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| | __NOTOC__ |
| {{Infobox_Disease | | | {{Infobox_Disease | |
| Name = {{PAGENAME}} |
| | Name = {{PAGENAME}} | |
| ICD10 = {{ICD10|R|27|0|r|25}} |
| | ICD10 = {{ICD10|R|27|0|r|25}} | |
| ICD9 = 781.3
| | ICD9 = 781.3 |
| }} | | }} |
| {{SI}} | | {{Ataxia}} |
| {{CMG}}
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| '''Associate Editor in Chief:''' {{MUT}} | | '''For patient information click [[{{PAGENAME}} (patient information)|here]]''' |
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| {{Editor Help}} | | {{CMG}}; '''Associate Editor in Chief:''' {{MUT}}; {{RT}} {{APG}} |
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| ==Overview==
| | '''''Synonyms and keywords:''''' Unsteady gait; ataxy; staggering gait; impaired coordination; lack of coordination; incoordination; incoordination of muscle movement |
| '''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. | |
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| 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>
| | ==[[Ataxia overview|Overview]]== |
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| ==Types of ataxia== | | ==[[Ataxia classification scheme|Classification]]== |
| ===Cerebellar ataxia===
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| 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.
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| '''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]]).
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| '''Spino-cerebellar dysfunction''' presents with a wide-based "drunken sailor" gait, characterised by uncertain start and stop, lateral deviations, and unequal steps.
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| '''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).
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| ===Sensory ataxia=== | | ==[[Ataxia pathophysiology|Pathophysiology]]== |
| 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
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| 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").
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| ===Vestibular ataxia=== | | ==[[Ataxia causes|Causes]]== |
| 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.
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| ==Complete Differential Diagnosis of Ataxia== | | ==[[Ataxia differential diagnosis|Differentiating Ataxia from other Diseases]]== |
| * [[Acanthocytosis]]
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| * [[Acoustic neurinoma]]
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| * [[Alcoholism]]
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| * Alpha-beta lipoproteinemia
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| * [[Arnold-Chiari Malformation]]
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| * [[Arteriosclerosis]]
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| * [[Ataxia-teleangiectasia]]
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| * Basilar impression / [[vertebral-basilar artery ischemia]]
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| * [[Brainstem]] or cortical lesions
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| * [[Cerebellar]] [[abscess]]
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| * [[Cerebellar]] cortex [[atrophy]]
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| * [[Cerebellar heredoataxia]]
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| * [[Chickenpox]]
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| * Circulation disorders in area of [[brain stem]]
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| * [[Cirrhosis]]
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| * [[Diabetic neuropathy]]
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| * [[Encephalitis]]
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| * [[Epiphyseal]] [[tumor]]
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| * [[Familial periodic ataxia]]
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| * [[Fisher's Syndrome]]
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| * [[Fredreich's Ataxia]]
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| * [[Frontal lobe]] lesion
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| * [[GALOP Syndrome]] ('''g'''ait disorder, '''a'''utoantibodies, '''l'''ate age '''o'''nset, '''p'''olyneuropathy)
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| * [[GM2-gangliosidoses]]
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| * [[Hartnup Syndrome]]
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| * [[Hemorrhage]]
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| * [[Hereditary spastic paraparesis]]
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| * [[HIV|HIV infection]]
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| * [[Hypothyroidism]]
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| * Hysterical gaits
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| * [[Infection]]
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| * Inherited neuropathies
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| * [[Intoxication]]
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| *:* [[Alcohol]]
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| *:* [[Benzodiazepines]]
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| *:* [[Carbon monoxide]]
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| *:* [[Morphine]]
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| *:* [[Barbituates]]
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| * [[Malabsorption]]
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| * [[Mann Syndrome]]
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| * [[Marinescu-Garland Syndrome]]
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| * [[Measles]]
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| * Mitochondrial cytopathy
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| * Mononeuropathy/[[radiculopathy]] affecting the lower extremities
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| * [[Morgagni's Syndrome]]
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| * [[Multiple Sclerosis]]
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| * [[Myelopathy]]
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| * [[Myopathy]]
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| * [[Normal pressure hydrocephalus]]
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| * Orthopedic issues affecting the hip, knee, leg, foot, ankle
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| * [[Paraneoplastic syndrome]]
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| * [[Parietal lobe]] lesion
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| * [[Parkinsonism]]
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| * [[Peripheral neuropathy]]
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| * [[Polyneuritis]]
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| * [[Smallpox]]
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| * [[Spinal cord compression]] (and pressure on dorsal nerve roots)
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| * [[Spinocerebellar ataxia]]
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| * [[Tabes dorsalis]]
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| * [[Thalamic syndrome]]
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| * [[Thiamine deficiency]]
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| * [[Trauma]]
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| * [[Tumor]]
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| * [[Vestibular apparatus]] diseases
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| * [[Vitamin B12 Deficiency]]
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| * [[Wernicke's Encephalopathy]]
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| * [[Westphal's Disease]]
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| * [[Wilson's Disease]]
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| ===Complete Differential Diagnosis of the Causes of Ataxia=== | | ==[[Ataxia epidemiology and demographics|Epidemiology and Demographics]]== |
| (By organ system)
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| {|style="width:80%; height:100px" border="1"
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| |style="height:100px"; style="width:25%" border="1" bgcolor="LightSteelBlue" | '''Cardiovascular'''
