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Ataxia 130514030409-phpapp01

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Ataxia 130514030409-phpapp01

  1. 1. APPROACH TO ATAXIA DR Y RAGHU NANDHINI UNIT 4
  2. 2. INTRODUCTION Important concepts in Ataxia ATAXIA MIMICKERS Tests of Cerebellar dysfunction Step-wise approach to Cerebellar Ataxias Summary ALGORITHM for cerebellar ataxias
  3. 3. ‘In Simple Terms…” • ATAXIA- “Absence of ORDER” (Greek Word) • In Neurological Terms- “Incoordination of movement” • A major feature of a disease or just one of the various clinical features of a disease
  4. 4. Definition • Ataxia is the inability to make smooth, accurate and coordinated movements • Arises from disorders of: ––Cerebellum ––Sensory pathways (Sensory Ataxia) ––Posterior columns, dorsal root ganglia, peripheral nerves ––Frontal lobe lesions ––Extra pyramidal system ––Vestibular system
  5. 5. Tests to differentiate Various systems in Ataxia- “The Ataxia Mimickers”
  6. 6. Cerebellar Ataxia Cortical Ataxia Myopathy Labrynthine Ataxia Sensory Ataxia (Posterior Column) Thalamic Ataxia Sensory Ataxia (Peripheral Neuropahy))
  7. 7. SENSORY ATAXIA “Disturbances in the sensory input to the cerebellum” •Tests of proprioception- Joint sense, passive movement “The corrective effects of the Visual system” •Classical Sensory Ataxic Gait •Romberg’s sign •Loss of tendon reflexes •Features of Peripheral neuropathy
  8. 8. Labrynthine Disorders • Input to cerebellum • Dizziness, light headedness, perception of “movement”, rotatory nystagmus • Infections, neoplasms, vascular causes
  9. 9. Cortical Ataxias  FRONTAL LOBE ATAXIA refers to disturbed coordination due to dysfunction of the contralateral frontal lobe; -Results from disease involving the frontopontocerebellar fibers en route to synapse in the pontine nuclei. • hyperreflexia, increased tone and Release reflexes
  10. 10. Thalamic Ataxias - transient ataxia affecting contralateral limbs after lesion of anterior thalamus - may see associated motor (pyramidal tract) signs from involvement of internal capsule - also can result in asterixis in contralateral limbs (hemiasterixis)
  11. 11. CEREBELLAR ATAXIAS “ATAXIA IS THE MOST IMPORTANT FEATURE AMIDST OTHER CLINICAL SIGNS OF CEREBELLAR DYSFUNCTION”
  12. 12. NEO CEREBELLUM FLOCCULONODULAR LOBE SPINO CEREBELLUM
  13. 13. Cerebellar Dysfunction: Anatomy Cerebellar parts Functions Signs Posterior (Flocculo-nodular lobe) Archicerebellum Body equilibrium Eye movements Eye movement disorders: Nystagmus; Vestibulo-ocular reflex (VOR) Postural and gait dysfunction Midline (Vermis; vermis of ant. lobe pyramid , uvula and paraflocculus) Paleocerebellum Input from spinal cord Muscle tone Axial stance and gait Truncal & gait ataxia Hemisphere (middle portion of vermis, cerebellar hemisphere) Neocerebellum Connected with Pons and cortex through thalalmus Planning and initiation of movements Regulation of fine limb movements Limb ataxia: Dysmetria, Dysdiadochokinesis, "intention" tremor Dysarthria Hypotonia
  14. 14. TESTS OF CEREBELLAR DYSFUNCTION
  15. 15. ATAXIA “errors in the RATE, RANGE, FORCE & DIRECTION of movement” •GAIT ATAXIA •TRUNCAL ATAXIA •LIMB ATAXIA
