Neurophysiological localisation of ulnar neuropathy at the elbow: Validation of diagnostic criteria developed by a taskforce of the Danish Society of clinical neurophysiology.

OBJECTIVE: This study validates consensus criteria for localisation of ulnar neuropathy at elbow (UNE) developed by a taskforce of the Danish Society of Clinical Neurophysiology and compares them to the existing criteria from the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM). The Danish criteria are based on combinations of conduction slowing in the segments of the elbow and forearm expressed in Z-scores, and difference between the segments in m/s. Examining fibres to several muscles and sensory fibres can increase the certainty of the localisation. METHODS: Diagnostic accuracy for UNE was evaluated on 181 neurophysiological studies of the ulnar nerve from 171 peer-reviewed patients from a mixed patient-group. The diagnostic reference standard was the consensus diagnosis based on all available clinical, laboratory, and electrodiagnostic information reached by a group of experienced Danish neurophysiologists. RESULTS: The Danish criteria had high specificity (98.4%) and positive predictive value (PPV) (95.2%) and fair sensitivity (76.9%). Compared to the AANEM criteria, the Danish criteria had higher specificity (p<0.001) and lower sensitivity (p=0.02). CONCLUSIONS: The Danish consensus criteria for UNE are very specific and have high PPV. SIGNIFICANCE: The Danish criteria for UNE are reliable and well suited for use in different centres as they are based on Z-scores.

Clinical and laboratory-reconfirmed myasthenia gravis: a population-based study.

The aim of this study was to compare the clinically based prevalence of myasthenia gravis (MG) with the prevalence of laboratory-confirmed cases. All patients with a diagnosis of MG living in Estonia as on 1 January 1997 were asked to participate in re-examination. The criteria for laboratory-supported MG were weakness and rapid fatigue and a positive outcome of at least one of three laboratory tests: (i) blinded acetylcholinesterase inhibitor test; (ii) determination of antibodies to acetylcholine receptor and (iii) neurophysiological examination using repetitive nerve stimulation and single-fibre EMG. Eighty-nine patients were re-examined and 70 patients (79%) fulfilled the criteria of laboratory-supported MG. The corrected prevalence ratio was 78 per million. In the non-confirmed MG group, there was more women (92%) than men (43%) whose diagnosis was established within 1 year from onset of symptoms (P = 0.016). In all women with non-confirmed MG the diagnosis was established within 1 year from referral to the physician, whereas 68% of women with confirmed MG was diagnosed within 1 year (P < 0.0001). Thus, we conclude that, in Estonia the prevalence of MG based on medical records seems overestimated by 21% and women are at higher risk of obtaining an uncertain diagnosis of MG.

A population based epidemiological study on myasthenia gravis in Estonia.

OBJECTIVE: To describe the occurrence of myasthenia gravis in the Baltic area. METHODS: Data were obtained from hospital files recorded during the period 1942 to 1996 from neurologists and the patient organisation. Survival data were checked with the Estonian Citizenship and Migration Board. Prevalence was determined on 1 January 1997. A questionnaire on the course of myasthenia gravis was sent to all the prevalent patients. RESULTS: The size of the population surveyed was 1 462 130. The average annual incidence from 1970 to 1996 was 4.0 per million (women, 5.2; men, 2.6). The point prevalence was 99 per million (women, 133; men 59). The incidence in the younger age group (<50 years) was 3.4 per million (women, 4.8; men, 1.9) and in the older age group (>or=50 years), 5.5 (women, 5.9; men, 4.9). The prevalence ratio was twofold higher in the older age group (p<0.0001)-for men (p = 0.034) as well as for women (p<0.001). CONCLUSIONS: Prevalence and incidence values of myasthenia gravis from Estonia are similar to those reported in most studies from Europe and north America. However, there seems to be a higher frequency in the elderly (>or=50 years) in Estonia.

The risk of motor neurone disease and multiple sclerosis is different in Estonians and Russians. Data from South Estonia.

Epidemiological studies were performed in South Estonia to establish the prevalence rate of multiple sclerosis (MS) and motor neurone disease (MND). The case finding method included information from the hospital records of the central hospital in the region-the University Hospital (for MS from 1942 to 1989), from all neurologists in the region, from the Estonian MS Society and Association of Muscular Disorders, and from nursing homes in the region. The prevalence day was 31 December 1989. MND incidence was established for the period of 1986-1995. The results demonstrated high prevalence rates of MS among native Estonians (55.3 per 100 000), somewhat lower prevalence among native-born representatives of other nationalities (43.6 per 100 000) and the lowest prevalence rate of MS among non-Estonian immigrants (26.6 per 100 000). The differences were not statistically significant. The results for MND demonstrated the opposite pattern. The mean annual incidence rate of MND for 10 years was statistically significantly higher among people of other nationalities (2.5 per 100 000) and Russians (2.6 per 100 000), and lower in native-born Estonians (1.1 per 100 000). No differences in health care or clinical picture were established. The reasons for the demonstrated differences in MND incidence remain unclear.

Motor neurone disease in South Estonia. Diagnosis and incidence rate.

The current study evaluated the diagnostic standards of MND and epidemiological markers of MND in Estonia. A total of 108 patients were referred to the University Hospital from 1986 to 1995 with the first suggested diagnosis or final diagnosis of amyotrophic syndrome, amyotrophic lateral sclerosis (ALS), progressive bulbar paralysis (PBP) or progressive muscular atrophy (PMA). In addition neurologists of the region and the National Society of Neuromuscular disorders were contacted. Some 94 patients satisfied the diagnostic criteria. The annual incidence rate in South Estonia and in the city of Tartu ranged from 0.5 to 2.8 per 100,000. The mean annual incidence rate in Tartu is 1.98 and in South Estonia in general 1.3. The highest incidence rate was 8.3 for men in the age group 60 to 64 years and 7.49 in the age group 70-74; among female patients the highest incidence rate -4.6 was in the age group from 65 to 69.