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'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''
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{{Infobox_Disease |
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ICD9 = 781.3
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{{SI}}
{{Ataxia}}
{{CMG}}; '''Associate Editor in Chief:''' {{MUT}} {{RT}}


'''''Synonyms and keywords:''''' unsteady gait, ataxy, staggering gait
'''For patient information click [[{{PAGENAME}} (patient information)|here]]'''


==Overview==
{{CMG}}; '''Associate Editor in Chief:''' {{MUT}}; {{RT}} {{APG}}
'''Ataxia''' (from Greek ''α-'' [used as a negative prefix] + ''-τάξις'' [order], meaning "lack of order") is a [[neurology|neurological]] sign and symptom consisting of gross incoordination of [[muscle]] movements. <ref>{{Dorlands|a_69|12166205}}</ref> Ataxia is an aspecific clinical manifestation implying dysfunction of parts of the [[nervous system]] that coordinate movement, such as the [[cerebellum]]. Several possible causes exist for these patterns of neurological dysfunction. The term "dystaxia" is rarely used as a synonym.


The International Ataxia Awareness Day is observed on September 25th each year.<ref name="titleNational Ataxia Foundation - International Ataxia Awareness Day">{{cite web |url=http://www.ataxia.org/events/international-ataxia-awareness-day.aspx |title=National Ataxia Foundation - International Ataxia Awareness Day |accessdate=2008-03-25 |format= |work=}}</ref>
'''''Synonyms and keywords:''''' Unsteady gait; ataxy; staggering gait; impaired coordination; lack of coordination; incoordination; incoordination of muscle movement


==Classification of ataxia==
==[[Ataxia overview|Overview]]==
===Cerebellar ataxia===
The term '''cerebellar ataxia''' is employed to indicate ataxia due to dysfunction of the cerebellum. This causes a variety of elementary neurological deficits, such as antagonist [[hypotonia]], [[asynergy]], [[dysmetria]], dyschronometria, and [[dysdiadochokinesia]]. How and where these abnormalities manifest depend on which cerebellar structures are lesioned, and whether the lesion is bilateral or unilateral.
'''Vestibulo-cerebellar dysfunction''' presents with [[postural instability]], in which the person tends to separate the feet on standing to gain a wider base, and avoid oscillations (especially posterior-anterior ones); instability is therefore worsened when standing with the feet together (irrespective of whether the eyes are open or closed: this is a negative [[Romberg's test]]).
'''Spino-cerebellar dysfunction''' presents with a wide-based "drunken sailor" gait, characterised by uncertain start and stop, lateral deviations, and unequal steps.
'''Cerebro-cerebellar dysfunction''' presents with disturbances in carrying out voluntary movements, including [[intention tremor]] (coarse trembling, accentuated over the execution of voluntary movements, possibly involving the head and eyes as well as the limbs and torso), peculiar writing abnormalities (large, unequal letters, irregular underlining), and a peculiar pattern of [[dysarthria]] (slurred speech, sometimes characterised by explosive variations in voice intensity despite a regular rhythm).


===Sensory ataxia===
==[[Ataxia classification scheme|Classification]]==
The term '''sensory ataxia''' is employed to indicate ataxia due to loss of [[proprioception]] (sensitivity to joint and body part position), which generally depends on dysfunction of the [[dorsal columns]] of the [[spinal cord]], since they carry proprioceptive information up to the brain; in some cases, the cause may instead be dysfunction of the various brain parts that receive that information, including the cerebellum, [[thalamus]], and [[parietal lobe|parietal lobes]]. Sensory ataxia presents
with an unsteady "stomping" gait with heavy [[heel]] strikes, as well as postural instability that is characteristically worsened when the lack of proprioceptive input cannot be compensated by [[sight|visual input]], such as in poorly lit environments. Doctors can evidence this during [[physical examination]] by having the patient stand with his / her feet together and [[eye|eyes]] shut, which will cause the patient's instability to markedly worsen, producing wide oscillations and possibly a fall (this is called a positive [[Romberg's test]]). Worsening of the finger-pointing test with the eyes closed is another feature of sensory ataxia. Also, when the patient is standing with arms and hands extended toward the examiner, if the eyes are closed, the patient's finger will tend to "fall down" and be restored to the horizontal extended position by sudden extensor contractions ("ataxic hand").


===Vestibular ataxia===
==[[Ataxia pathophysiology|Pathophysiology]]==
The term '''vestibular ataxia''' is employed to indicate ataxia due to dysfunction of the [[vestibular system]], which in acute and unilateral cases is associated with prominent [[vertigo (medical)|vertigo]], [[nausea]] and [[vomiting]]. In slow-onset, chronic bilateral cases of vestibular dysfunction, these characteristic manifestations may be absent, and dysequilibrium may be the sole presentation.


==Causes==
==[[Ataxia causes|Causes]]==
===Common Causes===
* [[Stroke]]
* [[Brain tumor]]
* [[Multiple sclerosis]]
* [[Alcoholism]]
* [[Head trauma]]
* [[Cerebral palsy]]
* [[Drug intoxication]]
* [[Heavy metal poisoning]]
* [[Hereditary ataxias]]
** [[Spinocerebellar ataxia]]s
** [[Friedreich's ataxia]]
** [[Ataxia-telangiectasia]]
* [[Wilson's disease]]


===Causes by Organ System===
==[[Ataxia differential diagnosis|Differentiating Ataxia from other Diseases]]==


