Transcript ataktos

Ataxia and Dizziness
Jesse Sturm, MD
Pediatric Fellow’s Conference
June 25, 2008
Outline
 Definitions
 Ataxia
 Causes
 Workup – labs and specific exam findings
 Dizziness
 Causes
 Algorithmic approach
 Conclusion
Definitions
 Ataxia: disturbance in smooth accurate
coordination of movements, unsteady gait
 Dizziness: non specific term
 Includes vertigo, disequilibrium, pre-syncope
 Vertigo – symptom of illusory movement, sense of
swaying or tilting
 Some perceive self-movement, others perceive motion of the
environment
 Due to asymmetry in vestibular system (labyrinth, central
structures in brainstem)
 Vertigo is a symptom, not a diagnosis
Ataxia
 Ataxia: ataktos – “lacking order” (Greek)
 Disturbance in smooth accurate movements –
commonly unsteady gait
 Often result of cerebellar dysfunction
 Disturbance at multiple sensory levels can affect
coordination
 i.e. loss of proprioception = sensory ataxia
 Acute ataxia is rare, most often benign
presenting complaint
Cerebellum
 A: midbrain
 B: pons
 C: medulla
 D: spinal cord
 E: 4th ventricle
 G: tonsil
 H: ant lobe
 I: post lobe
Cerebellum
 Vermis - midline
 dysarthria
 truncal titubation
 symmetric ataxia
 Hemispheres
 ipsilateral limb
dysmetria
 hypotonia
 tremor
 ataxia in direction
of affected hemisphere
Causes of Ataxia
 Review of 80 admitted pediatric cases:
 80% of acute ataxias had diagnosis of acute
cerebellar ataxia, toxic ingestion, GuillaineBarre syndrome
Gieron-Korthals, MA. Acute ataxia in childhood: a 10-year experience. J. Child
Neurology 1994: 9:381.
Differential of Acute Ataxia
Infectious/immune mediated disorders
Acute cerebellar ataxia
ADEM
Systemic infections
Brainstem encephalitis
Multiple Sclerosis
Toxic: alcohol and drug related
Mass lesions
Tumor
Vascular lesions
Abscesses
Hydrocephalus
Trauma
Cerebellar contusion or hemorrhage
Posterior fossa hematoma
Post-concussion syndrome
Vertebrobasilar dissection
Stroke
Vertebrobasilar dissection or
thromboembolism
Cerebellar hemorrhage
Paraneoplastic disorders
Opsoclonus-myoclunus syndrome
Sensory ataxia
Guillain-Barre syndrome
Miller Fisher syndrome
Paretic ataxia
Upper motor neuron syndrome
Lesions of frontal lobe
Lower motor neuron syndrome
Spinal cord
transverse myelitis, cord compression
Peripheral nerve
GBS, MF, tick paralysis
Inborn errors of metabolism
Basilar Migraines
Non-convulsive seizures
Wernicke’s encephalopathy
Causes of Acute Ataxia
 Life threatening conditions
 Tumors, Stroke, Infection
 Common conditions
 Acute cerebellar ataxia, GBS, Labyrinthitis,
Toxins, Migraine syndromes, Trauma
 Rare disorders
Causes of Acute Ataxia
 Life threatening conditions
 Tumors, Stroke, Infection
 Common conditions
 Acute cerebellar ataxia, GBS, Labyrinthitis,
Toxins, Migrane syndromes, Trauma
 Rare disorders
Ataxia - Tumors
 45-60% of all childhood brain tumors arise
in brainstem or cerebellum
 Can present with progressive ataxia
 Symptoms of increased ICP
 Papilledema, cranial neuropathies, HA, emesis
 Rarely midline supratentorial tumors
 Opsoclonus-Myoclonus (rapid dancing eye
movements and rhythmic jerking)
 Paraneoplastic - neuroblastoma in up to 50%
Ataxia - Stroke
 Hemmorhage into cerebellum or posterior fossa
from trauma or vascular malformation
 Vertebral or basilar artery disease
 Sickle cell
 Hypercoagulable states
 Vertebrobasilar artery
dissection following neck
injury can present as
acute ataxia
Ataxia - Infection
 Cerebellar abscesses – contiguous spread from
ASOM or mastoiditis
 Ataxia/fever +/- signs of increased ICP
 Brainstem encephalitis
