Cognitive Problems Common Nonmotor Symptom in Patients With Cerebellar Ataxias

Stopping Chain Reaction
Finger is stopping chain reaction of dominos who are about to fall in front of a dark background.
Presenting at the 2022 AAN Annual Meeting, researchers assessed the reported prevalence of nonmotor symptoms among adult patients with different types of progressive cerebellar ataxia.

The following article is part of conference coverage from the 2022 American Academy of Neurology (AAN) Annual Meeting. Neurology Advisor’s staff will be reporting breaking news associated with research conducted by leading experts in neurology. Check back for the latest news from the 2022 AAN Annual Meeting.


Nonmotor symptoms (NMS) were commonly present in adult patients with cerebellar ataxias and may be underrecognized, according to study results presented at the 2022 American Academy of Neurology (AAN) Annual Meeting, held from April 2 to April 7 in Seattle, Washington, and virtually from April 24-26, 2022.

Cerebellar ataxias include many heterogeneous disorders that involve both motor and NMS. The objective of the current study was determine the prevalence of NMS in adult patients with different types of progressive cerebellar ataxia.

In the current study, researchers systematically reviewed studies published in English of cerebellar ataxias that involved more than 5 patients who had been assessed for NMS. They found the studies by searching the PUBMED and EMBASE databases from 1947 to 2020.

The researchers identified 32 papers. The papers included data from 1181 cases of patients aged 39.8±17.7 years with autosomal dominant spinocerebellar ataxia (SCA), 160 cases of patients aged 47.8±21.6 years with autosomal recessive cerebellar ataxia (ARCA), and 488 cases of patients aged 58.3±7.3 years with multiple system atrophy (MSA).

The studies identified cognitive problems in anywhere from 25% to 100% of patients with SCA. The researchers reported that cognitive deficits may be less common and/or less severe in SCA6. They found that, among individuals with SCA, depression prevalence ranged from 23% to 69% and sleep disorders prevalence ranged from 20% to 80%. Sixty percent of patients reported pain, particularly in SCA3. Fifty-seven percent of patients reported fatigue.

The researchers found that studies reported the prevalence of cognitive problems in 12.5% to 100% in ARCA. Depression prevalence ranged from 21% to 50%.

Prevalence of autonomic failure in individuals with MSA was 48% to 96.5% at baseline, the researchers reported.

They concluded that NMS commonly presents in patients with cerebellar ataxias.

“The reporting of NMS in progressive cerebellar ataxia is selective and not systematized,” the researchers stated. “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.”


Malek N, Al-Sami Y, Aslanyan A, et al. A systematic review of the spectrum and prevalence of non-motor symptoms in adults with progressive cerebellar ataxias. Presented at: the 2022 AAN Annual Meeting; April 2-7, 2022; Seattle, Washington; April 24-26, 2022; Virtual Meeting. Abstract P18.001.