The Aetiologic Spectrum of Cerebellar Ataxia: Acquired Causes of Ataxia
Peter K Todd, University of Michigan Medical School, Ann Arbor, Michigan, USA
J Paul Taylor, St. Jude Children' Research Hospitals, Memphis, Tennesee, USA
Published online: March 2009
DOI: 10.1002/9780470015902.a0021592
Abstract
Ataxia is a common finding in patients seen in neurological practice and can be the result of a wide variety of causes. Although
cerebellar degeneration can be chronic and slowly progressive, acute cerebellar swelling due to infarction, oedema or haemorrhage
can have rapid and catastrophic effects and is a true neurological emergency. Here we set out to briefly describe the clinical/anatomical
correlates of cerebellar disease and to provide a broad differential diagnosis for patients who present with cerebellar ataxia.
This article specifically focuses on acquired causes of cerebellar ataxia. A separate complementary article discusses common
hereditary causes of cerebellar ataxia.
Key Concepts
-
Clinical syndromes associated with cerebellar dysfunction may be divided into syndromes that predominantly affect the midline
cerebellar structures (gait and ocular dysfunction predominant) or the cerebellar hemisphere(s) (ipsilateral appendicular
ataxia, dysarthria).
-
Acute onset of cerebellar ataxia is most commonly associated with vascular disorders (stroke or haemorrhage), toxins or infectious
aetiologies.
-
Subacute onset cerebellar ataxia can result from a variety of causes, including infectious, neoplastic, autoimmune and metabolic
abnormalities. Many of these causes of ataxia are treatable.
-
Most slowly progressive cerebellar ataxias result from neurodegenerative aetiologies, either inherited or sporadic.
Keywords: multiple sclerosis; paraneoplastic cerebellar degeneration; vitamin E deficiency; multiple system atrophy; toxins
References
Abele M,
Burk K,
Schols L et al.
(2002)
The aetiology of sporadic adult‐onset ataxia.
Brain
125:
961.
Abele M,
Weller M,
Mescheriakov S et al.
(1999)
Cerebellar ataxia with glutamic acid decarboxylase autoantibodies.
Neurology
52:
857.
Allen RK,
Sellars RE and
Sandstrom PA
(2003)
A prospective study of 32 patients with neurosarcoidosis.
Sarcoidosis, Vasculitis, and Diffuse Lung Diseases
20:
118.
Bushara KO,
Goebel SU,
Shill H et al.
(2001)
Gluten sensitivity in sporadic and hereditary cerebellar ataxia.
Annals of Neurology
49:
540.
Fisher M
(1956)
An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia).
New England Journal of Medicine
255:
57.
Hadjivassiliou M,
Davies‐Jones GA,
Sanders DS and
Grunewald RA
(2003)
Dietary treatment of gluten ataxia.
Journal of Neurology, Neurosurgery, and Psychiatry
74:
1221.
Hadjivassiliou M,
Grunewald RA,
Chattopadhyay AK et al.
(1998)
Clinical, radiological, neurophysiological, and neuropathological characteristics of gluten ataxia.
Lancet
352:
1582.
Honnorat J,
Saiz A,
Giometto B et al.
(2001)
Cerebellar ataxia with anti‐glutamic acid decarboxylase antibodies: study of 14 patients.
Archives of Neurology
58:
225.
Kappos L,
Polman CH,
Freedman MS et al.
(2006)
Treatment with interferon beta‐1b delays conversion to clinically definite and McDonald MS in patients with clinically isolated syndromes.
Neurology
67:
1242.
Kinkel RP,
Kollman C,
O'Connor P et al.
(2006)
IM interferon beta‐1a delays definite multiple sclerosis 5 years after a first demyelinating event.
Neurology
66:
678.
Liu GT,
Volpe NJ and
Galetta SL
(1995)
Eye movement disorders.
Current Opinion in Ophthalmology
6:
27.
Narang HK
(2001)
A critical review of atypical cerebellum‐type Creutzfeldt‐Jakob disease: its relationship to “new variant” CJD and bovine spongiform encephalopathy.
Experimental Biology and Medicine (Maywood)
226:
629.
Ravi V and
Rozen TD
(2000)
Acute cerebellitis: MRI findings.
Neurology
54:
213.
Sanchez‐Juan P,
Green A,
Ladogana A et al.
(2006)
CSF tests in the differential diagnosis of Creutzfeldt‐Jakob disease 10.1212/01.wnl.0000230159.67128.00.
Neurology
67:
637.
Sawaishi Y and
Takada G
(2002)
Acute cerebellitis.
Cerebellum
1:
223.
Further Reading
Fahn S,
Jankovic J,
Hallett M, and
Jenner P
(2007)
Principles and Practice of Movement Disorders.
Churchill Livingstone, PA: Elsevier Health Sciences.
The National Ataxia Foundation. http://www.ataxia.org/about/national‐ataxia‐foundation.aspx