 CNeuropathies, AMS, seizures
 Causes: listeria, lyme disease, EBV, HSV
 CSF pleocytosis
 Acute post-infectious demyelinating
encephalomyelitis (ADEM), multiple sclerosis
 Seizures, CNeuropathies, weakness, sensory deficits,
transverse myelitis
Causes of Acute Ataxia
 Life threatening conditions
 Tumors, Stroke, Infection
 Common conditions
 Acute cerebellar ataxia, GBS, Labyrinthitis,
Toxins, Migrane syndromes, Trauma
 Rare disorders
Acute Cerebellar Ataxia (ACA)
 Post infectious cerebellar demyelination and/or direct
cerebellar infection (seen on MRI)
 35% of acute childhood ataxia
 Autoimmune phenomena against cerebellar epitopes
 Onset 5-10 days after precipitating infection (70%)
 Peak age 2-4yo (case series ages 1.5yo – 12.5yo)
 Symptoms maximal at onset
 Truncal ataxia severe, extremity ataxia < trunk
 Seen in sitting position
 Vomiting, horizontal nystagmus, dysarthria may occur
 Mental status normal, no fever, no meningismus
Acute Cerebellar Ataxia (ACA)
 Most common findings on exam are
nystagmus and dysmetria (50%)
 Small retrospective study (n=39):
 Mean CSF WBC 16 (0-40)
 >5 WBC in 48%, all with lymph predominance
 Mean CSF protein 20 (>40 in 23%)
 CT done in 14 patients, all normal
 Recent studies show + MRI findings in
classic ACA
Acute Cerebellar Ataxia (ACA)
 Varicella implicated in >25% cases
 Rare cases due to VZV vaccine
 Echovirus, EBV, Measles, Mumps, HSV,
Parvovirus
 MMR vaccine implicated in rare cases
Acute Cerebellar Ataxia (ACA)
 Symptoms take several weeks to resolve
 Mean ~ 1.5 weeks
 Complete recovery in >90% patients
 Ataxia symmetric
 Findings in cerebellar ataxia remain
unchanged whether eyes open or closed
 No evidence that immunosupressive
therapies improve outcomes
Acute Cerebellar Ataxia (ACA)
 Clinical features do not distinguish from
other causes of acute ataxia
 Diagnosis of exclusion
Ataxia - Guillain-Barre Syndrome
 Ascending paralysis, areflexia, progressive
 15% of children with GBS also lose sensory
input to cerebellum --- develop sensory ataxia
 + Romberg, dec DTR
 Miller Fisher syndrome: GBS with triad of
ataxia, areflexia, opthalmoplegia
Ataxia - Labyrinthitis
 Inflammation of vestibular
apparatus
 Bacterial or viral
 Symptoms of hearing loss,
vomiting, extreme vertigo
 Vertigo often exacerbated
by head movements
 Dix-Hallpike maneuver
Ataxia - Toxin Exposure
 Responsible for up to 30% acute ataxia
 Anticonvulsants – phenytoin, carbamazepine,
phenobarbitol, antihistamines
 Lead, carbon monoxide, inhalants, Etoh,
Benzos
 Usually accompanied by AMS
Ataxia - Migraine Syndromes
 Basilar migraines and familial hemiplegic
migraine syndromes present with ataxia
 Associated headache and vomiting
distinguish from other acute ataxias
 Visual auras common
Ataxia - Trauma
 Post concussive ataxia
 Directed traumatic force to labyrinth structures
 May be associated with hemotympanum and
temporal fractures
Causes of Acute Ataxia
 Life threatening conditions
 Tumors, Stroke, Infection
 Common conditions
 Acute cerebellar ataxia, GBS, Labyrinthitis,
Toxins, Migrane syndromes, Trauma
 Rare disorders
Ataxia – Rare Causes
 Tick paralysis
 unsteady gait, ascending paralysis/weakness, areflexia
 neurotoxin in tick saliva
 Hypoglycemia
 Seizure disorder
 simple non-convulsive seizures may manifest as ataxia alone
 Conversion disorder
 narrow gait, elaborate near falls
 Inborn error metabolism
 Urea cycle, aminoacidopathies (MSUD), organics acidemias
 Congenital anomolies
 Chiari malformation, encephaloceles, cerebellar aplasia/hypoplasia
 Genetic conditions
 ataxia telangectasia etc.
