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.

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