Table 1 Clinical characteristics of the five FFI patients.

From: Clinical Features and Sleep Analysis of Chinese Patients with Fatal Familial Insomnia

 

Case 1

Case 2

Case 3

Case 4

Case 5

Gender

M

F

F

F

M

Age at onset (years)

62

60

19

36

55

Onset to diagnosis duration (months)

3

5

3

8

11

Onset to death duration (months)

12

11

8

10

18

Cluster A—sleep-related symptoms

Insomnia

+

+

+

+

+

Sleep-related involuntary movements

+

+

+

+

+

Sleep-related dyspnea

+

+

+

+

+

Laryngeal stridor

+

+

+

+

+

Cluster B—neuropsychiatric symptoms

RPD

+

+

+

+

+

Psychiatric symptoms

+

+

−

+

+

Ataxia

−

+

+

+

+

Pyramidal sign

−

−

+

+

−

Parkinsonism

−

−

−

−

+

Cluster C–progressive sympathetic symptoms

Autonomic symptoms

Hypertension

+

+

−

+

−

Sweating

+

+

−

+

+

Tachycardia

+

−

+

+

−

Irregular breathing

−

−

+

−

−

  1. RPD: rapidly progressive dementia. +: symptom/sign observed, −: symptom/sign not observed. Psychiatric syndromes included hallucination, personality change, depression, anxiety, aggressiveness, disinhibition, listlessness etc. Autonomic signs include hypertension, sweating, tachycardia, irregular breathing etc. Sleep-related involuntary movements included frequent changes in the body position and twitchy non-purposeful movements of limbs during sleep.