Polyglucosan bodies in brain tissue: a systematic study.

The relation between age, sex and presence of polyglucosan bodies in the brain has been systematically studied in 64 patients who did not suffer from neurological brain disorders and in 2 cases with Lafora's disease. In the normal brain the number of polyglucosan bodies is related to increasing age. Under the age of 40, polyglucosan bodies can be found sporadically in cases without a neurological disorder, but in the cases of Lafora's disease their number is uncomparatively higher.

X-linked ataxia, weakness, deafness, and loss of vision in early childhood with a fatal course.

An X-linked recessive disease with, in almost all patients, a fatal course in early childhood, occurring in a five-generation family is described. The 12 affected boys had early-onset floppiness, ataxia, liability to infections especially of the upper respiratory tract, deafness, and later, a flaccid tetraplegia and areflexia. Eleven boys died before the age of 5 years.

Histologic factors in the grading and prognosis of astrocytoma grade I-IV.

The prognostic value of histologic or cytologic features were examined in 317 patients with an astrocytic glioma. Of 46 features examined 7 morphologic characteristics of nucleus and 5 of mesenchyma correlated well with grading according to Kernohan. The morphological characteristics of nucleus are cellularity, atypical nuclei, polymorphism, multinucleated cells, hyperchromasia, gigantic nuclei and mitosis.

Long-lasting epidural sensory blockade by n-butyl-p-aminobenzoate in the terminally ill intractable cancer pain patient.

An aqueous suspension of n-butyl-p-aminobenzoate (BAB), a highly lipid-soluble congener of benzocaine, was applied epidurally in terminally ill cancer patients with intractable pain. The suspension consisted of 10% BAB and 0.025% of the nonionic surfactant polysorbate 80 in 0.

[Postoperative irradiation in grade III and IV astrocytoma].

The aim of this paper is to contribute to the discussion on the value of postoperative radiotherapy in patients with astrocytoma grade III-IV. One hundred and fifteen of the 243 patients with histologically proven astrocytoma grade III-IV received postoperative radiotherapy at the St. Radboud University Hospital Nijmegen.

Intermediate filament proteins in spindle cell carcinoma of the larynx and tongue.

In an analysis of intermediate filament protein expression of spindle cell carcinoma, a variant of squamous cell carcinoma, occurring in larynx and tongue, vimentin positivity was found in sarcomatoid areas in 12 of 13 patients. Scattered expression of keratin was observed in sarcomatoid areas in tumours of 10 patients. However, large parts of sarcomatoid areas of such tumours were negative for keratin.

Myelopathy due to cervical spine rheumatoid arthritis. A case report.

A 62-year-old woman with cervical myelopathy due to cervical spine rheumatoid arthritis is presented. Transoral decompression of the spinal cord, followed by posterior spine fusion in the same session was performed with good results. Unfortunately she died 3 months later due to cardiovascular disease.

[An unusual cause of subcutaneous postsacral swelling].

A subcutaneous sacrococcygeal located myxopapillary ependymoma is a rare presentation of this malignancy. It has a tendency to metastasize, even after a long latent period. This is a report of such a case together with proposals for treatment and follow-up.

Leigh syndrome, a mitochondrial encephalo(myo)pathy. A review of the literature.

Results of a literature survey of 173 patients with Leigh syndrome are presented, with emphasis on signs and symptoms in relation to age at onset, contributions of technical investigations to the diagnosis, pathophysiology, genetic considerations and therapeutic aspects. Based on this study we are of the opinion that it is possible to come to a diagnosis of "most probable Leigh syndrome" durante vitamin on the combination of clinical signs and symptoms, autosomal recessive mode of inheritance, association with a defect of energy metabolism, and CT or MRI abnormalities.

Lafora disease: a quantitative morphological and biochemical study of the cerebral cortex.

Morphological and biochemical studies were performed on a brain biopsy from a patient with typical Lafora disease. Qualitative morphological investigation of the cortex showed that the Lafora bodies were most abundant in layers III and V of the cerebral cortex. They were exclusively located in the neurons and their processes.

Malignant fibrous histiocytoma of the tongue.

Malignant fibrous histiocytoma (MFH) can occur as a rare mesenchymal neoplasm of the deep structures of the head and neck region. An unusual case of MFH of the tongue is described in a 61-year-old male. The primary tumour measured 90 X 50 X 30 mm.

Lafora's disease. Comparison of inclusion bodies in skin and in brain.

A patient had the clinical and neuropathologic signs of Lafora's disease. Skin biopsy specimens from the midcalf area confirmed earlier findings by showing numerous periodic acid-Schiff-positive inclusion bodies in eccrine sweat gland duct cells. In our patient, however, inclusion bodies were more abundantly present in the apocrine sweat gland duct cells of the axilla skin.