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| |style="height:100px"; style="width:75%" border="1" bgcolor="Beige" |
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| [[Arteriosclerosis]],
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| Circulation disorders in area of [[brain stem]],
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| [[Morgagni's Syndrome]],
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| | ==[[Ataxia risk factors|Risk Factors]]== |
| |-bgcolor="LightSteelBlue"
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| | '''Chemical / poisoning'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Dermatologic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Drug Side Effect'''
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| |bgcolor="Beige"|
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| [[Barbituates]], | |
| [[Benzodiazepines]],
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| [[Morphine]],
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| | ==[[Ataxia natural history|Natural History, Complications and Prognosis]]== |
| |-bgcolor="LightSteelBlue"
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| | '''Ear Nose Throat'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Endocrine'''
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| |bgcolor="Beige"|
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| [[Diabetic neuropathy]], | |
| [[Hypothyroidism]],
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| |- | | ==Diagnosis== |
| |-bgcolor="LightSteelBlue" | | [[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]] |
| | '''Environmental''' | |
| |bgcolor="Beige"| No underlying causes | |
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| |-bgcolor="LightSteelBlue" | |
| | '''Gastroenterologic''' | |
| |bgcolor="Beige"| | |
| [[Cirrhosis]], | |
| [[Malabsorption]], | |
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| | <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> |
| |-bgcolor="LightSteelBlue"
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| | '''Genetic'''
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| |bgcolor="Beige"| | |
| [[Familial periodic ataxia]],
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| [[Fredreich's Ataxia]],
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| [[Hartnup Syndrome]],
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| [[Hereditary spastic paraparesis]],
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| Inherited neuropathies,
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| | ==Treatment== |
| |-bgcolor="LightSteelBlue"
| | 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. |
| | '''Hematologic'''
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| |bgcolor="Beige"|
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| [[Acanthocytosis]],
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| [[Ataxia-teleangiectasia]],
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| |-bgcolor="LightSteelBlue"
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| | '''Iatrogenic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Infectious Disease'''
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| |bgcolor="Beige"|
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| [[Chickenpox]],
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| [[HIV infection]],
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| [[Smallpox]],
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Musculoskeletal / Ortho'''
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| |bgcolor="Beige"|
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| [[Myopathy]],
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Neurologic'''
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| |bgcolor="Beige"|
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| [[Acoustic neurinoma]],
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| [[Arnold-Chiari Malformation]],
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| Cerebellar abscess,
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| [[Cerebellar]] cortex [[atrophy]],
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| [[Cerebellar heredoataxia]],
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| [[Encephalitis]],
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| [[GALOP Syndrome]] (gait disorder, autoantibodies, late age onset, polyneuropathy),
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| [[Multiple Sclerosis]],
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| [[Normal pressure hydrocephalus]],
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| [[Parkinsonism]],
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| [[Peripheral neuropathy]],
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| [[Polyneuritis]],
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| [[Spinal cord compression]](and pressure on dorsal nerve roots),
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| [[Tabes dorsalis]],
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| [[Wernicke's Encephalopathy]],
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Nutritional / Metabolic'''
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| |bgcolor="Beige"|
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| [[Alcohol]],
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| Alpha-beta lipoproteinemia,
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| [[GM2-gangliosidoses]],
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| [[Thiamine deficiency]],
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| [[Wilson's Disease]],
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Obstetric/Gynecologic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Oncologic'''
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| |bgcolor="Beige"|
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| Epiphyseal tumor,
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| [[Paraneoplastic syndrome]],
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Opthalmologic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Overdose / Toxicity'''
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| |bgcolor="Beige"|
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| [[Intoxication]],
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Psychiatric'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Pulmonary'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Renal / Electrolyte'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Rheum / Immune / Allergy'''
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| |bgcolor="Beige"|
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| [[Fisher's Syndrome]],
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Sexual'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Trauma'''
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| |bgcolor="Beige"|
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| [[Brainstem]] or cortical lesions,
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| [[Frontal lobe]]lesion,
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| [[Mann Syndrome]],
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| [[Parietal lobe]]lesion,
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Urologic'''
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| |bgcolor="Beige"| No underlying causes
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| |-
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| |-bgcolor="LightSteelBlue"
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| | '''Miscellaneous'''
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| |bgcolor="Beige"|Mitochondrial cytopathy,
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| |-
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| |}
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| ==Causes of ataxia==
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| The three types of ataxia have overlapping causes, and can therefore either coexist or occurr in isolation. | |
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| ===Focal lesions===
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| Any type of focal lesion of the [[central nervous system]] (such as [[stroke]], [[brain tumour]], [[multiple sclerosis]] will cause the type of ataxia corresponding to the site of the lesion: cerebellar if in the cerebellum, sensory if in the dorsal spinal cord (and rarely in the [[thalamus]] or [[parietal lobe]]), vestibular if in the vestibular system (including the vestibular areas of the [[cerebral cortex]]).
