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Journal of Child Neurology
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0883073808315622v1
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Article

Spinocerebellar Ataxia Type 2 Presenting With Cognitive Regression in Childhood

Melissa B. Ramocki, MD, PhD1, Lynn Chapieski, PhD1, Ryan O. McDonald, MD2, Fabio Fernandez, MD1, and Amy D. Malphrus, MD1*

1 Department of Pediatrics, Baylor College of Medicine, Houston, Texas
2 Department of Adult Neurology, Baylor College of Medicine, Houston, Texas

* To whom correspondence should be addressed. E-mail: malphrus{at}bcm.edu.


  Abstract
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.

First published on March 14, 2008, doi:10.1177/0883073808315622

Journal of Child Neurology 2008;23:999.

A more recent version of this article appeared on September 1, 2008

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