Leigh syndrome in an infant: autopsy and histopathology findings.

Leigh syndrome is an inherited neurodegenerative disorder of infancy that typically manifests between 3 and 12 months of age. The common neurological manifestations are developmental delay or regression, progressive cognitive decline, dystonia, ataxia, brainstem dysfunction, epileptic seizures, and respiratory dysfunction. Although the disorder is clinically and genetically heterogeneous, the histopathological and radiological features characteristically show focal and bilaterally symmetrical, necrotic lesions in the basal ganglia and brainstem.

Cerebrospinal Fluid Spread in a Child with Pleomorphic Xanthoastrocytoma: Report with Cytopathologic Evidence.

Background: Pleomorphic xanthoastrocytoma (PXA), a relatively uncommon, World Health Organization grade II tumor of astrocytic origin, occurs in children and in general carries good prognosis. The recently identified anaplastic PXA (grade III) type shows poor outcome. Rarely, these said tumors can display an aggressive clinical course with features of leptomeningeal spread.

Spastic Quadriparesis and Communicating Hydrocephalus as Late Sequel of Rosai-Dorfman Disease: A Case Report and Review of Literature.

Rosai-Dorfman disease (RDD) predominantly affects cervical lymph nodes and presents with fever and pancytopenia. Central nervous system involvement though uncommon is often reported. Hydrocephalus and paraparesis as a consequence of RDD is an extremely rare entity.

Radiologic Dilemma in an Extra-Axial Petroclival Lesion: Low Grade versus High Grade.

Background: Radiologic differentiation between a low-grade and high-grade lesion is crucial when deciding extent of resection and prognostication. Occasionally, imaging features can be confusing between the 2 lesions. We report a case that presented such a radiologic dilemma.

Malignant cerebellar peduncle lesions - rapid progression and poor outcome.

Background: Tumors arising from cerebellar peduncle are extremely rare and behave aggressively. The inclusion of these into either cerebellar or brainstem gliomas is contentious.

Case Description: We performed clinicopathological review of three patients treated at our institute and surveyed the literature for previous such reported cases.

Spontaneous hemorrhages in pediatric supratentorial pilocytic astrocytomas. Malignant presentation of a benign entity.

Background: Hemorrhage as a presenting feature in pilocytic astrocytoma is an extremely rare phenomenon. When seen in children, most of such tumors exist in the cerebellum. Rarely, a supratentorial pilocytic astrocytoma can present with bleeding.