1 / 55

Ataxia and Dizziness

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.

alamea
Download Presentation

Ataxia and Dizziness

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript


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

  2. Outline • Definitions • Ataxia • Causes • Workup – labs and specific exam findings • Dizziness • Causes • Algorithmic approach • Conclusion

  3. 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

  4. 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

  5. Cerebellum • A: midbrain • B: pons • C: medulla • D: spinal cord • E: 4th ventricle • G: tonsil • H: ant lobe • I: post lobe

  6. Cerebellum • Vermis - midline • dysarthria • truncal titubation • symmetric ataxia • Hemispheres • ipsilateral limb dysmetria • hypotonia • tremor • ataxia in direction of affected hemisphere

  7. Causes of Ataxia • Review of 80 admitted pediatric cases: • 80% of acute ataxias had diagnosis of acute cerebellar ataxia, toxic ingestion, Guillaine-Barre syndrome Gieron-Korthals, MA. Acute ataxia in childhood: a 10-year experience. J. Child Neurology 1994: 9:381.

  8. Differential of Acute Ataxia

  9. Causes of Acute Ataxia • Life threatening conditions • Tumors, Stroke, Infection • Common conditions • Acute cerebellar ataxia, GBS, Labyrinthitis, Toxins, Migraine syndromes, Trauma • Rare disorders

  10. Causes of Acute Ataxia • Life threatening conditions • Tumors, Stroke, Infection • Common conditions • Acute cerebellar ataxia, GBS, Labyrinthitis, Toxins, Migrane syndromes, Trauma • Rare disorders

  11. 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%

  12. 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

  13. 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

  14. Causes of Acute Ataxia • Life threatening conditions • Tumors, Stroke, Infection • Common conditions • Acute cerebellar ataxia, GBS, Labyrinthitis, Toxins, Migrane syndromes, Trauma • Rare disorders

  15. 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

  16. 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

  17. 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

  18. 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

  19. Acute Cerebellar Ataxia (ACA) • Clinical features do not distinguish from other causes of acute ataxia • Diagnosis of exclusion

  20. 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

  21. 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

  22. 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

  23. 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

  24. Ataxia - Trauma • Post concussive ataxia • Directed traumatic force to labyrinth structures • May be associated with hemotympanum and temporal fractures

  25. Causes of Acute Ataxia • Life threatening conditions • Tumors, Stroke, Infection • Common conditions • Acute cerebellar ataxia, GBS, Labyrinthitis, Toxins, Migrane syndromes, Trauma • Rare disorders

  26. 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.

  27. Diagnostic workup • Temporal course • Acute, episodic, chronic • Associated neurological findings • History • PE • Targeted diagnostic workup

  28. Ataxia – Temporal Course • Rapid onset: traumatic, infectious or post-infectious, or toxic etiology • Progressive onset (few days): metabolic syndromes, GBS • Insidious onset (days to weeks): brainstem and cerebellar tumors

  29. History • Recent infection, vaccination • Previous episode of ataxia • Migraine-related syndrome, seizure, IEM • Family history • Migraine syndromes, hereditary ataxias, IEM

  30. 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

  31. Physical Exam • 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

  32. 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

  33. 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)

  34. 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

  35. 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

  36. 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%

  37. Algorithmic Approach

  38. Algorithmic Approach

  39. 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

  40. 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

  41. 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

  42. Vestibular System • Semicircular canals • rotation • Vestibule structures • linear acceleration

  43. Vertigo: Common Causes • Supperative or serous labyrinthitis • Vestibular neuronitis • Benign paroxysmal vertigo • Migraine • Ingestions • Seizure • Motion sickness

  44. Vertigo: Labyrinthitis Inflammation of David Bowie as ______ the ______ King

  45. 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

  46. 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

  47. 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)

  48. Vertigo: Ingestions • Ototoxic drugs: • Aminoglycosides, lasix, minocycline, aspirin, ethanol, anticonvulsants

  49. Vertigo: Seizures • Vestibular seizures • Sudden onset vertigo with or without nausea, emesis, headache • Followed by period of altered consciousness • EEG abnormal • Anticonvulsants of benefit

More Related