Ataxia
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| ICD-10 | R27.0 |
|---|---|
| ICD-9 | 438.84, 781.3 |
| MeSH | D001259 |
Ataxia (from Greek α- [used as a negative prefix] + -τάξις [order], meaning "lack of order") is a neurological sign and symptom consisting of gross lack of coordination of muscle movements. Ataxia is a non-specific 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 25 each year.[1]
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[edit] Types of ataxia
[edit] 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.
- Dysfunction of the vestibulocerebellum impairs balance and control of eye movements. This 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, or more accurately, denotes the inability to carry out the test as the individual is unstable even with open eyes).
- Dysfunction of the spinocerebellum presents with a wide-based "drunken sailor" gait, characterised by uncertain start and stop, lateral deviations, and unequal steps. This part of the cerebellum regulates body and limb movements.
- Dysfunction of the cerebrocerebellum presents with disturbances in carrying out voluntary, planned 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).
[edit] Sensory ataxia
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 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 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 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").
[edit] Vestibular ataxia
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, 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.
[edit] Causes of ataxia
The three types of ataxia have overlapping causes, and can therefore either coexist or occur in isolation.
[edit] Focal lesions
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).
[edit] Exogenous substances
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 reversible cerebellar and vestibular ataxia. Other examples include various prescription drugs (e.g. most antiepileptic drugs have cerebellar ataxia as a possible adverse effect), marijuana ingestion[2] and various other recreational drugs (e.g. ketamine, PCP or dextromethorphan, all of which are NMDA receptor antagonists that produce a dissociative state at high doses).
[edit] Vitamin B12 deficiency
Vitamin B12 deficiency may cause, among several neurological abnormalities, overlapping cerebellar and sensory ataxia.
[edit] Causes of isolated sensory ataxia
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.
[edit] Non-hereditary cerebellar degeneration
Non-hereditary causes of cerebellar degeneration include chronic ethanol abuse, paraneoplastic cerebellar degeneration, high altitude cerebral oedema, coeliac disease, normal pressure hydrocephalus and cerebellitis.
[edit] Hereditary ataxias
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) and Niemann Pick disease, 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.
[edit] Arnold-Chiari Malformation
Arnold-Chiari malformation is a malformation of the brain. It consists of a downward displacement of the cerebellar tonsils and the medulla through the foramen magnum, sometimes causing hydrocephalus as a result of obstruction of CSF (cerebrospinal fluid) outflow.
[edit] Treatment
There is no specific treatment for ataxia, although the consequent disability may also 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. Severe cases may require the use of a wheelchair.
[edit] Other uses of the term
The term "ataxia" is sometimes used in a broader sense to indicate lack of coordination in some physiological process. Examples include optic ataxia (lack of coordination between visual inputs and hand movements, resulting in inability to reach and grab objects, usually part of Balint's syndrome, but can be seen in isolation with injuries to the superior parietal lobule, as it represents a disconnection between visual-association cortex and the frontal premotor and motor cortex[3]), and ataxic respiration (lack of coordination in respiratory movements, usually due to dysfunction of the respiratory centres in the medulla oblongata).
[edit] References
- ^ "National Ataxia Foundation - International Ataxia Awareness Day". http://www.ataxia.org/events/international-ataxia-awareness-day.aspx. Retrieved 2008-03-25.
- ^ "Inadvertent Ingestion of Marijuana --- Los Angeles, California, 2009". http://www.cdc.gov/mmwr/preview/mmwrhtml/mm5834a2.htm?s_cid=mm5834a2. Retrieved 2009-09-03.
- ^ Giuseppe Vallar, MD, Spatial Neglect, Balint-Holmes’ and Gerstmann’s Syndromes, and Other Spatial Disorders CNS Spectr. 2007;12(7):527-536
[edit] External links
- Brasil, Rio Grande do Sul - Associação dos Amigos, Parentes e Portadores de Ataxias Dominantes
- Overview at National Institute of Neurological Disorders and Stroke (NINDS)
- National Ataxia Foundation
- Ataxia UK
- International Ataxia Awareness Day
- Ataxia Forums
- LivingWithAtaxia Forums & Community
- Canadian Association for Familial Ataxias - Claude St-Jean Foundation
- The latest news and research on Ataxia
- Video from the US Department of Agriculture of a sheep with scrapie
- GeneReview/NIH/UW entry on Hereditary Ataxia Overview
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