Global InformationVestibulocerebellar syndrome information
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| Basal view of the human brain including the cerebellum |
Vestibulocerebellar syndrome, also known as vestibulocerebellar ataxia, is a progressive neurological disorder that causes a variety of medical problems. Initially symptoms present as periodic attacks of abnormal eye movements but may intensify to longer-lasting motor incapacity. The disorder has been localized to the vestibulocerebellum, specifically the flocculonodular lobe.[1] Symptoms of vestibulocerebellar syndrome may appear in early childhood but the full onset of neurological symptoms including nystagmus (involuntary eye movement), ataxia (loss of voluntary muscle coordination), and tinnitus (perception of sound in the absence of external stimulation) does not occur until early adulthood.[2] To date, vestibulocerebellar syndrome has only been identified in three families but has affected multiple generations within them. Based on the familial pedigrees it has been characterized as an autosomal dominant disorder, although the exact genetic locus has not been identified.[2][3] It has been found to be genetically distinct from other seemingly similar forms of neurological syndromes such as episodic ataxia types 1 and 2. Due to its rarity, however, little is known about specific details of the pathology or long-term treatment options.[2] There is currently no cure for vestibulocerebellar syndrome, although some drug therapies have been effective in alleviating particular symptoms of the disorder.
- ^ Theunissen EJ, Huygen PL, Verhagen WI (February 1989). "Familial vestibulocerebellar dysfunction: a new syndrome?". Journal of the Neurological Sciences. 89 (2–3): 149–55. doi:10.1016/0022-510x(89)90016-6. PMID 2926446. S2CID 31498395.
- ^ a b c Damji KF, Allingham RR, Pollock SC, et al. (April 1996). "Periodic vestibulocerebellar ataxia, an autosomal dominant ataxia with defective smooth pursuit, is genetically distinct from other autosomal dominant ataxias". Archives of Neurology. 53 (4): 338–44. doi:10.1001/archneur.1996.00550040074016. PMID 8929156.
- ^ Cite error: The named reference
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