A systematic review of the spectrum and prevalence of non-motor symptoms in adults with progressive cerebellar ataxias
Naveed Malek1, Yaqub Al-Sami1, Aram Aslanyan1, Rajith Desilva1
1Dept. of Neurology, Queen's Hospital, Romford, Essex, United Kingdom.
Objective:

We wanted to ascertain the reported prevalence of NMS in different types of progressive cerebellar ataxia in adults. 

Background:
Cerebellar ataxias comprise a large group of heterogeneous disorders with both motor and non-motor symptoms (NMS).
Design/Methods:
Systematic review of studies of cerebellar ataxia (involving >5 patients) who were assessed for NMS, published in the English literature in PUBMED and EMBASE databases from 1947-2020.
Results:
32 papers, with data from 1181 cases of autosomal dominant spinocerebellar ataxia (SCA), 160 cases of autosomal recessive cerebellar ataxia (ARCA) and 488 cases of multiple system atrophy (MSA) were included. Mean age for SCA cases was 39.8 (SD 17.7) years, ARCA cases was 47.8 (SD 21.6) years, and for MSA cases was 58.3 (SD 7.3) years. The prevalence of cognitive problems in SCA was between 25-100%. Cognitive deficits may be less common and/or severe in SCA6.The prevalence of depression in SCA was between 23-69% and sleep disorders 20-80%. Pain was reported by 60% of patients, especially in SCA3, and fatigue by 57%. The prevalence of cognitive problems in ARCA varied from 12.5 -100% and depression between 21-50%. The prevalence of autonomic failure in MSA was between 48-96.5% at baseline.
Conclusions:
The presence of NMS in cerebellar ataxias is common.  The reporting of NMS in progressive cerebellar ataxia is selective and not systematized.  There is overlap of reported NMS in the different types of ataxia.  In routine clinical practice too, NMS in the cerebellar ataxias are probably under-recognized, and consequently unlikely to be managed appropriately.