Table 3.
Frequency of clinical symptoms and signs in fatal familial insomnia patients (n=23)
| Symptoms and signs | N | % | |
|---|---|---|---|
| Category A | Organic sleep disturbances* | 22 | 96 |
| Category B | Cognitive/mnestic deficits | 20 | 87 |
| Spatial disorientation | 15 | 65 | |
| Psychiatric | 20 | 87 | |
| Hallucinations† | 14 | 61 | |
| Personality change | 13 | 57 | |
| Depression | 5 | 22 | |
| Anxiety | 4 | 17 | |
| Aggressiveness | 2 | 9 | |
| Disinhibition | 2 | 9 | |
| Listlessness | 2 | 9 | |
| Others‡, each | 1 | 4 | |
| Ataxia | 19 | 83 | |
| Myoclonus | 16 | 70 | |
| Visual | 16 | 70 | |
| Double vision | 12 | 52 | |
| Blurred vision | 5 | 22 | |
| Others§, each | 1 | 4 | |
| Dysarthria¶ | 14 | 61 | |
| Pyramidal | 10 | 43 | |
| Extrapyramidal** | 8 | 35 | |
| Category C | Loss of weight | 19 | 83 |
| Vegetative signs | 19 | 83 | |
| Hyperhidrosis | 13 | 57 | |
| Newly diagnosed arterial hypertonia | 6 | 26 | |
| Tachycardia | 4 | 17 | |
| Obstipation | 3 | 13 | |
| Hyperthermia | 2 | 9 | |
| Others††, each | 1 | 4 | |
| Bulbar symptoms | 10 | 43 | |
| Husky voice | 5 | 22 | |
| Dysphagia | 3 | 13 | |
| Bulbar speech | 3 | 13 | |
| Tongue fasciculation | 1 | 4 | |
| Pruritus | 3 | 13 |
*Including polysomnography; insomnia, hypersomnia, restless sleep and sleep attacks.
†Optical in 14 patients, additionally acoustic in two of them.
‡Paranoia, fearfulness and rage to clean up.
§Seeing of flashes, poor vision, sliding field of vision and bad spatial vision.
¶Pseudobulbar and cerebellar.
**Nearly equally rigour, tremor and dystonia, mostly in combination.
††Tachypnoea, arterial hypotonia, intolerance of warmth, goose bumps, cardiac arrhythmia and abrogated day–night rhythm of blood pressure.