  16. 16. CLASSIC FEATURES AND TESTS Dyssynergia: results in jerky decomposed movements (heel-knee-shin test) Dysmetria: due to delayed activation of antagonists •- often correction to target by series of jerky corrections (finger nose test) •- may lead to intention tremor in limbs with finger-to-nose or foot-to-target testing as rhythmic oscillation emerges close to target Dysdiadochokinesis: irregularities of force, speed, and rhythm
  17. 17. Other features Hypotonia: decrease in resistance to passive movement of muscles related to depression of gamma motor neuron activity (usually seen transiently in acute phase of cerebellar lesions), pendullar knee jerk Rebound phenomenon: related to poor tone and weak check response, so when tap or displace limb, wider range of movement in return to static position, incl. Holmes phenomenon when suddenly release flexed arm held against resistance - unable to stop flexion and arm strike self (delay in activation of antagonist triceps muscle) Dysarthria: often scanning type with irregularities in tone, with words broken into syllables; often slow with occasional rapid portions ("explosive speech")
  18. 18. Other features Ocular Motor Abnormalities: - usually if vestibular connections or flocculonodular lobe affected - pursuit movements no longer smooth, but saccadic - may over- or under-shoot target with attempts at fixation (ocular dysmetria) - in primary position may see saccadic intrusions (such as macro square-wave jerks) or primary nystagmus (incl. vertical, esp up-beat nystagmus) or periodic alternating nystagmus -rebound nystagmus can occur with contralateral-beating nystagmus on return of eyes to primary position after eccentric gaze evoked nystagmus to one side Writing abnormalities Positional projectile vomiting (posterior fossa lesions)
  19. 19. APPROACH TO CEREBELLAR ATAXIA IN ADULTS
  20. 20. THE “FOUR” QUESTIONS???? • Mode of ONSET ? • PROGRESSION ? • Focal /Symmetric involvement ? • Localisation of the cerebellar lesion ? HISTORY EXAMINATION
  21. 21. MODE OF ONSET • ACUTE- hours to days • SUB ACUTE- days to weeks • CHRONIC- months to years
  22. 22. ACUTE ONSET ATAXIA • INTOXICATION: alcohol , lithium , phenytoin , barbiturates • POST INFECTIOUS: Acute Viral Cerebellitis, VZV • VASCULAR: Infarction (AICA, PICA syndromes), Haemorrhage, Subdural hematoma
  23. 23. SUB ACUTE ATAXIA • INTOXICATION: Mercury, Solvents, Glue • NUTRITIONAL: B1 and B12 deficiency • INFECTION: HIV • DEMYELINATING: Multiple Sclerosis • NEOPLASTIC: Glioma, Metastases
  24. 24. CHRONIC ATAXIA • AUTOIMMUNE CAUSES : Paraneoplastic syndromes, Gluten hypersensitivity, Anti GAD abs. • HYPOTHYROIDISM • INFECTIONS: Syphilis (Tabes Dorasalis) • CONGENITAL LESIONS: Arnold-Chiari and Dandy Walker Syndromes • INHERITED ATAXIAS: AD,AR,XR,XD,Mitochondrial
  25. 25. PROGRESSION • Progressive • Static • Intermittent symptoms • Reversible Ataxias
  26. 26. STATIC ATAXIAS • Vascular causes REVERSIBLE ATAXIAS • Infectious causes – post viral • Thyroid • Drugs • Toxins INTERMITTENT SYMPTOMS • Episodic Ataxias (Inherited etiology)
  27. 27. FOCAL / SYMMETRIC ATAXIAS • Cerebellar symptoms on same side of lesion, or • Bilateral symptoms FOCAL ATAXIAS Vascular causes, Multiple Sclerosis, Cerebellar abscess, cerebellar glioma, PML (HIV), Congenital causes SYMMETRIC ATAXIAS Intoxication, Nutritional, Post inhectious, Hypothyroid, Autoimmune causes
  28. 28. THE NEXT STEP …RULE OUT ACQUIRED ATAXIAS INHERITED ATAXIAS SPORADIC or IDIOPATHIC ATAXIAS
  29. 29. ACQUIRED ATAXIAS • First rule out the Structural causes (MRI Brain/ CT head) -CVJ anomalies -Posterior fossa tumors -Demyelinating diseases -Hypoxic encephalopathies -Vascular causes- infarct, haemorrhage
