Cerebellar Cognitive Affective Syndrome in Children with Acute Postinfectious Cerebellar Ataxia

Publikation: KonferencebidragPosterForskningpeer review

Acute post-infectious cerebellar ataxia is the most common cause of acute ataxia in childhood. A previous case study has suggested that acute post-infectious cerebellar ataxia may be comorbid with the cerebellar cognitive affective syndrome.

The cerebellar cognitive affective syndrome is characterized by (1) disturbances of executive functioning, including deficient planning, set-shifting, abstract reasoning, working memory, and decreased verbal fluency; (2) impaired spatial cognition, visual spatial disorganization, and impaired visual spatial memory; (3) personality changes, flattening or blunting of affect, and disinhibited and inappropriate behavior; and (4) linguistic difficulties, dysprosody, agrammatism, and mild anomia.

Children aged three to 15 years with a confirmed diagnosis of acute post-infectious cerebellar ataxia were invited to participate. Three patients were included and assessed by a pediatrician, neuropsychologist, and logopedist at the subacute stage (less than 14 days post-onset) and after six months and one year of follow-up.
All three children complied with the diagnostic criteria of cerebellar cognitive affective syndrome (Table 1). The severity of cognitive and affective cerebellar symptoms seem to correspond to the severity of the cerebellar motor symptoms. The cognitive and affective symptoms seem to persist longer than the cerebellar motor symptoms.

Child A (girl, aged three years and eight months) was most severely affected with slow progression of motor cerebellar symptom; the cerebellar cognitive affective symptoms had not entirely remitted at one-year follow-up.

Child B (boy, aged four years and four months) had more subtle motor cerebellar symptoms that swiftly remitted within the first week; the cerebellar cognitive affective symptoms were also more subtle.

Child C (boy, aged seven years and eleven months) was considerably affected by motor cerebellar symptoms but showed marked improvement within the first month; the cerebellar cognitive affective symptoms had not entirely remitted at one-year follow-up.

The cognitive affective cerebellar syndrome may be an overlooked complication of acute post-infectious cerebellar ataxia. The severity of cerebellar cognitive affective symptoms seemed to correspond to the severity of the cerebellar motor symptoms, but the improvement was remarkably slower.

No single assessment method can undercover all symptoms of the cerebellar cognitive affective syndrome. A combination of objective and subjective multidisciplinary methods are needed to fully assess the cerebellar cognitive affective syndrome in children with acute post-infectious cerebellar ataxia.

Udgivelsesår25 aug. 2021
StatusUdgivet - 25 aug. 2021
Begivenhed14th Nordic Meeting in Neuropsychology: From Cradle to cognitive Reserve - Scandic Falkoner, Copenhagen, Danmark
Varighed: 24 aug. 202127 aug. 2021


Konference14th Nordic Meeting in Neuropsychology
LokationScandic Falkoner

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