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 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.
van Alfen
N
N
Neuromuscular Centre Nijmegen, Institute of Neurology, University Hospital Nijmegen, The Netherlands.
van Engelen
B G
BG
Reinders
J W
JW
Kremer
H
H
Gabreëls
F J
FJ
D000293:Adolescent; D000328:Adult; D001341:Autonomic Nervous System / Q000503:physiopathology; D020968:Brachial Plexus Neuritis / Q000150:complications / Q000209:etiology / Q000235:genetics* / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D005260:Female; D008040:Genetic Linkage / Q000235:genetics; D006801:Humans; D008297:Male; D008875:Middle Aged; D009069:Movement Disorders / Q000209:etiology / Q000503:physiopathology; D009426:Netherlands; D010146:Pain / Q000503:physiopathology; D010166:Palliative Care; D010375:Pedigree; D020127:Recovery of Function; D012677:Sensation