Slobodan Aleksic

Melanotic ependymoma and subependymoma

We report two examples of melanin production by human gliomas. One was a grossly pigmented, well-differentiated ependymoma resected from the left frontoparietal region of a 13-year-old girl. The patient received radiotherapy and was free of tumor 12 years after operation. The second example was a pigmented subependymoma incidentally discovered at the autopsy of a 52-year-old man. Neoplastic cells containing an intracytoplasmic pigment satisfying histochemical criteria for melanin were present in both cases. Electron microscopic study of the melanotic ependymoma revealed electron-dense granules in the cytoplasm of cells forming rosettes. Premelanosomes were not detected. While the mechanism of melanogenesis in these cases is obscure, they support the potential of glial derivatives to produce melanin and indicate that melanogenesis in such neoplasms may have no adverse prognostic import.

Retinall and Optic Nerve Findings in Goldenhar-Gorlin Syndrome

Involvement of the posterior segment of the eye in Goldenhar-Gorlin syndrome is more common than is generally appreciated. We examined seven patients with this syndrome. Abnormalities included diminished visual acuity, tilted optic disc, optic nerve hypoplasia, tortuous retinal vessels, macular hypoplasia and heterotopia, microphthalmia and anophthalmia. In one case, pathologic study showed agenesis of the optic nerve. It is proposed that retinal, optic nerve and craniofacial abnormalities in this condition may reflect an asynchrony in the migration of the neural crest cells in the early stages of embryonal development.

Intracranial Lipomas, Hydrocephalus and Other CNS Anomalies in Oculoauriculo-Vertebral Dysplasia (Goldenhar-Gorlin Syndrome)

13 cases of Goldenhar-Gorlin syndrome are presented in which numerous central nervous system anomalies have been found. These include occipital encephalocele, hydrocephalus, aqueductal stenosis, agenesis of corpus callosum, multiple congenital lipomas and many others. Pertinent literature has been reviewed. It is concluded that any part of the central nervous system can be involved in this condition and that careful evaluation is indicated in order to rule out a treatable intracranial anomaly.

Herpes zoster oticus and facial paralysis (Ramsay Hunt syndrome)

Abstract Two patients with herpes zoster oticus and facial paralysis are presented, in 1 of whom the geniculate ganglion showed at autopsy unequivocal pathological changes consistent with previous herpetic inflammation. The other patient showed a large area of sensory loss to pain and touch in the concha and external acoustic meatus during recovery from the acute episode. The clinical and pathological features are discussed in the light of a review of the literature. We conclude that the term “Ramsay Hunt syndrome” should be used only for cases of herpes oticus with facial paralysis and that herpetic geniculate ganglionitis and facial neuritis represent its anatomic substrate.

Coincidental pituitary adenoma and cerebral aneurysm with pathological findings.

Abstract A rare case of pituitary adenoma associated with an intrasellar cerebral aneurysm is presented with autopsy findings. The pertinent literature is reviewed.

Supraophthalmic intracarotid infusion of BCNU for malignant glioma.

We treated five patients with 11 supraophthalmic infusions of BCNU at 200 mg/m2 every 2 months. All three patients with residual tumors showed marked CT response after one infusion. Two patients with bilateral tumors had no response on the contralateral side. All four evaluable cases showed evidence of BCNU neurotoxicity. CT findings superficially resembled tumor recurrence, but white matter changes, nonspecific gyral enhancement, and delayed calcification were more indicative of neurotoxicity. There were no procedure-related complications. One autopsy suggested that direct parenchymal damage might be responsible for delayed neurotoxicity. Supraophthalmic BCNU infusion, at this dosage, is too toxic for cerebral tissue.

Congenital ophthalmoplegia in oculoauriculovertebral dysplasia‐hemifacial microsomia (Goldenhar‐Gorlin syndrome) A clinicopathologic study and review of the literature

Two patients with Goldenhar-Gorlin syndrome showed paralysis of one or more extraocular eye movements on neurologic examination. At autopsy, a third patient showed unilateral agenesis of trochlear and abducens nerves and corresponding brain stem nuclei. Congenital ophthalmoplegia is not infrequent in Goldenhar-Gorlin syndrome and may be due to hypoplasia or agenesis, or both, of extraocular muscles, extraocular nerves, and brain stem nuclei.

Pure motor hemiplegia with occlusion of the extracranial carotid artery

Abstract Two patients with the syndrome of pure motor hemiplegia are presented in whom occlusion of the carotid artery in the neck was demonstrated angiographically. A relationship between extracranial vascular disease and pure motor hemiplegia is suggested and potential implications in the management and treatment of such patients are discussed.

Cerebellocele and Associated Central Nervous System Anomalies in the Meckel Syndrome

Three cases with the Meckel syndrome were autopsied and found to have: arhinencephaly , polymicrogyria , aqueductal stenosis, heterotopia of glial tissue, hypoplasia or agenesis of the cerebellar vermis, cranium bifidum associated with large occipital ventriculocele and others. The anomalies at the level of posterior fossa in this condition are classified as those belonging to the Chiari type III group of anomalies. This unusual set of anomalies which forms pathogenetic link between the Dandy-Walker and Chiari-Arnold group of anomalies in the posterior fossa seems to be very frequent in the Meckel syndrome. The therapeutic emphasis is on genetic counseling in view of the recessive inheritance of the syndrome.

Intractable facial pain associated with a ganglioglioma of the cervicomedullary junction: report of a case.

A 6-year-old child with a brain stem tumor presented with the unusual complaint of intractable facial pain resembling trigeminal neuralgia in the absence of other symptoms or signs referable to the 5th cranial nerve. The radiological evaluation included a computed tomographic scan with intravenous contrast administration, which demonstrated an enhancing intramedullary lesion extending from the obex to C-4. After radiation and chemotherapy had failed to achieve symptomatic relief, the tumor, later proven to be a ganglioglioma, was radically removed with the ultrasonic aspirator. Postoperatively the patient experienced full pain relief.