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Symptomatic congenital hydrocephalus in the elderly simulating normal pressure hydrocephalus

From the Department of Neurology (Dr. Graff-Radford), Division of Cognitive Neuroscience and Behavioral Neurology, and Department of Surgery (Dr. Godersky), Division of Neurosurgery, The University of Iowa College of Medicine, Iowa City, IA.

In a series of 30 older patients shunted for symptomatic hydrocephalus, we found 3 with a head circumference at or greater than the 98th percentile. In 2, we demonstrated deterioration over 6 and 12 months by serial videotaping of gait and neuropsychological testing. In the 3rd, serial lumbar punctures over a 6-month period gave temporary improvement. In each, CTs showed ventriculomegaly without transependymal flow. One patient had an Arnold-Chiari type I abnormality identified by MRI. All had systemic hypertension. CSF pressure monitoring showed CSF pressure > 15 mm Hg 39% of the time in 1 patient, and 100% in another. All improved with ventriculoperitoneal shunting. Patients with probable compensated congenital hydrocephalus who functioned well throughout most of their lives may become symptomatic as they age but improve with shunt surgery. The head circumference should be measured in all older hydrocephalic patients.

Address correspondence and reprint requests to Dr. Graff-Radford, Department of Neurology, Mayo Clinic Jacksonville, 4500 San Pablo Drive, Jacksonville, FL 32224.

Received March 29, 1989. Accepted for publication in final form June 9, 1989.

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