ataxia and dizziness jesse sturm, md pediatric fellow’s conference june 25, 2008

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Ataxia and Ataxia and Dizziness Dizziness Jesse Sturm, MD Jesse Sturm, MD Pediatric Fellow’s Pediatric Fellow’s Conference Conference June 25, 2008 June 25, 2008

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Ataxia and DizzinessAtaxia and Dizziness

Jesse Sturm, MDJesse Sturm, MD

Pediatric Fellow’s ConferencePediatric Fellow’s Conference

June 25, 2008June 25, 2008

OutlineOutline

DefinitionsDefinitions AtaxiaAtaxia

CausesCauses Workup – labs and specific exam findingsWorkup – labs and specific exam findings

DizzinessDizziness CausesCauses Algorithmic approachAlgorithmic approach

ConclusionConclusion

DefinitionsDefinitions

Ataxia: disturbance in smooth accurate Ataxia: disturbance in smooth accurate coordination of movements, unsteady gaitcoordination of movements, unsteady gait

Dizziness: non specific termDizziness: non specific term Includes vertigo, disequilibrium, pre-syncopeIncludes vertigo, disequilibrium, pre-syncope Vertigo – symptom of illusory movement, sense of Vertigo – symptom of illusory movement, sense of

swaying or tiltingswaying or tilting Some perceive self-movement, others perceive motion of the Some perceive self-movement, others perceive motion of the

environmentenvironment Due to asymmetry in vestibular system (labyrinth, central Due to asymmetry in vestibular system (labyrinth, central

structures in brainstem)structures in brainstem) Vertigo is a symptom, not a diagnosisVertigo is a symptom, not a diagnosis

AtaxiaAtaxia

Ataxia: Ataxia: ataktosataktos – “lacking order” (Greek) – “lacking order” (Greek) Disturbance in smooth accurate movements – Disturbance in smooth accurate movements –

commonly unsteady gaitcommonly unsteady gait Often result of cerebellar dysfunctionOften result of cerebellar dysfunction

Disturbance at multiple sensory levels can affect Disturbance at multiple sensory levels can affect coordinationcoordination i.e. loss of proprioception = sensory ataxiai.e. loss of proprioception = sensory ataxia

Acute ataxia is rare, most often benign Acute ataxia is rare, most often benign presenting complaintpresenting complaint

CerebellumCerebellum

A: midbrainA: midbrain B: ponsB: pons C: medullaC: medulla D: spinal cordD: spinal cord E: 4E: 4thth ventricle ventricle G: tonsilG: tonsil H: ant lobeH: ant lobe I: post lobeI: post lobe

CerebellumCerebellum

Vermis - midlineVermis - midline dysarthriadysarthria truncal titubationtruncal titubation symmetric ataxiasymmetric ataxia

HemispheresHemispheres ipsilateral limb ipsilateral limb dysmetriadysmetria hypotoniahypotonia tremortremor ataxia in direction ataxia in direction of affected hemisphereof affected hemisphere

Causes of AtaxiaCauses of Ataxia

Review of 80 admitted pediatric cases:Review of 80 admitted pediatric cases: 80% of acute ataxias had diagnosis of acute 80% of acute ataxias had diagnosis of acute

cerebellar ataxia, toxic ingestion, Guillaine-cerebellar ataxia, toxic ingestion, Guillaine-Barre syndromeBarre syndrome

Gieron-Korthals, MA. Acute ataxia in childhood: a 10-year experience. J. Child Neurology 1994: 9:381.

