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  Vol. 35 No. 5, May 1978 TABLE OF CONTENTS
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  CHILD NEUROLOGY
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Megalencephaly in Infants and Children

The Possible Role of Increased Dural Sinus Pressure

Harold D. Portnoy, MD; Paul D. Croissant, MD

Arch Neurol. 1978;35(5):306-316.


Abstract

• Seven children studied because of clinical macrocephaly and suspected hydrocephalus ultimately proved to have megalencephaly apparently due to an increase in sagittal sinus venous pressure as established from infusion studies. Unexplainably, these patients were all males. All were seen initially between 2 and 8 months of age. Head enlargement exceeded two standard deviations in all seven. Pneumoencephalography, ventriculography, or computerized tomography demonstrated normal or minimally enlarged ventricles that did not progress in size. Isotope cisternography was abnormal. Studies of CSF formation and absorption demonstrated normal absorption rates but high calculated sagittal sinus pressures. Though therapy was usually not required, in one unusual infant, severe progressive macrocephaly with minimal hydrocephalus required a shunt. Another had a transient episode of acute hydrocephalus associated with a low CSF absorption rate and ventricular enlargement.

In this report, we review the intracranial hydrodynamics of benign intracranial hypertension (BIH), communicating hydrocephalus, and the pathogenesis of megalencephaly. Benign intracranial hypertension and the type of megalencephaly demonstrated by our patients appear to develop similarly except that the presence of open cranial sutures may allow a transient nonhydrostatic loading of brain parenchyma in infants, resulting in mild, nonprogressive macrocephaly.



Author Affiliations

From the Howard L. Gregor, Jr, Hydrocephalus and Spina Bifida Unit, Crittenton Hospital, Rochester, Mich, and Oakland Neurological Clinic, Professional Corp, Bloomfield Hills, Mich.


Footnotes

Accepted for publication Aug 8, 1977.

Reprint requests to Oakland Neurological Clinic, Professional Corp, 1431 Woodward Ave, Bloomfield Hills, MI 48013 (Dr Portnoy).



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THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

The Pseudotumor Syndrome: Disorders of Cerebrospinal Fluid Circulation Causing Intracranial Hypertension Without Ventriculomegaly
Johnston et al.
Arch Neurol 1991;48:740-747.
ABSTRACT  

Megalencephaly Due to Impaired Cerebral Venous Return in a Sturge-Weber Variant Syndrome
Fishman and Baram
J Child Neurol 1986;1:115-118.
ABSTRACT  

Macrocephaly With Head Growth Parallel to Normal Growth Pattern: Neurological, Developmental, and Computerized Tomography Findings in Full-term Infants
Pettit et al.
Arch Neurol 1980;37:518-521.
ABSTRACT  





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