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Journal of Child Neurology
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Spinocerebellar Ataxia Type 2 Presenting With Cognitive Regression in Childhood

Melissa B. Ramocki, MD, PhD

Section of Pediatric Neurology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas.

Lynn Chapieski, PhD

Section of Pediatric Neurology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas.

Ryan O. McDonald, MD

Department of Adult Neurology Baylor College of Medicine, Houston, Texas

Fabio Fernandez, MD

Section of Pediatric Neurology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas.

Amy D. Malphrus, MD

Section of Pediatric Neurology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas, malphrus{at}bcm.edu

Spinocerebellar ataxia type 2 typically presents in adulthood with progressive ataxia, dysarthria, tremor, and slow saccadic eye movements. Childhood-onset spinocerebellar ataxia type 2 is rare, and only the infantile-onset form has been well characterized clinically. This article describes a girl who met all developmental milestones until age 31/2 years, when she experienced cognitive regression that preceded motor regression by 6 months. A diagnosis of spinocerebellar ataxia type 2 was delayed until she presented to the emergency department at age 7 years. This report documents the results of her neuropsychologic evaluation at both time points. This case broadens the spectrum of spinocerebellar ataxia type 2 presentation in childhood, highlights the importance of considering a spinocerebellar ataxia in a child who presents with cognitive regression only, and extends currently available clinical information to help clinicians discuss the prognosis in childhood spinocerebellar ataxia type 2.

Key Words: spinocerebellar ataxia • cognitive regression

This version was published on September 1, 2008

Journal of Child Neurology, Vol. 23, No. 9, 999-1001 (2008)
DOI: 10.1177/0883073808315622
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