Gli autori descrivono il caso di una giovane paziente sottoposta all'età di 16 anni ad intervento chirurgico di anastomosi spleno-renale per trombosi delle vene sovraepatiche conseguente ad incongrua manovra di cateterizzazione della vena ombelicale nelle prime 48 ore di vita. All'età di 20 ani la paziente giunge alla nostra osservazione per amenorrea secondaria; l'esame RM evidenzia un incremento del segnale nelle sequenze poderate in T1 in corrispondenza dei globi pallidi, della regione subtalamica e dell'adenoipofisi.
Gli autori discutono le possibili cause ed il significato clinico delle alterazioni di segnale nella regione dei nuclei della base in soggetti con insufficienza epatica cronica associata o meno a shunts porto-sistemici.
AdamsRD: Acquired hepatocerebral degeneration. In: VinkenPJBruynGW, (eds): Handbook of clinical neurology. Vol 6. Diseases of the basal ganglia. North-Holland, Amsterdam1968: 279–297.
2.
BarbeauA: Manganese and extrapyramidal disorders. Neurotoxicology5: 13–361, 1984.
3.
BernthalPHaysA: Cerebral CT scan abnormalities in cholestatic and hepatocellular disease and their relationship to neuropsychologic test performance. Hepatology7: 107–114, 1987.
4.
BrumbergJAKanalE: Chronic acquired hepatic failure: MR imaging of the brain at 1, 5 T. Am J Neurorad12: 909–914, 1991.
5.
DevenyiAGBarronTFMamourianAC: Dystonia, hyperintense basal ganglia, and high whole blood manganese levels in Alagille's Syndrome. Gastroenterology106: 1068–1071, 1994.
6.
DonaldsonJLaBellaFSGesserD: Enhanced autoxidation of dopamine as a possible basis of manganese neurotoxicity. Neurotoxicology2: 53–64, 1981.
7.
FellJMEReynoldsAP: Manganese toxicity in children receiving long-term parenteral nutrition. Lancet347: 1218–1221, 1996.
8.
FinlaysonMHSupervilleB: Distribution of cerebral lesions in acquired hepatocerebral degeneration. Brain104: 79–95, 1981.
9.
GriggsRC: Recognition and treatment of hepatic encephalopathy. Annual Course of American Academy of Neurology. Vol VII, course n° 226. S. Diego1995.
10.
HauserRAZesiewiczTA: Manganese intoxication and chronic liver failure. Annals of Neurology36/6: 871–875, 1994.
11.
HuangCCLuCS: Progression after chronic manganese exposure. Neurology43: 1479–1483, 1993.
12.
InoueEShinichiH: Portal-systemic encephalopathy: Presence of basal ganglia lesions with high signal intensity on MR images. Radiology179: 551–555, 1991.
13.
KulisevskyJRuscadellaJGrauJM: MR imaging of acquired hepatocerebral degeneration. Am J Neurorad12: 527–528, 1991.
14.
KulisevskiJPujolJ: Pallidal hyperintesity on magnetic resonance imaging in cirrhotic patients: Clinical correlations. Hepatology16: 1382–1388, 1992.
15.
KulisevskyJPujolJ: MRI pallidal hyperintensity and brain atrophy in cirrhotic patients: Two different MRI patterns of clinical deterioration?Neurology43: 2570–2573, 1993.
16.
KulisevskyJPujolJ: Persistence of MRI hyperintensity of the globus pallidus in cirrhotic patients: A 2-year follow-up study. Neurology45: 995–997, 1995.
17.
LangstonJW: Industrial toxins in Parkinsonism. Annual Course of American Academy of Neurology. Vol. IV, course n° 440. S. Francisco1996.
18.
LangstonJW: Chemicals and the shakes: Designer steel, cloths and drugs. (Story of Parkinsonism among manganese miners, steel and rayon industry & MPTP drugs). Annual Course of American Academy of Neurology. Vol VIII, course n° 125. S. Francisco1996.
19.
LaursenH: Cerebral vessels and glial cells in liver disease. A morphometric and electron microscopic investigation. Acta Neurol Scand65: 381–412, 1982.
20.
MirowitzSAWestrichTJHirschJD: Hyperintense basal ganglia on T1-weighted MR images in patients receiving parenteral nutrition. Radiology181: 117–120, 1991.
21.
MirowitzSAWeistrichTJ: Basal ganglia signal intensity alterations: Reversal after discontinuation of parenteral manganese administration. Radiology185: 535–536, 1992.
22.
PujolAPujolJ: Hyperintense globus pallidus on T1-weighted MRI in cirrhotic patients is associated with severity of liver failure. Neurology43: 65–69, 1993.
