Volume 13, Number 9—September 2007
Research
Family Clustering of Viliuisk Encephalomyelitis in Traditional and New Geographic Regions
Table 1
Feature |
Family 1 |
Family 2 |
Family 3 |
Family 4 |
Family 5 |
Family 6 |
|||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1-1 |
1-2 |
1-3 |
2-1 |
2-2 |
3-1 |
3-2 |
4-1 |
4-2 |
4-3 |
5-1 |
5-2 |
6-1 |
6-2 |
6-3 |
|
Sex | F | F | F | F | F | F | M | M | F | F | F | F | M | F | F |
Age at onset, y | 16 | 38 | 26 | 16 | 44 | 31 | 21 | 51 | 47 | 36 | 20 | 34 | 46 | 37 | 53 |
Febrile stage of illness | |||||||||||||||
Duration, wk | 7 | 11 | 2 | 2 | 10 | ND | 2 | ND | ND | 4 | ND | 1 | ND | 6 | 26 |
Maximum temperature, oC | 40.5 | 39 | 38 | 37.9 | 39.8 | 39.0 | 39.0 | ND | 38.5 | 38.8 | |||||
Reduced level of consciousness | ++ | +++ | + | +++ | + | ++ | + | +++ | |||||||
Generalized seizures | ++ | + | + | ||||||||||||
Neck rigidity and Kernig sign | + | ++ | + | ++ | + | + | + | ||||||||
Nausea, vomiting | + | ++ | + | ++ | ++ | + | |||||||||
Upper motor neuron pattern of muscle weakness | + | + | + | + | |||||||||||
Relapse of febrile illness | + | + | + | + | |||||||||||
Outcome |
a |
d |
a |
a |
d |
a |
a |
a |
a |
a |
a |
a |
a |
a |
d |
Advanced disease | |||||||||||||||
Cognitive decline | ++ | + | ++ | m.i. | ++ | ++ | ++ | ++ | ++ | ++ | + | ++ | |||
Apathy | + | + | ++ | + | + | + | + | ||||||||
Dysarthria | +++ | ++ | ++ | ++ | + | ++ | + | ++ | + | + | |||||
Dysphagia | + | + | + | + | |||||||||||
Brisk deep tendon reflexes | ++ | ++ | ++ | ++ | ++ | ++ | + | ++ | + | ++ | + | ++ | |||
Spastic tetraparaparesis | +++ | + | ++ | + | ++ | + | ++ | +++ | – | + | + | – | |||
Increased muscle tone | + | + | + | + | ++ | + | + | ++ | – | + | + | ||||
Babinski sign | + | + | + | + | + | + | + | + | |||||||
Spastic gait | +++ | ++ | ++ | + | +++ | + | + | + | + | + | + | + | |||
Falls | + | ++ | + | + | + | ||||||||||
Sphincter dysfunction | + | ++ | ++ | + | |||||||||||
Extrapyramidal rigidity | + | + | ++ | + | ++ | ++ | + | + | + | + | |||||
Obesity | + | + | + | + | |||||||||||
Cachexia |
+ |
+ |
++ |
+ |
|||||||||||
Overall duration of illness | 18 mo | 11 wk | A | 48 mo | 10 wk | 17 y | 24 mo | 9 y | 6 y | 11 mo | 11 y | 23 mo | 13 y | 27 mo | 26 wk |
*ND, not documented; m.i., memory impairment; +, mild; ++, moderate; +++, strong; –, not expressed; d, died within acute phase; a, advanced disease developed; A, alive.
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