{|style="width:80%; height:100px" border="1"
==[[Ataxia epidemiology and demographics|Epidemiology and Demographics]]==
|style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Cardiovascular'''
|style="height:100px"; style="width:75%" border="1" bgcolor="Beige" | [[Arteriosclerosis]], Circulation disorders in area of [[brain stem]], [[Morgagni's Syndrome]]
|-
|-bgcolor="LightSteelBlue"
| '''Chemical / poisoning'''
|bgcolor="Beige"| [[2,4,6-Trichlorophenol]], [[Acrylamide]], [[Aftershave]], [[Amitraz]], [[Aniline]], [[Antifreeze]], [[Apple seed poisoning]], [[Arizona Bark Scorpion poisoning]], [[Benzene]], [[Blue-ringed octopus poisoning]], [[Buckeye poisoning]], [[Carbon monoxide poisoning]], [[Death Camas poisoning]], [[English Ivy poisoning]], [[Gasoline]], [[Lantana poisoning]], [[Lead]], [[Mayapple poisoning]], [[Mercury]], [[Mescal poisoning]], [[Minamata disease]], [[Mistletoe poisoning]], [[Thallium poisoning]], [[Tolterodine toxicity]], [[Toxic mushrooms -- Anticholinergic]], [[Toxic mushrooms -- Monomethylhydrazine]], [[Toxic mushrooms -- Psychedelic]], [[Varnish makers' and painters' Naptha]], [[Xylene]]
|-
|-bgcolor="LightSteelBlue"
| '''Dermatologic'''
|bgcolor="Beige"| [[Erythrokeratodermia with ataxia]], [[Tranebjaerg-Svejgaard syndrome]], [[DeSanctis-Cacchione syndrome]], [[Xeroderma pigmentosum]]
|-
|-bgcolor="LightSteelBlue"
| '''Drug Side Effect'''
|bgcolor="Beige"| [[Amiodarone]], [[Barbituates]], [[Benzodiazepines]], [[Ethosuximide]], [[Gabapentin]], [[Lamotrigine]],  [[Levetiracetam]], [[Metronidazole]], [[Morphine]], [[Oxcarbazepine]], [[Primidone]]
|-
|-bgcolor="LightSteelBlue"
| '''Ear Nose Throat'''
|bgcolor="Beige"| [[Acoustic neurinoma]], [[Benign Paroxysmal Positional Vertigo]], [[Reardon-Wilson-Cavanagh syndrome]], [[Richards-Rundle syndrome]], [[Susac syndrome]], [[Treft-Sanborn-Carey syndrome]]
|-
|-bgcolor="LightSteelBlue"
| '''Endocrine'''
|bgcolor="Beige"| [[Adult-onset adreno leukodystrophy]], [[Diabetic neuropathy]], [[Functioning pancreatic endocrine tumor]], [[Furukawa-Takagi-Nakao syndrome]], [[Hypogonadism]], [[Hypothyroidism]], [[Insulinoma]], [[Metastatic insulinoma]], [[Morgagni's Syndrome]]
|-
|-bgcolor="LightSteelBlue"
| '''Environmental'''
|bgcolor="Beige"| [[Acute altitude sickness]]
|-
|-bgcolor="LightSteelBlue"
| '''Gastroenterologic'''
|bgcolor="Beige"| [[Bassen-Kornzweig syndrome]], [[Celiac disease]], [[Cirrhosis]], [[Dykes-Markes-Harper syndrome]], [[Hepatocerebral degeneration]], [[Wilson's Disease]]
|-
|-bgcolor="LightSteelBlue"
| '''Genetic'''
|bgcolor="Beige"| [[Alpers Syndrome]], [[Angleman syndrome]], [[Bangstad syndrome]], [[Bassen-Kornzweig syndrome]], [[Bhaskar-Jagannathan syndrome]], [[Cerebellar ataxia]], [[Cockayne syndrome]], [[Dravet syndrome]], [[Familial isolated deficiency of vitamin E]], [[Familial periodic ataxia]], [[Feline spongiform encephalopathy]], [[Friedreich's ataxia]], [[Griscelli disease]], [[Hallervorden-Spatz disease]], [[Hereditary spastic paraparesis]], [[Krabbe leukodystrophy]], [[Richards-Rundle syndrome]], [[Roussy-Levy syndrome]], [[Rubinstein-Taybi syndrome]], [[Sialidosis type 1 and 3]], [[Tranebjaerg-Svejgaard syndrome]], [[Xeroderma pigmentosum]], [[X-linked sideroblastic anaemia]]
|-
|-bgcolor="LightSteelBlue"
| '''Hematologic'''
|bgcolor="Beige"| [[Acanthocytosis]], [[Aceruloplasminemia]], [[Macrocytic anemia]], [[Pernicious anemia]], [[Revesz Debuse syndrome]], [[X-linked sideroblastic anaemia]]
|-
|-bgcolor="LightSteelBlue"
| '''Iatrogenic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Infectious Disease'''
|bgcolor="Beige"| [[Brain abscess]], [[Cerebellar abscess]], [[Chickenpox]], [[Diphtheria]], [[Encephalitis]], [[HIV infection]], [[Measles]], [[Quaternary syphilis]], [[Ramsay-Hunt syndrome]], [[Smallpox]], [[Tabes dorsalis]]
|-
|-bgcolor="LightSteelBlue"
| '''Musculoskeletal / Ortho'''
|bgcolor="Beige"| [[Dinno-Shearer-Weisskopf syndrome]], [[Furukawa-Takagi-Nakao syndrome]], [[Myopathy]]
|-
|-bgcolor="LightSteelBlue"
| '''Neurologic'''
|bgcolor="Beige"| [[Acute Disseminated Encephalomyelitis]], [[Alexander Syndrome]], [[Alpers Syndrome]], [[Arachnoid Cysts]], [[Arnold-Chiari Malformation]], [[ARTS syndrome]], [[Ataxia deafness reardon type]], [[Ataxia tapetoretinal degeneration]], [[Ataxia telangiectasia / Louis-Bar syndrome]], [[Ataxia-oculomotor apraxia syndrome]], [[Basal ganglia calcification]], [[Basilar artery migraine]], [[Vertebro-basilar artery ischemia]], [[Brain abscess]], [[Brain cancer]], [[Brain cyst]], [[Brain injury]], [[Brainstem or cortical lesions]], [[Central nervous system lymphoma, primary]], [[Cerebellar abscess]], [[Cerebellar ataxia]], [[Cerebellar cortex atrophy]], [[Cerebellar degeneration]], [[Cerebellar haemorrhage]], [[Cerebellar heredoataxia]], [[Cerebellar hypoplasia]], [[Cerebellar infarct]], [[Cerebellar mass]], [[Cerebral Amyloid Angiopathy, Familial]], [[Cerebral hemorrhage]], [[Cerebral infarction]], [[Cerebral palsy]], [[Cerebrorenodigital syndrome]], [[Cerebrovascular accident]], [[Ceroid lipofuscinosis]], [[Cervical myelopathy]], [[Chorea familial benign]], [[Chronic progressive traumatic encephalopathy]], [[Cockayne syndrome]], [[Combarros Calleja Leno syndrome]], [[Corneal cerebellar syndrome]], [[Creutzfeldt-Jakob disease]], [[Cutler-Bass-Romshe syndrome]], [[Dandy-Walker Syndrome]], [[Dementia pugilis]], [[Demyelinating disorder]], [[DeSanctis-Cacchione syndrome]], [[Dinno-Shearer-Weisskopf syndrome]], [[Dravet syndrome]], [[Dykes-Markes-Harper syndrome]], [[Dysequilibrium syndrome]], [[Encephalitis]], [[Encephalocele frontal]], [[Epiphyseal tumor]], [[Episodic ataxia]], [[Erythrokeratodermia with ataxia]], [[Feline spongiform encephalopathy]], [[Fisher's Syndrome]], [[Friedreich's ataxia]], [[Frontal lobe lesion]], [[GALOP Syndrome]] (gait disorder, autoantibodies, late age onset, polyneuropathy), [[Glioma]], [[Granulomatous Angiitis of the central nervous system]], [[Guillain-Barré syndrome]], [[Hallervorden-Spatz disease]], [[Heidenhain syndrome]], [[Hepatocerebral