Differential of Acute AtaxiaDifferential of Acute AtaxiaInfectious/immune mediated disordersInfectious/immune mediated disorders

Acute cerebellar ataxiaAcute cerebellar ataxia

ADEMADEM

Systemic infectionsSystemic infections

Brainstem encephalitisBrainstem encephalitis

Multiple SclerosisMultiple Sclerosis

Toxic: alcohol and drug relatedToxic: alcohol and drug related

Mass lesionsMass lesions

TumorTumor

Vascular lesionsVascular lesions

AbscessesAbscesses

HydrocephalusHydrocephalus

TraumaTrauma

Cerebellar contusion or hemorrhageCerebellar contusion or hemorrhage

Posterior fossa hematomaPosterior fossa hematoma

Post-concussion syndromePost-concussion syndrome

Vertebrobasilar dissectionVertebrobasilar dissection

StrokeStroke

Vertebrobasilar dissection or Vertebrobasilar dissection or thromboembolismthromboembolism

Cerebellar hemorrhageCerebellar hemorrhage

Paraneoplastic disordersParaneoplastic disorders

Opsoclonus-myoclunus syndromeOpsoclonus-myoclunus syndrome

Sensory ataxiaSensory ataxia

Guillain-Barre syndromeGuillain-Barre syndrome

Miller Fisher syndromeMiller Fisher syndrome

Paretic ataxiaParetic ataxia

Upper motor neuron syndromeUpper motor neuron syndrome

Lesions of frontal lobeLesions of frontal lobe

Lower motor neuron syndromeLower motor neuron syndrome

Spinal cord Spinal cord

transverse myelitis, cord compressiontransverse myelitis, cord compression

Peripheral nervePeripheral nerve

GBS, MF, tick paralysisGBS, MF, tick paralysis

Inborn errors of metabolismInborn errors of metabolism

Basilar MigrainesBasilar Migraines

Non-convulsive seizuresNon-convulsive seizures

Wernicke’s encephalopathyWernicke’s encephalopathy

Causes of Acute AtaxiaCauses of Acute Ataxia

Life threatening conditionsLife threatening conditions Tumors, Stroke, InfectionTumors, Stroke, Infection

Common conditionsCommon conditions Acute cerebellar ataxia, GBS, Labyrinthitis, Acute cerebellar ataxia, GBS, Labyrinthitis,

Toxins, Migraine syndromes, TraumaToxins, Migraine syndromes, Trauma

Rare disordersRare disorders

Causes of Acute AtaxiaCauses of Acute Ataxia

Life threatening conditionsLife threatening conditions Tumors, Stroke, InfectionTumors, Stroke, Infection

Common conditionsCommon conditions Acute cerebellar ataxia, GBS, Labyrinthitis, Acute cerebellar ataxia, GBS, Labyrinthitis,

Toxins, Migrane syndromes, TraumaToxins, Migrane syndromes, Trauma

Rare disordersRare disorders

Ataxia - TumorsAtaxia - Tumors

45-60% of all childhood brain tumors arise 45-60% of all childhood brain tumors arise in brainstem or cerebellumin brainstem or cerebellum Can present with progressive ataxiaCan present with progressive ataxia Symptoms of increased ICPSymptoms of increased ICP

Papilledema, cranial neuropathies, HA, emesisPapilledema, cranial neuropathies, HA, emesis

Rarely midline supratentorial tumorsRarely midline supratentorial tumors Opsoclonus-Myoclonus (rapid dancing eye Opsoclonus-Myoclonus (rapid dancing eye

movements and rhythmic jerking)movements and rhythmic jerking) Paraneoplastic - neuroblastoma in up to 50%Paraneoplastic - neuroblastoma in up to 50%

Ataxia - StrokeAtaxia - Stroke

Hemmorhage into cerebellum or posterior fossa Hemmorhage into cerebellum or posterior fossa from trauma or vascular malformationfrom trauma or vascular malformation

Vertebral or basilar artery diseaseVertebral or basilar artery disease Sickle cellSickle cell Hypercoagulable statesHypercoagulable states Vertebrobasilar artery Vertebrobasilar artery

dissection following neck dissection following neck

injury can present as injury can present as

acute ataxiaacute ataxia

Ataxia - InfectionAtaxia - Infection

Cerebellar abscesses – contiguous spread from Cerebellar abscesses – contiguous spread from ASOM or mastoiditisASOM or mastoiditis Ataxia/fever +/- signs of increased ICPAtaxia/fever +/- signs of increased ICP

