Extraocular muscle dystonia due to acquired (non-Wilsonian) hepatocerebral degeneration.
We present a video report of a patient with advanced non-Wilsonian cirrhotic liver disease who developed extraocular muscle dystonia (oculogyric crisis) and severe orofaciolingual dyskinesias. Acquired hepatocerebral degeneration causes choreic movements, especially of cranial muscles, but dystonic ocular spasm is an infrequent manifestation of this disorder. This case illustrates that AHD should be considered in the differential diagnosis of extraocular muscle dystonia.
Ferrara
Joseph
J
Department of Medicine, Division of Neurology, Duke University Medical Center, Durham, North Carolina, USA.
Gupta
Deepak
D
Foster
Emily
E
Garman
Katherine
K
Stacy
Mark
M
D003937:Diagnosis, Differential; D020820:Dyskinesias / Q000209:etiology; D020821:Dystonic Disorders / Q000209:etiology*; D005260:Female; D005917:Globus Pallidus / Q000473:pathology; D006527:Hepatolenticular Degeneration / Q000209:etiology / Q000503:physiopathology*; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009801:Oculomotor Muscles / Q000503:physiopathology*; D013378:Substantia Nigra / Q000473:pathology; D019896:alpha 1-Antitrypsin Deficiency / Q000150:complications