degeneration]], [[Hydrocephalus]], [[Infantile polymyoclonus]], [[Inherited neuropathies]], [[Joubert syndrome]], [[Juvenile pilocytic astrocytoma]], [[Kearns-Sayre Syndrome]], [[Kuru]], [[Lhermitte-McAlpine syndrome]], [[Machado-Joseph Disease]], [[Macrogyria, pseudobulbar palsy and mental retardation]], [[Mann Syndrome]], [[Marinesco-Sjogren-like syndrome]] (MSLS), [[Marinescu-Garland Syndrome]], [[Medulloblastoma]], [[Meinecke syndrome]], [[Meningoencephalocele]], [[Mental retardation progressive spasticity, X-linked]], [[Mental retardation, X-linked, with cerebellar hypoplasia and distinctive facial appearance]], [[Metachromatic Leukodystrophy]], [[Miller Fisher Syndrome]], [[Mitochondrial encephalomyopathy]], [[Mononeuropathy/radiculopathy affecting the lower extremities]], [[Multiple sclerosis]], [[Myelopathy]], [[Myoclonus]], [[Neuronal intranuclear hyaline inclusion disease]], [[Normal pressure hydrocephalus]], [[Olivopontocerebellar atrophy]], [[Opsoclonus myoclonus syndrome]], [[Parietal lobe lesion]], [[Parkinson's disease]], [[Partington X-linked mental retardation syndrome]], [[Pelizaeus-Merzbacher disease]], [[Peripheral neuropathy]], [[Polyneuritis]], [[Polyneuropathy]], [[Post-infective polyradiculopathy]], [[Posterior column ataxia with retinitis pigmentosa]], [[Posterior fossa tumor]], [[Progressive Multifocal Leukoencephalopathy]], [[Reardon-Wilson-Cavanagh syndrome]], [[Rett syndrome]], [[Rhombencephalosynapsis]], [[Roussy-Levy syndrome]], [[Schroer-Hammer-Mauldin syndrome]], [[Segawa syndrome, autosomal recessive]], [[Shy-Drager syndrome]], [[Spastic paraplegia]], [[Spinal cord compression (and pressure on dorsal nerve roots)]], [[Spinal cord tumor]], [[Spinocerebellar ataxia]], [[Spinocerebellar degenerescence, book type]], [[Stroke]], [[Susac syndrome]], [[Tabes dorsalis]], [[Telencephalic leukoencephalopathy]], [[Thalamic syndrome]], [[Thoracic dysplasia -- hydrocephalus syndrome]], [[Tranebjaerg-Svejgaard syndrome]], [[Transient ischemic attack (TIA)]], [[Transverse myelitis]], [[Vascular dementia]], [[Vertebral Artery Dissection]], [[Vertebrobasilar dolichoectasia]], [[Vertigo, benign paroxysmal]], [[Westphal's Disease]], [[Wilson's Disease]]
|-
|-bgcolor="LightSteelBlue"
| '''Nutritional / Metabolic'''
|bgcolor="Beige"| [[Abetalipoproteinemia]], [[Alpha-ketoglutarate dehydrogenase deficiency]], [[Aminoaciduria]], [[Argininosuccinase lyase deficiency - late onset]], [[Arginosuccinate synthetase deficiency]], [[Ataxia with Vitamin E Deficiency]], [[Biotinidase deficiency]], [[Ceruloplasmin deficiency]], [[Cholestanol storage disease]], [[Coenzyme Q 10 (CoQ10), deficiency]], [[Complex 2 mitochondrial respiratory chain deficiency]], [[Congenital Disorders of Glycosylation]], [[Cystinuria -- lysinuria]], [[Fabry's Disease]], [[Gangliosidosis GM1 type 3]], [[Gaucher disease type 2]], [[Glutathione synthase deficiency]], [[Glycine encephalopathy, atypical mild form]], [[GM2-gangliosidoses]], [[Hartnup Disease ]], [[Hexosaminidase deficiency]], [[Homozygous hypobetalipoproteinemia]], [[Hydroxyacyl-coa dehydrogenase, type 2, deficiency]], [[Infantile sialic acid storage disorder]], [[Lactic acidosis congenital infantile]], [[Lipoamide dehydrogenase deficiency]], [[Maple syrup urine disease]], [[Mevalonic aciduria]], [[Mitochondrial cytopathy]], [[Niemann-Pick disease]], [[Pellagra-like syndrome]], [[Phosphoribosylpyrophosphate synthetase superactivity]], [[Purine nucleoside phosphorylase deficiency]], [[Pyruvate dehydrogenase deficiency]], [[Refsum Disease]], [[Selective vitamin E deficiency]], [[Sialidosis type 1 and 3]], [[SSADH deficiency (succinic semialdehyde dehydrogenase deficiency)]], [[Thiamine deficiency]], [[Triglyceride storage disease with impaired long-chain fatty acid oxidation]], [[Vitamin B12 deficieny]], [[Wernicke-Korsakoff psychosis]], [[Zinc deficiency]]
|-
|-bgcolor="LightSteelBlue"
| '''Obstetric/Gynecologic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Oncologic'''
|bgcolor="Beige"| [[Acoustic neurinoma]], [[Brain cancer]], [[Bronchogenic carcinoma]], [[Central nervous system lymphoma, primary]], [[Epiphyseal tumor]], [[Functioning pancreatic endocrine tumor]], [[Glioma]], [[Insulinoma]], [[Juvenile pilocytic astrocytoma]], [[Medulloblastoma]], [[Metastatic insulinoma]], [[Paraneoplastic syndromes]], [[Posterior fossa tumor]], [[Spinal cord tumor]]
|-
|-bgcolor="LightSteelBlue"
| '''Opthalmologic'''
|bgcolor="Beige"| [[Combarros Calleja Leno syndrome]], [[Corneal cerebellar syndrome]], [[Fisher's Syndrome]], [[Marinesco-Sjogren-like syndrome]] (MSLS), [[Posterior column ataxia with retinitis pigmentosa]], [[Refsum Disease]], [[Revesz Debuse syndrome]], [[Schroer-Hammer-Mauldin syndrome]], [[Susac syndrome]], [[Treft-Sanborn-Carey syndrome]]
|-
|-bgcolor="LightSteelBlue"
| '''Overdose / Toxicity'''
|bgcolor="Beige"| [[Lithium toxicity]], [[Phenytoin toxicity]]
|-
|-bgcolor="LightSteelBlue"
| '''Psychiatric'''
|bgcolor="Beige"| [[Hysteria]]
|-
|-bgcolor="LightSteelBlue"
| '''Pulmonary'''
|bgcolor="Beige"| [[Bronchogenic carcinoma]], [[Prolonged anoxia]]
|-
|-bgcolor="LightSteelBlue"
| '''Renal / Electrolyte'''
|bgcolor="Beige"| [[Cerebrorenodigital syndrome]], [[Cutler-Bass-Romshe syndrome]]
|-
|-bgcolor="LightSteelBlue"
| '''Rheum / Immune / Allergy'''
|bgcolor="Beige"| [[ARTS syndrome]], [[Griscelli disease]], [[Fisher's Syndrome]]
|-
|-bgcolor="LightSteelBlue"
| '''Sexual'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Trauma'''
|bgcolor="Beige"| [[Brainstem]] or cortical lesions, [[Frontal lobe]]lesion, [[Mann Syndrome]], [[Parietal lobe]]lesion, [[Head trauma]]
|-
|-bgcolor="LightSteelBlue"
| '''Urologic'''
|bgcolor="Beige"| No underlying causes
|-
|-bgcolor="LightSteelBlue"
| '''Miscellaneous'''
|bgcolor="Beige"| [[Alcohol intoxication]], [[Amyloidosis, oculoleptomeningeal]], [[Arteriosclerosis]], [[Bonnemann-Meinecke-Reich syndrome]], [[Chitayat-Moore-Del Bigio syndrome]], [[Hemangioblastoma]], [[Hyperammonemia]], [[sporadic ataxia]], [[Tick paralysis]], [[Transthyretin amyloidosis]]
|-
|}


