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 |
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| 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 |