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| ===Exogenous substances===
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| Exogenous substances that cause ataxia mainly do so because they have a depressant effect on central nervous system function. The most common example is [[ethanol]], which is capable of causing overlapping cerebellar and vestibular ataxia. Other examples include both prescription drugs (e.g. most antiepileptic drugs have cerebellar ataxia as a possible unwanted effect) and recreational drugs (e.g. [[ketamine]], [[PCP]]).
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| ===Vitamin B<sub><small>12</small></sub> deficiency===
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| [[cyanocobalamin|Vitamin B<sub><small>12</small></sub>]] deficiency may cause, among several neurological abnormalities, overlapping cerebellar and sensory ataxia.
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| ===Causes of isolated sensory ataxia===
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| [[Peripheral neuropathy|Peripheral neuropathies]] may cause generalised or localised sensory ataxia (e.g. a limb only) depending on the extent of the neuropathic involvement. Spinal disorders of various types may cause sensory ataxia from the lesioned level below, when they involve the dorsal columns.
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| ===Non-hereditary cerebellar degeneration===
| | In some cases, you may have continuing and disabling symptoms. Treatment includes: |
| Non-hereditary causes of cerebellar degeneration include chronic [[ethanol abuse]], [[paraneoplastic cerebellar degeneration]], [[high altitude cerebral oedema]], [[coeliac disease]], and [[normal pressure hydrocephalus]].
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| ===Hereditary ataxias===
| | * Corticosteroids |
| Ataxia may depend on [[hereditary]] disorders consisting of degeneration of the cerebellum and/or of the spine; most cases feature both to some extent, and therefore present with overlapping cerebellar and sensory ataxia, even though one is often more evident than the other. Hereditary disorders causing ataxia include [[autosomal dominant]] ones such as [[spinocerebellar ataxia]], [[episodic ataxia]], and [[dentatorubropallidoluysian atrophy]], as well as [[autosomal recessive]] disorders such as [[Friedreich's ataxia]] (sensory and cerebellar, with the former predominating), [[ataxia-telangiectasia]] (sensory and cerebellar, with the latter predominating), and [[abetalipoproteinaemia]]. An example of X-linked ataxic condition is the rare [[fragile X-associated tremor/ataxia syndrome]].
| | * IV immune globulin |
| | * Plasma exchange therapy |
| | * Medications to improve muscle coordination |
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| ==Treatment==
| | Occupational or physical therapy may also be needed. Changes to diet and nutritional supplements may also help. |
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| 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.
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| ==References==
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| {{Reflist}}
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| == External links ==
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| * [http://www.ninds.nih.gov/disorders/ataxia/ataxia.htm National Institute of Neurological Disorders and Stroke (NINDS)]
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| * [http://www.ataxia.org National Ataxia Foundation]
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| * [http://www.ataxia.org.uk Ataxia UK]
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| * [http://www.ataxiaawarenessday.org/ International Ataxia Awareness Day]
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| * [http://www.ataxiaforums.co.uk Ataxia Forums]
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| * [http://www.lacaf.org/en/ Canadian Association of Familial Ataxias – The Claude St-Jean Foundation]
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| * [http://www.youtube.com/watch?v=S4bhexPs1FA Video] from the US Department of Agriculture of a sheep with [[scrapie]]
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| {{Symptoms and signs}}
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| {{SIB}}
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| [[de:Ataxie]]
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| [[es:Ataxia]] | | [[es:Ataxia]] |
| [[fr:Ataxie]] | | [[fr:Ataxie]] |
| [[it:Atassia]]
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| [[hu:Ataxia]]
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| [[nl:Ataxie]]
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| [[pt:Ataxia]] | | [[pt:Ataxia]] |
| [[ru:Атаксия]] | | [[ru:Атаксия]] |
| [[sr:Атаксија]]
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| [[fi:Ataksia]]
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| [[sv:Ataxi]]
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| [[uk:Атаксія]]
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| [[ur:لاتراتب]]
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| [[pl:Ataksja]] | | [[pl:Ataksja]] |
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| [[Category:Neurological disorders]] | | [[Category:Medical signs]] |
| [[Category:Signs and symptoms]] | | [[Category:Signs and symptoms]] |
| [[Category:Neurology]] | | [[Category:Neurology]] |
| [[Category:Needs patient information]]
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