Trigeminal amyloidoma: case report and review of the literature.

The authors present a case of amyloid infiltration involving the trigeminal nerve that mimicked a malignant cavernous sinus tumor with perineural tumor infiltration. A 64-year-old man presented with trigeminal nerve numbness. Imaging revealed a plaque-like enhancing lesion along the right lateral cavernous sinus extending anteriorly into Meckel's cave and involving the proximal V2 and V3 branches of the trigeminal nerve. The patient underwent an extradural frontotemporal craniotomy with middle fossa exposure of the cavernous sinus to diagnose and treat the presumed malignant cavernous sinus tumor. A reddish mass involving the lateral dural wall of the cavernous sinus was resected. The gasserian ganglion, V2, and V3, the latter of which was biopsied, were enlarged. Permanent histopathological studies showed microscopic eosinophilic, amorphous material, which stained positive for Congo red, and an absence of neoplastic cells. The final diagnosis was amyloidoma. Thus, amyloidomas can involve the trigeminal nerve or ganglia and should be considered in the differential diagnosis of a cavernous sinus lesion mimicking a tumor. Patients may have symptomatic improvement of trigeminal neuropathy with resection of the amyloidoma outside the nerve capsule that is compressing the nerve, while resection of the lesion from within the capsule may result in permanent trigeminal nerve dysfunction.     

Educational level as a modulator of cognitive performance and neuropsychyatric features in Parkinson disease.

OBJECTIVES: To test a possible association between the educational level (EL), cognitive performance, and neuropsychiatric features in Parkinson disease (PD). BACKGROUND: An inverse association has been reported between EL and cognitive dysfunction in patients with senile dementia of Alzheimer type but it is yet unsettled whether education has a similar effect on cognition in PD. METHODS: Seventy-two PD patients (45 males, mean age 68.7+/-11.6 y) underwent a detailed neurologic examination, a battery of neuropsychologic tests, and questionnaires for the evaluation of psychosis, sleep disturbances, and depression. According to the number of educational years, patients were divided into 3 groups: low EL (0 to 8 y), (15 patients), intermediate EL (9 to 12 y) (28 patients), and high EL (>/=13 y) (29 patients). RESULTS: Patients with a higher EL had a better cognitive function and an association was found between the patients' EL and their scores in various neuropsychologic tests mainly those sensitive to frontal lobe dysfunction. Low education was associated with an increased risk for hallucinations and a trend for more depression, delusions, and sleep disturbances. CONCLUSIONS: The association between high educational attainment and the lower risk of cognitive dysfunction suggest that education might modulate cognitive performance in PD.     

Searching for the Favorable Donor Site for Fat Injection: In Vivo Study Using the Nude Mice Model

BACKGROUND: The use of suctioned fat grafts for correction of soft tissue defects is a widespread procedure in esthetic and reconstructive surgery. The main disadvantage of this simple and sensible procedure is the unpredictable absorption rate of the fat graft. A lot of research has been performed aiming for enhancement of the take of the fat grafts. OBJECTIVE: Our study was performed to find if there is any favorable donor site for fat harvesting. METHODS: This in vivo experiment using the nude mice model enables the study of the long-term survival of human fat in an animal model. The fat was harvested from three donor areas: the thigh, abdomen, and breast of a 48-year-old woman who came for an elective esthetic procedure. After centrifugation, 1 cc of fat was injected subcutaneously into the scalp of the nude mouse. There were 15 mice in each of the three groups, according to the selected donor sites. The animals were sacrificed 16 weeks after the procedure. The extracted fat was evaluated in terms of weight, volume, and six histologic parameters: integrity, vascularization, cyst formation, fibrosis, necrosis, and inflammation. RESULTS: This study could not find any statistically significant differences between the three investigated donor sites in the evaluated parameters. CONCLUSION: On the basis of this study, there is no favorable area for harvesting fat grafts. The donor site can be chosen according to the preference of the surgeon and the patient.     

Fine needle aspiration biopsy for skull tumors: technical note and two demonstrative case reports.

Fine needle aspiration biopsy was performed in two case of infiltrating skull lesions. The role played by this relatively simple procedure in avoiding a major operation is discussed.     

