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Volume 13, Number 9—September 2007

Research

Family Clustering of Viliuisk Encephalomyelitis in Traditional and New Geographic Regions

Vsevolod A. Vladimirtsev*, Raisa S. Nikitina*, Neil Renwick†, Anastasia A. Ivanova*, Al’bina P. Danilova*, Fyodor A. Platonov*, Vadim G. Krivoshapkin*, Catriona A. McLean‡, Colin L. Masters‡, D. Carleton Gajdusek§, and Lev G. Goldfarb¶Comments to Author 
Author affiliations: *Institute of Health–Sakha (Yakut) Republic, Yakutsk, Russian Federation; †Columbia University Medical Center, New York, New York, USA; ‡University of Melbourne, Parkville, Victoria, Australia; §Institut Alfred Fessard, Gif-sur-Yvette, France; ¶National Institutes of Health, Bethesda, Maryland, USA;

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Table 1

Clinical features of Viliuisk encephalomyelitis in affected members of 6 families*

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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