[Chronic acquired hepatocerebral degeneration. Two case reports].
BACKGROUND
Chronic acquired hepatocerebral degeneration (CAHD) is a rare heterogeneous disorder that may occur in patients with chronic liver disease, initially described in 1914 by van Woerkom. The spectrum of clinical presentations may be neuropsychiatric, a movement disorder, parkinsonism, cerebellar symptoms and myelopathy. The pathogenesis of CAHD has not been well clarified and is probably multifactorial. Neuroradiological patterns show a specific magnetic resonance (MR) imaging appearance with a hyperintense T1 signal in the basal ganglia and substantia nigra.


METHODS
We describe two cases with clinical features and MR imaging findings present in the CAHD. One had Budd-Chiari syndrome, myoclonus and T1 hyperintensity in pallidum and substantia nigra in MR. The other had focal dystonia of the fingers and T1 hyperintensity in pallidum in MR.


CONCLUSIONS
These two case reports have clinical and neuroradiological findings present in CAHD.
Martín Romano
L
L
Servicio de Neurología, Hospital Privado de Comunidad, Mar de Plata, Argentina. lromano@sna.org.ar
Alfredo Femminini
R
R
D002908:Chronic Disease; D005260:Female; D006527:Hepatolenticular Degeneration / Q000175:diagnosis*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged