B. Tomik et al., Phenotypic differences between African and white patients with motor neuron disease: a case-control study, J NE NE PSY, 69(2), 2000, pp. 251-253
There is increasing evidence that race may affect the phenotype in some neu
rodegenerative diseases. To investigate this in motor neuron disease a retr
ospective case-control study has been carried out on 15 negroid African and
45 white patients with the disease seen over 8 years. Each African was com
pared with three age and sex matched white patients with motor neuron disea
se. There were no statistically significant differences in age of onset or
the mean duration of disease in the two groups. The chance of presenting wi
th the "flail arm" variant of motor neuron disease was four times as high i
n the African group than the white group (odds ratio 4.33, p=0.05, 95% conf
idence interval 0.99-18.92). Although no overall differences in survival we
re seen between the two groups, in those with the flail arm variant, four o
ut of the six African patients had died whereas all six white arm patients
were alive at the censoring date of 1 January 1999 (median follow up 38.5 m
onths). It is concluded that race may influence the phenotype and progressi
on of motor neuron disease.