Journal of Child Neurology

 

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Journal of Child Neurology, Vol. 16, No. 9, 698-704 (2001)
DOI: 10.1177/088307380101600917

Visual Agnosia After Treatment of a Posterior Fossa Ependymoma in a 16-Month-Old Girl

Virginie Kieffer-Renaux, PA

Departments of Pediatrics

Christine Bulteau, MD

Departments of Pediatrics

Jacques Grill, MD

Departments of Pediatrics

Christine Levy-Piebois, MD

Department of Radiotherapy

Dominique Couanet, MD

Department of Radiology Gustave Roussy Institute

Alain Pierre-Kahn, MD

Department of Neurosurgery Necker Hospital Paris, France

Olivier Hartmann, MD

Department of Pediatrics Gustave Roussy Institute Villejuif, France

Chantal Kalifa, MD

Department of Pediatrics Gustave Roussy Institute Villejuif, France

We present the clinical observation of a 16-month-old girl treated for a posterior fossa ependymoma who experienced severe and delayed visual dysfunction. She was initially treated by surgery and conventional chemotherapy. When she relapsed at age 3 years, the salvage treatment combined high-dose chemotherapy, second surgery, and local irradiation. At age 4 years, disturbed gait and dysarthric speech appeared rapidly, and she became unable to recognize objects and people. Computed tomography revealed bilateral calcifications in the cerebellum and temporal and occipital lobes but no relapse. The neuropsychologic evaluations revealed signs of visual agnosia and marked intellectual impairment. The role of the different treatment modalities in the pathogenesis of this unusual syndrome is discussed. (J Child Neurol 2001;16:698-704).


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