engleski

Kinnsbourne Syndrome : A case report

Konsbourne syndrome is an acute cerebelar encephalopathy characterized with ataxia (often with opoclonus, polymioclonus and iritability) associated with a solid malignant tumor- neuroblastoma.Our patient is a girl aged 18 months, referred to our hospital with dg: cerebral palsy, wich occured for the first time at the age of 8 months of the child, who had been healthy till then. A dominant neurologic symptom was trunkal ataxia, generalized hypotonia and expressive weakness of lower extremities, alternating converegent strabism and a lack of speech development. At the age of 21 months a solid abdominal tumor- neuroblastoma was diagnosed by ultrasound and computerized tomography. Further worsening in neurological status was registered. Concerning tumor markers, increased values of feritin and noradrenalin were found. In bone marrow tumor cells were found. Scintigraphy of skeleton show a multiple pathologic forming in bines, Concerning the spreading of the disease- stage IV - a chemotherapy was chosen. The girl received cycles of Adriamycin-DTIC-Endoxan-Vincristin and Platimit Endoxan-VP-16. After the chemotherapy had been applied a local finding firstly showed a regression with inproved of finding of skeleton scintigraphy "normalization" of catecholamine values. After the 10th cycle of chemotherapy a further worsening of the disease appeared with a lethal outcome.

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