resident tutorial: ataxia

Surat Tanprawate, MD, FRCPTNorthern Neuroscience Center

Chiangmai University

Resident Tutorial Long case Board

Examination

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“Fear factors in Long Case Neuro Examination”

and“How to approach to ataxia”

By

Dr. Surat

Present

Fear Factors

• Hard to get neurological history

• Hard to do the physical neurological exam

• Hard to conclude the results

• Hard to make neurological diagnosis

Fear Factors

• Hard to get neurological history

• Hard to do the physical neurological exam

• Hard to conclude the results

• Hard to make neurological diagnosis

Symptomatology

Practice the physical exam

Practice to list the problem

To know the common diseases

Key Concept

Symptoms approach

“Algorithm”

Key Concept

Symptoms&Signs

“Specific examination”

Key Concept

To diagnose

“Problem list and Conclusion”

Symptomatology

Syndrome

• Disorder of consciousness

• Level of consciousness

• Content of consciousness

• Mental disorder• Memory• Intelligence• Personality• Behavioral • Dementia

• Visual disorder• Visual loss

• Ocular motility disorder

• Diplopia

• Abnormal ocular ossilation

Symptomatology

Lower cranial nerve disorder

• Deafness/tinnitus

• Vertigo

• Balance/staggering

• Swallowing

• Voice change

Symptomatology

Multiple Cranial Nerve Disorders

• Sensory disorder• Pain disorder

• Headache and facial pain• Others pain disorder

• Numbness/tingling

• Motor disorder • Weakness• Movement disorder• Gait abnormality• Ataxic disorder

• Sphincter disorder

• Episodic disorder– Seizure/epilepsy– Syncope– TIA– Abnormal movement– Migraine

Symptomatology

Syndrome• Amnistic and Dementia syndrome

• Neuro-opthalmology syndrome

• Syndrome of Multiple cranial nerve disorder

• Stroke syndrome

• Cortical stroke syndrome

• Lacunar stroke syndrome

• Brain stem stroke syndrome

• Spinal cord syndrome

Ataxia

Algorithm

Specific examination

Conclusion and Diagnosis

Ataxia

Gait abnormality

Specific examination-confirm ataxic disorder-for categorized ataxia

GreekwordA=nega.ve

Taxi=order

Ataxic symptoms?-Nystagmus-Dysarthria-Trunkcal ataxia-Limb and gait -ataxia

Ataxic symptoms mimicker?

Ataxia: disease other than cerebellum

Cerebellar’s disease-Where’s the lesion (cerebellum, cerebellar peduncle, cerebellar tract) -What’s the lesion

True Ataxia• Mildweakness• Apraxia• Abnormalmovement• Gaitabnormality

AtaxiaAlgorithm

NeuroanatomyNeurophysiology

• Coordinating skill voluntary movement

• Muscle activity

• Control equilibrium

• Muscle tone

Function of cerebellum

• Lesion

• Incoordination (ataxia) of volitional movement

• Tremor (ataxic or intention tremor)

• Disorder of equilibrium and gait

• Diminish muscle tone

Function of cerebellum

Toa7ributedsensorimotornetwork:

• cerebralcortex

• basalmotornuclei

• thalamus

• re:cularforma:on

Cerebellar pathway

Func.onalZone

Functional divisions

Deep Nuclei Connections Functions

Vermis(face, proximal

body)

Fastigial Vestibular nucleusReticular formationMedial descending system

Axial and proximal muscle controlProgressive movement

Intermediate zone (spinal cord)

Interposed Red nucleusMotor cortexLateral descending system

Distal muscle controlProgressive movement

Lateral zone (cortex, pons)

Dentate Red nucleusThalamusMotor, premotor cortex

Motor planningInitiationTiming

Flocculonodular lobe

Vestibular nuclei, visual system

Vestibular nuclei Axial equilibriumEye movementsVestibular reflexes

Part of Cerebellum

Cerebellar peduncles

Tracts connect to brain stem

Major pathways Connections

Superior Brachium conjunctivuum

Afferent Efferent

Rubral, thalamic, Dentate, spinal cord

Middle Brachium pontis Afferent only

Pontine nuclei

Inferior Restiform body Afferent Efferent

Vestibular, olive, Spinal cordReceives from Flocculonodular lobe

BasicAnatomy–Cerebellarpeduncles

Blood Vessel

Ataxic symptoms?-Nystagmus-Dysarthria-Trunkcal ataxia-Limb and gait -ataxia

Ataxic symptoms mimicker?