Major depressives' and dysthmics' performance on the Wisconsin Card Sorting Test

Despite awareness that depression may affect performance on commonly used neuropsychological tests, little research has been conducted on the relative impact of severity and type of depression on higher problem-solving ability. Major depressives (n = 13), dysthymics (n = 17), and non-psychiatric comparison subjects (n = 18) were administered the Wisconsin Card Sorting Test (WCST). Although diagnostic category was related only marginally to some WCST measures after controlling for intellectual function, depressive symptom severity emerged as an independent predictor of Total Errors, Perseverative Responses, and Failure to Maintain Set. These findings lend support to the suggestion that neuropsychological test batteries should include assessment for depression and suggest that even relatively mild depression may affect WCST scores.     

Neuroactive steroids: their mechanism of action and their function in the stress response

Steroids are usually identified as genomic regulators, yet recently a body of evidence has accumulated demonstrating specific plasma membrane effects, as well as coordinative effects, of some steroids on both membrane and intracellular receptors. The resulting rapid (<1 min) modulation of cellular activity has strongly suggested a non-genomic, and possibly modulatory, role for certain steroid compounds, and dramatic effects on membranes of excitable as well as other tissues have been demonstrated. Steroid synthesis and metabolism have been shown to exist in the CNS, and the effects have been seen in both the central and peripheral nervous systems. The major groups of neuroactive steroids, and their metabolites, have been progesterone, deoxycorticosterone, and some androgens, notably dihydroxyepiandrosterone (DHEA). These compounds show increased concentrations both in blood and in the brain following stress and they have also been associated with anxiolytic effects and antiepileptic activity. In the periphery, some of these compounds show remarkable inhibitory effects on the secretion of catecholamines and other neurotransmitters. The mechanism for the majority of the effects of these steroids is via their effect on receptor-mediated binding to ligand-gated ion channels. Activation of the GABAA receptor complex, resulting in the opening of its central chloride channel, is the major target of the neuroactive steroids, resulting in re-polarization of the plasma membrane and inhibition of further neuronal firing. The anxiolytic, anti-convulsant and sedative-hypnotic actions of these neuroactive steroids have resulted in their being used as therapeutic agents for the treatment of anxiety, epilepsy, insomnia, and possibly for the alteration of pain thresholds.     

Increased yield of full GBA sequencing in Ashkenazi Jews with Parkinson's disease

Background

Variants in GBA are the most common genetic risk factor for Parkinson's disease (PD), and are especially prevalent in the Ashkenazi Jewish (AJ) population. However, most studies on GBA in AJ genotype only seven selected Gaucher-associated pathogenic variants rather than sequencing the whole gene, which may leave carriers of PD-associated GBA variants undiscovered.

Cellular localization of complement C3 and C4 transcripts in intestinal specimens from patients with Crohn's disease

It has been suggested that the increase in C3 and C4 levels in jejunal perfusates of patients with Crohn's disease (CD) results from local intestinal synthesis of complement. The present study evaluated the expression of these complement genes in inflamed tissues from patients with CD. Surgically resected specimens from patients with CD and control tissue obtained from subjects with adenocarcinoma of the colon were evaluated for C3 and C4 gene expression by the use of 35S-labelled anti-sense RNA probes. All tissue samples, diseased and normal tissue, expressed C4 mRNA throughout in the intestinal epithelium. C3 mRNA was not detected in epithelial cells in histologically normal tissue, but in diseased specimens there was a focal distribution of C3 mRNA in epithelial cells of the crypts, but not in villous epithelium. Focal C3 gene expression correlated with crypt abscess formation and the presence of polymorphonuclear leucocytes in the lumen of the crypts. In addition, C3 mRNA was also found in macrophages of the submucosa. These macrophages were CD68+, fusiform with faint cytoplasm and morphologically different from the large rounded lamina propria macrophages, which do not express C3 mRNA. Multinucleated giant cells did not express either C3 or C4 genes. In addition to its presence in intestinal epithelium, C4 mRNA was also expressed in mast cells, which however did not express C3 mRNA. These observations identify cells in the intestinal wall expressing complement genes and support the hypothesis that there is local regulated production of complement in the intestine of patients with CD, and subsequent complement activation may contribute to the inflammatory process.