The Cohen syndrome. Retinal lesions and granulocytopenia.

[cohen syndrome]

A patient with the Cohen syndrome is described and the literature is reviewed . Electroretinography (ERG ) showed that the patient had a tapeto-retinal degeneration . The authors believe that a retinitis pigmentosa -like phenotype is an obligatory sign in patients with the Cohen syndrome . Assessment of a tapeto-retinal dystrophy by electroretinography , however , is often not available for mentally retarded persons . The authors ' patient also had granulocytopenia . This was previously described in Finnish patients with Cohen 's syndrome . They speculate that this sign is more prevalent in the disorder than would appear from the literature . Alternatively , there may be a type I Cohen syndrome with granulocytopenia and a type II without .