We investigated the survival of patients with amyotrophic lateral sclerosis (ALS) in a follow-up study of all patients hospitalized with the disease in 2 Danish counties during the period 1974 to 1986. There were 186 patients, with a mean age at diagnosis of 64.3 years. The median survival time was 12 months from diagnosis, the 3-year survival rate was 12%, and the 5-year survival rate 4%. (The corresponding figures from onset were 23 months, 26%, and 7%). Old age and bulbar findings at onset were negative prognostic factors. For each clinical category, the annual death rate remained constant throughout the observation period, indicating the effects of a steadily progressing degenerative disorder. We found no evidence of the existence of a separate variety of ALS, associated with long survival.
Patients Twelve women and 10 men with a mean age of 59 3 (range 44-76) years comprised the study group. They had had idiopathic Parkinson's disease for an average of 9-8 (range 3-4-19-2) years, and when in on phase they were in Hoehn and Yahr stages 2-0-40.The mean duration of levodopa treatment was eight (range 3-19) years. Mean body weight was 69-9 (range 47-0-98-0) kg.All patients had daily, disabling off periods, defined as change in motor state within five minutes, and described by the patient and by relatives or a physician. Diphasic dyskinesia was an exclusion criterion.
A total of 186 cases of motor neuron disease (MND) was identified in two Danish counties during the period 1974–1986. The average annual incidence rate was 1.4/100,000 population, and the male:female ratio of incidence rates was 1.5. Mean age at diagnosis was 64.3 ± 10.0 years. The incidence rates increased significantly with advancing age and reached a maximum at age 60–79 years, followed by a nonsignificant decrease. The average point prevalence was 3.1/100,000 population. Bulbar symptoms were part of the initial symptoms in 65 % of cases, and patients with bulbar onset were older than patients with spinal onset. Age- and sex-specific incidence rates indicated a marked male preponderance amongst the youngest patients, in contrast to a female preponderance in patients above 60 years with bulbar onset of MND. Familial MND accounted for 2.7% of cases.
Discontinuation of indomethacin in chronic paroxysmal hemicrania (CPH) usually causes relapse of symptoms within a few days. A patient with characteristic symptoms of CPH is reported. The patient had daily attacks of unilateral headache accompanied by ipsilateral lacrimation and conjunctival injection, a frequency of attacks between four and fourteen per day, and never a headache-free day. He was treated with indomethacin, and all symptoms disappeared within 24 h. After three months indomethacin was discontinued. Six months after that the patient was still symptom-free.
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