Frank O. Bastian

Malignant Transformation in Craniopharyngioma

Malignant transformation in a craniopharyngioma has not been described previously. A 49-year-old woman presented with recurrence of a suprasellar craniopharyngioma diagnosed 35 years previously. The patient had been treated surgically for recurrence on five occasions. Radiation therapy had been administered 7 years before the final presentation. Tissue obtained from the fifth operation revealed malignant degeneration in a typical craniopharyngioma.

Myopathy in Marinesco-Sjogren Syndrome

Progressive muscular weakness, hypotonia and atrophy are among the cardinal signs of the Marinesco-Sjogren syndrome but have not been extensively investigated. Our study focused on 6 related patients who are members of an inbred population. Muscle biopsies revealed myopathic alterations with variation of fiber size, rounding, degeneration and regeneration of fibers, internalization of nuclei and endomysial fat and fibrosis. Most patients had elevated serum creatine kinase levels. One patient revealed endstage neuromuscular disease and had normal serum creatine kinase levels. Of particular interest was the finding of conspicuous myopathy in 2 young children. Thus far, it has not been appreciated that myopathy represents an early sign of the Marinesco-Sjogren syndrome.

Gliosarcoma developing from an irradiated ependymoma

Abstract A 17-year-old girl was operated for a cystic mass located deep within the left parieto-occipital white matter. Histologically the tumor was an ependymoma with a vascular stroma. In spite of irradiation the tumor recurred locally twice, 1 and 2 years respectively after the original operation. The ependymoma portion of the tumor remained unchanged, but the stroma showed increased vascular hyperplasia at the time of the second operation and transformation into a fibrosarcoma in the third operative specimen. Proliferating cell markers (MIB-1) were positive only in the ependymoma cell nuclei in the first two specimens, but were also extensively present in the nuclei of the fibrosarcoma cells in the third specimen. In the latter, the fibrosarcoma portion greatly overwhelmed the residual ependymoma islands, but remained sharply delineated from them. This is the first observed case of a gliosarcoma originating from an ependymoma. The histological pattern of this mixed tumor clearly indicates that the source of the sarcomatous portions was the neoplastically transformed fibrovascular stroma of the original tumor, rather than “desmoplastic” alterations of the neoplastic ependymal cells themselves.

Neurovirulence in an experimental focal herpes encephalitis: relationship to observed seizures.

An animal model of focal herpes simplex encephalitis was used to study several strains of type-1 herpes simplex virus. Rabbits were inoculated in the olfactory bulb by a standardized technique. Virus strains resulting in mortality of greater than 70% produced seizures of 3 types, and all animals that seized became moribund or died. In contrast, a virus strain resulting in a 20% mortality produced no seizures. Administration of 60 mg phenobarbital intramuscularly daily reduced mortality significantly in animals given the epileptogenic viruses. Cultures from temporal and frontal lobes showed viral growth more frequently than did cultures of other brain areas. Microscopic examination of routine and immunoperoxidase-stained brain sections confirmed the focal nature of the infection. Clinical syndromes such as seizures arising from viral brain disease may influence mortality in animal model systems.

Nonfulminant herpes simplex encephalitis as a cause for mesial temporal sclerosis

Although mesial temporal sclerosis has been recognized for more than 100 years, its etiology remains unknown. It is proposed that a common infectious agent, herpes simplex virus type-1, may cause this disorder by means of a nonfulminant infection of mesial temporal lobe structures, which is resolved by the immune system and becomes gliotic in the course of healing by the central nervous system. Brain sections from a long-term experiment in a model of herpes simplex encephalitis reveal such a scar, which shows a high concentration of glial fibrillary acidic protein, without any evidence of residual herpes antigen, by immunocytochemistry.

Polymorphic Genotype Matching in Acquired Creutzfeldt-Jakob Disease: An Analysis of Donor/Recipient Case Pairs

Two different expressed polymorphisms have been identified in the chomosome 20 (PRNP) gene encoding the amyloid precursor protein of infectious cerebral amyloidosis: codon 129 may specify either methionine or valine (at an allelic ratio of 0.62 to 0.38 in Caucasians), and a 24 base pair deletion may occur in the region of octapeptide coding repeats between codons 51 and 91 (at a frequency of about 2% in Caucasians). Although neither polymorphism is by itself pathogenic, codon 129 homozygosity is over-represented in both sporadic and iatrogenic forms of Creutzfeldt-Jakob disease (CJD) [1–4], and influences certain phenotypic features of sporadic and familial forms of disease [5–8], and octapeptide coding deletions can occur in association with CJD and other degenerative diseases [9, 10]. It therefore seemed of more than passing interest to learn whether polymorphic ‘matching’ could be demonstrated in those rare instances of proven or possible human-to-human contact infection in which tissue from identifiable donors and recipients was still available. We here report our findings in seven patients involved in three separate occurrences of surgically transmitted CJD, and in one conjugal pair in which both spouses died of CJD.

Backache, Unsteady Gait, Incontinence, and Large Thoracic Epidural Mass

Unsteady gait with frequent falls and urinary incontinence appeared over a period of a few days in a 6‐year‐old girl with a long history of backache. A large posterior epidural mass involved the lower thoracic vertebral canal and extended over several vertebral levels, suggesting a diagnosis of epidural hemorrhage with spinal cord compression. Examination of a surgical specimen led to a different diagnosis.