===Causes by Alphabetical Order===
==[[Ataxia risk factors|Risk Factors]]==
{{MultiCol}}
*[[2,4,6-Trichlorophenol]]
*[[Abetalipoproteinemia]]<ref name="pmid19066957">{{cite journal |author=Chardon L, Sassolas A, Dingeon B, ''et al.'' |title=Identification of two novel mutations and long-term follow-up in abetalipoproteinemia: a report of four cases |journal=Eur. J. Pediatr. |volume=168 |issue=8 |pages=983–9 |year=2009 |month=August |pmid=19066957 |doi=10.1007/s00431-008-0888-6 |url=}}</ref>
*[[Acanthocytosis]]
*[[Aceruloplasminemia]]<ref name="pmid20301666">{{cite journal |author=Pagon RA, Bird TD, Dolan CR, Stephens K, Adam MP, Miyajima H |title= |journal= |volume= |issue= |pages= |year= |pmid=20301666 |doi= |url=}}</ref>
*[[Acoustic neurinoma]]
*[[Acrylamide]]
*[[Acute altitude sickness]]<ref name="pmid22776381">{{cite journal |author=Oliver SJ, Sanders SJ, Williams CJ, ''et al.'' |title=Physiological and psychological illness symptoms at high altitude and their relationship with acute mountain sickness: a prospective cohort study |journal=J Travel Med |volume=19 |issue=4 |pages=210–9 |year=2012 |month=July |pmid=22776381 |doi=10.1111/j.1708-8305.2012.00609.x |url=}}</ref>
*[[Acute Disseminated Encephalomyelitis]]
*[[Adult-onset adreno leukodystrophy]]
*[[Aftershave]]
*[[Alcohol intoxication]]
*[[Alexander Syndrome]]
*[[Alpers Syndrome]]
*[[Alpha-ketoglutarate dehydrogenase deficiency]]<ref name="pmid1640293">{{cite journal |author=Bonnefont JP, Chretien D, Rustin P, ''et al.'' |title=Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis |journal=J. Pediatr. |volume=121 |issue=2 |pages=255–8 |year=1992 |month=August |pmid=1640293 |doi= |url=}}</ref>
*[[Aminoaciduria]]
*[[Amiodarone]]
*[[Amitraz]]
*[[Amyloidosis, oculoleptomeningeal]]
*[[X-linked sideroblastic anaemia]]
*[[Angleman syndrome]]
*[[Aniline]]
*[[Antifreeze]]
*[[Apple seed poisoning]]
*[[Arachnoid Cysts]]
*[[Argininosuccinase lyase deficiency - late onset]]
*[[Arginosuccinate synthetase deficiency]]
*[[Arizona Bark Scorpion poisoning]]
*[[Arnold-Chiari Malformation]]
*[[Arteriosclerosis]]
*[[ARTS syndrome]]
*[[Ataxia deafness reardon type]]
*[[Ataxia tapetoretinal degeneration]]
*[[Ataxia telangiectasia / Louis-Bar syndrome]]
*[[Ataxia with Vitamin E Deficiency]]
*[[Ataxia-oculomotor apraxia syndrome]]
*[[Bangstad syndrome]]
*[[Barbituates]]
*[[Basal ganglia calcification]]
*[[Basilar artery migraine]]
*[[Basilar impression / vertebral-basilar artery ischemia]]
*[[Bassen-Kornzweig syndrome]]
*[[Benign Paroxysmal Positional Vertigo]]
*[[Benzene]]
*[[Benzodiazepines]]
*[[Bhaskar-Jagannathan syndrome]]
*[[Biotinidase deficiency]]
*[[Blue-ringed octopus poisoning]]
*[[Bonnemann-Meinecke-Reich syndrome]]
*[[Brain abscess]]
*[[Brain cancer]]
*[[Brain cyst]]
*[[Brain injury]]
*[[Brainstem or cortical lesions]]
*[[Bronchogenic carcinoma]]
*[[Buckeye poisoning]]
*[[Carbon monoxide poisoning]]
*[[Celiac disease]]
*[[Central nervous system lymphoma, primary]]
*[[Cerebellar abscess]]
*[[Cerebellar ataxia]]
*[[Cerebellar cortex atrophy]]
*[[Cerebellar degeneration]]
*[[Cerebellar haemorrhage]]
*[[Cerebellar heredoataxia]]
*[[Cerebellar hypoplasia]]
*[[Cerebellar infarct]]
*[[Cerebellar mass]]
*[[Cerebral Amyloid Angiopathy, Familial]]
*[[Cerebral hemorrhage]]
*[[Cerebral infarction]]
*[[Cerebral palsy]]
*[[Cerebrorenodigital syndrome]]
{{ColBreak}}
*[[Cerebrovascular accident]]
*[[Ceroid lipofuscinosis]]
*[[Ceruloplasmin deficiency]]
*[[Cervical myelopathy]]
*[[Chickenpox]]
*[[Chitayat-Moore-Del Bigio syndrome]]
*[[Cholestanol storage disease]]
*[[Chorea familial benign]]
*[[Chronic progressive traumatic encephalopathy]]
*[[Cirrhosis]]
*[[Cockayne syndrome]]
*[[Coenzyme Q 10 (CoQ10), deficiency]]
*[[Combarros Calleja Leno syndrome]]
*[[Complex 2 mitochondrial respiratory chain deficiency]]
*[[Congenital Disorders of Glycosylation]]
*[[Corneal cerebellar syndrome]]
*[[Creutzfeldt-Jakob disease]]
*[[Cutler-Bass-Romshe syndrome]]
*[[Cystinuria -- lysinuria]]
*[[Dandy-Walker Syndrome]]
*[[DeSanctis-Cacchione syndrome]]
*[[Death Camas poisoning]]
*[[Dementia pugilis]]
*[[Demyelinating disorder]]
*[[Diabetic neuropathy]]
*[[Dinno-Shearer-Weisskopf syndrome]]
*[[Diphtheria]]
*[[Dravet syndrome]]
*[[Dykes-Markes-Harper syndrome]]
*[[Dysequilibrium syndrome]]
*[[Encephalitis]]
*[[Encephalocele frontal]]
*[[Encephalopathy due to sulphite oxidase deficiency]]
*[[English Ivy poisoning]]
*[[Epiphyseal tumor]]
*[[Episodic ataxia]]
*[[Erythrokeratodermia with ataxi]]
*[[Ethosuximide]]
*[[Fabry's Disease]]
*[[Familial isolated deficiency of vitamin E]]
*[[Familial periodic ataxia]]
*[[Feline spongiform encephalopathy]]
*[[Fisher's Syndrome]]
*[[Friedreich's ataxia]]
*[[Frontal lobe lesion]]
*[[Functioning pancreatic endocrine tumor]]
*[[Furukawa-Takagi-Nakao syndrome]]
*[[Gabapentin]]
*[[GALOP Syndrome (gait disorder, autoantibodies, late age onset, polyneuropathy)]]
*[[Gangliosidosis GM1 type 3 - gait ataxia]]
*[[Gasoline]]
*[[Gaucher disease type 2]]
*[[Glioma]]
*[[Glutathione synthase deficiency]]
*[[Glycine encephalopathy, atypical mild form]]
*[[GM2-gangliosidoses]]
*[[Granulomatous Angiitis of the Central Nervous System]]
*[[Griscelli disease]]
*[[Guillain-Barré syndrome]]
*[[Hallervorden-Spatz disease]]
*[[Hartnup Disease ]]
*[[Head trauma]]
