Creutzfeldt-jakob syndrome (spastic pseudosclerosis; corticostriatospinal degeneration; disseminated encephalopathy; heidenhaim syndrome; presenile dementia with spastic paralysis; presenile dementia-cortical degeneration syndrome)    321

General: Heredofamilial occurrence; caused by degenerative changes in cerebral cortex, basal ganglion, and spinal cord; disease is progressive; begins in middle or later age; occurs in both sexes.

Ocular: Cortical blindness; myoclonic conjugate eye movements; paralysis of seventh nerve; ptosis; dyschromatopsia; homonymous hemianopsia; nystagmus; slow vertical saccades; mild demyelination of the optic nerve.

Clinical: Mental deterioration; psychosis; stupor; weakness and stiffness of extremities; slow development of pyramidal signs; loss of reflexes; tremor, rigidity; dysarthria; aphasia; ataxia; myoclonus; convulsive seizures; cerebellar ataxia decerebrate posture.

Creutzfeldt HG. Uber Fine Eigenartige Herdformige Erkrankung des Zentralnervensystems. In: Nissl F, Alzheimer A, eds. Histologie und Histopathologie. Jena: Fischer, Arbeit Erganzungs-band, 1921; and Z Ges Neurol Psychiat 1920; 57:1.

Fraunfelder FT, Roy FH. Current Ocular Therapy, 5th ed. Philadelphia: WB Saunders, 2000.

Geeraets WJ. Ocular Syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976.

Grant MP, et al. Abnormal eye movements in Creutzfeldt-Jakob disease. Ann Neurol 1993; 34:192-l97.

Jacob A. Uber Eine Eigenartige Erkrankung des Zentralnervensystems mit Bemerkenswertem Anatomischen Befund. Z Ges Neural Psychiatr 1921; 64:147.

Roos R, et al. The Clinical characteristics of transmissible Creutzfeldt-Jakob disease. Brain 1973; 96:1-20.