Objective: To determine the prevalence and causation of late onset cerebellar ataxia (LOCA) in south east Wales, United Kingdom. Methods: A population based study of LOCA was conducted in a defined geographical region...Objective: To determine the prevalence and causation of late onset cerebellar ataxia (LOCA) in south east Wales, United Kingdom. Methods: A population based study of LOCA was conducted in a defined geographical region with a total population of 742 400. Multiple sources of ascertainment were used to identify all cases prevalent on 1 January 2001. The inclusion criteria were: a predominantly progressive cerebellar ataxia with onset of symptoms at age ≥ 18 years; and disease duration of ≥ 1 year. Cases with known acquired ataxias, atoxic syndromes with associated prominent autonomic dysfunction and/or atypical parkinsonism suggestive of multiple system atrophy and disorders with ataxia as a minor feature were excluded. Results: We identified 76 index cases of LOCA, of whom 63 were sporadic, idiopathic LOCA (ILOCA) and 13 were familial LOCA, of whom six had either spinocerebellar ataxia type 6, Friedreich’ s ataxia or dominant episodic ataxia. The mean annual incidence rate for the period 1999- 2001 was 0.3/100 000 population/year. The crude prevalence rates were 8.4 per 100 000 (95% CI 7.2 to 11.6) for ILOCA and 1.8 per 100 000 (95% CI 0.8 to 2.7) for inherited LOCA. Of the 54/63 (85.7% ) patients with ILOCA who were assessed, mean (SD) age at onset of symptoms was 53.8 (14.1) years (range 19 to 78) with a male:female ratio of 2.1:1. The mean disease duration was 8.7 (6.3) years (range 1 to 31). The most frequent presenting complaint was disturbance in gait (90.7% ). Onethird had a relatively pure cerebellar syndrome (33.3% ) and two thirds (66.7% ) had additional extracerebellar neurological features. The majority (92% ) were ambulant but only 9.3% were independently self caring. Conclusion: This population based study provides insight into LOCA within a defined region and will inform decisions about the rational use of healthcare resources for patients with LOCA.展开更多
文摘Objective: To determine the prevalence and causation of late onset cerebellar ataxia (LOCA) in south east Wales, United Kingdom. Methods: A population based study of LOCA was conducted in a defined geographical region with a total population of 742 400. Multiple sources of ascertainment were used to identify all cases prevalent on 1 January 2001. The inclusion criteria were: a predominantly progressive cerebellar ataxia with onset of symptoms at age ≥ 18 years; and disease duration of ≥ 1 year. Cases with known acquired ataxias, atoxic syndromes with associated prominent autonomic dysfunction and/or atypical parkinsonism suggestive of multiple system atrophy and disorders with ataxia as a minor feature were excluded. Results: We identified 76 index cases of LOCA, of whom 63 were sporadic, idiopathic LOCA (ILOCA) and 13 were familial LOCA, of whom six had either spinocerebellar ataxia type 6, Friedreich’ s ataxia or dominant episodic ataxia. The mean annual incidence rate for the period 1999- 2001 was 0.3/100 000 population/year. The crude prevalence rates were 8.4 per 100 000 (95% CI 7.2 to 11.6) for ILOCA and 1.8 per 100 000 (95% CI 0.8 to 2.7) for inherited LOCA. Of the 54/63 (85.7% ) patients with ILOCA who were assessed, mean (SD) age at onset of symptoms was 53.8 (14.1) years (range 19 to 78) with a male:female ratio of 2.1:1. The mean disease duration was 8.7 (6.3) years (range 1 to 31). The most frequent presenting complaint was disturbance in gait (90.7% ). Onethird had a relatively pure cerebellar syndrome (33.3% ) and two thirds (66.7% ) had additional extracerebellar neurological features. The majority (92% ) were ambulant but only 9.3% were independently self caring. Conclusion: This population based study provides insight into LOCA within a defined region and will inform decisions about the rational use of healthcare resources for patients with LOCA.
