaining milestones, but about 1 yearbehind, slight dysarthria, myo*lonus 'hen alling asleep/'a.ing up,signiB*ant behaioral outbursts 'hen he doesn4t get his 'ay

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Reeren*espoate9 Approa*h to the *hild 'ith a*ute

ata)ia9

poate9 A*ute *erebellar ata)ia in *hildren9

poate9 +pso*lonus myo*lonus ata)ia9