Table 1.

Clinical features of patients with the Heidenhain variant of sCJD

N°	Gender	Age	Visual symptoms (VS)	VS duration (mo)	Other symptoms	Disease duration (mo)
1	F	54	Visual field restriction, loss of vision, disturbed perception of colors or objects, hallucinations	2.5	Delirium, confusion, disorientation, dystonia, pyramidal signs, akinetic mutism	5
2	M	63	Blurred vision, visual field restriction,	2	Aphasia, attentive deficit, cerebellar signs, myoclonus	4
3	M	67	Blurred vision, visual field restriction, vision loss up to cortical blindness	1.5	Cognitive disturbances, abnormal behavior, myoclonus, tremor, pyramidal signs, akinetic mutism	5
4	M	80	Blurred vision, disturbed perception of colors or structures, hallucinations	1	Ataxia, disorientation	4
5	M	69	Blurred vision, vision loss	1	Dysarthria, myoclonus, cognitive and gait disturbances	2.5
6	F	70	Blurred vision, vision loss up to cortical blindness, disturbed perception of colors or structures	2	Ataxia, cognitive decline, myoclonus, akinetic mutism (several months with parenteral nutrition in vegetative state)	10
7	F	80	Disturbed perception of colors or objects, optical anosognosia	1	Cognitive decline, cerebellar and pyramidal signs, seizures, myoclonus, akinetic mutism	5
8	M	78	Disturbed perception of structures, optical hallucinations	1.5	Alien arm syndrome, apraxia, involuntary movements, ataxia	3.5
9	M	69	Visual field restriction, vision loss, optical hallucination (palinopsia)	2	Pyramidal signs, myoclonus, abrupt stupor, coma	4
10	F	64	Blurred vision, disturbed perception of objects (simultanagnosia)	1.5	Myoclonus, memory loss, disorientation, ataxic gait, akinetic mutism	2.5
11	F	77	Visual field restriction, vision loss up to cortical blindness, optical anosognosia	1	Myoclonus, ideomotor apraxia, disorientation	3.5
12	F	63	Visual field restriction, vision loss up to cortical blindness, disturbed perception of objects (distortion)	2	Memory loss, aphasia, weakness, myoclonus	3.5
13	M	57	Visual field restriction, blurred vision, disturbed perception of objects	2	Dementia, myoclonus, rigidity, seizures, coma	3
14	F	79	Disturbed perception of colors and objects, visual field restriction	2	Ataxia, myoclonus, dementia, aphasia, coma	4
15	F	70	Disturbed perception of colors or objects, visual field restriction	1	Ataxic gait, ideomotor apraxia, dystonia, memory loss, myoclonus, rigidity, apallic state	3
16	F	70	Vision loss, disturbed perception of colors or structures, optical anosognosia	6	Ataxia, dysphonia, dysphagia, cognitive disturbances, pyramidal signs	26
17	M	54	Optical hallucination, environmental agnosia	12	Discalculia, pyramidal signs, disorientation, psychosis	44
18	M	48	Blurred vision, visual field restriction, vision loss up to cortical blindness	2.5	Dysarthria, dysphagia, disorientation, myoclonus	6