Diagnostic workup
 Temporal course
 Acute, episodic, chronic
 Associated neurological findings
 History
 PE
 Targeted diagnostic
workup
Ataxia – Temporal Course
 Rapid onset: traumatic, infectious or postinfectious, or toxic etiology
 Progressive onset (few days): metabolic
syndromes, GBS
 Insidious onset (days to weeks): brainstem
and cerebellar tumors
History
 Recent infection, vaccination
 Previous episode of ataxia
 Migraine-related syndrome, seizure, IEM
 Family history
 Migraine syndromes, hereditary ataxias, IEM
Concurrent Symptoms
 Otalgia, vertigo, vomiting
 Suggest labyrinthitis, often see nystagmus
 Recurrent headaches, behavior changes
 May represent increased ICP
 Abnormal mental status
 Mass lesions, CNS infection, toxin exposure,
trauma (head/neck), stroke, inborn error
metabolism
 Access to drugs of abuse, ethanol, anticonvulsants
Physical Exam
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Vitals: bradycardia, HTN, resp pattern, fever
Anterior fontanelle
Ipsilateral head tilt (posterior fossa tumor)
Papilledema
Nystagmus (vestibular, cerebellar, brainstem disorder)
Opsoclonus (occult neuroblastoma)
AOM, hearing loss +/- vomiting/vertigo (acute labyrinthitis)
Meningismus
Healing rash/viral exanthem
Tick attachment
Neurologic Exam
 General mental status
 AMS suggests ADEM, CNS infection, stroke, ingestion
 Cranial neuropathies
 Suggest posterior fossa lesion, encephalitis, GBS with MFS
 Motor exam
 “paretic ataxia” -if weak may stagger to compensate
 GBS, Botulism, transverse myelitis, myasthenia, tick paralysis
 Check reflexes, strength
 Sensory exam
 Proprioceptive input may cause ataxia (seen in GBS)
 Romberg test – when close eyes remove visual compensation
 Cerebellar exam
 May be normal even with specific lesions
Cerebellar Exam
 Gait, Speech, Coordination
i.e. DRUNK
 Gait – wide based, unsteady,
lurching
 Titubation – difficulty with
truncal position
 Speech – clarity, rhythm,
tone, volume
 Coordination –
over/undershooting on FTN,
difficulty with RAM
(dysdiadochokinesia)
Diagnostic Testing
 Toxicology Screen
 Drug of abuse, specific drug levels
 35% of UDS were + in one retrospective series in children (n=90)
(Gieron-Korthals, 1994), HIGHEST YIELD
 Glucose
 Metabolic Evaluation
 Especially for acute episodic ataxia to identify IEM
 Serum lactate, pyruvate, amino acids, ammonia, pH
 CSF examination
 Rarely indicated unless clinically concerned for meningoencephalitis
 Moderate protein elevation and pleocytosis occurs in 25-50% ACA,
ADEM, MS, GBS
 Cytoalbuminologic dissociation in GBS (high protein >40, low cells<10)
 Neuroimaging
 Prior to LP if any concern for increased ICP
Imaging
 Obtain for acute ataxia with:
 AMS, focal neuro signs, cranial neuropathies,
asymmetry of ataxia, history of trauma, concern for
mass lesion, no improvement in 1-2wks
 MRI
 superior for posterior fossa lesions
 demyelinating disease better visualized
 CT
 conditions needing urgent intervention
EEG and EMG
 EEG if concerned concurrent seizure
 Obtain if fluctuating clinical signs
 60% of children with ACA will have abnormal
EEG, epileptiform activity or slowing
 EMG sensitive tests for GBS (sensory
ataxias), may not be helpful early in
disease
 EMG findings in 90%
Algorithmic Approach
Algorithmic Approach
Dizziness
 Dizziness: non specific term
 Includes vertigo, disequilibrium, pre-syncope
 Vertigo – symptom of illusory
movement/rotation, sense of swaying or tilting
 Some perceive self-movement, others perceive
motion of the environment
 Due to asymmetry in vestibular system (labyrinth,
central structures in brainstem)
 Vertigo is a symptom, not a diagnosis
Vertigo
 True vertigo
 Subjective sense of rotation of environment relative to
patient or patient to environment
