Dysembryoplastic neuroepithelial tumors: nonspecific histological forms -- a study of 40 cases.

OBJECTIVE: To demonstrate that DNTs include a large morphological spectrum of tumors that cannot be histologically distinguished from conventional categories of gliomas. METHODS: All tumors from patients who underwent epilepsy surgery in Sainte-Anne hospital (Paris) that histologically resembled gliomas and did not conform to current histological criteria for DNTs or gangliogliomas were entered in the study. RESULTS: According to the WHO histological classification, the 40 tumors resembled: pilocytic astrocytomas (4 cases), astrocytomas (16 cases), anaplastic astrocytoma (1 case), oligodendrogliomas (10 cases), oligo-astrocytomas (8 cases) or anaplastic oligo-astrocytomas (1 case). However foci of cortical dysplasia could be observed in 47% of the cases. Clinical presentation and imaging features were strikingly similar to that observed in typical DNTs. Although surgical removal was incomplete in 28% of the cases and none of the patients underwent chemo or radiotherapy, none of the tumors recurred (mean follow-up: 7 years). Moreover, serial preoperative imaging in 26 patients (mean follow-up: 4.5 years) demonstrated that these lesions were perfectly stable. CONCLUSIONS: Whatever the histological appearance of a glial tumor, the diagnosis of DNT must be considered when all the following criteria are associated: (1) partial seizures, with or without secondary generalization, beginning before the age 20 years, (2) no neurological deficit or stable congenital deficit, (3) cortical topography of the lesion as better demonstrated by MRI and (4) no mass effect on imaging.

Dysembryoplastic neuroepithelial tumors located in the caudate nucleus area: report of four cases.

OBJECTIVE AND IMPORTANCE: Dysembryoplastic neuroepithelial tumors (DNTs) histologically resemble gliomas but behave as stable lesions. These tumors initially were considered to be located exclusively within the supratentorial cortex. The four reported cases demonstrate that DNTs may also arise in the area of the caudate nuclei. Moreover, the peculiar topography of these lesions, which suggests a derivation from the subependymal plate, is in accordance with the putative origin of DNTs from secondary germinal layers. CLINICAL PRESENTATION: The patients experienced partial seizures (two patients), generalized seizures (one patient), or headaches (one patient). All patients were young (17-26 yr) at the onset of symptoms, and all had normal results from their neurological examinations. INTERVENTION: All lesions demonstrated a pseudocystic appearance on computed tomographic scans, were hypointense on T1-weighted magnetic resonance imaging scans, hyperintense on T2-weighted magnetic resonance imaging scans, and did not show contrast enhancement. The four tumors similarly lined the left or right caudate nuclei and expanded within the homolateral ventricle (three patients) or both lateral ventricles (one patient). In one patient, the tumor also involved the adjacent paraolfactory cortex. CONCLUSION: In all patients, stereotactic biopsies helped to identify a specific glioneuronal element of DNTs. None of the tumors was operated on. Radiotherapy was performed in only one patient. A long pre- and/or postbiopsy imaging follow-up, which was available in two nontreated patients (3 yr and 16 yr), demonstrated the perfect stability of the lesion. The occurrence of DNTs in this peculiar location needs to be considered to avoid misidentification as "ordinary" gliomas and prevent useless aggressive treatment.

Somatostatin messenger RNA-containing neurons in Alzheimer's disease: an in situ hybridization study in hippocampus, parahippocampal cortex and frontal cortex.

The level of expression of somatostatin messenger RNA-containing neurons in human brain was visualized and quantified by in situ hybridization with a 35S-labelled oligonucleotide complementary to amino acids 96-111 of the preprosomatostatin complementary DNA sequence. The analysis was carried out in the frontal and parahippocampal cortices and hippocampus of six age- and post mortem delay-matched Alzheimer's disease and control brains. By northern blot analysis, in frontal cortex samples, 18S rRNA degradation was identical in control and Alzheimer brains and somatostatin messenger RNAs migrated as a single band of 1 kb. By in situ hybridization, specificity was demonstrated by abolition of the signal using either an excess of unlabelled antisense probe or using a labelled sense probe. Somatostatin messenger RNA-containing neurons displayed a similar regional and subregional distribution in control subjects and patients with Alzheimer's disease, being more abundant in the frontal cortex, followed by the hippocampus and the parahippocampal cortex. An overall reduction of labelled cell density was observed in patients with Alzheimer's disease (frontal cortex gray matter:--41%; white matter:--66%; hippocampus:--44%; parahippocampal cortex white matter:--40%). Due to a great variation between brains, this decrease only reached significance in the parahippocampal cortex (-59%, P < 0.05). A significantly lower level of expression of somatostatin messenger RNA per somatostatinergic cell was observed in the hippocampus of Alzheimer's disease patients (-47%, P < 0.05), but not in frontal cortex gray (-17%) and white (-36%) matter and parahippocampal cortex gray (-42%) and white (-29%) matter. These data are in accordance with the distribution of somatostatin cells as visualized by immunohistochemistry in human brain. They indicate that the ability of cortical cells to express somatostatin messenger RNA is partially preserved in Alzheimer disease brains and that the decrease in the amount of somatostatin messenger RNA per cell is restricted to the hippocampal formation.

Heterogeneity and selectivity of the degeneration of cholinergic neurons in the basal forebrain of patients with Alzheimer's disease.

Cholinergic neurons were studied by immunohistochemistry, with an antiserum against choline acetyltransferase (ChAT), in the basal forebrain (Ch1 to Ch4) of four patients with Alzheimer's disease (AD) and four control subjects. ChAT-positive cell bodies were mapped and counted in Ch1 (medial septal nucleus), Ch2 (vertical nucleus of the diagonal band), Ch3 (horizontal nucleus of the diagonal band) and Ch4 (nucleus basalis of Meynert). Compared to controls, the number of cholinergic neurons in AD patients was reduced by 50% on average. The interindividual variations in cholinergic cell loss were high, neuronal loss ranging from moderate (27%) to severe (63%). Despite the small number of brains studied, a significant correlation was found between the cholinergic cell loss and the degree of intellectual impairment. To determine the selectivity of cholinergic neuronal loss in the basal forebrain of AD patients, NPY-immunoreactive neurons were also investigated. The number of NPY-positive cell bodies was the same in controls and AD patients. The results (1) confirm cholinergic neuron degeneration in the basal forebrain in AD and the relative sparing of these neurons in some patients, (2) indicate that degeneration of cholinergic neurons in the basal forebrain contributes to intellectual decline, and (3) show that, in AD, such cholinergic cell loss is selective, since NPY-positive neurons are preserved in the basal forebrain.

Palatal myoclonus and inferior olivary lesions: MRI-pathologic correlation.

In a patient with palatal myoclonus, MR imaging demonstrated bilateral hyperintense lesions in the ventral part of the medulla. Microscopic examination of the inferior olives showed gliosis, enlargement and vacuolation of the neurons, and demyelinization.