Brainstem encephalitisBrainstem encephalitis CNeuropathies, AMS, seizuresCNeuropathies, AMS, seizures Causes: listeria, lyme disease, EBV, HSVCauses: listeria, lyme disease, EBV, HSV CSF pleocytosisCSF pleocytosis

Acute post-infectious demyelinating Acute post-infectious demyelinating encephalomyelitis (ADEM), multiple sclerosisencephalomyelitis (ADEM), multiple sclerosis Seizures, CNeuropathies, weakness, sensory deficits, Seizures, CNeuropathies, weakness, sensory deficits,

transverse myelitistransverse myelitis

Causes of Acute AtaxiaCauses of Acute Ataxia

Life threatening conditionsLife threatening conditions Tumors, Stroke, InfectionTumors, Stroke, Infection

Common conditionsCommon conditions Acute cerebellar ataxia, GBS, Labyrinthitis, Acute cerebellar ataxia, GBS, Labyrinthitis,

Toxins, Migrane syndromes, TraumaToxins, Migrane syndromes, Trauma

Rare disordersRare disorders

Acute Cerebellar Ataxia (ACA)Acute Cerebellar Ataxia (ACA)

Post infectious cerebellar demyelination and/or direct Post infectious cerebellar demyelination and/or direct cerebellar infection (seen on MRI)cerebellar infection (seen on MRI) 35% of acute childhood ataxia35% of acute childhood ataxia Autoimmune phenomena against cerebellar epitopesAutoimmune phenomena against cerebellar epitopes

Onset 5-10 days after precipitating infection (70%)Onset 5-10 days after precipitating infection (70%) Peak age 2-4yo (case series ages 1.5yo – 12.5yo)Peak age 2-4yo (case series ages 1.5yo – 12.5yo)

Symptoms maximal at onsetSymptoms maximal at onset Truncal ataxia severe, extremity ataxia < trunkTruncal ataxia severe, extremity ataxia < trunk

Seen in sitting positionSeen in sitting position

Vomiting, horizontal nystagmus, dysarthria may occurVomiting, horizontal nystagmus, dysarthria may occur Mental status normal, no fever, no meningismusMental status normal, no fever, no meningismus

Acute Cerebellar Ataxia (ACA)Acute Cerebellar Ataxia (ACA)

Most common findings on exam are Most common findings on exam are nystagmus and dysmetria (50%)nystagmus and dysmetria (50%)

Small retrospective study (n=39):Small retrospective study (n=39): Mean CSF WBC 16 (0-40)Mean CSF WBC 16 (0-40)

>5 WBC in 48%, all with lymph predominance>5 WBC in 48%, all with lymph predominance Mean CSF protein 20 (>40 in 23%)Mean CSF protein 20 (>40 in 23%) CT done in 14 patients, all normalCT done in 14 patients, all normal

Recent studies show + MRI findings in Recent studies show + MRI findings in classic ACAclassic ACA

Acute Cerebellar Ataxia (ACA)Acute Cerebellar Ataxia (ACA)

Varicella implicated in >25% casesVaricella implicated in >25% cases Rare cases due to VZV vaccineRare cases due to VZV vaccine

Echovirus, EBV, Measles, Mumps, HSV, Echovirus, EBV, Measles, Mumps, HSV, ParvovirusParvovirus

MMR vaccine implicated in rare casesMMR vaccine implicated in rare cases

Acute Cerebellar Ataxia (ACA)Acute Cerebellar Ataxia (ACA)

Symptoms take several weeks to resolveSymptoms take several weeks to resolve Mean ~ 1.5 weeksMean ~ 1.5 weeks Complete recovery in >90% patientsComplete recovery in >90% patients