*[[Heidenhain syndrome]]
*[[Hemangioblastoma]]
*[[Hepatocerebral degeneration]]
*[[Hereditary spastic paraparesis]]
*[[Hexosaminidase deficiency]]
*[[HIV infection]]
*[[Homozygous hypobetalipoproteinemia]]
{{ColBreak}}
*[[Hydrocephalus]]
*[[Hydroxyacyl-coa dehydrogenase, type 2, deficiency]]
*[[Hyperammonemia]]
*[[Hypogonadism]]
*[[Hypothyroidism]]
*[[Hysteria]]
*[[Infantile polymyoclonus]]
*[[Infantile sialic acid storage disorder]]
*[[Inherited neuropathies]]
*[[Insulinoma]]
*[[Joubert syndrome]]
*[[Juvenile pilocytic astrocytoma]]
*[[Kearns-Sayre Syndrome]]
*[[Krabbe leukodystrophy]]
*[[Kuru]]
*[[Lactic acidosis congenital infantile]]
*[[Lamotrigine]]
*[[Lantana poisoning]]
*[[Lead]]
*[[Levetiracetam]]
*[[Lhermitte-McAlpine syndrome]]
*[[Lipoamide dehydrogenase deficiency]]
*[[Lithium toxicity]]
*[[Machado-Joseph Disease]]
*[[Macrocytic anemia]]
*[[Macrogyria, pseudobulbar palsy and mental retardation]]
*[[Mann Syndrome]]
*[[Maple syrup urine disease]]
*[[Marinesco-Sjogren-like syndrome (MSLS)]]
*[[Marinescu-Garland Syndrome]]
*[[Mayapple poisoning]]
*[[Measles]]
*[[Medulloblastoma]]
*[[Meinecke syndrome]]
*[[Meningoencephalocele]]
*[[Mental retardation progressive spasticity, X-linked]]
*[[Mental retardation, X-linked, with cerebellar hypoplasia and distinctive facial appearance]]
*[[Mercury]]
*[[Mescal poisoning]]
*[[Metachromatic Leukodystrophy]]
*[[Metastatic insulinoma]]
*[[Metronidazole]]
*[[Mevalonic aciduria]]
*[[Miller Fisher Syndrome]]
*[[Minamata disease]]
*[[Mistletoe poisoning]]
*[[Mitochondrial cytopathy]]
*[[Mitochondrial encephalomyopathy]]
*[[Mononeuropathy/radiculopathy affecting the lower extremities]]
*[[Morgagni's Syndrome]]<ref name="pmid818785">{{cite journal |author=Henschen F |title=[Morgagni's syndrome] |language=German |journal=Virchows Arch A Pathol Anat Histol |volume=370 |issue=1 |pages=1–11 |year=1976 |month=April |pmid=818785 |doi= |url=}}</ref>
*[[Morphine]]
*[[Multiple sclerosis]]
*[[Myelopathy]]
*[[Myoclonus]]
*[[Myopathy]]
*[[Neuronal intranuclear hyaline inclusion disease]]
*[[Niemann-Pick disease]]
*[[Normal pressure hydrocephalus]]
*[[Olivopontocerebellar atrophy]]
*[[Opsoclonus myoclonus syndrome]]
*[[Oxcarbazepine]]
*[[Paraneoplastic syndromes]]
*[[Parietal lobe lesion]]
*[[Parkinson's disease]]
*[[Partington X-linked mental retardation syndrome]]
*[[Pelizaeus-Merzbacher disease]]<ref name="pmid22511562">{{cite journal |author=Kitsiou-Tzeli S, Konstantinidou A, Sofocleous C, ''et al.'' |title=Familial Pelizaeus-Merzbacher disease caused by a 320.6-kb Xq22.2 duplication and the pathological findings of a male fetus |journal=Birth Defects Res. Part A Clin. Mol. Teratol. |volume=94 |issue=6 |pages=494–8 |year=2012 |month=June |pmid=22511562 |doi=10.1002/bdra.23015 |url=}}</ref>
*[[Pellagra-like syndrome]]
*[[Peripheral neuropathy]]
*[[Pernicious anemia]]
*[[Phenytoin toxicity]]
{{ColBreak}}
*[[Phosphoribosylpyrophosphate synthetase superactivity]]
*[[Polyneuritis]]
*[[Polyneuropathy]]
*[[Post-infective polyradiculopathy]]
*[[Posterior column ataxia with retinitis pigmentosa]]
*[[Posterior fossa tumor]]
*[[Primidone]]
*[[Progressive Multifocal Leukoencephalopathy]]
*[[Prolonged anoxia]]
*[[Purine nucleoside phosphorylase deficiency]]
*[[Pyruvate dehydrogenase deficiency]]
*[[Quaternary syphilis]]
*[[Ramsay-Hunt syndrome]]
*[[Reardon-Wilson-Cavanagh syndrome]]
*[[Refsum Disease]]
*[[Rett syndrome]]
*[[Revesz Debuse syndrome]]
*[[Rhombencephalosynapsis]]
*[[Richards-Rundle syndrome]]<ref name="pmid6425460">{{cite journal |author=Franceschi M, Parmigiani F, Zamproni P, Cairoli G, Canal N |title=Richards-Rundle syndrome, cochleovestibular dysfunction and neurofibromatosis in a family |journal=J. Neurol. |volume=231 |issue=1 |pages=11–3 |year=1984 |pmid=6425460 |doi= |url=}}</ref>
*[[Roussy-Levy syndrome]]
*[[Rubinstein-Taybi syndrome]]
*[[Schroer-Hammer-Mauldin syndrome]]
*[[Segawa syndrome, autosomal recessive]]
*[[Selective vitamin E deficiency]]
*[[Shy-Drager syndrome]]
*[[Sialidosis type 1 and 3]]
*[[Smallpox]]
*[[Spastic paraplegia]]
*[[Spinal cord compression (and pressure on dorsal nerve roots)]]
*[[Spinal cord tumor]]
*[[Spinocerebellar ataxia]]
*[[Spinocerebellar degenerescence, book type]]
*[[sporadic ataxia]]
*[[SSADH deficiency (succinic semialdehyde dehydrogenase deficiency)]]
*[[Stroke]]
*[[Susac syndrome]]
*[[Tabes dorsalis]]
*[[Telencephalic leukoencephalopathy]]
*[[Thalamic syndrome]]
*[[Thallium poisoning]]
*[[Thiamine deficiency]]
*[[Thoracic dysplasia -- hydrocephalus syndrome]]
*[[Tick paralysis]]
*[[Tolterodine toxicity]]
*[[Toxic mushrooms -- Anticholinergic]]
*[[Toxic mushrooms -- Monomethylhydrazine]]
*[[Toxic mushrooms -- Psychedelic]]
*[[Tranebjaerg-Svejgaard syndrome]]
*[[Transient ischemic attack (TIA)]]
*[[Transthyretin amyloidosis]]
*[[Transverse myelitis]]
*[[Treft-Sanborn-Carey syndrome]]
*[[Triglyceride storage disease with impaired long-chain fatty acid oxidation]]
*[[Varnish makers' and painters' Naptha]]
*[[Vascular dementia]]
*[[Vertebral Artery Dissection]]
*[[Vertebrobasilar dolichoectasia]]
*[[Vertigo, benign paroxysmal, in childhood - ataxic gait]]
*[[Vitamin B12 deficieny]]
*[[Wernicke-Korsakoff psychosis]]
*[[Westphal's Disease]]
*[[Wilson's Disease]]
*[[Xeroderma pigmentosum]]
*[[Xylene]]
*[[Zinc deficiency]]
{{EndMultiCol}}


