A rare type of presenile dementia associated with cortical blindness, ataxia, dysarthria, athetotic movements and rigidity of all four limbs. Neuronal lesions with proliferation of astrocytes are seen in the cerebral cortex and to a lesser degree in the basal ganglion, with status spongiosus.
This and the Nevin syndrome are variants of the Creutzfeldt-Jakob disease. First described by Adolf Heidenhain in 1929.
Described in three patients by Heidenhain in 1929. Meyer and associates added a case in 1954 and suggested the eponymic designation. All patients were males between the ages of 38 and 55 years.
See also Creutzfeldt-Jakob disease, or progressive encephalopathy with spongioform degeneration of the brain, under Hans Gerhard Creutzfeldt, German neuropathologist, 1885-1964.
- A. Heidenhain:
Klinische und anatomische Untersuchungen über eine eigenartige anorganische Erkrankung des Zentralnervensystems im Praesenium.
Zeitschrift für die gesamte Neurologie und Psychiatrie, 1929, 118: 49-114.