Ataxia symmetricAtaxia symmetric Findings in cerebellar ataxia remain Findings in cerebellar ataxia remain

unchanged whether eyes open or closedunchanged whether eyes open or closed No evidence that immunosupressive No evidence that immunosupressive

therapies improve outcomestherapies improve outcomes

Acute Cerebellar Ataxia (ACA)Acute Cerebellar Ataxia (ACA)

Clinical features do not distinguish from Clinical features do not distinguish from other causes of acute ataxiaother causes of acute ataxia

Diagnosis of exclusionDiagnosis of exclusion

Ataxia - Guillain-Barre SyndromeAtaxia - Guillain-Barre Syndrome

Ascending paralysis, areflexia, progressiveAscending paralysis, areflexia, progressive 15% of children with GBS also lose sensory 15% of children with GBS also lose sensory

input to cerebellum --- develop sensory ataxiainput to cerebellum --- develop sensory ataxia + Romberg, dec DTR+ Romberg, dec DTR

Miller Fisher syndrome: GBS with triad of Miller Fisher syndrome: GBS with triad of ataxia, areflexia, opthalmoplegiaataxia, areflexia, opthalmoplegia

Ataxia - LabyrinthitisAtaxia - Labyrinthitis

Inflammation of vestibular Inflammation of vestibular apparatusapparatus Bacterial or viralBacterial or viral

Symptoms of hearing loss, Symptoms of hearing loss, vomiting, extreme vertigovomiting, extreme vertigo

Vertigo often exacerbated Vertigo often exacerbated by head movementsby head movements

Dix-Hallpike maneuverDix-Hallpike maneuver

Ataxia - Toxin ExposureAtaxia - Toxin Exposure

Responsible for up to 30% acute ataxiaResponsible for up to 30% acute ataxia Anticonvulsants – phenytoin, carbamazepine, Anticonvulsants – phenytoin, carbamazepine,

phenobarbitol, antihistaminesphenobarbitol, antihistamines Lead, carbon monoxide, inhalants, Etoh, Lead, carbon monoxide, inhalants, Etoh,

BenzosBenzos Usually accompanied by AMSUsually accompanied by AMS

Ataxia - Migraine SyndromesAtaxia - Migraine Syndromes

Basilar migraines and familial hemiplegic Basilar migraines and familial hemiplegic migraine syndromes present with ataxiamigraine syndromes present with ataxia

Associated headache and vomiting Associated headache and vomiting distinguish from other acute ataxiasdistinguish from other acute ataxias Visual auras commonVisual auras common

Ataxia - TraumaAtaxia - Trauma

Post concussive ataxiaPost concussive ataxia Directed traumatic force to labyrinth structuresDirected traumatic force to labyrinth structures May be associated with hemotympanum and May be associated with hemotympanum and

temporal fracturestemporal fractures

Causes of Acute AtaxiaCauses of Acute Ataxia

Life threatening conditionsLife threatening conditions Tumors, Stroke, InfectionTumors, Stroke, Infection

Common conditionsCommon conditions Acute cerebellar ataxia, GBS, Labyrinthitis, Acute cerebellar ataxia, GBS, Labyrinthitis,

Toxins, Migrane syndromes, TraumaToxins, Migrane syndromes, Trauma

Rare disordersRare disorders

Ataxia – Rare CausesAtaxia – Rare Causes Tick paralysisTick paralysis

unsteady gait, ascending paralysis/weakness, areflexiaunsteady gait, ascending paralysis/weakness, areflexia neurotoxin in tick salivaneurotoxin in tick saliva

HypoglycemiaHypoglycemia Seizure disorderSeizure disorder

simple non-convulsive seizures may manifest as ataxia alonesimple non-convulsive seizures may manifest as ataxia alone Conversion disorderConversion disorder

narrow gait, elaborate near fallsnarrow gait, elaborate near falls Inborn error metabolismInborn error metabolism

Urea cycle, aminoacidopathies (MSUD), organics acidemiasUrea cycle, aminoacidopathies (MSUD), organics acidemias Congenital anomoliesCongenital anomolies

Chiari malformation, encephaloceles, cerebellar aplasia/hypoplasiaChiari malformation, encephaloceles, cerebellar aplasia/hypoplasia Genetic conditionsGenetic conditions

ataxia telangectasia etc.ataxia telangectasia etc.