Co-expression of glial fibrillary acidic protein- and vimentin-type intermediate filaments in human astrocytomas.

The expression of intermediate filament (IF) proteins was studied in 71 cases of malignant human astrocytoma and in 17 cases of reactive gliosis, using immunocytochemical techniques with polyclonal and monoclonal antibodies to glial fibrillary acidic protein (GFAP) and vimentin. In all cases of astrocytoma, varying in degree of malignancy from grade I to grade IV, co-expression of GFAP and vimentin was found. No change in vimentin- or GFAP-IF expression with increasing anaplasia was seen.

Completely thrombosed giant aneurysm of the basilar artery trunk: a case report.

A patient with a completely thrombosed giant aneurysm arising from the trunk of the basilar artery is described. Although it is difficult to differentiate this anomaly from a posterior fossa tumor, negative angiographic findings combined with certain computed tomographic (CT) signs may point to the correct diagnosis. Our case demonstrates that one of these CT signs (viz.

[Neonatal meningitis, ventriculitis, intraventricular therapy or not?].

Neonatal meningitis still remains a disease with high mortality in spite of systemic treatment with in vitro active antibiotics. Of 42 consecutive cases, the fatality rate was 36%; Among the 27 survivors, there were 7 patients with extensive neurological and psychological impairment. The pathogenesis of the ventriculitis is discussed on the basis of the histomorphological structure of the ependyma, the subependymal tissue and the glycogen-rich choroid plexus within the first few weeks after birth.

Subacute necrotizing encephalomyelopathy (Leigh syndrome) associated with disturbed oxidation of pyruvate, malate and 2-oxoglutarate in muscle and liver.

We studied a 17-year-old girl with subacute necrotizing encephalomyelopathy (Leigh syndrome). Lactate and pyruvate levels were increased in serum and cerebrospinal fluid. The oxidation rates of all substrates tested, i.

Carcinoid of the larynx: a report of three cases and a review of the literature.

Carcinoid tumors are among the exceptional neoplasms of the larynx. The laryngeal carcinoid was first described in 1969 by Goldman, et al. Only 16 cases could be traced from the literature.

Computed tomography in the diagnosis of Wernicke's encephalopathy: a radiological-neuropathological correlation.

A case of Wernicke's encephalopathy with pathological verification is reported. Results of computed tomography scanning performed one day after admission of the patient, a male alcoholic, strongly suggested Wernicke's encephalopathy and were in agreement with the subsequent postmortem findings.

Defects in citric acid cycle and the electron transport chain in progressive poliodystrophy.

We will present 8 children with progressive infantile or juvenile poliodystrophy (Alpers' disease), associated with a defect in pyruvate metabolism. Laboratory studies showed elevated levels of lactate in CSF and, in 4 children, elevated levels in serum. Histopathologic studies revealed lipid storage in liver and/or muscle tissue, sometimes myopathy with abnormal mitochondria and slight axonal degeneration in the peripheral nerve.

Malignant tumours of the nasopharynx: retrospective review of 74 cases.

Seventy-four patients with histologically confirmed malignant tumours of the nasopharynx were treated by the Departments of ENT and Radiotherapy of the Sint Radboud Academic Hospital, Nijmegen, The Netherlands. Selected prognostic factors were examined. The 5-year overall survival for the malignant epithelial tumours was 35%.

Ultrasound detection of congenital arteriovenous aneurysm of the great cerebral vein of Galen.

Using combined echoencephalography and Doppler flow determination the diagnosis Arteriovenous aneurysm of the great cerebral vein of Galen could be made in two infants. Without vascular surgery one patient died, the other recovered completely. CT scanning confirmed the diagnosis.

Progressive infantile poliodystrophy (Alpers' disease) with a defect in citric acid cycle activity in liver and fibroblasts.

We present the case history of a boy, who died at the age of 3 1/2 years after a rapidly progressive neurologic disorder, characterized by psychomotor retardation, hypotonia, hemiparesis, seizures and myoclonic contractions. Histopathologic studies showed slight lipid storage in liver. Autopsy showed the characteristic features of progressive infantile poliodystrophy (Alpers' disease); ultrastructural examination showed an increased density of mitochondria in cerebral gray matter.

Familial lissencephaly with extreme neopallial hypoplasia.

Two siblings, male and female, with identical lethal brain malformation are described. Their anomaly is characterized by very low brain weight, lissencephaly, wide ventricles and thin neopallium (colpocephaly) varying in thickness between 0.2 and 3 mm.