Data Pendukung Cerebellum

7/23/2019 Data Pendukung Cerebellum

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DATA PENDUKUNGSirkuit Internal Cerebellum

Jaras Propriosepsi

Spinocerebella tract

Vestibulo-cerebellar connection

Sindrom cerebellum

Infark cerebellum

Sindrom kognitif afektif cerebellum

Cerebropontocerebellar tract

Sirkuit serebellum

Gerakan volunter

Fisiologi cerebellum

Tumor cerebellum

Malformasi cerebellum

isfungsi cerebellum dan alko!ol

Vacularisasi Cerebellum

Pemeriksaan Cerebellum


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Vascularisasi cerebellum


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Vascularisasi Cerebellum


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"rteri inferior posterior cerebelli #PIC"$

- cabang terbesar%

- men&uplai bagian basal !emisfer cerebelli'bagian ba(a! vermis' sebagian nukleicerebelli' ple)us c!oroideus ventrikelkeempat' dorsolateral medulla

"rteri inferior anterior cerebelli - *occulus serebelli' anterior !emisfer

cerebelli%

"rteri superior cerebelli

- rostral !emisfer cerebelli' bagian atas vermis


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Serebellum


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Jaras Propiosepsi


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Jaras Propriosepsi


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Spinocerebellar Tract


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CerebropontoCerebellar


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Spinocerebellar Tractorsal SpinocerebellarTract

Cuneocerebellar Tract

+ote from (!ere t!esetracts originate%

Travels in ipsilateral

lateral column%,ot! of t!ese tracts entercerebellum t!roug! t!eipsilateral ipsilateralinferior cerebellar

peduncle%Sensor& info comes

from perip!er&

S i b ll T


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Spinocerebellar TractDescending Pathways:

Ventral Spinocerebellar Tract travels

after a decussation in the ventral

portion of the lateral column and enter

cerebellum via the superior cerebellar

peduncle.

Once in cerebellum, fibres cross

again so, input is ipsilateral!

"ostral Spinocerebellar

#oth relay internal feedbac$ signals

reflecting amounts of neural activity in

descending pathways.

,order ofventral andintermediateone of sc


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Sirkuit cerebellum


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"scenden Pat!(a& Cerebellum


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"scenden Pat!(a& Cerebellum


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Voluntary movement re%uires comple& interaction of

the corticospinal (pyramidal) tracts, basal

ganglia, and cerebellum'the center for motor

coordination( to ensure smooth, purposeful

movement without e&traneous muscular

contractions. The pyramidal tracts pass through the

medullary pyramids to connect the cerebral corte& to

lower motor centers of the brain stem and spinal

cord.


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Fisiologi Cerebellum Menerima informasi dari eksteroreseptor dan

proprioreseptor' reseptor visual dan auditorik'

formasio retikularis batang otak dan korteks cerebri%

Setela! mengola! impuls-impuls tersebut di atas' ia

memancarkan aktivitasn&a pada pusat pengelolaan

motorik di korteks serebri dan batang otak' se!ingga

gerakan &ang timbul memperli!atkan kelancaran dan

ketangkasan &ang teratur dan efektif%


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Fisiologi CerebellumControl of eye movements

)natomically, the flocculonodular lobe and the paraflocculus are

strongly interconnected with the vestibular nuclei. The cerebellum

controls the vestibuloocular refle& 'VO"( and the opto$inetic

refle&es. The VO" stabili*es vision during head turning by counter+

rotating the eyes in the orbit.

The gain 'eye velocityhead velocity( can be adapted by visual+

vestibular mismatch. Visual suppression of the VO" by fi&ating a

target moving simultaneously is reduced in cerebellar patients. The

initiation of the opto$inetic nystagmus, evo$ed when watching a

target moving rapidly in one direction, is often characteri*ed by either

e&aggerated or decreased e&cursions. Smooth pursuit depends on

the integrity of the cerebellum.


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Fisiologi Cerebellum

Control of eye movements

Single+unit recordings show a ma-or role of the fastigial

nucleus related to eye movements, control of head

position, and regulation of muscle activities during stance

and gait. The rostral fastigial nucleus controls head

orientation and eyehead ga*e shifts 'Pelisson et al.,

/001(.

The caudal fastigial nucleus controls oculomotor aspects,

such as saccades or smooth pursuit '2uchs et al., /003(.


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Fisiologi Cerebellum

Control of speech

4esions of the superior paravermal region are commonly

associated with speech deficits '4echtenberg 5 6ilman,

/071(.

