The natural history of hereditary neuralgic amyotrophy in the Dutch population: Two distinct types?
On investigation of 101 attacks in 24 patients with hereditary neuralgic amyotrophy (HNA) from nine different families, we found that HNA can run two distinct courses: a `classic' relapsing–remitting and a chronic undulating type with exacerbations. Only one type occurred per family, suggesting genetic heterogeneity. This is supported by the finding that only in a family with `classic type' HNA are data of linkage analysis compatible with linkage to the 17q24–q25 interval which harbours a locus for the disease. The average number of attacks per patient during a follow-up of 26 years was four i... Mehr ...
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Dokumenttyp: | TEXT |
Erscheinungsdatum: | 2000 |
Verlag/Hrsg.: |
Oxford University Press
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Schlagwörter: | Articles |
Sprache: | Englisch |
Permalink: | https://search.fid-benelux.de/Record/base-27411002 |
Datenquelle: | BASE; Originalkatalog |
Powered By: | BASE |
Link(s) : | http://brain.oxfordjournals.org/cgi/content/short/123/4/718 |
On investigation of 101 attacks in 24 patients with hereditary neuralgic amyotrophy (HNA) from nine different families, we found that HNA can run two distinct courses: a `classic' relapsing–remitting and a chronic undulating type with exacerbations. Only one type occurred per family, suggesting genetic heterogeneity. This is supported by the finding that only in a family with `classic type' HNA are data of linkage analysis compatible with linkage to the 17q24–q25 interval which harbours a locus for the disease. The average number of attacks per patient during a follow-up of 26 years was four in the classic form of HNA and five in the chronic undulating type. All patients suffered from residual symptoms on follow-up, with a median Rankin score of 2 in both groups, showing that long-term prognosis is less favourable than previously reported.