The Electrophoretic Pattern of Cerebrospinal Fluid Proteins in the Cutis Verticis Gyrata and Mental Retardation Syndrome

Abstract

The electrophoretic evaluation of the cerebrospinal fluid (CSF) proteins was performed in the cutis verticis gyrata and mental retardation (CVG-MR) syndrome, as the assumed handicapped CSF circulation might result in an altered electrophoretic pattern. In seven patients with the miscellaneous form of the syndrome the electrophoretic findings did not deviate significantly from the normal. In nine patients with the primary form’ of the syndrome there was a significant increase in α₁-globulin (p<0.05) and β-globulin (p<0.01) fractions as well as a decrease in albumin fraction (p<0.05). The ratio between albumin and globulins was 1.5 in the primary group and 1.8 in the miscellaneous group. In the primary group the total amount of β-globulins was 66 mg/1 and in the miscellaneous 46 mg/1 (p<0.05). Consequently, CSF electrophoresis may serve as a diagnostic clue for the primary form of the syndrome, which appears to be a separate condition of its own.

The Cutis Verticis Gyrata and Mental Retardation Syndrome: Electroencephalographic Analysis of Nineteen Cases

“…We believe that it is justified to classify patients with the primary form of the syndrome into the aetiological category of mental retardation due to unknown prenatal infiuence. PEG findings such as narrow posterior fossa and microventriculy (Palo el al., 1970), cerebrospinal fiuid abnormalities such as high ^-and a-1-globulin and low albumin (Iivanainen and Kostiainen, 1974) are findings suggestive of disturbed cerebrospinal fluid circulation. Diffuse and generalised EEG abnormalities suggest generalised cerebral involvement and are thus in agreement with the hypothesis of disturbed cerebrospinal fiuid circulation.…”