) preparative loop includes the supplementary motor area,

dorsolateral frontal corte& including #roca area, anterior

insula, and superior cerebellum. The e&ecutive loop

comprises the sensorimotor corte&, basal ganglia,

thalamus, and inferior cerebellum.


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Control of limb movements

Single-unit recordings in t!e intermediatecerebellar corte) and t!e interpositus nucleus

!ave demonstrated t!at t!e& control re*e)

movements (!en a !olding position is suddenl&perturbed #Fr&singer et al%' ./01$%

T!e interpositus nucleus is implicated in

somest!etic re*e)es t!at control antagonist

muscles to damp 2oint oscillations and to correct

movements via feedback initiated b& t!e

movement itself #Vilis 3 4ore' ./05$%


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The dentate nucleus regulates reaction time through

initiation of movements triggered by vision or mental

percepts and accuracy of single+-oint and multi+-oint

goal+directed movements, such as reaching.

4esions of the dentate nuclei are associated typically

with an overshoot of the target 'hypermetria( and a

decomposition of multi+-oint movements 'Thach et

al., /008 #astian et al., /009(.


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Posture and gait

Due to its anatomical connections, the medial cerebellar*one can integrate spinal and vestibular inputs to

influence vestibulospinal and reticulospinal tracts.

The intermediate *one can integrate spinal and cortical

inputs to influence wal$ing via pro-ections to motor

cortical areas.

"egarding the lateral cerebellum, it influences wal$ing via

cortical interactions and contributes to the voluntary

modifications of the locomotor cycle '#astian 5 orton,

8;;7(


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Sitting, stance, and gait are usually impaired in midline cerebellar

lesions. Lesions in the medial and intermediate zones of the

cerebellum, especially in the anterior lobe, disturb movements

lin$ed to an e%uilibrium function '


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V!"#$%L&' !!"& central role in the maintenance of

e*uilibrium and gaze stability."he vestibular system, by means of its

receptors for the perception of linear andangular acceleration, plays a central role inorientation.

+esigned to answer two basic *uestions

-hich way is up

-here am # going


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Very elusive to test

/ive peripheral 0receptors1 (three semicircularcanals, utricule, saccule)

2erve (sub3divisions)

Central connections

Cortical area


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"he otoliths register linear acceleration and

static tilt


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Vestibular system


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Vestibular 2uclei (V2)

Vestibular signals originating in the two labyrinthsfirst interact with signals from other sensory systemsin the V2.

4nly one fraction of the neurons in the V2 receive

direct vestibular input, and most neurons receiveafferent input from other sensory systems (visual orproprioceptive) or regions of the C2! (cerebellum,reticular formation, spinal cord and contralateral V2).

Conse*uently the output of neurons from the V2

reflect the interaction of many systems.


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III

oculomotor IV

abducens


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Vestibulo3ocular and vestibulo3spinal refle5es


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Vestibulocerebellar and vestibulospinal pathways and

connections between vestibular and ocular motor nuclei


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Vestibular3cerebellar connectionsSome fibers of the vestibular nerve transmit impulses

directly via the -u&tarestiform tract 'ne&t to the ?>P( andruns to the flocculonodular lobe of the cerebellum.

@fferents from the fastigial nucleus turn through the

uncinate fasciculus of "ussell bac$ to the vestibular nuclei

and via the vestibular nerve to the hair cells of thelabyrinth 'predominantly inhibitory(

The flocculonodular lobe of the cerebellum also receives

secondary fibers from the superior, medial and inferior

vestibular nuclei. ?t returns efferent stimuli directly to thevestibular nuclei and spinal motor neurons via

cerebelloreticular and reticulospinal connections.

ach side of the cerebellum e5erts an influence on the

vestibular nuclei of both sides


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Vestibulo-okular re*eks)natomically, the flocculonodular lobe and the

paraflocculus are strongly interconnected with

the vestibular nuclei. The cerebellum controls the

vestibuloocular refle& 'VO"( and the opto$inetic

refle&es. The VO" stabili*es vision during head

turning by counter+rotating the eyes in the orbit.


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Vestibular Palsy

rapid horizontal head rotation toward thelesioned

side elicits compensator re!"ation saccades#


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C & L 4 ' # C " ! " # 2 6

"hermal convectivetheory

7eating or cooling the

e5ternal ear canal

causes convection

current in the

endolymph and

subse*uent

movement of the

cupula.


