Leukoencephalopathy, intracranial calcifications, and cysts (LCC) is a very rare cerebral disorder, first described in 3 children in 1996. It has subsequently been reported in adults and children from Europe and America, but has not so far been reported from Asia. We report an adult patient with pathologically proven LCC from a tertiary care hospital in South India. He presented with features of ataxia and raised intracranial pressure. Magnetic resonance imaging of the brain showed multiple bilateral cerebral cystic lesions along with diffuse white matter lesions in the cerebral and cerebellar white matter, and computed tomography of brain showed multiple calcifications in the white matter and basal ganglia. A large right cerebellar cyst causing mass effect was surgically excised. Histopathologic features were consistent with earlier reports of LCC and showed Rosenthal fibers, angiomatous changes, and calcifications. Our report suggests that although it is rare, LCC has a global distribution.
Cirrhosis presenting as Parkinsonism is a distinct subset of acquired chronic hepatocerebral degeneration. The entity is not rare, and unless suspected, cirrhosis can easily be overlooked. We report our experience with three such patients. They presented to us, over a period of two years, with symmetrical Parkinsonism and were later diagnosed to have cirrhosis with portal hypertension. All patients had minimal or absent tremors. Reversal of serum albumin to globulin ratio and evidence of cirrhosis on abdominal ultrasound were consistent. All three patients had the characteristic MRI abnormality of symmetrical T1 hyperintensity in basal ganglia and anterior midbrain. They improved to variable extents after treatment for cirrhosis, along with dopa agonists. We stress the importance of recognizing this syndrome and briefly review the relevant literature.
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