  30. 30. Acquired Causes • HYPOTHYROIDISM- Mild gait ataxia PLUS Systems of hypothyroidism
  31. 31. ALCOHOLISM • Vermian Atrophy
  32. 32. TOXINS • Cancer chemotherapeutics 5 FU, Cytarabine • Metals Bismuth, Mercury (parasthesiass, restricted visual defects), Lead • SolventsPaint thinners , toluene (Cognitive defects PLUS pyramidal tract signs) • AnticonvulsantsPhenytoin (purkinje cell loss)avoid in epileptics with ataxia
  33. 33. INFECTIONS • VZV in children • EBV in children • Bickerstaff’s encephalitis (brain stemophthalmoplegia,ataxia,lower c.n palsies) • HIV ( Lymphomas, PML, Infections, Toxoplasmosis) • CJD (17% classic CJD, Ataxic variant of CJD) • Syphilis (Tabes Dorsalis) • Whipple’s disease
  34. 34. AUTOIMMUNE CAUSES PARANEOPLASTIC SYNDROMES •ANTI Hu abs. Small Cell Cancer Lung (extrapyramidal signs) •ANTI Yo abs. Ovarian cancer •ANTI Ri abs. Breast cancer (opsoclonus – saccadomania, Trunk ataxia) •ANTI Tr abs. Hodgkin’s lymphoma (hearing loss)
  35. 35. • GLUTEN SENSITIVITY - Anti Gliadin abs. (ataxia, brisk reflexes, peripheral neuropathies) • ANTI GAD abs. – Diabetes, hypothyroidism, peripheral neuropathySTIFF PERSON syndrome AUTOIMMUNE CAUSES
  36. 36. NUTRITIONAL CAUSES • FAT MALABSORPTION- Vit. E deficiency • Vit. B12 , B1 deficiencies
  37. 37. INHERITED ATAXIAS • AD • AR • MITOCHONDRIAL DISTURBANCES • X LINKED RECESSIVE • X LINKED DOMINANT
  38. 38. INHERITED ATAXIAS
  39. 39. AUTOSOMAL DOMINANT
  40. 40. • SPINO CEREBELLAR ATAXIAS (Types131)- previously olivopontocerebellar atrophies • DentatoRubroPallidoLuysian Atrophy • EPISODIC ATAXIAS (Types 17) AUTOSOMAL DOMINANT
  41. 41. SCA SALIENT FEATURES • 3-5th decade of life ONSET, loss of ambulation over 10-15 yrs. from onset • Phenomena called ANTICIPATION and PENETRANCE differs from each SCAresponsible for various ages of presentation and variable phenotypic expression • CAG repeat expansion in most of them
  42. 42. Spinocerebellar Ataxia (SCA) Dominant SCA syndromes have many overlapping signs: Difficult to distinguish on clinical grounds Common features to all: Gait ataxia; Dysarthria Features in some ataxias: Ocular D; Extrapyramidal; Peripheral nerve; Intellectual D; Seizures Features with some predictive value for specific gene defects
  43. 43. SCA: Clinical Syndromes •SCA 1: Hypermetric saccades; ++Tendon reflexes; Evoked motor potentials Long conduction times •SCA 2 Slowing saccads; Myoclonus or action tremor •SCA 3/Machado-Joseph: Gaze-evoked nystagmus; Prominent spasticity or neuropathy •SCA 4: Cerebellar syndrome; Sensory neuropathy •SCA 5: Pure cerebellar syndrome •SCA 6: Pure cerebellar syndrome; -ve family history; Late onset > 50 •SCA 7: Retinal degeneration; Hearing loss; Onset in 1st decade •SCA 8: Pure cerebellar syndrome •SCA 10: Pure cerebellar syndrome ± Seizures •SCA 11: Pure cerebellar syndrome •SCA 12: Early arm tremor; Late dementia •SCA 13: Early childhood onset; Mental retardation •SCA 14: Ataxia; Myoclonus (with early onset)
  44. 44. Relationship between ADCAs and SCAs ADCA type SCA type I -Cerebellar plus (Pyramidal, Extra-pyramidal, Ophthalmoplegia, & Dementia) 1,2,3,4,12,16,17, DRPLA II Cerebellar + pigmentary maculopathy 7 III pure cerebellar ± Mild pyramidal signs 5,6,8,11,14,15,22 Ataxia and epilepsy 10 Early onset with mental retardation 13
  45. 45. SCA 5 SCA 2MJD SCA 7