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Vestibular nuclei


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$indrom Cerebellar1 ge2ala cardinal6

.% "ta)ia

7% 4&potonus

8% Tremor

1% "st!enia #kelema!an' fatigue' malasbergerak$

$i d C b ll


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$indrom Cerebellar"ta)ia6 ataksia trunkal' limb' gait' stance%

Tremor 6 - ekstremitas #intention tremor' end-point'

kinetic$

- kepala' dan trunkal #titubasi$&sart!ria

+&stagmus

4&potonia

Peruba!an kepribadian&sdiadokinesia

&smetri


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Dsarthria

>erebellar disorders are typically associated with

slow speech accompanied by slurring.

>omprehension is spared. Temporal dysregulation

may lead to unintelligible words 'Aent et al., /007(.

Speech may turn out to be e&plosive, ta$ing a

staccato rhythm and a nasal character


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akibat lesi paravermis%

Gangguan sinergi otot-otot bicara%

Pasien bicara pelan-pelan' terputus-putus'artikulasi buruk' penekanan abnormal dandatar di setiap suku kata%


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&smetria Aetida$mampuan menghenti$an gera$an terarah

tepat pada wa$tunya, bermanifestasi 'misalnya(

sebagai gera$an -ari melewati lo$asi target .

Aegagalan fungsi cerebellum mengu$ur -ara$ $e

target.


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Dysmetria represents an error in tra-ectory of movement.


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&sdiadokinesia

gangguan gera$an bergantian secara cepat a$ibat

$erusa$an $oordinasi $etepatan wa$tu beberapa

$elompo$ otot antagonisti$: gera$an seperti pronasi

dan supinasi tangan secara cepat men-adi lambat,

terputus+putus, dan tida$ berirama.


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Tremor

%ntention tremor bermanifestasi selama

gerakan volunter' meningkat ketika

mendekati target%

Tremor postural $eti$a pasien mempertahan$an

tangan yang sedang pronasi tepat di depan, dengan

lengan terang$at.


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4&potonia dan

!&pore*eksPada lesi akut !emisfer cerebelli' resistensiotot ter!adap gerakan pasif meng!ilang'

postur abnormal #misaln&a pada tangan$%

9e*eks otot intrinsik meng!ilang pada otot

!ipotonik%

9e*eks pendular


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&esi 'estib(locerebell(m

6angguan fungsional lobus flo$ulonodularis atau

nu$leus fastigii:

/. Pasien $urang dapat menempat$an dirinya pada

lapangan gravitasi bumi.

8. Pasien tida$ dapat memfi$sasi tatapan pada obye$

yang diam saat $epalanya bergera$.


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isekuilibrium"stasia 6 sulit berdiri tegak

"basia 6 sulit ber2alan

"taksia trunkal 6

Ga&a ber2alan pasien lebar-lebar dan tidak stabil'

men&erupai ga&a ber2alan orang &ang mabuk%

4eel-to-toe (alking tidak dapat dilakukan%

:etidakseimbangan bukan karena de;siensi impuls

proprioseptif mencapai kesadaran' tapi akibat

koordinasi respon otot-otot ter!adap gravitasi &ang

sala!


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Gangguan okulomotor' nistagmus6angguan $emampuan mempertahan$an tatapan

terhadap ob-e$ diam atau bergera$.

Bi$a pasien mencoba mengi$uti ob-e$ yang bergera$

dengan matanya, ter-adi s%uare+wave -er$s, yang

terlihat oleh pemeri$sa.

6a*e+evo$ed nystagmus $eti$a mata bergera$ $e

sisi lesi serebellum dan menghilang bila pandangan

dipertahan$an $e sisi tersebut. #ila mata diarah$an

$e tengah, rebound nystagmus.


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6angguan refle$s vestibulo+o$ular, yaitu berupa

senta$an sa$adi$ mata $eti$a menoleh$an $epala.

?ndividu yang sehat dapat mene$an refle$ ini dengan

mempertahan$an tatapannya pada sebuah ob-e$.

&esi $pinocerebell(m


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&esi $pinocerebell(mSpinocerebellum berfungsi mengontrol tonus otot

dan $oordinasi $er-a $elompo$ otot antagonisti$

yang berpartisipasi pada postur dan gaya ber-alan.

4esi lobus anterior dan superior vermis :

ata$sia stance dan ata$sia gait 'lebih berat(.

>ara ber-alan yang lebar dan tida$ stabil, berdeviasi

$e sisi lesi, $ecenderungan -atuh di sisi lesi.


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)ta$sia stance terlihat dengan tes "omberg:

Pasien berdiri dengan mata tertutup, dorongan ringan pada sternum

a$an menyebab$an pasien berayun $e depan dan $e bela$ang

dengan fre$uensi 8+=


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&esi $erebrocerebell(mTida$ menimbul$an paralisis.