 Acute attacks often accompanied by nystagmus
 Pseudovertigo
 Complaints of lightheadedness, flushing, weakness,
ataxia, unsteadiness, pallor, anxiety, stress, fear
True Vertigo
 Disturbance of peripheral or central components
of vestibular system
 CN8 carries impulses to nuclei in cerebellum
 Additional impulses carried to CN 3,4,6
 Almost all patients have fast component of nystagmus in
same direction as perceived rotation
 Rare in young children, average age 10yo
 Peripheral – semicircular canals and vestibule
 Hearing may be impaired
 Central – brainstem, cerebellum, cortex
 Hearing usually spared
Vestibular System
 Semicircular canals
 rotation
 Vestibule structures
 linear acceleration
Vertigo: Common Causes
 Supperative or serous labyrinthitis
 Vestibular neuronitis
 Benign paroxysmal vertigo
 Migraine
 Ingestions
 Seizure
 Motion sickness
Vertigo: Labyrinthitis
Inflammation of David Bowie
as ______ the ______ King
Vertigo: Labyrinthitis
 Supperative otitis with
effusion – may extend
directly into labyrinth
 Cholesteatoma of TM can
causes fistula into labyrinth
 Direct viral infections of
labyrinth, w/o effusion
 Vestibular neuronitis
 Measles, mumps, EBV,
Zoster of canal and CN7
(Ramsay-Hunt)
 Resolves in 1-3 wks
 Steroids shorten course
Benign Paroxsysmal Vertigo (BPV)
 Considered to be form of migraine
 Peaks 1-5yo
 Recurrent attacks, sudden onset – emesis, pallor,
sweating, nystagmus
 Episodes last minutes
 Mistaken for seizures
 EEG normal, no altered consciousness
 Disorder spontaneously resolves after 2-3 years
 Distinct from benign paroxysmal positional vertigo
 Short vertigo attacks from certain positional movements (adult
phenomena)
 Dix Hallpike maneuver
Vertigo: Migraine
 Up to 19% of children have vertiginous
symptoms during aura of migraine
 HA pain often absent
 Basilar migraines – throbbing occipital HA
with brainstem dysfunction (vertigo, ataxia,
tinnitus, dysarthria)
Vertigo: Ingestions
 Ototoxic drugs:
 Aminoglycosides, lasix, minocycline, aspirin,
ethanol, anticonvulsants
Vertigo: Seizures
 Vestibular seizures
 Sudden onset vertigo with or without nausea,
emesis, headache
 Followed by period of altered consciousness
 EEG abnormal
 Anticonvulsants of benefit
Vertigo: Motion Sickness
 Mismatch of information provided to brain
by vestibular and visual systems
 Occurs during periods of unfamiliar rotation
and acceleration
 Prevent attacks by watching environment
move in direction opposite body
movement
 i.e. looking out window of moving car
Vertigo: Meniere’s Disease
 Episodic attacks of vertigo, hearing loss,
tinnitus, autonomic symptoms of pallor,
nausea, emesis (1-3hrs)
 Between episodes may have impaired balance
 Uncommon < 10yo
 Caused by overaccumulation of endolymph
in labyrinth
Vertigo: Physical Exam
 Nystagmus is highly specific signs for both central and
peripheral vertiginous disorders
 Peripheral vertigo: slow component to affected side
 Central vertigo: fast component to affected side
 Dix-Hallpike maneuver to stress vestibular system
 Central vertigo onset of nystagmus is immediate
 Peripheral onset of nystagmus delayed several seconds
 Cold calorics tests integrity of peripheral vestibular system
 10cc ice water into EAC with child 60º
 Slow eye movement toward cold, fast movement away
(COWS)
 Warm water has inverse
 Lack of response implies peripheral vestibular damage
Approach to True Vertigo
Conclusion
 Acute childhood ataxia often benign
condition requiring little workup
 Asymmetry to exam, neuropathies, progressive
onset more concerning
 Dizziness encompasses multiple symptoms
 Differentiate true vertigo from pseudovertigo
 Careful physical exam with focus on
cerebellar testing often uncovers diagnosis