  46. 46. SPINOCEREBELLAR ATAXIA 1 ;SCA 1 SPINOCEREBELLAR ATORHY I OLIVOPONTOCREBELLAR ATORPHY I; OPCA 1 OPCA I MENZEL TYPE OPCA  CLINICAL SYNOPSIS  Neurological:  Miscellaneous:  Labs:  Gene Map Locus:6p 22-p23 CAG 40-83 ( N 6-40)  cerebellar ataxia  chorea  upper motor neuron signs  extensor planter, hyperreflexia  lower bulbar palsies  gaze paresis 50% , slow saccades 100 %  scanning and explosive speech  inco-ordination  onset third/fourth decade  earlier onset when inherited from father , anticipation  axonal neuropathy  atrophy of cerebellum, pons, olive,lower CN nuclei, dorsal columns and spinocerebellar tracts  Reduced aspartic acid in brain  mutant protein Ataxin- 1, Intranuclear inclusions
  47. 47. SPINOCEREBELLAR ATAXIA 2; SCA 2 SPINOCEREBELLAR ATROPHY II OLIVOPONTOCEREBELLAR ATROPHY, HOLGUIN TYPE OLIVOPONTOCEREBELLAR ATROPHY 2 SPINOCEREBELLAR ATAXIA, CUBAN TYPE  CLINICAL SYNOPSIS  Neurological  Limbs  Miscellaneous  Labs  Gene Map Locus :12q23-24.1 CAG 34-59 ( N 14-31)  adult onset progressive cerebellar ataxia  palatal myoclonus , myokimia  slow saccadic eye movements 100%  dysarthria  ophthalmoparesis 40%, optic atrophy  pyramidal signs 20%  peripheral sensory loss, abolished tendon reflexes  dementia  extrapyramidal signs in Tunisian kindred  bladder dysfunction  no parkinsonian features  flexion contracture of legs  onset 2 - 65 yrs, 40% < 25 yrs, anticipation, may be sporadic  involvement of cerebellum & inferior. olivary nuc.,pons,spinal cord Ataxin - 2 , nuclear aggregates
  48. 48. SPINOCEREBELLAR ATAXIA 3 ; SCA 3 MACHODO-JOSEPH DIEASE,MJD AZOREAN NEUROLOGIC DISEASE NIGROSPINODENTATAL DEGENERATION, SPINOPONTINE AROPHY  CLINICAL SYNOPSIS  Neurological:  Eyes  Muscle  Endocrinal  Miscellaneous  Labs  Gene Map Locus : 14q32..1 CAG 56-86 (N -12-38)  ataxia  parkinsonian features  facial and lingual fasciculations  muscle fasciculations  loss of leg reflexes  cerebellar tremors  extensor planter  bulging eyes, limited eye movements, nystagmus  muscle atrophy  diabetes mellitus  onset after 40 yr, paternal> maternal anticipation  I- earlier onset(5-30), II- intermediate(~36 yr),  III- cerebellar,PN,Optho(>40yr), IV- parkinsonian, fasciculations, sensory(38-47yr levodopa responsive)  neuronal loss and gliosis in SN, STN,GP,Dedtate nuclei, nuclei pontis, vestibular & cranial nerve nuclei,,post.columns and ant. Horns  abnormal EOG  ATAXIN- 3, nuclear/cytoplasmic inclusions
  49. 49. SCA 3 • ALSO KNOWN AS MACHEDO JOSEPH DISEASE • 3 TYPES • TYPE 1 –ALS PARKINSONS DYSTONIA TYPE • WEAKNESS ND SPASTICITY OF LIMBS • DYSTONIA OF FACE, NECK,TRUNK • PHARYNGEAL WEAKNESS,HORIZANTAL ND VERTICAL NYSTAGMUS • OPYHALMOPARESIS,FACIAL FASICULATIONS ND MYOKINESIA
  50. 50. TYPE 2-ATAXIC CEREBELLAR DEFECTS PYRAMIDAL ND EXTAPYRAMYDAL SYMOTOMS OPTHALMOPARESIS FACIAL ND LINGUAL FASICULATIONS
  51. 51. • TYPE 3-ATAXIC AMYYOTROPHIC • CEREBELLAR SYMPTOMS • DISITAL SENSORY LOSS • DISITAL ATROPY • DEPRESSED OR ABSENT DTR • NO PYRAMIDAL OR EXTRA PYRAMIDAL SYMPTOMS
  52. 52. DENTATORUBRAL-PALLIDOLUYSIAN ATROPHY; DRPLA MYOCLONIC EPILEPSY WITH CHOREOATHETOSIS NAITO-OYANAGI DISEASE; NOD ATROPHIN 1, INCLUDED  CLINICAL SYNOPSIS  Neurological:  Miscellaneous:  Labs:  Gene Map Locus: 12p13.31 CAG 49-75 (N<24)  Myoclonus  epilepsy ( longer repeats)  Dementia  Ataxia  Choreoathetosis  Onset usually in the 20s and death in the 40s  commaon in Japan  Combined degeneration of dentatorubral and pallidoluysian systems  DRPLA protein , neuronal cytoplasm