Aerusa$an berat pada e$se$usi gera$an volunter.

anifestasi $linis selalu ipsilateral terhadap lesi

penyebabnya.

"ebound phenomenon

Pasien mene$an tangan pemeri$sa dengan $e$uatan

ma$simum, pemeri$sa tiba+tiba menari$ tangannya,

gera$an pasien tida$ dapat dihenti$an, lengan terayun

memu$ul pemeri$sa.

C b ll $ d


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Cerebellar $ndromes9ostral vermis s&ndrome result from

alco!olism and nutritional de;cienc&%Caudal vermis s&ndrome implies a midline

cerebellar neoplasm 6 medulloblastoma'epend&moma' astroc&toma%

Cerebellar !emisp!er s&ndrome comes fromacute destructive lesion6 infarct' !emorr!age'neoplasm' abcess' trauma%

Pancerebellar s&ndrome6 vitamine de;sienc&'#vitamin


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Dist(rbancesGenerall& s&mptoms of anot!er disease

"ta)iaFailure to produce smoot! intentional

movements

Gait isturbance

Inabilit& to perform smoot! coordinated gaitMa& be described b& patient as

-=eakness -iiness

-Stroke -Falling


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Pat!op!&siolog&9esult from an& condition t!at a>ects t!e

central and perip!eral nervous s&stems

"ta)ia6 T&pesMotor ata)ia Sensor& ata)ia


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Motor "ta)iaCaused b& cerebellar disorders Intact sensor& receptors and a>erent pat!(a&s

Integration of proprioception is fault&Midline cerebellar lesions cause truncal ata)ia ?ateral cerebellar lesions cause limb ata)iaT!alamic infarcts ma& cause contralateral ata)ia

(it! sensor& loss


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Sensor& "ta)iaFailure of proprioceptive information to t!e

central nervus s&stem

Ma& be due to disorders of spinal cord orperip!eral nerves

Can be compensated for b& visual inputs


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i>erential iagnosesInto)ication


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e;nitions,est to use descriptive terms for gait

disturbances

Motor ata)ia6 (ide-based (it! irregular'unstead& steps

Sensor& ata)ia6 abrupt leg movement andslapping impact of feet

Festinating gait6 narro(-based miniatures!uBing steps% Commonl& seen in P


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"pra)ic gait6 dicult initiating gait% Ma& beseen in +P4 and P


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4istor&Dnset

9apidit&

Previous s&mptomsPM4

Medications

Social

"lco!ol intake Illicit drug use

"ssociated S&mptoms

4eadac!e ro(siness iiness VertigoTinnitus Fever +auseaEvomiting =eakness Parest!esia


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P!&sical


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Speci;c PopulationsGeriatric PatientGait normall& c!anges (it! age

S!ortened stride =idened baseSlo( gait

Senile gait ma& represent neuronal loss' reducedproprioception' slo(ing of corrective responses and(eakness

Can also be present in ot!er neurodegenerativediseases

Dccurs in 7H of elderl& populationTreatmentS&mptomatic

suall& admitted to rule out ot!er life-t!reatening


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T!e "lco!olic Patient"n& gait abnormalit& in an alco!olic patient s!ould

raise concern about nutritional de;ciencies

If acute ata)ia is associated (it! confusion and e&emovement abnormalities Wernickeencephalopathyneeds to be considered

Still ot!er intracranial pat!olog& needs to be ruledout

Treatment IV !&dration' Vit ,. and de)trose

Most often need to be admitted


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C!ildrenMa& appear (ell' but

(obbl& (!en sitting

Into)ications are mostcommon' follo(ed b&infectionEin*ammation

"sk about famil&member !omemedications

PM4

PF4

i>erential diagnoses

rug Into)ication Infection or in*ammation+eoplasmTrauma Inborn errors of

metabolism4&drocep!alus Idiopat!ic

isposition9ule out life t!reatening

processesMost are admittedPediatric neurolog&

consult


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i i


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Tipe "ta)ia

!imtom

Vestibular&ta5ia

Cerebellar&ta5ia

!ensory&ta5ia

Vertigo Possible K

Cystagmus K

Disartria K Possible K

)ta&iae&tremitas

K #onl& in legs$

Tes "omberg K #bot! (it!open andclosed e&es$

#(it! closede&es$


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Dccupational t!erap& L to ma)imie use oft!e limbs and to ma)imie safet& (!en(alking and doing transfers

Safet& bars' gait assist aids or (!eelc!airs=eig!ted bracelets ma& improve !and controlSpeec! and s(allo(ing t!erap& L to improve

communication and s(allo(ing safet&

#especiall& to prevent c!oking andpneumonia$ communication assist devicesPneumova) and in*uena vaccinations

General ata)ia treatments

i i d i !


