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.
Superior vena cava (SVC) syndrome refers to the clinical manifestation due to an obstruction in the SVC; resulting in decreased venous return from the head, neck and upper extremities. The obstruction can occur either due to tumour invasion of the vessel wall with associated thrombus or due to vessel wall compression by the tumour mass. The patient being reported is a young male who presented with recurrent episodes of syncope and was found to have mediastinal Gardner fibroma causing SVC syndrome. Gardner fibroma is a benign soft tissue lesion; and its occurrence in the mediastinum resulting in SVC syndrome has not been reported yet.
Red Baby Syndrome is a new disease seen in infants and young children. Dramatic onset of clinical symptoms with high intensity, short duration and lack of similarity with other cutaneous lesions makes it distinct. Of 50 such patients studied over a period of 5 years, half were below one year of age. Abrupt onset of high fever and generalized erythema involving the entire skin, which is swollen and tender is characteristic. These children were highly irritable and had paradoxical cry when cuddled. Rapid resolution of symptoms occurred in 7-10 days with extensive desquamation. Routine investigations were normal, C-reactive protein was raised only in 10 patients. Human Parvo virus B-19 IgM antibodies were positive in 15 out of 24 patients. Real time polymerase chain reaction was positive for human parvovirus B 19 DNA in one. Histopathological changes in the skin biopsy showed post infectious vascular injury pattern.
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