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Acute Infantile Bilateral Striatal Necrosis: Single-Photon Emission Computed Tomography (SPECT) Imaging and Review

Department of Pediatrics C Schneider Children's Medical Center of Israel, Petah Tikva 49202, Israel, nzevit{at}hotmail.com

Adam Steinmetz, MD

Department of Nuclear Medicine, Rabin Medical Center, Beilinson Campus, Sackler Faculty of Medicine, Tel Aviv University, Israel

Liora Kornreich, MD

Department of Pediatric Radiology Tel Aviv University

Rachel Straussberg, MD

Department of Pediatric Neurology Schneider Children's Medical Center of Israel, Sackler Faculty of Medicine, Tel Aviv University

Acute infantile bilateral striatal necrosis is a rarely described acute neurological syndrome associated with radiological findings. Its etiology and pathogenic mechanisms are unknown. Clinically, the syndrome usually follows respiratory illnesses and presents with an array of neurological findings, including axial ataxia, grimacing, mutism, head nodding, and high-pitched cry. This study follows a child with acute infantile bilateral striatal necrosis both clinically and radiologically. In addition, for the first time, the authors describe the serial findings of single-photon emission computed tomography (SPECT) from onset of illness through 20 months. Their findings indicate an initial insult apparent on both magnetic resonance imaging and SPECT localized to the basal ganglia, which, although improved over time, does not fully regress. The residual lesion on SPECT was clinically associated with only mild attention deficit disorder and no motor pathology. The authors review the published literature concerning acute infantile bilateral striatal necrosis and suggest possible mechanisms of this poorly understood and probably underreported condition.

Key Words: basal ganglia • striatal necrosis • single-photon emission computed tomography

Journal of Child Neurology, Vol. 22, No. 10, 1222-1226 (2007)
DOI: 10.1177/0883073807304194


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