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"ta)ia Friedreic!Sebelum usia 75 ta!un

4ilangn&a sel-sel ganglion radi) dorsalisdegenerasi kolumna posterior%

:linis6

- gangguan sensasi posisi' diskriminasi 7 titik'stereognosis%

- ataksia progresif tanpa diketa!ui pen&ebabn&a%

- romberg tes #$

- re*eks tendon dalam di ekstremitas ba(a! #-$ - disartria dalam ta!un setela! onset%

- autosomal resesif


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Tabes Dorsalis

egenerasi kolumna dorsalis dan dorsal root%Sensor& ata)ia' lutut pasien diangkat tinggi

ketika ber2alan' kemudian diturunkan cepat%

Gangguan sense of position' kompensasi ole!

visual%9omberg tes #$

Gangguan vibrasi

Pupil arg&ll robertson' serologi sip!&lis #$


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Motion Sickness


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Motion Sicknessotion sic$ness is a very common disturbance of the

inner ear that is caused by repeated motion such as fromthe swell of the sea, the movement of a car, the motion of

a plane in turbulent air, etc.

?n the inner ear, motion sic$ness affects the sense of

balance and e%uilibrium and, hence, the sense of spatialorientation.


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otion is sensed by the brain through three different

pathways of the nervous system that send signalscoming from the inner ear 'sensing motion, acceleration,

and gravity(, the eyes 'vision(, and the deeper tissues of

the body surface 'proprioceptors(.

hen there is unintentional movement of the body, asoccurs, the brain is not coordinating the input, and there

is thought to be discoordination or conflict among the

input from the three pathways.

?t is hypothesi*ed that the conflict among the inputs isresponsible for motion sic$ness.


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Infark serebellar

"ppro)imatel& )*+ of patients (it!

cerebellar infarction present (it! verti,o and

no localizin, ne(rolo,ic de!cits% T!e

ma2orit& of t!ese ma& !ave ot!er signs of

central vertigo' speci;call& direction-c!anging

n&stagmus and severe ata)ia%


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Cerebellar infarction represents appro)imatel& -./ + of

ac(te stro0es overall%7/

T!ese can result from occl(sion of t!e superior

cerebellar arter& #SC"$' anterior inferior cerebellar

arter& #"IC"$' or t!e posterior inferior cerebellar arter&

#PIC"$%

?arger cerebellar infarcts produce s&mptoms and signs

localiing to t!e brainstem' suc! as diplopia1

dsarthria1 limb ata"ia1 dspha,ia1 and wea0ness

or n(mbness.
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2791733/#b29-wjem-10-273http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2791733/#b29-wjem-10-273


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"ppro)imatel& .5H of patients (it! cerebellar

infarction can present (it! isolated vertigo' t!at

is' vertigo (it! no localiing ;ndings on motor'

sensor&' re*e)' cranial nerve' or limb

coordination e)amination%

Most of t!ese are infarcts of t!e medial branc!

of t!e PIC" #/OH$%


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First' stroke in general tends to present (it!t!e s(dden and immediate onset ofsmptoms' usuall& reac!ing ma)imal

intensit& at once%Second1 vasc(lar ris0 factors raise the

prior probabilit of disease% 4&pertensionand cardioaortic diseases are found in t!e

ma2orit& of patients (it! cerebellar infarction'and an embolic source is found in 71L15H%78'7/

Finall&' t(o easil& overlooked p!&sical signs!ave been s!o(n to indicate cerebellar

infarction%
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2791733/#b23-wjem-10-273http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2791733/#b29-wjem-10-273http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2791733/#b29-wjem-10-273http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2791733/#b23-wjem-10-273


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Tumor Cerebellum/. >erebellar )strocytoma + ==E tumor fossa posterior pada ana$.

+ 8FE tumor pediatri$.

+ rata+rata pada umur 0 tahun.

+ benigna, $isti$.

+ hemisfer vermis.