Diagnostic workupDiagnostic workup

Temporal courseTemporal course Acute, episodic, chronicAcute, episodic, chronic

Associated neurological findingsAssociated neurological findings HistoryHistory PEPE

Targeted diagnostic Targeted diagnostic

workupworkup

Ataxia – Temporal CourseAtaxia – Temporal Course

Rapid onset: traumatic, infectious or post-Rapid onset: traumatic, infectious or post-infectious, or toxic etiologyinfectious, or toxic etiology

Progressive onset (few days): metabolic Progressive onset (few days): metabolic syndromes, GBSsyndromes, GBS

Insidious onset (days to weeks): brainstem Insidious onset (days to weeks): brainstem and cerebellar tumorsand cerebellar tumors

HistoryHistory

Recent infection, vaccinationRecent infection, vaccination Previous episode of ataxia Previous episode of ataxia

Migraine-related syndrome, seizure, IEMMigraine-related syndrome, seizure, IEM

Family historyFamily history Migraine syndromes, hereditary ataxias, IEMMigraine syndromes, hereditary ataxias, IEM

Concurrent SymptomsConcurrent Symptoms

Otalgia, vertigo, vomitingOtalgia, vertigo, vomiting Suggest labyrinthitis, often see nystagmusSuggest labyrinthitis, often see nystagmus

Recurrent headaches, behavior changesRecurrent headaches, behavior changes May represent increased ICPMay represent increased ICP

Abnormal mental statusAbnormal mental status Mass lesions, CNS infection, toxin exposure, Mass lesions, CNS infection, toxin exposure,

trauma (head/neck), stroke, inborn error trauma (head/neck), stroke, inborn error metabolismmetabolism Access to drugs of abuse, ethanol, anticonvulsantsAccess to drugs of abuse, ethanol, anticonvulsants

Physical ExamPhysical Exam

Vitals: bradycardia, HTN, resp pattern, feverVitals: bradycardia, HTN, resp pattern, fever Anterior fontanelleAnterior fontanelle Ipsilateral head tilt (posterior fossa tumor)Ipsilateral head tilt (posterior fossa tumor) PapilledemaPapilledema Nystagmus (vestibular, cerebellar, brainstem disorder)Nystagmus (vestibular, cerebellar, brainstem disorder) Opsoclonus (occult neuroblastoma)Opsoclonus (occult neuroblastoma) AOM, hearing loss +/- vomiting/vertigo (acute labyrinthitis)AOM, hearing loss +/- vomiting/vertigo (acute labyrinthitis) MeningismusMeningismus Healing rash/viral exanthemHealing rash/viral exanthem Tick attachmentTick attachment

Neurologic ExamNeurologic Exam General mental statusGeneral mental status

AMS suggests ADEM, CNS infection, stroke, ingestionAMS suggests ADEM, CNS infection, stroke, ingestion Cranial neuropathiesCranial neuropathies

Suggest posterior fossa lesion, encephalitis, GBS with MFSSuggest posterior fossa lesion, encephalitis, GBS with MFS Motor examMotor exam

““paretic ataxia” -if weak may stagger to compensateparetic ataxia” -if weak may stagger to compensate GBS, Botulism, transverse myelitis, myasthenia, tick paralysisGBS, Botulism, transverse myelitis, myasthenia, tick paralysis Check reflexes, strengthCheck reflexes, strength

