Summary of clinical and pathologic findings in six cases of pCJD

Case No.	Age (y)/Sex	Duration of Illness (mo)	Clinical Findings (mo after onset)	Brain Weight (gs)	Histologic Findings (gray matter: spongiform change, neuronal loss and gliosis; white matter: loss of myelin and axons)
Case No.	Age (y)/Sex	Duration of Illness (mo)	Clinical Findings (mo after onset)	Brain Weight (gs)	Cerebral Cortex	Cerebral White Matter	Internal Capsule	Basal Ganglia	Thalamus	Brain Stem	Middle Cerebellar Peduncle
1	66/M	8	Dementia (1); change of character (0); myoclonus (3); ataxia (2); akinetic mutism (4); dysarthria (2); PSD (4)	1210	++	+–++	−	ST: ++	++	CP: −	−
								GP: ++		PLF: −
										PN: −
										PTF: −
2	64 /F	15	Dementia (1); ataxia (0); myoclonus (1); tremor (1); akinetic mutism (2); dystonia (1); PSD	900	+++	+++	−	ST: +++	++	CP: −	−
								GP: ++		PLF: +
										PN: −
										PTF: +
3	78 /F	22	Dementia (0); tremor (1); myoclonus (2); diplopia (1); akinetic mutism (3); PSD (2)	740	+++	+++	−	ST: +++	++–+++	CP: −	++
								GP: +++		PLF: −
										PN: +
										PTF: ++
4	76 /F	25	Dementia (2); tremor (0); myoclonus (2); hemiplegia (1); akinetic mutism (3); PSD	720	+++	+++	−	ST: +++	++–+++	CP: +	+
								GP: ++		PLF: +
										PN: +
										PTF: ++
5	68 /F	28	Dementia (1); ataxia (0); myoclonus (2); chorea (1); akinetic mutism (2); PSD	650	+++	+++	++	ST: +++	++–+++	CP: +++	+++
								GP: ++		PLF: +++
										PN: +++
										PTF: +++
6	82 /F	30	Dementia (0); gait disturbance (5); myoclonus (5); akinetic mutism (6); PSD	740	+++	+++	++	ST: +++	++–+++	CP: +++	+++
								GP: ++		PLF: +++
										PN: +++
										PTF: +++

Note.—PSD, periodic synchronous discharge on electroencephalogram; ST, striatum; GP, globus pallidus; CP, cerebral peduncle; PN, pontine nucleus; PTF, pontine transverse fibers; PLF, pontine longitudinal fibers; +, mild; ++, moderate; +++, marked.