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8. edulloblastoma

+ infratentorial

+ malignan

+ cerebellar hemisfer

+ mendesa$ ventri$el $e 3hydrocephalus

Tumor cerebellum


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Tumor cerebellum$enign cerebellar tumors

Pilocytic astrocytoma, may be problematic in that

they often grow %uite large before producing

symptoms, because of the plasticity of the

cerebellum. Papilledema, an indirect sign of an

intracranial mass, may be lac$ing for a long time,

particularly in adults it is present in about 7FE of

affected children


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%n most cases 23* +4' cerebellar tumors

manifest t!emselves initiall& (it!

occipitocervical headache and na(sea

and vomitin, on an empt& stomac! #dr&

!eaves$% " forced !ead tilt is a clinical sign of

impending !erniation of t!e cerebellar tonsils

t!roug! t!e foramen magnum


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edulloblastoma is a malignant tumor that

preferentially affects children and adolescents and

accounts for one+third of all brain tumors in this age

group '1E of all brain tumors regardless of age(.

?t often arises from the roof of the fourth ventricle

and then grows into the vermian portion of the

flocculonodular lobe.


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#ecause this type of tumor often begins in the

vestibulocerebellum, its typical initial sign is

dyse*uilibrium: the affected child has a broad+based,

swaying, and staggering gait.

2urther cerebellar manifestations including ata5ia,

dysmetria, asynergia, adiadocho8inesia, andintention tremorgradually arise as the tumor grows

further and begins to affect the lateral portions of the

cerebellum 'the hemispheres(.

?n advanced stages of tumor growth, bloc$age of thefourth ventricle or of the cerebral a%ueduct causes

occlusive hydrocephalus, with clinical signs of

intracranial hypertension%


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&coustic neuroma (i.e., vestibular schwannoma).

This tumor arises from the Schwann cells of the

eighth cranial nerve 'usually its vestibular portion(

and is thus found in the cerebellopontine angle.


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5alformasi Cerebell(m


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5alformasi Cerebell(m.% "rnold-C!iari

- !erniasi tonsil cerebellum ke foramenmagnum%

- kadang menimbulkan !&drocep!alus non-communicating akibat dari obstruksi aliran

?CS%

- ge2ala klinik6

n&eri kepala' fatigue' kelema!an otot

(a2a! dan kepala' sulit menelan' diiness'nausea' gangguan koordinasi' paralisis#berat$%


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"da 1 tipe6

.% Tipe I

asimtomatik selama masa kanak-kanak'

kadang bermanifestasi dengan n&erikepala dan ge2ala cerebellar%

4erniasi tonsil cerebellar' 8 mm di ba(a!foramen magnum%

S&ringom&elia cervicot!oracic spinal cord%

iagnosa dan terapi sulit


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$a,ittal 56% 2T)4

shows cerebellartonsils -7/ cm belowforamen ma,n(m1where the C$8 spaceis narrow. There is

no srin" in thecervical cord 9 the:thventricle is normalsize 9 con!,(ration


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7% Tipe II

tonsilar !erniation di ba(a! foramenmagnum

displacement luas dari vermis?umbar m&elomeningocele


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C!iari II #"rnold-C!iariCerebellar tonsillar !erniationSmall posterior fossa


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8% Tipe III

- occipital encep!alocele%

- 2aringan neuroectodermal abnormal%

1% Tipe IV

- gangguan perkembangan cerebellum%

- cerebellum dan batang otak di dalam fossa

posterior' tapi tidak ber!ubungan denganforamen magnum%

Dand7;al0er


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Dand ;al0er

5alformation+andy3-al8er syndrome)genesis of cerebellar vermis

cystic dilatation of 3th venticle

enlargement of posterior fossa

Variable clinical manifestations


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DAND< ;A&KE6

$


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C'$LL&' &L/4'&"#42!

V'#&2 (P&L4C'$LL%4

9oubert syndrome>linical manifestations include episodic hyperpnea, ata&ia,

eye movement abnormalities, hypotonus, and retardasi

mental.

2amilial

)genesis of vermis, cystic dilatation of 3th venticle 'but

less than DS(

icroscopically normal cerebellar corte& with numerous

subcortical heterotopias



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Cara ber:alan (gait) dan berdiri (stance)

?nspe$si pasien saat berdiri. )da$ah goyangan $eti$a

berdiri, -uga cara ber-alan dysta&ia. Gntu$

meng$ompensasi $etida$stabilan dari cara berdiri

dan cara ber-alan, cerebellum memerintah$an berdiri

dan ber-alan dengan melebar$an $edua $a$i.

Pasien ber-alan melalui garis lurus. Dengan -alantandem. )mati ada$ah $ecenderungan ayunan $e

salah satu sisi.



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Pemeriksaan Cerebellum+ysta5ia lengan

/. Pasien diperintah$an untu$ melurus$an $edua

lengan secara horisontal. )mati ada$ah postural

tremor.