  53. 53. EPISODIC ATAXIA, TYPE 1; EA1 PAROXYSMAL ATAXIA WITH NEUROMYOTONIA, HEREDITARY EPISODIC ATAXIA WITH MYOKYMIA; EAM ATAXIA, EPISODIC, WITH MYOKYMIA; AEM; AEMK MYOKYMIA WITH PERIODIC ATAXIA  CLINICAL SYNOPSIS  Neurological  Miscellaneous  Labs  Treatment  Gene Map Locus: 12p13  Myokymia  Continuous muscle movement  Periodic ataxia  Continuous muscle movement  Periodic ataxia  Ataxic attacks provoked by abrupt postural change, emotional stimulus, and caloric-vestibular stimulation, startle  Onset in second decade  Hand posture resembling carpopedal spasm  Potassium voltage-gated channel gene mutation  Continuous spontaneous activity on EMG at rest  Muscle biopsy consistent with denervation, with enlargement of muscle fiber  Phenytoin, not Acetazolamide
  54. 54. EPISODIC ATAXIA, TYPE 2; EA2 PERIODIC VESTIBULOCEREBELLAR CEREBELLOPATHY, HEREDITARY PAROXYSMAL ATAXIA, FAMILIAL PAROXYSMAL ATAXIA ACETAZOLAMIDE-RESPONSIVE PAROXYSMAL CEREBELLARATAXIA; APCA EPISODIC ATAXIA, NYSTAGMUS-ASSOCIATED CEREBELLAR ATAXIA  CLINICAL SYNOPSIS  Neurological  Miscellaneous  Labs  Treatment  Gene Map Locus: 19p13  Episodic ataxia  Cerebellar ataxia  Vertigo  Diplopia  Downbeat nystagmus  Ataxia precipitated by stress or excitement, not by startle  attacks last 1/2 to 6 hrs.  point mutation alpha 1A calcium voltage dependant channel  allelic with SCA6 & familial hemiplegic migraine  Response to oral acetazolamide
  55. 55. AUTOSOMAL RECESSIVE ATAXIAS
  56. 56. • FRIEDERICK’S ATAXIA • ATAXIA TELANGIECTASIA • ATAXIA WITH ISOLATED VIT.E DEFICIENCY • ABETALIPOPROTEINEMIA • ENZYME DEFICIENCIES (Maple Syrup urine disease, Urea cycle defects, Sialidosis, Adrenoleucodystrophy,Organic aciduria, Pyruvate dehydogenase def.) AUTOSOMAL RECESSIVE ATAXIAS
  57. 57. Friederick’s ataxia • Unstable expansion of GAA repeatsFRATAXIN proteiniron accumulation in mitochondriamito injuryneuronal injury • DORSAL GANGLION CELLS- absent reflexes • DORSAL COLUMN DEGENERATION- dec.post.column senses • SPINOCEREBELLAR TRACT-gait atxia, dysarthria • CORTICOSPINAL TRACT- Babinski Positive • OTHER SIGNS- dysphagia,optic atrophy, spinal and foot deformities, diabetes (10%), cardiac defects (50%)
  58. 58. Friederick’s ataxia
  59. 59. • NATURAL HISTORY: -onset <25 yrs. At ADOLESCENCE -loss of ambulation 15 yrs. Since onset -Death usualyy due to cardiac complications • VARIANTS: -FA with Retained reflexes -Late onset FA Friederick’s ataxia
  60. 60. ATAXIA TELANGIECTASIA • OCULOMOTOR APRAXIA , TELANGIECATSIAS IN EYES, SKIN • Hematological malignancies (defective DNA repairs) • Infections (Ig deficiencies) • Other features-peripheral neuropathy, choreoathetosis
  61. 61. ATAXIA TELANGIECTASIA
  62. 62. Mitochondrial Inheritance • MERRF • MELAS • NARP • RP degeneration • Short stature, Endocrine deficiencies,
  63. 63. X linked ATAXIAS • X linked Dominant- Fragile X syndrome • CGG repeats’ expansion
  64. 64. • X linked Recessive Ataxias- Sideroblastic anemia with ataxia X linked ATAXIAS
  65. 65. SPORADIC or IDIOPATHIC ATAXIAS • Unknown genetic defects after ruling out acquired causes • Old age of onset • Presents with Dysautonomia –Orthostatic hypotension, erectile dysfunction, Urinary incontinence
