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Biomedical subjects

N Heldt

Publications and source records attributed to N Heldt.

At least 19 recordsLinked to original sources

Malignant change in an intradiploic epidermoid cyst.

We describe a unique case of an intra-diploic epidermoid cyst of the parietal and occipital right bone in a 43 year-old man. The tumor remained quiescent for 12 years and then spontaneously showed malignant changes with intra-cerebral involvement. Fatal outcome occurred one year after surgery, radiation therapy and chemotherapy because of a recurrence. Malignant transformation of the epithelium in epidermoid cyst is very rare. Our case is the first one where this transformation occurred spontaneously without previous surgery.

Adult↗

Low grade supratentorial astrocytomas. Management and prognostic factors.

BACKGROUND: The authors examined the principal prognostic factors for supratentorial low grade astrocytomas and the effects of treatment on survival time. METHODS: Fifty-eight patients, 32 males and 26 females, treated from January 1970 through December 1990 are reported. Thirty-five patients underwent surgery, of whom three died. Among the survivors there were 7 gross total resections, 13 subtotal resections, and 12 partial resections. Of the 23 remaining patients, diagnosis was obtained by stereotactic biopsy. Twenty-five patients were irradiated and eight received chemotherapy. The histologic material, originally graded 1 or 2 according to Kernohan's system, was reviewed using the St. Anne-Mayo Clinic system, with which there were 18 Grade 3 tumors. RESULTS: Six patients had pilocytic astrocytomas (mean age, 18 years), of which three astrocytomas could be removed, and the patients were well 128, 152, and 254 months, respectively, after diagnosis. Among the other three patients, one with a hypothalamic localization refused any form of treatment and died rapidly. The remaining two patients also had deep-seated tumors and were irradiated: one died 17 months later, and the other remained relapse free 56 months after diagnosis. The 49 patients with ordinary astrocytomas who survived surgery (mean age, 37 years) had a median survival time of 64 months and 5- and 10-year survival rates of 54.6% and 34.1%, respectively. Patients with Grade 1 or 2 tumors had a median survival time of 108.6 months, compared with 39.4 months for patients with Grade 3 tumors. Gross total and subtotal resections were significantly associated with longer survival time only in patients with Grade 1 and 2 tumors: in this group, the 5- and 10-year survival rates were 87.5% and 68.2%, respectively, when tumor was completely resected, instead of 57% and 31.2% in patients with incomplete or no surgical resection. There was a trend suggesting that antimitotic drugs and X-ray therapy could benefit patients with Grade 3 tumors. CONCLUSION: The importance of an accurate histologic grading system and a gross total resection of Grade 1 and 2 astrocytomas is emphasized.

Actuarial Analysis↗

[Cerebral amyloid angiopathy apropos of a case. Review of the literature].

The authors present the case of an 83 year-old man with cerebral amyloid angiopathy who had three successive lobar intracerebral hematomas within 4 weeks. There were neither a predisposing familial factor nor cardiovascular risk factors nor any other cause favouring cerebral bleeding. The autopsy revealed amyloid deposits in small cortical and meningeal arteries. No other lesions of the Alzheimer's disease was found.

Aged↗

Immunogold light and electron microscopic detection of amyloid plaques in transmissible spongiform encephalopathies.

The antigenicity of the 'prion' protein amyloid fibrils was shown to be preserved after glutaraldehyde/OsO4 fixation in uranyl acetate-stained brain tissue blocks from patients with Gerstmann-Sträussler syndrome (GSS) and from mice infected with Creutzfeldt-Jakob disease (CJD). Amyloid plaques were demonstrated by light microscopy in immunogold silver-intensified semithin sections. Under the electron microscope, the amyloid fibrils were labelled in immunogold-reacted ultrathin sections using an antiserum prepared against GSS amyloid plaque cores and mouse amyloid fibrils respectively. The influence of various oxidizing agents (hydrogen peroxide, sodium metaperiodate) on the tissue preservation and the immunohistochemical detection was tested.

Amyloidosis↗

Immunochemical, molecular genetic, and transmission studies on a case of Gerstmann-Sträussler-Scheinker syndrome.

Using immunostaining with anti-prion protein (PrP) antiserum, we detected numerous kuru plaques in the brain of a 24-year-old man with Gerstmann-Sträussler-Scheinker syndrome. Immunoreactivity on Western blotting of the protease-resistant PrP fraction from the frozen brain was weak. PrP gene analysis showed substitution of alanine to valine in codon 117 but no substitution in codon 102. As the experimental transmission of the disease to mice was negative, a pathogen of a relatively low infectivity may cause the disease in predisposed family members.

