Spontaneous resolution of a pericardial cyst.
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Biomedical subjects
Publications and source records attributed to J Michaud.
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Several authors have recently reported a neurological disorder associated with chronic vitamin E deficiency in man. Except in one patient, this deficiency has always been secondary to an underlying disease resulting in lipid malabsorption. We report a second case of such a neurological syndrome in a patient in whom vitamin E deficiency was an isolated finding. The clinical picture in our patient was characterized by a diffuse muscle weakness most prominent distally and in the lower limbs, generalized areflexia, a decrease in proprioception and vibration sense and slight limb and gait ataxia. His condition improved on alpha tocopherol therapy so that it is very likely that vitamin E deficiency is responsible for his neurological deficit. Since in our patient as well as in several other reported cases this condition has been treatable, it is important that this syndrome be recognized in children presenting a suggestive clinical picture even if they do not have lipid malabsorption.
Bilateral retinoblastoma was diagnosed in a 12-month-old boy. The left eye was enucleated; the choroid and the optic nerve were not invaded by the tumour. The right eye had three lesions: one was photocoagulated, and the other two were irradiated. Two years later, symptoms and signs of intracranial hypertension prompted computerized tomography, which demonstrated a mass in the pineal region and secondary hydrocephalus. The right eye showed papilledema but no evidence of tumour activity in the three retinal scars. Radiation therapy and chemotherapy had only a transient effect, and the child died at 42 months of age. At autopsy the pineal region and the third ventricle were found to be replaced by a pinealoblastoma with retinomatous differentiation. Only 11 other cases of pinealoblastoma associated with bilateral (heritable) retinoblastoma have been reported. The embryologic relationship between the pineal gland and the retina has given rise to the concept of trilateral retinoblastoma, a concept important in the investigation and follow-up of patients with bilateral retinoblastoma.
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A 33-year-old man, an immunosuppressed renal allograft recipient, developed fever of unknown etiology, multiple pulmonary densities, space-occupying lesions in the liver, confusion, disorientation, and seizures with cerebral infarcts. At autopsy, changes classical of active lymphomatoid granulomatosis (LYG) were found in the brain but those studied in the lungs and liver were in the healing phase. In addition, one cerebral focus in the right temporo-occipital lobe showed a lymphomatous transformation. Immunofluorescence studies done on this focus showed monoclonal IgG-lambda cytoplasmic staining in the perivascular infiltrate. Immunoperoxidase studies confirmed the monoclonal nature of this focus but showed a polyclonal pattern in the other foci. This finding is discussed along with the relationship between LYG and lymphoma.
We report the case of a five-year-old child who had a hard painless left testis discovered on a routine physical examination. He underwent a left inguinal orchiectomy, and the pathology examination revealed a dermoid cyst of the testis. This is a rare tumor, considering the low incidence of testicular tumors in childhood. Histologic criteria and surgical management are also presented. We believe this report is the first well-documented case.
Ameloblastoma is a locally invasive, histologically nonmalignant tumor that may on very rare occasions give rise to metastases. A patient with a mandibular ameloblastoma presenting typical histologic appearances developed a pulmonary metastasis confirmed by histology as arising from the primary tumor. Two groups of these extremely rare malignant ameloblastomas can be distinguished: those without histologic signs of degeneration that give rise to metastases identical to the primary lesion, and those with degenerative signs provoking metastases with the histologic appearance of undifferentiated carcinoma. No relation appears to exist between size of primary tumor, histologic type of ameloblastoma, or its course and tendency to produce metastases.
A case of a rare form of giant ameloblastoma of the mandible is reported, and the lack of relationship between the size and malignancy of such tumors emphasized, as well as the value of using a modified Pons technique through an enlarged orobuccal approach.
We report herein eight cases of microcystic meningiomas. This unusual variant was originally described by Masson, who labeled it "humid." Grossly, tumors were usually soft, with occasionally a glistening cut surface. Two tumors were cystic and three invaded bone structures. By light microscopy, tumor cells were stellate, with long processes delimitating clear spaces. Whorls were found in all tumors. Intracytoplasmic glycogen, fatty droplets, and phosphotungstic acid-hematoxylin-positive fibrils were sometimes seen. Tumor cells, however, were all negative with the peroxidase-antiperoxidase technique for glial fibrillary acidic protein. Mitoses, hemorrhages, and necrosis were absent. By electron microscopy, interdigitations of cytoplasmic processes and desmosomes were found. The large extracellular spaces were filled with a finely granular substance intermingled with fibrils and collagenic fibers. The clinical and morphological findings point toward a benign course. We also discuss the differential diagnosis and the pathogenesis.