Sensory examSensory exam Proprioceptive input may cause ataxia (seen in GBS)Proprioceptive input may cause ataxia (seen in GBS) Romberg test – when close eyes remove visual compensationRomberg test – when close eyes remove visual compensation

Cerebellar examCerebellar exam May be normal even with specific lesionsMay be normal even with specific lesions

Cerebellar ExamCerebellar Exam

Gait, Speech, Coordination Gait, Speech, Coordination i.e. DRUNKi.e. DRUNK

Gait – wide based, unsteady, Gait – wide based, unsteady, lurchinglurching Titubation – difficulty with Titubation – difficulty with

truncal positiontruncal position

Speech – clarity, rhythm, Speech – clarity, rhythm, tone, volumetone, volume

Coordination – Coordination – over/undershooting on FTN, over/undershooting on FTN, difficulty with RAM difficulty with RAM (dysdiadochokinesia)(dysdiadochokinesia)

Diagnostic TestingDiagnostic Testing Toxicology ScreenToxicology Screen

Drug of abuse, specific drug levelsDrug of abuse, specific drug levels 35% of UDS were + in one retrospective series in children (n=90) 35% of UDS were + in one retrospective series in children (n=90)

(Gieron-Korthals, 1994), HIGHEST YIELD(Gieron-Korthals, 1994), HIGHEST YIELD GlucoseGlucose Metabolic EvaluationMetabolic Evaluation

Especially for acute episodic ataxia to identify IEMEspecially for acute episodic ataxia to identify IEM Serum lactate, pyruvate, amino acids, ammonia, pHSerum lactate, pyruvate, amino acids, ammonia, pH

CSF examinationCSF examination Rarely indicated unless clinically concerned for meningoencephalitisRarely indicated unless clinically concerned for meningoencephalitis Moderate protein elevation and pleocytosis occurs in 25-50% ACA, Moderate protein elevation and pleocytosis occurs in 25-50% ACA,

ADEM, MS, GBSADEM, MS, GBS Cytoalbuminologic dissociation in GBS (high protein >40, low cells<10)Cytoalbuminologic dissociation in GBS (high protein >40, low cells<10)

NeuroimagingNeuroimaging Prior to LP if any concern for increased ICPPrior to LP if any concern for increased ICP

ImagingImaging

Obtain for acute ataxia with:Obtain for acute ataxia with: AMS, focal neuro signs, cranial neuropathies, AMS, focal neuro signs, cranial neuropathies,

asymmetry of ataxia, history of trauma, concern for asymmetry of ataxia, history of trauma, concern for mass lesion, no improvement in 1-2wksmass lesion, no improvement in 1-2wks

MRI MRI superior for posterior fossa lesionssuperior for posterior fossa lesions demyelinating disease better visualizeddemyelinating disease better visualized

CTCT conditions needing urgent interventionconditions needing urgent intervention

EEG and EMGEEG and EMG

EEG if concerned concurrent seizureEEG if concerned concurrent seizure Obtain if fluctuating clinical signsObtain if fluctuating clinical signs 60% of children with ACA will have abnormal 60% of children with ACA will have abnormal

EEG, epileptiform activity or slowingEEG, epileptiform activity or slowing

EMG sensitive tests for GBS (sensory EMG sensitive tests for GBS (sensory ataxias), may not be helpful early in ataxias), may not be helpful early in diseasedisease EMG findings in 90%EMG findings in 90%

Algorithmic ApproachAlgorithmic Approach

Algorithmic ApproachAlgorithmic Approach

DizzinessDizziness

Dizziness: non specific termDizziness: non specific term Includes vertigo, disequilibrium, pre-syncopeIncludes vertigo, disequilibrium, pre-syncope Vertigo – symptom of illusory Vertigo – symptom of illusory

movement/rotation, sense of swaying or tiltingmovement/rotation, sense of swaying or tilting Some perceive self-movement, others perceive Some perceive self-movement, others perceive

motion of the environmentmotion of the environment Due to asymmetry in vestibular system (labyrinth, Due to asymmetry in vestibular system (labyrinth,

central structures in brainstem)central structures in brainstem) Vertigo is a symptom, not a diagnosisVertigo is a symptom, not a diagnosis

