Myoclonic Form of Hallervorden-Spatz Disease: Clinical Aspect Simulating a Subacute Spongy Encephalopathy

DESCRIBES THE RAPID AND PROGRESSIVE DEVELOPMENT OF SYMPTOMS OF A DEMENTIAL AND MYOCLONIC SYNDROME IN A MALE S DEAD AT AGE 42, 5 MO. AFTER ONSET OF WALKING DIFFICULTIES, SLOW SPEECH, DYSARTHRIA; TREMBLING WITH A PARKINSONIAN ASPECT, SLIGHT MYOCLONIC TREMORS OF THE RIGHT SUPERIOR LIMB; AND MENTAL DETERIORATION. RAPID EEG TRACES REVEALED TRACKS OF THETA WAVES MOSTLY FROM THE TEMPORAL REGIONS. LESIONS OBSERVED DURING MACROSCOPIC AND MICROSCOPIC EXAMINATION RESEMBLED THOSE OF THE HALLERVORDEN-SPATZ DISEASE, AN ILLNESS WHICH EVOLVES IN SEVERAL YEARS: ATHETOSIC AND CHOREIFORM MOVEMENTS, A STATE OF RIGIDITY, AND BUCCO-PHARYNGEAL SPASMS. SIMILARITY TO DEMENTIAL DEGENERATION CASES WERE NOTED FOLLOWING THE PRESENTATION OF DIAGNOSTIC DIFFERENTIALS WITH THE CREUTZFELDT-JAKOB SYNDROME. (REPORT PRESENTED AT THE SOCIETE FRANCAISE DE NEUROLOGIE, FEBRUARY 2, 1967.) ((c) 1997 APA/PsycINFO, all rights reserved)