  66. 66. Investigations • MRI Brain and Upper cervical cord • CT Head • Vit. E, B12 levels • Total cholesterol levels, Thyroid hormones • NCV and EMG studies (to rule out other systems’ involvement) • Toxicology screen (includes phenytoin levels) • Serology screen (for autoantibodies) • CSF analysis • Genetic Analyses (GAA, CGG, CAG repeat analyses)
  67. 67. TREATMENT • Reversible causes to be identified and treated • Structural lesions to be considered for surgery • Dietary modifications • IDEBENONE- in Friederick’s Ataxia • RILUZOLE- in Friederick’s Ataxia • ACETAZOLAMIDE- in Episodc Ataxia • GENETIC COUNSELLING
  68. 68. HISTORY SUMMARY 1. Duration: acute, subacute vs chronic 2. Rate of Progression: static vs progressive 3. Constant vs Paroxysmal 4. Associated features: - headache & vomiting suggesting mass lesion with raised ICP - previous neurological events (similar with ataxia - as in episodic ataxias, or other as in multiple sclerosis or vertebrobasilar TIAs) 5. Medical History: - recent infection, Hx of malignancy or weight loss, breast mass / tenderness, cough / hemoptysis - drug use / intoxication, medications, alcohol, smoking, environmental exposures 6. Family History positive or negative (in siblings or cousins but not parents suggesting autosomal recessive or parents and/or sibs suggesting autosomal dominant inheritance
  69. 69. EXAMINATION SUMMARY General examination: - signs of primary neoplasm (with paraneoplastic or metastatic ataxia), vascular disease (stroke), cardiac abnormality (Friedreick's) or Kayser-Fleischer rings (Wilson's) -short stature and cataracts with mitochondrial disease Higher Mental Functions: - confusion associated with ataxia in Wernicke's, drug or environmental toxicity, prion diseases or any condition obstructing 4th ventricle leading to hydrocephalus with raised ICP
  70. 70. Cranial Nerves: - ophthalmoplegia seen in Wernicke's, brainstem infarcts, demyelinating lesions, and Miller-Fisher syndrome (MFS) - nystagmus common in most vestibulocerebellar (or pancerebellar) disorders but prominent if drug toxicity (eg. phenytoin), Wernicke's and multiple sclerosis (also episodic ataxia-2) - associated brainstem (cranial nerve) dysfunction if concomitant involvement of brainstem or compression of it by mass effect from cerebellum - hearing loss or tinnitus with lesions of the cerebellopontine angle (eg. vestibular schwannoma or meningioma) EXAMINATION SUMMARY
  71. 71. EXAMINATION SUMMARY Motor: - weakness associated with ataxia is uncommon but can be seen ipsilaterally with infarcts (or other lesions) of the basis pontis or internal capsule (ataxic hemiparesis syndrome) - also seen in MFS (with concomitant demyelinating polyneuropathy), cord dysfunction (in paraneoplastic syndromes or demyelinating multifocal disease) - tremor associated either as intention tremor of cerebellar origin or postural tremor in FXTAS (Fragile X), multiple sclerosis, Wilson's disease - myoclonus in prion disorders with cerebellar involvement - parkinsonism with ataxia in multiple systems atrophy (also dystonia and chorea if DRPLA)
  72. 72. SUMMARY • RULE OUT “ATAXIA MIMICKERS” • CONFIRM PREDOMINANT CEREBELLAR INVOLVEMENT WITH RESPECTIVE TESTS • ANSWER THE “FOUR” QUESTIONS (Onset, progression, Symmetry, Localisation of lesion) • RULE OUT ACQUIRED CAUSES • LARGE PEDIGREE CHART • GENETIC ANALYSES
  73. 73. ALGORITHM PEDIGREE CHART ACQUIRED CAUSES AD IMAGING (MRI,CT) SCA1,2 MJD SCA6,7 SCA10,12 DRPLA SCA17 FA AT AVED ABETALIPOPROTEINEMIA

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