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This careful and thoroughgoing monograph on the juvenile form of amaurotic family idiocy merits, and will no doubt receive, a place in the standard literature on this subject. The large number of cases of this notoriously rare disease, investigated by the same observer, alone makes the work unique. The patients were mostly from state institutions for blind children in Sweden. In all, 120 examples are reported, and of these 39 were examined clinically by the author. In 18 cases the diagnosis was certified anatomically, either post mortem or from tissue obtained by puncture of the brain. The remainder of the cases were culled from the complete and reliable records kept in the institutions for many years past. The book contains excellent clinical data for all the cases.
The difficulty of making a clinical diagnosis of the Vogt-Spielmeyer form of amaurotic family idiocy is well known. According to the best opinion
Die juvenile amaurotische Idiotie.. Am J Dis Child. 1933;45(6):1376. doi:10.1001/archpedi.1933.01950190218021