Ataxia: disease other than cerebellum

Cerebellar’s disease-Where’s the lesion (cerebellum, cerebellar peduncle, cerebellar tract) -What’s the lesion

True Ataxia• Mildweakness• Apraxia• Abnormalmovement• Gaitabnormality

AtaxiaAlgorithm

What is the cerebellar syndrome?

What is the associated signs?

Pure cerebellum

ClassifiedCerebellarsyndrome

WithBrainstem

signs

ClassifiedBrainstemSyndrome?

With mild hemiparesis

Involve fronto-Ponto-

CerebellarPathway“Ataxic

hemiparesis”

Associated signs

Cerebellar hemispheric syndrome

Rostral vermis

syndrome

Caudal vermis

syndrome

Pancerebellar syndrome

Unilateral intermediate, lateral zones

Ant, sup vermis

Flucculonodular,

post vermis

All regions

Classifiedcerebellarsyndrome

Cerebellarsyndromes

Regionsinvolved

Distribu.onsofdeficits

Commoncauses

Cerebellarhemispheresyndrome

Cerebellar hemisphere

Ipsilateralhead&body

Infarct,neoplasm,abscess,demyelina:on

Rostralvermissyndrome

Ant,supvermis Gait,trunk Alcoholism,thiaminedef

Caudalvermissyndrome

Flucculonodular,postvermis

Axialdiisequilibrium

Midlineneoplasm

Pancerebellarsyndrome

Allregions Bilateralsymmetricalsignsofcerebellardysfunc:on

Toxic,metabolic,infec:ous,paraneoplas:c,degenera:ve

Cerebellar syndrome and its disorders

Posterior inferior cerebellar artery (PICA)Anterior inferior cerebellar artery (AICA)

Superior cerebellar artery (SCA)Posterior cerebral artery (PCA)

PICA = lateral medulla & inferior cerebellumAICA = lateral caudal pons & part of cerebellum

SCA = superior cerebellum & rostral laterodorsal ponsPCA = midbrain, thalamus, medial surface of occipital lobe, inferior and medial

surfaces of temporal lobe

Brainstem Vascular Territories

SYNDROME STRUCTURES DAMAGED CLINICAL SYMPTOM

Lateral medullary (PICA, Wallenberg’s)syndrome

Damage Level:

Lateral medullar

Corticospinal tract(pyramid)

Contralateral hemiplegia

Trigerminal spinal nucleus

Ipsilateral facial hemisensory loss

Ipsilateral palatal, pharyngeal, vocal cord paralysisDysarthria, dysphagia

Spinothalamic tract Contralateral hemisensory loss

Vascular supplyPICA

Nucleus ambiguous

Sympathetic fiber Ipsilateral Horner’s syndrome

Vertigo, N/VVestibular nuclei

Cerebellum Ipsilateral cerebellar sign

SYNDROME STRUCTURES DAMAGED CLINICAL SYMPTOM

Lateral inferior pontine syndrome

(AICA stroke syndrome)Damage Level:

Lateral inferior pons

Vestibular nucleus Ipsilateral vertigo, N/V, nystagmus

Contralateral hemisensory loss

Nuclear of CN7 Ipsilateral facial palsy

Ipsilateral deafnessCochlear nucleus

Cerebellum Ipsilateral ataxia

Spinothalamic tract

CN 5 Ipsilateral hemisensory loss of face

Neurological examination in

ataxia

Nystagmus

Patient Video

Intention tremor

Patient Video

Finger to Nose Test

Patient Video

Ataxic gait

Patient Video

Ataxic speech

Patient Video

What’s lesion?

Time, Caused, OnsetMedical History

Neurological Signs

• Sudden

• Vascular

• Acute

• Intoxication

• Viral, Post infectious

Time Caused Onset

• Subacute

• Intoxication

• Chronic

• Hereditary

• Paraneoplastic syndrome

• Hypothyroid

• Intoxication

• Acquired

• Wernicke’s encephalopathy

• Miller Fisher syndrome

• Hereditary

• SCA

• FA

Symmetrical ataxia plus syndrome

Imaging • to exclude identifiable

structural lesion• Atrophy of cerebellum or

spinal cord

AD

Acquired causes-Alcohol/medication

-Hypothyroid-Vit.B12-Anti-HIV

-Paraneoplastic study-GAD Ab

Evaluate •Accurate family history• Phenotype

Singleton patient

AR

SCA1,2MJD

SCA6,7SCA10,12DRPLASCA17

FAAT

AVEDAbetalipoproteinemia

AOAMitochondrialdisorder

Pt.withprogressiveataxia

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Next Episode: Neuro-opthalmology approachDiplopia, Visual loss, Ptosis