Polyneuritis cranialis as a presentation of malignant lymphoma.

Two cases of polyneuritis cranialis resulting from infiltration of nerve roots by malignant lymphoma are described, and 8 previously reported cases discussed. In these, cranial nerve involvement was the presenting feature of lymphoma, and lesions at other sites were inconspicuous until necropsy. In one patient infectious mononucleosis developed concurrently with the neurological deficit. The course of this condition is progressive and fatal.

Myelin composition in acute and chronic multiple sclerosis in relation to cerebral lysosomal activity.

The neuropathology of 3 cases of acute multiple sclerosis was correlated with biochemical analyses. Astrocytosis was a characteristic feature of the diffuse demyelinating lesions in one case and lymphocytic cuffing characterized the well-defined plaques present in the white matter of the other two cases. No abnormalities were found in the protein or lipid composition of isolated myelin, despite a wide range of recovery. Nevertheless, the gel electrophoretic protein pattern of white matter adjacent to plaque areas showed selective loss of myelin basic protein. Lysosomal acid proteinase and beta-glucuronidase levels were very significantly increased in all white matter samples in which astrocytosis was a major neuropathological feature. Levels were less markedly raised in samples containing discrete active plaques. Enzyme changes were also found in the apparently normal white matter of 2 of the cases. Acid proteinase activity was in the normal range but the activities of beta-glucuronidase and acetylcholine esterase were elevated. The significance of these results in relation to glial cell activity in the early stages of demyelination is discussed.

Klinefelter's syndrome, cerebral germinoma, Chiari malformation, and syrinx: a case report.

A case of Klinefelter's syndrome associated with intracranial germinoma and Chiari malformation is described. The case is discussed in view of the literature linking Klinefelter's syndrome with skeletal defects and germinoma.

Metastasis of carcinoma to hemangioblastoma cerebelli: case report.

A patient with a long-standing posterior fossa tumor developed symptoms that it was growing rapidly. The increasing growth resulted from secondary prostatic carcinoma complicating the cystic cerebellar hemangioblastoma.

Malignant behavior of myxopapillary ependymoma. Report of three cases.

From a series of 66 cases of central nervous system ependymomas, three cases of well differentiated ependymoma of the cauda equina are described. These tumors recurred and spread to produce intracranial deposits. No extraneural lesions were found.

Cerebellar desmoplastic medulloblastomas. A further immunohistochemical characterization of the reticulin-free pale islands.

We studied by immunohistochemistry the features of differentiation in 24 desmoplastic and 16 classic medulloblastomas (median patient ages, 18 and 6.5 years, respectively) with the use of a panel of cytoskeletal and synaptosomal markers. A distinctive pattern of immunoreactivity with a series of monoclonal antibodies (Mabs) was documented in the polar tumor cells forming the reticulin-free pale islands of the desmoplastic variant, denoting overt neuritogenesis. These comprised the following: (1) Mab Tp-NFP1A3 recognizing an epitope in the high-molecular-weight (Mr) isoform of neurofilament protein; (2) Mab AP18 to the high-Mr microtubule-associated protein 2; (3) Mab TUJ1 recognizing the class III beta-tubulin isotype (human h beta 4); and (4) Mab SY38 to synaptophysin. Immunoblot analysis confirmed the expression of h beta 4 in three medulloblastomas, yielding strong single bands in two desmoplastic medulloblastomas and a considerably weaker band in one classic medulloblastoma. Glial fibrillary acidic protein-positive tumor cells frequently formed an integral component of the pale islands. Oligodendrogliallike areas in one classic and in three desmoplastic medulloblastomas were immunopositive for the Mabs to synaptophysin, microtubule-associated protein 2, and h beta 4, indicating a neuroblastic nature. We propose that the reticulin-free structures of desmoplastic medulloblastomas constitute neoplastic foci with features of predominantly neuronal and, to a lesser degree, astroglial differentiation.

The incidence of multifocal cerebral gliomas. A histologic study of large hemisphere sections.