8. Tes telun-u$+hidung

Pasien disuruh melurus$an salah satu lengan,

$emudian menggera$$an -ari telun-u$ $e u-ung

hidung.

?nterpretasi:

#ila muncul tremor $eti$a mende$ati hidung disebut

intension tremor. Dan bila pasien gagal menyentuh

hidung secara tepat, disebut dysmetria.


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Pemeriksaan Cerebellum 4a$u$an tes tumit+tulang $ering di e$stremitasbawah.

Glang pemeri$saan = $ali bila hasil meragu$an.


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Pemeriksaan Cerebellum+ysdiado8inesia)dalah gangguan $oordinasi otot selama gera$an

cepat.

Pasien disuruh mela$u$an gera$an yangberlawanan secara cepat. isalnya pronasi dan

supinasi.

Pemeriksaan cerebellum


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Pemeriksaan cerebellum4vershooting test

/. Pasien diminta menutup mata. Aemudian

melurus$an lengan. Pemeri$sa ber$ata $epada

pasien bila pemeri$sa a$an mene$an salah satu

lengan. Pasien disuruh menahan.

?nterpretasi: normal bila lengan pasien $embali $e

posisi semula secara cepat.


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9ebound sign of 4olmes

- pemeriksa memegang pergelangan tanganpasien%

- pasien menggenggam erat tanganpemeriksa%

- kemudian tangan pemeriksa dilepaskan tiba-tiba@

- interpretasi6

normal6 lengan pasien bera&un dengan2arak minimal' dan kembali ke posisi

semula%



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Cerebellar Cognitive ">ective S&ndrome


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Cerebellar Cognitive ">ective S&ndrome

Simptom 6

Gangguan fungsi eksekutif' visuo-spasial'ba!asa' peruba!an emosi dan kepribadian%

Causa6

cerebellar agenesis' d&splasia dan !ipoplasia'stroke serebellar' tumor' serebellitis'trauma'dan pen&akit neurodegeneratif%

era2at kepara!an CC"S tergantung lokasi

dan perluasan lesi%

Cerebellar Cognitive ">ective


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S&ndrome

Fungsi cerebellum adala! bertanggung2a(abregulasi motorik' kognitif dan emosional be!aviour%

Cerebellum ber!ubungan dengan regulasi emosi%

"rea kognitif di corte) cerebri &ang berpro&eksi ke

cerebellum6.% Corte) parietal posterior #spatial a(areness$%

7% G&rus temporal superior #ba!asa$

8% "rea para!ipocampal superior #spatial memor&$

1% "sosiasi visual% Corte) prefrontal #atensi' (orking memor&'

2udgement$

>erebellar >ognitive )ffective Disorder


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>erebellar >ognitive )ffective Disorder

4esions of the posterior corte&and vermis?mpairment of e&ecutive functions

Planning, verbal fluency, abstract reasoningDifficulties with spatial cognition

Visuo+spatial organi*ation, visual memory

Personality changes#lunting of affect, inappropriate behaviors

4anguage disorders)grammatism, disprosodia


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The cerebellar circuits are involved in many aspects

of memory, in particular, in nondeclarative memory.

This latter includes procedural learning (s8ills and

habits), priming and perceptual learning, basic

associative learning'including simple classical

conditioning of emotional and s$eletal muscle

responses(, and non3associative learning

Pschiatric Aspects of Cerebellar


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Disorders

#.$ Cerebellar Cognitive ">ective S&ndrome

#7$ "natomicall& Speci;c Ps&c!iatric "spectsof Cerebellar isorders

#8$ Dt!er Ps&c!iatric "spects of Cerebellarisorders

;. Cerebellar Cognitive &ffective !yndrome

(S h h 5 Sh /001(


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(Schmahman 5 Shermen, /001(.

Cerebellar lesions in ,enerale%g% acAuired lesions'congenital cerebellar malformations' cerebellar tumourresection' etc can cause motor impairments pl(s thefollowin,#Sc!ma!man et al' 755R Tavano et al' 755R ?eviso!net al' 7555$

Co,nitive impairments6 E"ec(tive dsf(nctionse%g% in (orking memor& and

planning 'is(o7spatial abnormalitiese%g% in visual memor& and

visuo-spatial organisation

&in,(istic dsf(nctione%g% d&sprosodia' agrammatism andanomia

A>ective impairments6 an)iet&' let!arg&' depression' lack of empat!&'

ruminativeness' perseveration' an!edonia and aggression

'8( )natomically Specific Psychiatric )spects of


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>erebellar Disorders

'ermal A,enesis severe ?' "utism 3abnormal motor development #Tavano et al' 755R$%

