We describe two patients and a previously reported patient who acquired unique pendular vergence oscillations of the eyes and concurrent contractions of the masticatory muscles, i.e., oculomasticatory myorhythmia (OMM). The smooth disjunctive eye movements cycled with a frequency of 0.8 to 1.2 Hz. An analysis of peak velocities (15 to 200 degrees/sec) with respect to peak amplitudes (5 to 25 degrees) revealed dynamics characteristic of normal vergence movements. The pathological alterations resulting in pendular vergence oscillations implicate a separately functioning, physiologically normal vergence system within the brainstem. In addition to paralysis of vertical gaze, each patient also experienced progressive somnolence and intellectual deterioration. An intestinal biopsy in 1 patient established a diagnosis of Whipple's disease, which led to appropriate treatment and amelioration of the OMM. A pathological diagnosis of Whipple's disease of the central nervous system was made in the other 2 patients; results of an intestinal biopsy in one of these patients were normal. No patient had palatal myoclonus, and olivary pseudohypertrophy was not found in two autopsy examinations. Thus, OMM is a distinct movement disorder and has been recognized only in Whipple's disease. We conclude that patients with OMM should be treated presumptively for Whipple's disease of the central nervous system, even if a jejunal biopsy is normal.
The presence and distribution of glial fibrillary acidic protein in fixed, paraffin embedded tissue were studied in 85 human intracranial neoplasms, using the peroxidase-anti-peroxidase method. In some cases, indirect immunofluorescence of frozen sections was used as well. In normal tissue, only the cell processes and perikarya of fibrous astrocytes were stained. Immunostaining was also observed in the following glial neoplasms: astrocytomas (all varieties), astroblastoma, subependymal giant cell astrocytoma, subependymoma, glioblastoma multiforme and ependymoma. The astrocytic elements of mixed gliomas and of medulloblastomas undergoing glial differentiation were likewise strongly stained. In contrast, oligodendrogliomas, meningiomas, pituitary adenomas, sarcomas, lymphomas and metastatic carcinomas were negative. Either a perikaryal or a diffuse fibrillary staining pattern was observed. Combination of the two patterns occasionally occurred. The perikaryal staining was prominent in gemistocytic astrocytomas and in astroblastomas. A distinct negative correlation existed between the degree of anaplasia and the intensity of immunostaining.
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