Agenesis of the cerebellar vermis with fusion of the dentate nuclei and cerebellar hemispheres (rhombencephalosynapsis) is a rare cerebral malformation. We report the case of a 7-h-old girl whose mother had taken the drug phencyclidine during the first 6 weeks of pregnancy. Absence of septum pellucidum, hypoplasia of the commissural system, optic nerves, chiasm and tracts, moderate hydrocephalus, and agenesis of the posterior lobe of the pituitary were also found. Extracranial congenital anomalies involved the cardiovascular, respiratory, urinary, and musculoskeletal systems. Death was secondary to severe respiratory distress and bradycardia. The literature concerning this rare form of cerebellar malformation is reviewed.
Two cases of acute haemorrhagic leucoencephalitis localised to the brainstem and cerebellum are reported. One followed the insertion of a ventriculoatrial shunt and the other an upper respiratory tract infection. The rare previously reported cases of this condition involving mainly the posterior fossa structures are reviewed.
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An 18-year-old girl died following a slowly progressive neurodegenerative disease of nine years duration. At 9 years of age, she developed intellectual deterioration associated with speech difficulty, pseudobulbar palsy and ataxia. The progression included spastic quadriplegia, anarthria, severe dysphagia, ophthalmoplegia, and pes cavus. There was no family history. The brain was uniformly small and the substantia nigra was not pigmented. Neuronal loss and gliosis involving globus pallidus, subthalamic nucleus, thalamic nuclei, brain stem, cerebellum, and spinal cord gave the picture of multisystem atrophy. Intranuclear hyaline inclusions were observed in numerous neurons of the central and peripheral nervous system. These were auto-fluorescent and were made up of intermingled straight filaments (8-9 nm in diameter). Only two previously reported cases showing these same inclusions are known. They are reviewed, compared, and discussed in relation to primary neuronal degenerations.
Permanent constrictions of the jaws are of various types depending on the site of the lesion: temporomaxillary ankylosis, extra-articular constrictions of bone, skin, muscle, or mucosal origin, and those arising from tumors. The commonest cause is injury, those due to infection being currently less frequently observed, which cannot be said for those of tumoral origin. The consequences are difficulty in taking foud and poor buccodental hygiene, while temporomaxillary ankylosis in children provokes mandibular growth disturbances. Surgical treatment is aimed at removing the constriction. Total resection of the ankylosed block is essential to avoid recurrences, while reeducation of buccal opening must be started early and continued for long periods in all cases.
A choroid plexus papilloma was found in the right lateral ventricle of a five-month-old boy. Though it was associated with an important communicating hydrocephalus, it was seen at operation to be freely mobile and did not directly obstruct the flow of the cerebrospinal fluid. Computerized axial tomography was the most helpful para-clinical investigation for diagnostic purposes, and for follow-up study. Tumor removal alone was sufficient to cure the hydrocephalus, and no shunt was necessary. We feel that this case demonstrates that hyperproduction of CSF is sufficient alone to cause communicating hydrocephalus in patients with choroid plexus papilloma of the lateral ventricle.
Papillomas of the choroid plexus arising in and occupying the cerebellopontine angle are rare. Such a case occurring in a 41-year-old woman is reported. The signs and symptoms are described. The literature is reviewed.
Pycnogenols is the term put forward by the authors to designate flavan-3-ol derivatives, so as to distinguish these substances, upon chemical and pharmacological grounds, from the heterogeneous group of flavonoid compounds.
This study was designed to measure the effects of contingent and noncontingent EMG feedback on hand temperature, anxiety, and locus of control. Two groups of six subjects each were selected on the basis of high test-anxiety scores. The groups participated in a reverse design study in which Group 1 received five sessions of contingent EMG ffedback followed by five sessions of noncontingent feedback. Group 2 received noncontingent feedback followed by contingent feedback. Results indicate a significant order of treatment effect. Subjects who received contingent feedback first produced lower EMG readings, lower test-anxiety scores, and higher hand temperatures during noncontingent feedback sessions. Receiving noncontingent feedback first may actually have interfered with utilizing contingent feedback.