The natural history of dystonia.
We have analyzed 226 patients with a diagnosis of dystonia musculorum deformans seen in our clinica between 1955 and 1974. These were evenly divided between male and female, but of the 226 patients, 225 were white and only one was black. Forty-two percent were Jewish, as contrasted with 3% Jewish population in the United States. It is interesting to note that a family history was obtained in 28% of both the Jewish and the non-Jewish groups. Thirty of the patients reported a significant viral infection within 3 months preceding the onset of symptoms. Only six patientss had a history of one or more remissions during the course of their illness. The mean duration of symptoms before examination in this series was 8.6 years with a duration of 1 to 42 years. Fifty-six of the patients had been diagnosed as having conversion hysteria at some time during the course of their illness. The majority of the patients had the onset of symptoms between the ages of 5 and 10, although the age span was from 2 to 45 years of age. Seventy-six of the patients had an IQ statistically significant above average (3). Each symptom, rate of progress of the disease, and various other factors in the history were corrolated with age, sex, ethnic group, mode of onset, and 20 other variables. The most pertinent subgroupings affecting the natural history were the age on onset and the ethnic group and family history. Trunkal involvement was most common in the non-Jewish group with a positive family history. This particular sub-group of patients showed predominately midline symptomatology. In the groups below the age of 13, the onset was almost invariably in one of the four limbs. However, in the group 14 years of age and older, 30% had their initial dystonic symptoms in the neck and a total of 40% of the patients whose onset was at age 14 or later, had marked nuchal symptoms. This clear-cut predominance of limb involvement in the youngest groups is also indicated by the observation that 38% in the youngest group had become confined to a wheelchair because of gait abnormalities, whereas none of those whose age of onset was 14 or older were disabled to this degree by gait abnormality. The most rapidly progressive and incapacitating symptomatology was seen in those patients with onset of symptoms below the age of 8 so that in general the younger the age at onset, the more rapid the progress of the symptoms.
D000203:Activities of Daily Living; D000293:Adolescent; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D004422:Dystonia Musculorum Deformans / Q000150:complications / Q000235:genetics; D005006:Ethnicity; D005260:Female; D007839:Functional Laterality; D005684:Gait; D006801:Humans; D007223:Infant; D007360:Intelligence; D007585:Jews; D008297:Male; D012075:Remission, Spontaneous; D012737:Sex Factors; D013064:Speech Disorders / Q000209:etiology