|Year : 2006 | Volume
| Issue : 1 | Page : 46-48
Extrahepatic portal vein obstruction with parkinsonism and symmetric hyperintense basal ganglia on T1 weighted MRI
Sita S Jayalakshmi, Rupam Borgohain, S Mohandas
Department of Neurology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad - 500082, A.P, India
Sita S Jayalakshmi
Department of Neurology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad - 500082
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Abnormal high signal in the globus pallidus on T1 weighted magnetic resonance imaging (MRI) of the brain has been well described in patients with chronic liver disease. It may be related to liver dysfunction or portal-systemic shunting. We report a case of extra hepatic portal vein obstruction with portal hypertension and esophageal varices that presented with extra pyramidal features. T1 weighted MRI brain scans showed increased symmetrical signal intensities in the basal ganglia. Normal hepatic function in this patient emphasizes the role of portal- systemic communications in the development of these hyperintensities, which may be due to deposition of paramagnetic substances like manganese in the basal ganglia.
Keywords: Extra-hepatic portal vein obstruction, hyper intense basal ganglia, portal-systemic encephalopathy
|How to cite this article:|
Jayalakshmi SS, Borgohain R, Mohandas S. Extrahepatic portal vein obstruction with parkinsonism and symmetric hyperintense basal ganglia on T1 weighted MRI. Ann Indian Acad Neurol 2006;9:46-8
|How to cite this URL:|
Jayalakshmi SS, Borgohain R, Mohandas S. Extrahepatic portal vein obstruction with parkinsonism and symmetric hyperintense basal ganglia on T1 weighted MRI. Ann Indian Acad Neurol [serial online] 2006 [cited 2021 May 8];9:46-8. Available from: https://www.annalsofian.org/text.asp?2006/9/1/46/22823
Abnormal high signal in the globus pallidus has been described in 50 to 75% of patients with chronic liver disease on T1 weighted MR imaging. The basis of this signal alteration is unclear but may be related to liver dysfunction or portal- systemic shunting and is independent of the etiology of the liver disease.,, Liver transplantation is known to resolve this abnormal signal suggesting that hepatic dysfunction could account for the development of these changes in the striatum. Increased symmetrical signal intensities in the globus pallidus are not necessarily indicative of chronic hepatic encephalopathy (CHE) and could result from large portal - systemic collaterals. We report such MRI changes in a patient with extra hepatic portal vein obstruction (EHPO), extrapyramidal manifestations and normal hepatic function emphasizing the role of portal-systemic communications in the development of extrapyramidal features associated with hyperintense T1 weighted MR images of the basal ganglia.
| Case Report|| |
A 30-year-old lady presented to the neurological services with complaints of slowness in activities of daily living, stiffness of all four limbs, alteration in speech and tremor of the left upper limb of four months duration. A decade prior to the present admission, she was admitted to the gastroenterology department in this institution with recurrent bouts of hemetemesis and was diagnosed to have extrahepatic portal vein obstruction, with portal hypertension and esophageal varices. She underwent sclerotherapy for the varices and had remained asymptomatic until the present admission. During the present admission, general physical examination did not reveal anything of note except for mild anemia. Cardiovascular and respiratory system examination was normal. Abdominal examination showed moderate splenomegaly but no evidence of hepatomegaly or ascites. Neurological examination revealed normal mental functions. Speech was slow, monotonous and hypophonic. Restriction of upward gaze and decreased blink rate were present with preserved horizontal gaze and vergence movements. The cranial nerves were normal. Bradykinesia and cogwheel rigidity were present in all the four limbs with rest tremor in the left upper limb. No motor weakness was discernible but deep tendon reflexes were symmetrically exaggerated bilaterally with extensor plantar responses. Coordination was normal. Complete blood picture and erythrocyte sedimentation rates were normal. Blood urea, creatinine, serum proteins, ceruloplasmin and urinary copper were within normal limits. Mild elevation of serum transaminases was noted. Abdominal ultrasound showed multiple collaterals replacing the portal vein. Technetium 99m sulphur colloid hepatic scan showed small sized liver with splenomegaly but no scintigraphic evidence of hepatic parenchymal dysfunction. MRI of the brain showed bilaterally symmetric hyperintense signals in the region of caudate, putamen and globus pallidii on T1 weighted images [Figure - 1]. T2 weighted images were normal. Liver biopsy was essentially normal. She responded to L-dopa with significant improvement of rigidity and tremor.
