TABLE 1.

Patients' histories and clinicopathological features of the atypical CJD cases^a

Feature	Case 1	Case 2
Gender	Male	Female
Age at onset (yrs)	54	75
Occupational history	Neurosurgeon consulted on a case of CJD in which a myelogram was performed but not known to have participated in the procedure (7 yrs before onset)
Medical historyb	Surgical operations for perinephric abscess, inguinal herniorrhaphy, and removal of ureteric calculus (28 yrs, 8 yrs, and 32 mos before onset, respectively)	Neurosurgery for cerebellar hemangioblastoma (14 yrs before onset)
Initial symptomsc	Abnormal sleep patterns, hallucinations, myoclonic twitching of the extremities, paresthesia of the toes, loss of vibration sense, gait disturbance	Drowsiness, gait disturbance
Duration of illness (mos)	17	11
PSWCs on EEGd	Negative	Negative
PRNP genee	129M/M, 219E/E; no pathogenic mutations	129M/M, 219E/E; no pathogenic mutations (24 bp deletion in octapeptide repeat region)
PrP depositionf	Kuru plaques	Kuru plaques

^aDetails of the clinicopathological features of these patients have been reported elsewhere (10, 11). Underlined data were not described in the original reports and were obtained in the present study.

^bThere was no medical history of the use of dura mater grafts, pituitary hormone, corneal transplants, or electroencephalogram (EEG) needles in either patient.

^cCase 1 had necrotizing cutaneous lesions with vasculitis in the lower extremities. A part of his initial symptoms might have been related to these lesions.

^dPSWCs on EEG, the presence of periodic sharp-wave complexes (PSWCs) in electroencephalogram (EEG) results, which is a characteristic of sCJD-MM1/MV1.

^ePRNP gene, the genotype at polymorphic codon 129 (methionine [M] or valine [V]) or codon 219 (glutamate [E] or lysine [K]) of the PRNP gene.

^fPrP deposition, the patterns of PrP deposition in the brain.