ATAXIAS: General Classification

ATAXIAS: General

Classification

Congenital

Hereditary
Autosomal Dominant
Autosomal Recessive
X-linked

Congenital
DNA repair defects
Episodic
Metabolic disorders
Mitochondrial
Multisystem disorders

Comparative pathology
External link

Immune
Acute cerebellar ataxia
Miller-Fisher
Multiple sclerosis
Opsoclonus-Myoclonus
Paraneoplastic
Infections: CNS
Acute ataxia: Viral
Creutzfeldt-Jakob
Meningitis

Mass lesion
Abscess
Neoplasm
Sarcoid

Paroxysmal
Epilepsy
Febrile
Hereditary
Migraine

Polyneuropathy
Anti-MAG Syndrome
GALOP Syndrome
Sensory Neuronopathy
Large fiber

Supratentorial
Gait disorders
Elderly
Extrapyramidal
Hydrocephalus

Systemic
Amyloid
Autoimmune
GAD antibodies
Endocrine
Hypoparathyroid
Thyroid
GI disorders
Celiac disease; Sprue
Vitamin E malabsorption
Whipple's

Trauma

Vascular

Vestibular
Toxins & Drugs

Ataxia: Differential diagnostic testing 1


CEREBELLAR DYSFUNCTION: ANATOMY

Cerebellar lesion Signs or Function
Posterior
(Flocculo-nodular lobe; Archicerebellum)
Eye movement disorders: Nystagmus; Vestibulo-ocular reflex (VOR)
Postural and gait dysfunction
Midline
(Vermis; paleocerebellum)
Truncal & gait ataxia
Hemisphere
(Neocerebellum)
Limb ataxia: Dysmetria, Dysdiadochokinesis, "Intention" tremor
Dysarthria
Hypotonia
Afferent defect: Mossy fibers
Vestibulopontine pathway
Spinocerebellar system (Clarke)
via: Inferior cerebellar peduncle
Dysmetria
Impaired stance & gait
Information about body parts in space
Vestibulocerebellar system
Vestibular nucleus to Flocculonodular lobe
Situation & location of head
Outflow pathways: Dentate nucleus Postural instability
Limb ataxia
Inferior olive Disturbed teaching error signals
Ataxia developing over time:
Especially trained limb movements (writing)

Ataxias: Comparative Pathology 2

Disease Dorsal root
ganglion
Clarke's
column
Vestibular
nucleus
Pontine
nucleus
Granule
cells
Purkinje
cells
Dentate
nucleus
Red
nucleus
Inferior
olive
DRPLA + +++ +
SCA6 + +++ ++ +
SCA31 +++
MSA + +++ + +++
SCA2 ++ +++ +++ +++ ++ + +++
SCA1 ++ + + ++ ++ ++ ++
SCA3 +++ ++ ++ + +++
FRDA +++ +++ ++ + +++
AOA1 +++ +++ + +++ + +


Patient Resources
Patient information

via http://neuromuscular.wustl.edu/ataxia/aindex.html

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