==Risk factors==
==[[Ataxia natural history|Natural History, Complications and Prognosis]]==
Those who have a family history of inherited ataxia (e.g Friedreich's ataxia) may be at higher risk for developing ataxia.  Genetic testing may be recommended in these cases.


==Diagnosis==
==Diagnosis==
===Symptoms===
[[Ataxia history and symptoms|History and Symptoms]] | [[Ataxia physical examination|Physical Examination]] | [[Ataxia laboratory tests|Laboratory Findings]] | [[Ataxia x ray|X Ray]] | [[Ataxia CT|CT]] | [[Ataxia MRI|MRI]] | [[Ataxia other imaging findings|Other Imaging Findings]] | [[Ataxia other diagnostic studies|Other Diagnostic Studies]]
Symptoms may include:
*Inability to stand without swaying
*A uneven gait that may look like the person is drunk
*Involuntary shifting of the eyes (nystagmus)
*Unusual speech patterns such as:
**Monotonous tone
**Either rushing words or placing large gaps between syllables
*Impaired motions
*Tremors in the limbs, particularly towards the end of movements


A patient with ataxia may also experience other neurological symptoms such as dementia, seizures, or a wide array of other disorders.
<ref name="pmid30786918">{{cite journal |vauthors=de Silva R, Greenfield J, Cook A, Bonney H, Vallortigara J, Hunt B, Giunti P |title=Guidelines on the diagnosis and management of the progressive ataxias |journal=Orphanet J Rare Dis |volume=14 |issue=1 |pages=51 |date=February 2019 |pmid=30786918 |pmc=6381619 |doi=10.1186/s13023-019-1013-9 |url=}}</ref>
 