| Discussion|| |
Sherlock et al. coined the term portal-systemic encephalopathy (PSE) for the neuropsychiatric state associated with chronic portal-systemic collaterals which includes both clinical and subclinical cases with structural cerebral changes. Abnormal high signal intensity on T1 weighted MR images symmetrically involving the globus pallidii, portions of the internal capsule and cerebral peduncles bilaterally has been increasingly recognized in the setting of advanced liver disease with portal-systemic shunts. The cause and mechanisms leading to the increased signal intensities are not clear. It has been postulated that the presence of porto-systemic shunting could allow toxic substances with paramagnetic properties to selectively accumulate within the globus pallidii. It may also be related to altered intracellular relaxation associated with the proliferation of astrocyte cytoplasmic organelles. The hyperintensities correlate to the severity of liver disease and are reversible when liver function returns to normal. The signal intensity significantly decreases or disappears after liver transplantation suggesting that liver dysfunction could be the cause of this MRI abnormality. This increased signal intensity could also be related to the degree of portal systemic shunting. As shown by Inoune et al., patients with large (more than 10 mm) portal systemic collateral vessels originating from the superior mesenteric vein in the angiographic studies, had hyperintense globus pallidii in the MRI. Those with smaller size (less than 5 mm) of the collateral vessels had normal MRI. Portal hypertension, particularly in extrahepatic portal vein obstruction, can lead to the opening of certain embryonic venous channels (retroperitoneal collaterals) more so after the obliteration of esophageal varices. Large splenoadrenorenal shunts can develop following sclerotherapy in the long term follow up, thereby preventing gastrointestinal bleeding but a theoretical possibility of development of encephalopathy exists. These large shunts might also reduce the hepatic detoxification because of diversion of blood. The present case suggests that paramagnetic substances like manganese could be deposited in the basal ganglia in patients with normal hepatic function by simply bypassing the liver through the large portosystemic channels and could be the basis for porto-systemic encephalopathy. Symmetrical pallidal and mesencephalic hyperintensity on T1 weighted MRI has been reported in chronic liver failure, prolonged parenteral nutrition and manganese toxicity. Manganese has been implicated in the pathogenesis of chronic hepatic encephalopathy (CHE) in the setting of hepatic dysfunction leading to excessive excretion into bile. It has been found that manganese accumulates within the basal ganglia with highest concentration in the caudate followed by the quadrigeminal plate and globus pallidus in patients with cirrhosis.
Development of extrapyramidal manifestations after 10 years of sclerotherapy underscores the slowly but definitely progressive volume of adreno-lieno-renal shunts. This patient with EHPO underwent sclerotherapy for esophageal varices a decade prior to the development of neurological symptoms probably facilitating the establishment of large spleno-adreno-renal shunts by opening up embryonic channels. Hyperintensities of the basal ganglia in patients with extra hepatic portal vein obstruction has not been reported so far and this is the first case being reported. The pathogenesis and MRI abnormalities may be the same as in other patients with chronic liver disease.
Symmetrical hyper intensities in the basal ganglia on T1 weighted MR images in advanced chronic liver disease have been attributed to hepatic failure and/or portal-systemic shunts. As advanced liver disease has been invariably associated with portal- systemic communications, it was thought that both factors contribute to the development of hyperintensities and PSE. Demonstration of such changes on MRI in a case of EHPO clearly indicates that hepatic dysfunction is not mandatory. This may be due to the deposition of paramagnetic substances like manganese in the basal ganglia in patients with large portal-systemic collateral vessels. Chelating agents have been suggested as a therapeutic option to prevent or reverse the neuro-psychiatric symptoms in patients with hepato-cerebral degeneration.
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[Figure - 1]