Wilson's disease: a great masquerader.
BACKGROUND
Wilson's disease (WD) is a treatable autosomal recessive metabolic disorder which could lead to protean hepatic or neurologic manifestations. WD could mimic many neurologic disorders and is often diagnosed with a long delay. This study describes central nervous system manifestations of a group of Iranian patients with neurologic WD.


METHODS
Data from case records of patients with neurologic WD presenting at a referral university hospital and a private clinic in Tehran from 1984 to 2004 were analyzed.


RESULTS
Fifty patients from 44 unrelated families with WD were identified, whose mean duration of follow-up was 51.8 (+/-58.5) months. The median age of onset of neurologic symptoms in 37 patients with primary neurologic or simultaneous hepatic-neurologic presentation was 16 (10-38) years, whereas in 13 patients with prior hepatic damage, this was 18.5 (11-34) years. The 6 most common manifestations were dysarthria (80%), drooling (48%), tremor in limbs (44%), abnormal gait (44%), psychiatric and/or sleep symptoms (44%), and dystonia in limbs (42%).


CONCLUSIONS
Neurologic WD has heterogeneous manifestations and should be considered in young patients presenting with dysarthria, drooling, any kind of movement disorders or psychiatric symptoms.
Soltanzadeh
Akbar
A
Department of Neurology, Shariati University Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Soltanzadeh
Payam
P
Nafissi
Shahriar
S
Ghorbani
Askar
A
Sikaroodi
Hajir
H
Lotfi
Jamshid
J
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D003937:Diagnosis, Differential; D004401:Dysarthria / Q000209:etiology; D004421:Dystonia / Q000209:etiology; D005260:Female; D020233:Gait Disorders, Neurologic / Q000209:etiology; D006527:Hepatolenticular Degeneration / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology*; D006801:Humans; D008297:Male; D001523:Mental Disorders / Q000209:etiology; D009422:Nervous System Diseases / Q000209:etiology*; D012798:Sialorrhea / Q000209:etiology; D014202:Tremor / Q000209:etiology