Hereditary haemochromatosis is unlikely to cause movement disorders--a critical review.
Hereditary haemochromatosis (HH) is a common autosomal recessive systemic iron overload disorder in which CNS manifestations, particularly movement disorders, have been reported. We report a 63-year-old woman with familial HH with a four-year history of progressive gait disturbance, chorea, and mild cervical and laryngeal dystonia. Her movement disorder was thought to be related to the haemochromatosis. On further investigation, analysis for the Huntington's disease expansion was positive. A review of the seven published cases of movement disorders associated with HH as well as data concerning brain iron deposition in this condition leads us to debate the causal link between movement disorders and HH. We suggest that movement disorders are rare in association with HH, and that such patients should be thoroughly investigated for another cause for their movement disorder.
Russo
Noemi
N
Sobell Department of Motor Neuroscience & Movement Disorders, Institute of Neurology, University College London, Queen Square, WC1N 3BG, UK.
Edwards
Mark
M
Andrews
Thomasin
T
O'Brien
Michael
M
Bhatia
Kailash P
KP
D001479:Basal Ganglia / Q000378:metabolism / Q000473:pathology / Q000503:physiopathology*; D003937:Diagnosis, Differential; D018450:Disease Progression; D005192:Family Health; D005260:Female; D006432:Hemochromatosis / Q000150:complications / Q000175:diagnosis*; D006501:Hepatic Encephalopathy / Q000175:diagnosis / Q000503:physiopathology; D006801:Humans; D006816:Huntington Disease / Q000150:complications / Q000175:diagnosis* / Q000235:genetics*; D007501:Iron / Q000378:metabolism / Q000633:toxicity; D008875:Middle Aged; D009069:Movement Disorders / Q000175:diagnosis* / Q000209:etiology / Q000235:genetics; D019680:Trinucleotide Repeat Expansion / Q000235:genetics
D007501:Iron