===Lab tests===
Diagnostic evaluation of [[Ataxia]] include:
* [[Complete blood count]]s and differential count
* [[Cerebro spinal fluid]] examination for cell count, proteins and glucose levels - if fever is present
* Toxicology screening
* [[CT]] brain with and without contrast - for [[intracranial bleeding]] and lesions
* [[MRI]] brain - for masses
* Genetic testing


==Treatment==
==Treatment==
There is no specific treatment for ataxia as such, altough there may be for the underlying cause. The disability of ataxia may be reduced by physical therapy, including exercises, along with leg braces or shoe splints, if foot alignment has been affected; a cane or walker is often used in the effort to prevent falls.
The ataxia that occurs in children can often can go away in a few months without any treatment. In cases where an underlying cause is identified, the cause will be treated.


* Recovery tends to be better in individuals with a single focal injury (such as [[stroke]] or a [[benign tumour]]), compared to those who have a neurological degenerative condition.<ref name="can rehabilitation help">{{cite journal | author = Morton SM, Bastian AJ | title = Can rehabilitation help ataxia? | journal = Neurology | volume = 73 | issue = 22 | pages = 1818–9 | year = 2009 | month = December | pmid = 19864635 | doi = 10.1212/WNL.0b013e3181c33b21 | url = }}</ref>
In some cases, you may have continuing and disabling symptoms. Treatment includes:


* The movement disorders associated with ataxia can be managed by pharmacological treatments and through [[physical therapy]] and [[occupational therapy]] to reduce [[disability]].<ref name="pmid17000340">{{cite journal | author = Perlman SL | title = Ataxias | journal = Clin. Geriatr. Med. | volume = 22 | issue = 4 | pages = 859–77, vii | year = 2006 | month = November | pmid = 17000340 | doi = 10.1016/j.cger.2006.06.011 }}</ref> Some drug treatments that have been used to control ataxia include: 5-hydroxytryptophan (5-HTP), idebenone, [[amantadine]], physostigmine, L-carnitine or derivatives, trimethoprim–sulfamethoxazole, vigabatrin, phosphatidylcholine, [[acetazolamide]], 4-aminopyridine, buspirone, and a combination of  [[coenzyme Q10]] and [[vitamin E]].
* Corticosteroids
* IV immune globulin
* [[Physical therapy]] requires a focus on adapting activity and facilitating [[motor learning]] for retraining specific functional motor patterns.<ref name="Intensive coordinative training improves motor performance in degenerative cerebellar disease">{{cite journal | author = Ilg W, Synofzik M, Brötz D, Burkard S, Giese MA, Schöls L | title = Intensive coordinative training improves motor performance in degenerative cerebellar disease | journal = Neurology | volume = 73 | issue = 22 | pages = 1823–30 | year = 2009 | month = December | pmid = 19864636 | doi = 10.1212/WNL.0b013e3181c33adf  }}</ref> A recent systematic review suggested that physical therapy is effective, but there is only moderate evidence to support this conclusion.<ref name="pmid19114434">{{cite journal | author = Martin CL, Tan D, Bragge P, Bialocerkowski A | title = Effectiveness of physiotherapy for adults with cerebellar dysfunction: a systematic review | journal = Clin Rehabil | volume = 23 | issue = 1 | pages = 15–26 | year = 2009 | month = January | pmid = 19114434 | doi = 10.1177/0269215508097853 }}</ref> The most commonly used physical therapy interventions for cerebellar ataxia are vestibular habituation, proprioceptive neurofaciliation, Frenkel exercises, and balance training; however, therapy is often highly individualized and gait and coordination training are large components of therapy. 
* Plasma exchange therapy
* Medications to improve muscle coordination


* Current research suggests that, if a person is able to walk with or without a [[mobility aid]], physical therapy should include an exercise program addressing five components: static balance, dynamic balance, trunk-limb coordination, stairs, and [[contracture]] prevention.  Once the physical therapist determines that the individual is able to safely perform parts of the program independently, it is important that the individual be prescribed and regularly engage in a supplementary home exercise program that incorporates these components to further improve long term outcomes. These outcomes include balance tasks, gait, and individual activities of daily living.  While the improvements are attributed primarily to changes in the brain and not just the hip and/or ankle joints, it is still unknown whether the improvements are due to adaptations in the cerebellum or compensation by other areas of the brain.<ref name="Intensive coordinative training improves motor performance in degenerative cerebellar disease"/> 
Occupational or physical therapy may also be needed. Changes to diet and nutritional supplements may also help.


