Alexia, without agraphia, due to brain tumor: a reversible syndrome.
Alexia without agraphia occurred in a 41-year-old man suffering from a left occipital brain tumor. The syndrome disappeared following excision of the neoplasm.
Biomedical subjects
Publications and source records attributed to Y Goldhammer.
Alexia without agraphia occurred in a 41-year-old man suffering from a left occipital brain tumor. The syndrome disappeared following excision of the neoplasm.
Severe dementia and ataxia in an elderly woman were related to hydrocephalus resulting from Paget disease of the skull, with basilar impression. Ventriculoatrial shunting resulted in dramatic improvement. This procedure, rather than suboccipital decompression, is probably the operation of choice, and should be carried out at an early stage.
The placing of a ventriculoatrial anastomosis because of elevated CSF pressure secondary to tuberculous meningitis resulted in repeated dissemination of Mycobacterium tuberculosis from the anastomosis. The consequent clinical picture showed recurrent appearance and clearing of miliary tuberculosis of the lung in spite of antituberculosis chemotherapy. While this possibility was considered early, the diagnosis was not established until the shunt was replaced shortly before the patient's death.
Electromyographic examination of vertical and lateral extraocular muscles was carried out in five patients suffering from progressive supranuclear palsy, and incapable of performing voluntary vertical eye movements. No evidence of a lower motor neurone lesion or paradoxical innervation of eye muscles was noted. Reciprocal inhibiton of antagonist vertical muscles though present in oculocephalic (doll's head) stimulation, was incomplete on attempted voluntary movement. This factor is held to be the probable immediate cause of the vertical gaze palsy.
Of 150 new cases of nasopharyngeal carcinoma diagnosed in Israel during a 9-year period (nationwide study 1960--1968), 74 patients developed neurological complications. In 92% of the cases, neurologic deficits were confined exclusively to cranial nerves. The majority of these patients presented general symptoms of disease such as neck masses, head and facial pain, or nasal and aural disorders. In 23 patients (34%), the initial presentation was of neurologic disability. The most frequently affected nerves were the abducens and the trigeminal. In the fully developed clinical picture, ocular and trigeminal lesions predominated. 5-year survival in patients with cranial nerve involvement was 21% as compared to 55% in those without such lesions. Survival was not influenced by delayed diagnosis.
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Two cases are described presenting a clinical picture of sudden headache, fever and vomiting, accompanied by unilateral ophthalmoplegia and acute deterioration of visual acuity. In one this amounted to monocular blindness with a field defect in the other, indicative of chiasmal compression. Necrotic pituitary adenoma material was removed by the transphenoidal route in both cases. Recovery of eye movements was obtained and considerable improvement of visual acuity, which in one patient, was restored completely.
Basal encephalocele should be suspected in child when hypertelorism, defect of the bridge of the nose, and other midfacial anomalies are present. Optic nerve anomalies may occur with basal encephalocele. Those previously described are pale discs, coloboma of the optic nerve head, and pit of the optic disc. Two additional disc anomalies that are associated with basal encephalocele are megalopapilla and optic nerve dysplasia. A mass in the nose or nasopharynx under these circumstances is most likely an encephalocele and biopsy is contraindicated.
A 2,144-g white girl was born with absence of the right ear and eye, cleft lip and palate, two basal encephaloceles, tricuspid atresia, ventricualr and atrial septal defects, detransposition of the great vessels, right aortic arch, and aberrant right subclavian artery. Through an oval defect in the center of the sphenoid bone, soft tissue protruded into the right nasopharynx. The medial portions of the roof of both orbits and the cribriform plate were absent and soft tissue protruded through this bony defect. Basal tomography was required to demonstrate the encephaloceles, which should be suspected in any child with a median cleft syndrome, a flat broad nasal root, and hypertelorism.
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