VertigoVertigo

True vertigoTrue vertigo Subjective sense of rotation of environment relative to Subjective sense of rotation of environment relative to

patient or patient to environmentpatient or patient to environment Acute attacks often accompanied by nystagmusAcute attacks often accompanied by nystagmus

PseudovertigoPseudovertigo Complaints of lightheadedness, flushing, weakness, Complaints of lightheadedness, flushing, weakness,

ataxia, unsteadiness, pallor, anxiety, stress, fearataxia, unsteadiness, pallor, anxiety, stress, fear

True VertigoTrue Vertigo

Disturbance of peripheral or central components Disturbance of peripheral or central components of vestibular systemof vestibular system

CN8 carries impulses to nuclei in cerebellumCN8 carries impulses to nuclei in cerebellum Additional impulses carried to CN 3,4,6Additional impulses carried to CN 3,4,6 Almost all patients have fast component of nystagmus in Almost all patients have fast component of nystagmus in

same direction as perceived rotationsame direction as perceived rotation Rare in young children, average age 10yoRare in young children, average age 10yo

Peripheral – semicircular canals and vestibulePeripheral – semicircular canals and vestibule Hearing may be impairedHearing may be impaired

Central – brainstem, cerebellum, cortexCentral – brainstem, cerebellum, cortex Hearing usually sparedHearing usually spared

Vestibular SystemVestibular System

Semicircular canalsSemicircular canals rotationrotation

Vestibule structuresVestibule structures linear accelerationlinear acceleration

jesse sturm

Vertigo: Common CausesVertigo: Common Causes

Supperative or serous labyrinthitisSupperative or serous labyrinthitis Vestibular neuronitisVestibular neuronitis Benign paroxysmal vertigoBenign paroxysmal vertigo MigraineMigraine IngestionsIngestions SeizureSeizure Motion sicknessMotion sickness

Vertigo: LabyrinthitisVertigo: Labyrinthitis

Inflammation of David Bowie as ______ the ______ King

Vertigo: LabyrinthitisVertigo: Labyrinthitis Supperative otitis with Supperative otitis with

effusion – may extend effusion – may extend directly into labyrinthdirectly into labyrinth

Cholesteatoma of TM can Cholesteatoma of TM can causes fistula into labyrinthcauses fistula into labyrinth

Direct viral infections of Direct viral infections of labyrinth, w/o effusionlabyrinth, w/o effusion Vestibular neuronitisVestibular neuronitis Measles, mumps, EBV, Measles, mumps, EBV,

Zoster of canal and CN7 Zoster of canal and CN7 (Ramsay-Hunt)(Ramsay-Hunt)

Resolves in 1-3 wksResolves in 1-3 wks Steroids shorten courseSteroids shorten course

Benign Paroxsysmal Vertigo (BPV)Benign Paroxsysmal Vertigo (BPV)

Considered to be form of migraineConsidered to be form of migraine Peaks 1-5yoPeaks 1-5yo Recurrent attacks, sudden onset – emesis, pallor, Recurrent attacks, sudden onset – emesis, pallor,

sweating, nystagmussweating, nystagmus Episodes last minutesEpisodes last minutes Mistaken for seizuresMistaken for seizures

EEG normal, no altered consciousnessEEG normal, no altered consciousness Disorder spontaneously resolves after 2-3 yearsDisorder spontaneously resolves after 2-3 years

Distinct from benign paroxysmal positional vertigoDistinct from benign paroxysmal positional vertigo Short vertigo attacks from certain positional movements (adult Short vertigo attacks from certain positional movements (adult

phenomena)phenomena) Dix Hallpike maneuverDix Hallpike maneuver

Vertigo: MigraineVertigo: Migraine

Up to 19% of children have vertiginous Up to 19% of children have vertiginous symptoms during aura of migrainesymptoms during aura of migraine HA pain often absentHA pain often absent