A series of 241 gliomas (astrocytomas, oligodendrogliomas, glioblastomas, and subependymal giant-cell astrocytomas) was studied. This represents all the gliomas examined post mortem over 25 years at one hospital. Two hundred and one cases (85%) were apparently solitary tumors; of the 40 cases with multiple tumor foci, 23 (9.5%) were true multicentric gliomas. After excluding cases in which there was concomitant disease (neurofibromatosis, tuberose sclerosis, or multiple sclerosis), 18 cases of multicentric tumor (7.5%) remained. Multicentric tumors with different histologic appearances accounted for 2.9% of the series. Celloidin-embedded whole brain sections proved invaluable for the detection of microscopic neoplastic foci and unsuspected diffuse spread. The estimated incidence of multiplicity in gliomas is higher than in most series, but the findings suggest that detection of multifocal neoplastic change in these tumors is directly related to the extent to which the brain is sampled, and that figures obtained in this study may well underestimate the true incidence.

Astrocytic differentiation in medulloblastoma.

A case of cerebellar medulloblastoma with transitional features towards malignant fibrillary astrocytoma is described. In the cerebellum the tumour is characterised by extensive subpial infiltration with cells of undifferentiated type, and the astrocytic component could only be identified by a positive glial fibrillary acidic protein reaction. In the brainstem the character of the growth transforms to that of diffuse astrocytoma. This demonstrates the potential for differentiation existing in a "primitive" neuroepithelial neoplasm.

A note on the nature of eosinophilic granular bodies in astrocytic gliomas.

Eosinophilic granular bodies are a characteristic microscopical finding in certain gliomas of astrocytic type. The nature of these structures has been the subject of debate. The finding of a positive reaction for glial fibrillary acidic protein confirms the astrocytic nature of this material.

Corpus callosum in multiple sclerosis.

A neuropathological study of 20 multiple sclerosis brains using celloidin-embedded slices was carried out to assess the extent of changes in the corpus callosum. Severe atrophy of the callosum was found in cases with marked hydrocephalus. Demyelination of the callosum varied in extent from slight involvement (with a few small plaques) to almost total myelin loss. A clinical history of mental deterioration was usual in the cases with severe callosal lesions, but no symptoms were recorded that indicated a specific disconnection syndrome. The ventricular enlargement noted in this series could not be explained either on the basis of obstruction to the flow of cerebrospinal fluid, or by the effects of shrinkage of the white matter.

Perivascular lymphocytic cuffing in astrocytomas.

A series of cases of astrocytoma of differing cell composition and degree of malignancy was examined for the presence of reactive perivascular cuffing. Only surgical biopsy material was used. A correlation was made between perivascular lymphocyte cuffing and the presence of neoplastic astrocytes of the gemistocytic type.

Patterns of proliferation in cerebral lymphoreticular tumours.

A study of eighteen cases of primary lymphoreticular tumours in the brain is described. In four of these there were extraneural lesions and in one macroglobulinaema. The use of whole brain sections embedded in celloidin, and of metallic impregnation methods, revealed certain constant patterns of proliferation. The tumours were diffuse and multicentric; the leptomeninges and perivascular spaces especially in the subependymal regions were frequently involved. Mature microglia were active both in infiltrated and in apparently tumour-free regions.

Cerebellar syndrome in myxoedema revisited: a published case with carcinomatosis and multiple system atrophy at necropsy.

One of six patients in a 1960 paper on "Cerebellar syndrome in myxoedema" was subsequently found to have adenocarcinoma. General post-mortem revealed carcinomatosis and basal pneumonia. Neuropathological examination revealed the changes of multiple system atrophy. The relationship between hypothyroidism, carcinoma, and cerebellar, pontine and striatonigral degeneration is discussed.

Aspergillosis of the nervous system. Report of two cases.

Two cases of Aspergillus infection of the nervous system are reported. In the first case it occurred in a healthy individual and presented as a space occupying lesion mimicking a tuberculous granuloma. In the second case it was an opportunistic infection in a patient with predisposing conditions and manifested as chronic meningitis with fatal arteritis.