'ermal lesions a>ective and relational

disorders #Sc!ma!man et al' 755R$%$pinocerebellar Ata"ia impairment in

attention' memor&' e)ecutive functions andt!eor& of mind #Garard et al' 7550$%


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(


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"ssessment6

#.$ Motor disorders in ps&c!iatric disordersas signs of cerebellar d&sfunctioning

#7$ +on-motor s&mptoms eAuivalent tomotor s&mptoms related to cerebellum

Treatments6

#8$ Cerebellar e)ercises

#1$ Transcranial Magnetic Stimulation #TMS$ #$ 9outine disorders

+&stagmus


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& gT!e localiing signi;cance of n&stagmus is often a

mere indication of d&sfunction some(!ere in t!e

posterior fossa #i%e%' vestibular end-organ' brain

stem' or cerebellum$% 4o(ever' certain n&stagmus

patterns are Auite speci;c and permit reasonabl&

accurate neuroanatomic diagnosis% =!en

possible' t!e speci;c and nonspeci;c forms are

separated on t!e basis of clinical appearance and

associated signs and s&mptoms%


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o(nbeato(nbeat n&stagmus is de;ned as n&stagmusgae position (it! t!e fast p!ase beating in ado(n(ard direction% Patients (it! brain stemdisease or drug into)ications usuall& lack gae-evoked do(n(ard n&stagmus despite n&stagmusin all ot!er ;elds of gae% T!us' n&stagmusbeating do(n(ard in t!e primar& position is astriking p!enomenon and is !ig!l& suggestive of a

disorder of t!e craniocervical 2unction' suc! as"rnold-C!iari malformations


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pbeatPrimar&-position n&stagmus (it! t!e fast p!asebeating up(ard rarel& re*ects drug into)ication%Most often' t!e n&stagmus is acAuired andindicates str(ct(ral disease1 (s(all of thebrain stem% T!e location of t!e lesions inpatients (it! upbeat n&stagmus after meningitis'=ernickes encep!alopat!&' or organop!osp!atepoisoning is uncertain% =it! convergence' upbeat

ma& en!ance or convert to do(nbeat%T!e slo(-p!ase (aveform is usuall& linear but ma& be anincreasing-velocit& e)ponential%

P


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V


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Gae evoked n&stagmus6

Dne of t!e most common forms of centraln&stagmus

Inabilit& to maintain eccentric gae Uleak& integrator -- miscalibration bet(een pulse

and step inputs

S&mmetric cerebellar *occulus implicated "ge' anti-convulsant t!erap&' alco!olic

degeneration' stroke' dem&elination ,aclofen e>ective


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o(nbeat n&stagmus6

efect in vertical gae !olding "s&mmetric inputs from vertical semi-circular canals

produce up(ard slo( drift of e&esefect in fastigial nuclei calibration Secondar& do(n(ard corrective fast p!aseDbe&s "le)ander@s la( ?ocalies to cervico-medullar& 2unction "rnold-C!iari malformationTreatment (it! baclofen' clonaepam' base-out

prisms


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pbeat n&stagmus6

Present in primar& position or upgae Classicall& localies to a lesion of anterior cerebellar vermis More generall& implicates posterior fossa disease

ective


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#runs nystagmus:

associated with >P) tumors

high fre%uency, low amplitude

nystagmus 'fast+phase away from lesion(

low fre%uency, large amplitude

nystagmus on ipsilateral ga*e 'fast phase

toward lesion(

shift from eye movement response to

vestibular imbalance to that of defective

ga*e holding


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See-sa( n&stagmus6

iscon2ugate vertical n&stagmus #pendular vs% 2erk$ p(ard moving e&e intorts (!ile do(nard e&e e)torts ?ocalies to lesions of diencep!alon Visual ;elds ma& be useful #disruption of a>erents to cerebellum$

Dcular *utterEopsoclonus6

,urst-like' incoordinated saccadic e)cursions (it! !ig! freAuenc&' lo(amplitude

+o intersaccadic latenc&

Purel& !oriontal6 ocular *utter Multiplanar6 opsoclonus 9e*ect pause cell d&sfunction #pons$ Must consider paraneoplastic etiolog&6 SCC of lung' ovarian' breast C" +euroblastoma in c!ildren


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=ernicke@s S&ndrome"kut

"bnormal e&e movements

"ta)ia

ConfusionCausa 6 de;siensi t!iamin e%c poor nutrition

#"IS'!&peremesis gravidarum't!&roto)icosis'C4F (it! longterm diuretict!erap&$

9educed cerebral metabolism a)onal

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