Basilar migraines – throbbing occipital HA Basilar migraines – throbbing occipital HA with brainstem dysfunction (vertigo, ataxia, with brainstem dysfunction (vertigo, ataxia, tinnitus, dysarthria)tinnitus, dysarthria)

Vertigo: IngestionsVertigo: Ingestions

Ototoxic drugs:Ototoxic drugs: Aminoglycosides, lasix, minocycline, aspirin, Aminoglycosides, lasix, minocycline, aspirin,

ethanol, anticonvulsantsethanol, anticonvulsants

Vertigo: SeizuresVertigo: Seizures

Vestibular seizuresVestibular seizures Sudden onset vertigo with or without nausea, Sudden onset vertigo with or without nausea,

emesis, headacheemesis, headache Followed by period of altered consciousnessFollowed by period of altered consciousness EEG abnormalEEG abnormal Anticonvulsants of benefitAnticonvulsants of benefit

Vertigo: Motion SicknessVertigo: Motion Sickness

Mismatch of information provided to brain Mismatch of information provided to brain by vestibular and visual systemsby vestibular and visual systems Occurs during periods of unfamiliar rotation Occurs during periods of unfamiliar rotation

and accelerationand acceleration

Prevent attacks by watching environment Prevent attacks by watching environment move in direction opposite body move in direction opposite body movementmovement i.e. looking out window of moving cari.e. looking out window of moving car

Vertigo: Meniere’s DiseaseVertigo: Meniere’s Disease

Episodic attacks of vertigo, hearing loss, Episodic attacks of vertigo, hearing loss, tinnitus, autonomic symptoms of pallor, tinnitus, autonomic symptoms of pallor, nausea, emesis (1-3hrs)nausea, emesis (1-3hrs) Between episodes may have impaired balanceBetween episodes may have impaired balance Uncommon < 10yoUncommon < 10yo

Caused by overaccumulation of endolymph Caused by overaccumulation of endolymph in labyrinthin labyrinth

Vertigo: Physical ExamVertigo: Physical Exam Nystagmus is highly specific signs for both central and Nystagmus is highly specific signs for both central and

peripheral vertiginous disordersperipheral vertiginous disorders Peripheral vertigo: slow component to affected sidePeripheral vertigo: slow component to affected side Central vertigo: fast component to affected sideCentral vertigo: fast component to affected side

Dix-Hallpike maneuver to stress vestibular systemDix-Hallpike maneuver to stress vestibular system Central vertigo onset of nystagmus is immediateCentral vertigo onset of nystagmus is immediate Peripheral onset of nystagmus delayed several secondsPeripheral onset of nystagmus delayed several seconds

Cold calorics tests integrity of peripheral vestibular systemCold calorics tests integrity of peripheral vestibular system 10cc ice water into EAC with child 6010cc ice water into EAC with child 60ºº Slow eye movement toward cold, fast movement away Slow eye movement toward cold, fast movement away

(COWS)(COWS) Warm water has inverseWarm water has inverse

Lack of response implies peripheral vestibular damageLack of response implies peripheral vestibular damage

Approach to True VertigoApproach to True Vertigo

ConclusionConclusion

Acute childhood ataxia often benign Acute childhood ataxia often benign condition requiring little workupcondition requiring little workup Asymmetry to exam, neuropathies, progressive Asymmetry to exam, neuropathies, progressive

onset more concerning onset more concerning

Dizziness encompasses multiple symptomsDizziness encompasses multiple symptoms Differentiate true vertigo from pseudovertigoDifferentiate true vertigo from pseudovertigo

Careful physical exam with focus on Careful physical exam with focus on cerebellar testing often uncovers diagnosiscerebellar testing often uncovers diagnosis