* Decomposition, simplification, or slowing of multijoint movement may also be an effective strategy that therapists may use to improve function in patients with ataxia.<ref name="pmid9184691">{{cite journal | author = Bastian AJ | title = Mechanisms of ataxia | journal = Phys Ther | volume = 77 | issue = 6 | pages = 672–5 | year = 1997 | month = June | pmid = 9184691 | doi = }}</ref> Training likely needs to be intense and focused—as indicated by one study performed with stroke patients experiencing limb ataxia who underwent intensive upper limb retraining.<ref name="stroke ataxia">{{cite journal | author = Richards L, Senesac C, McGuirk T, Woodbury M, Howland D, Davis S, Patterson T | title = Response to intensive upper extremity therapy by individuals with ataxia from stroke | journal = Top Stroke Rehabil | volume = 15 | issue = 3 | pages = 262–71 | year = 2008 | pmid = 18647730 | doi = 10.1310/tsr1503-262 }}</ref> Their therapy consisted of [[constraint-induced movement therapy]] which resulted in improvements of their arm function.<ref name ="stroke ataxia" /> Treatment should likely include strategies to manage difficulties with everyday activities such as walking. Gait aids (such as a cane or walker) can be provided to decrease the risk of falls associated with impairment of [[Balance (ability)|balance]] or poor [[Motor coordination|coordination]]. Severe ataxia may eventually lead to the need for a [[wheelchair]]. In order to obtain better results, possible coexisting motor deficits need to be addressed in addition to those induced by ataxia. For example, muscle weakness and decreased endurance could lead to increasing fatigue and poorer movement patterns.


* There are several assessment tools available to therapists and health care professionals working with patients with ataxia.  The [[International Cooperative Ataxia Rating Scale]] (ICARS) is one of the most widely used and has been proven to have very high reliability and validity.<ref>{{cite journal | author = Schmitz-Hübsch T, Tezenas du Montcel S, Baliko L, Boesch S, Bonato S, Fancellu R, Giunti P, Globas C, Kang JS, Kremer B, Mariotti C, Melegh B, Rakowicz M, Rola R, Romano S, Schöls L, Szymanski S, van de Warrenburg BP, Zdzienicka E, Dürr A, Klockgether T | title = Reliability and validity of the International Cooperative Ataxia Rating Scale: a study in 156 spinocerebellar ataxia patients | journal = Mov. Disord. | volume = 21 | issue = 5 | pages = 699–704 | year = 2006 | month = May | pmid = 16450347 | doi = 10.1002/mds.20781 }}</ref>  Other tools that assess motor function, balance and coordination are also highly valuable to help the therapist track the progress of their patient, as well as to quantify the patient's functionality.  These tests include, but are not limited to:
** The [[Berg Balance Scale]]
** Tandem Walking (to test for [[Tandem gait]]ability)
** Scale for the Assessment and Rating of Ataxia<ref name="pmid16769946">{{cite journal | author = Schmitz-Hübsch T, du Montcel ST, Baliko L, Berciano J, Boesch S, Depondt C, Giunti P, Globas C, Infante J, Kang JS, Kremer B, Mariotti C, Melegh B, Pandolfo M, Rakowicz M, Ribai P, Rola R, Schöls L, Szymanski S, van de Warrenburg BP, Dürr A, Klockgether T, Fancellu R | title = Scale for the assessment and rating of ataxia: development of a new clinical scale | journal = Neurology | volume = 66 | issue = 11 | pages = 1717–20 | year = 2006 | month = June | pmid = 16769946 | doi = 10.1212/01.wnl.0000219042.60538.92 }}</ref>
** tapping tests - The person must quickly and repeatedly tap their arm or leg while the therapist monitors the amount of [[dysdiadochokinesia]].<ref name="Notermans_1994">{{cite journal | author = Notermans NC, van Dijk GW, van der Graaf Y, van Gijn J, Wokke JH | title = Measuring ataxia: quantification based on the standard neurological examination | journal = J. Neurol. Neurosurg. Psychiatr. | volume = 57 | issue = 1 | pages = 22–6 | year = 1994 | month = January | pmid = 8301300 | pmc = 485035 | doi = 10.1136/jnnp.57.1.22 }}</ref>
** [[finger-nose testing]]<ref name="Notermans_1994"/> - This test has several variations including finger-to-therapist's finger, finger-to-finger, and alternate nose-to-finger.<ref name="urlOPETA: Neurologic Examination">{{cite web | url = http://medinfo.ufl.edu/other/opeta/neuro/NE_ch3.html | title = OPETA: Neurologic Examination | author = | date = | format = | work = Online physical exam teaching assistant | publisher = The UF College of Medicine Harrell Center | accessdate = 2012-05-07 }}</ref>


==References==
{{reflist|2}}


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Latest revision as of 17:05, 20 October 2020

Ataxia
ICD-10 R27.0
ICD-9 781.3

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor in Chief: M.Umer Tariq [2]; Raviteja Guddeti, M.B.B.S. [3] Apeksha Gupta, MBBS[4]

Synonyms and keywords: Unsteady gait; ataxy; staggering gait; impaired coordination; lack of coordination; incoordination; incoordination of muscle movement

Overview

Classification

Pathophysiology

Causes

Differentiating Ataxia from other Diseases

Epidemiology and Demographics

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | X Ray | CT | MRI | Other Imaging Findings | Other Diagnostic Studies

[1]

Treatment

The ataxia that occurs in children can often can go away in a few months without any treatment. In cases where an underlying cause is identified, the cause will be treated.

In some cases, you may have continuing and disabling symptoms. Treatment includes:

  • Corticosteroids
  • IV immune globulin
  • Plasma exchange therapy
  • Medications to improve muscle coordination

Occupational or physical therapy may also be needed. Changes to diet and nutritional supplements may also help.


Template:WikiDoc Sources

  1. ↑ de Silva R, Greenfield J, Cook A, Bonney H, Vallortigara J, Hunt B, Giunti P (February 2019). "Guidelines on the diagnosis and management of the progressive ataxias". Orphanet J Rare Dis. 14 (1): 51. doi:10.1186/s13023-019-1013-9. PMC 6381619. PMID 30786918.
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