Sporadic Creutzfeldt-Jakob Disease: Prion Pathology in Medulla Oblongata—Possible Routes of Infection and Host Susceptibility

Table 1

Main demographic and clinical data of all subjects in study. Group 2 is the second part of the table.

Case number	sCJD molecular type	Age at death/gender	Duration of disease (months)	Clinical signs at onset	Clinical signs at late clinical evaluation
1	MM1	70/f	3	Confusion	Myoclonus, ataxia, gait disorders, paraphasia, and coma
2	MM1	70/f	4	Cognitive impairment	Progressive cognitive impairment
3	MM1	63/m	6	Irritability, restlessness	Myoclonus, ataxia, motor perseverations, amnesia, loss of spontaneous movement, and aphasia
4	MM1	52/m	3	Amnesia, diplopia, and involuntary movements	Myoclonus, involuntary movements, bradikinesia, cognitive impairment, visual hallucinations, and aphasia
5	MM1	70/f	8	Confusion, hallucinations	Myoclonus, ataxia, progressive dementia, and coma
6	MM1	65/m	3	Confusion, gait disorders	Myoclonus, plastic hypertonia, ataxia, aphasia, and progressive cognitive impairment
7	MM1	71/f	16	Objective vertigo	Myoclonus evoked by auditory and tactile stimuli, ataxia hallucinations, paresthesias, diplopia, alien hand, and dementia
8	MM1	62/f	11	Ataxia, speech disorder	Myoclonus, behavioral changes (aggression), confusion, dysphagia, and spasticity
9	MM1	60/m	2	Confusion	Myoclonus, insomnia, visual hallucinations, apraxia, and cognitive impairment
10	MM1	69/m	6	Visual disorders	Myoclonus, ataxia, and cognitive impairment
11	MM2	61/f	11	Amnesia, impaired attention, and disorientation in time and place	Myoclonus, rigidity, alteration production and comprehension of the speech, agitation, altered sleep wake rhythm, and behavioral disorders
12	MM2	59/m	18	Depression	Amnesia, reduced ideational, ataxia insomnia, headache, cognitive impairment, amnesia, and reduced ideation
13	MV1	66/m	17	Aphasia	Tremor, involuntary movements, dysarthria, comprehension deficit, and akinetic mutism
14	MV2	79/m	6	Gait disorders, drowsiness	Myoclonus, motor slowing, ataxia, and impaired eye movements upwards
15	MV2	61/f	11	Ataxia	Myoclonus, ataxia dementia, and aphasia
16	MV2	48/m	48	Irritability, apathy, delusions, and paranoia	Rigidity, hypokinesia, visuospatial and memory disorders, and cognitive impairment
17	VV2	71/f	5	Dizziness	Myoclonus, photophobia, amnesia, confusion, cognitive impairment, drowsiness, and anxiety
18	VV2	64/f	13	Ataxia, cognitive impairment	Involuntary movements, ataxia depression, cognitive impairment, and delirium
19	VV2	62/f	3	Ataxia	Myoclonus, visual hallucinations, and gaze deviation to the left
20	VV2	68/f	6	Balance disorders	Bradikinesia, myoclonus, ataxia, disorders of attention, difficulty writing, and depression
21	VV2	69/f	6	Cognitive decline, myoclonus	Akinetic mutism