Adult↗

[Bilateral meningioma of the optic nerve. Discussion apropos of a case].

The authors report a case of a 14 years old girl with a bilateral optic neuropathy especially an optic atrophy on the left eye. They show the clinical and radiological diagnostic difficulty of these symptoms before coming to the bilateral optic nerve meningioma. The authors have compared this rare case to those described in literature and insist on the importance of the CT Scan and eventually the nerve surgical exploration.

Adolescent↗

CT findings in malignant meningiomas.

Recurrent meningiomas are due usually to incomplete removal as in the case of basal meningiomas where the tumour surrounding vessels and nervous structures cannot be completely excised. Recurrent meningiomas of the convexity are rare after resection. In most recurrent cases histological changes are noted which may explain the rapid growth, the aggressive nature and also the malignancy of the tumour. These changes include increased mitotic activity, necrosis and invasion of the adjacent brain parenchyma. CT findings in malignant meningiomas are related to the microscopic appearance of these tumours: tumoral necrosis determines heterogeneous enhancement; brain invasion explains the irregular outline of the tumour and sometimes the absence of surrounding low attenuation area. The authors report 5 malignant meningiomas in which the CT findings are correlated with the microscopic findings.

Aged↗

[Familial presenile dementia: Gerstmann-Sträussler-Scheinker's syndrome (author's transl)].

A similar affection has developed in eight members from four generations of a family living in the Alsace. The disease is characterized by the onset of a pyramidal, pseudobulbar syndrome and dementia during the third or fourth decade of life. The outcome is fatal after a mean period of three years. Cerebral biopsies in three cases have demonstrated multicentric amyloid plaques differing from senile plaques. Clinical and pathological findings are similar to those currently reported in the literature as being typical of Gerstmann-Sträussler-Scheinker's syndrome. The affection appears as a separate entity: the multicentric plaques, clinical symptomatology, pyramidal or pseudobulbar, cerebellar syndromes, usually preceding dementia, age of onset, course, and familial character or the disorder distinguish it among presenile dementias. Its clinical profile and course are very similar to that of familial cases of Alzheimer's disease, some of which are probably cases of Gerstmann-Strässler-Scheinker's syndrome. Transmission to animals, though inconstant, places it within the group of transmissible dementias among kuru, Creutzfeldt-Jakob's, and familial forms of Alzheimer's disease. The familial nature of the affection and the variability of clinical and pathological features in the same family illustrate the complex relationships between hosts and pathogenic agents in the clinicopathological expression of a disease.

Adult↗

Frontal horn coarctation: CT demonstration. A report of two cases.

The CT aspects of frontal horn coarctation by ependymal fusion in two cases are described with post-mortem verification. These ependymal fusions in the lateral ventricles are seen frequently on air ventriculograms, especially in the occipital horns. Ependymal fusions in the frontal horns are rare and not very well known. These fusions are clearly seen on CT and post-mortem examinations. The frontal horns appear asymmetrical on CT and this asymmetry evokes frontal horn compression (by an isodense tumour for instance), or contralateral frontal horn dilatation. Some CT details of ventricular coaractation makes it easy to differentiate tumoural compression from controlateral subcortical atrophy.

Aged↗

Angiographic changes in a case of herpes simplex encephalitis.

A case of herpes encephalitis predominating in the right temporal lobe with unusual angiographic changes is reported. Carotid angiography revealed a right temporal mass with vascular blush and early venous filling through irregular veins. Early venous filling and vascular blush have been known for a long time with cerebral inflammatory disease, but venous drainage through irregular veins is unusual.

Aged↗

[Cerebral tumors of primitive germinal origin].

The authors attempt to justify the term cerebral tumour of primitive germinal origin from four of their own cases and a review of the literature. They emphasise the specific features. The tumours are more common in boys and involve the pineal, the walls of the third ventricle, the hypothalamus and the posterior pituitary. Several histological types may be distinguished by the degree of differentiation but the stromal reaction, which is partly responsible for the symptoms, is always present. The clinical course of the illness is biphasic. The first is manifest by endocrine disorders and is of relatively long duration. Water homeostasis is always affected and may be associated with other hypothalamic disorders. In the second phase, neurological symptoms and raised intracranial pressure appear. Surgical removal is not always possible, but radiotherapy improves the outlook.

Adenoma↗