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February 8, 2000

Acute hydrocephalus in nonketotic hyperglycinemia

J.L. K. Van Hove, MD, PhD, P.S. Kishnani, MD, P. Demaerel, MD, S.G. Kahler, MD, C. Miller, MD, J. Jaeken, MD, PhD, and S.L. Rutledge, MDAuthors Info & Affiliations
February 8, 2000 issue
54 (3) 754
https://doi.org/10.1212/WNL.54.3.754
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Abstract

Article abstract We present four patients with typical neonatal onset nonketotic hyperglycinemia (NKH) who developed hydrocephalus requiring shunting in early infancy. Brain imaging revealed acute hydrocephalus, a megacisterna magna or posterior fossa cyst, pronounced atrophy of the white matter, and an extremely thin corpus callosum in all. The three older patients had profound developmental disabilities. This suggests that the development of hydrocephalus in NKH is an additional poor prognostic sign.

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Information & Authors

Information

Published In

Neurology®
Volume 54 • Number 3 • February 8, 2000
Pages: 754
PubMed: 10680820

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© 2000.

Publication History

Published online: February 8, 2000
Published in print: February 8, 2000

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Authors

Affiliations & Disclosures

J.L. K. Van Hove, MD, PhD
From the Departments of Pediatrics (Drs. Van Hove and Jaeken) and Radiology (Dr. Demaerel)University Hospital Gasthuisberg, Leuven, Belgium; the Departments of Pediatrics (Drs. Van Hove, Kishnani, and Kahler) and Radiology (Dr. Miller), Duke University Medical Center, Durham, NC; the Victorian Clinical Genetics Services (Dr. Kahler), The Murdoch Institute, Parkville, VIC, australia; and the Department of Neurology (Dr. Rutledge), University of Alabama at Birmingham, AL.
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P.S. Kishnani, MD
From the Departments of Pediatrics (Drs. Van Hove and Jaeken) and Radiology (Dr. Demaerel)University Hospital Gasthuisberg, Leuven, Belgium; the Departments of Pediatrics (Drs. Van Hove, Kishnani, and Kahler) and Radiology (Dr. Miller), Duke University Medical Center, Durham, NC; the Victorian Clinical Genetics Services (Dr. Kahler), The Murdoch Institute, Parkville, VIC, australia; and the Department of Neurology (Dr. Rutledge), University of Alabama at Birmingham, AL.
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P. Demaerel, MD
From the Departments of Pediatrics (Drs. Van Hove and Jaeken) and Radiology (Dr. Demaerel)University Hospital Gasthuisberg, Leuven, Belgium; the Departments of Pediatrics (Drs. Van Hove, Kishnani, and Kahler) and Radiology (Dr. Miller), Duke University Medical Center, Durham, NC; the Victorian Clinical Genetics Services (Dr. Kahler), The Murdoch Institute, Parkville, VIC, australia; and the Department of Neurology (Dr. Rutledge), University of Alabama at Birmingham, AL.
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S.G. Kahler, MD
From the Departments of Pediatrics (Drs. Van Hove and Jaeken) and Radiology (Dr. Demaerel)University Hospital Gasthuisberg, Leuven, Belgium; the Departments of Pediatrics (Drs. Van Hove, Kishnani, and Kahler) and Radiology (Dr. Miller), Duke University Medical Center, Durham, NC; the Victorian Clinical Genetics Services (Dr. Kahler), The Murdoch Institute, Parkville, VIC, australia; and the Department of Neurology (Dr. Rutledge), University of Alabama at Birmingham, AL.
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C. Miller, MD
From the Departments of Pediatrics (Drs. Van Hove and Jaeken) and Radiology (Dr. Demaerel)University Hospital Gasthuisberg, Leuven, Belgium; the Departments of Pediatrics (Drs. Van Hove, Kishnani, and Kahler) and Radiology (Dr. Miller), Duke University Medical Center, Durham, NC; the Victorian Clinical Genetics Services (Dr. Kahler), The Murdoch Institute, Parkville, VIC, australia; and the Department of Neurology (Dr. Rutledge), University of Alabama at Birmingham, AL.
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J. Jaeken, MD, PhD
From the Departments of Pediatrics (Drs. Van Hove and Jaeken) and Radiology (Dr. Demaerel)University Hospital Gasthuisberg, Leuven, Belgium; the Departments of Pediatrics (Drs. Van Hove, Kishnani, and Kahler) and Radiology (Dr. Miller), Duke University Medical Center, Durham, NC; the Victorian Clinical Genetics Services (Dr. Kahler), The Murdoch Institute, Parkville, VIC, australia; and the Department of Neurology (Dr. Rutledge), University of Alabama at Birmingham, AL.
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S.L. Rutledge, MD
From the Departments of Pediatrics (Drs. Van Hove and Jaeken) and Radiology (Dr. Demaerel)University Hospital Gasthuisberg, Leuven, Belgium; the Departments of Pediatrics (Drs. Van Hove, Kishnani, and Kahler) and Radiology (Dr. Miller), Duke University Medical Center, Durham, NC; the Victorian Clinical Genetics Services (Dr. Kahler), The Murdoch Institute, Parkville, VIC, australia; and the Department of Neurology (Dr. Rutledge), University of Alabama at Birmingham, AL.
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Notes

Address correspondence and reprint requests to Dr. Johan L.K. Van Hove, Department of Pediatrics, University Hospital Gasthuisberg, Herestraat 49, B-3000 Leuven, Belgium.

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