A case of neurofibromatosis type 1 with clival chordoma

Neurofibromatosis type-1 is an autosomal dominant tumour syndrome with a high clinical susceptibility to malignancies, especially nervous system malignancies. Here, we reported a case of neurofibromatosis type-1 in a male in his 50s, who presented with generalised weakness, headache associated with ear pain, a feeling of heaviness of the head, and giddiness for a duration of 1 week. On examination, he had axillary freckling and multiple neurofibromas over his body. Nystagmus and dysdiadokokinesia were present. MRI brain revealed an enhancing lesion in the sphenoid and clivus, extending into the sellar and supra-sellar region. The possibility of pituitary adenoma and clival chordoma were considered. He was referred to Neurosurgery and underwent Trans-nasal Trans-sphenoidal near-total resection of the tumour. Biopsy of the lesion was indicative of conventional clival chordoma, which is rarely reported with NF-1. The post-operative period was uneventful and the patient is planned for regular follow-up to detect recurrence.

What influences outcome of patients with suicidal hanging.

AIM: Suicidal hanging is an uncommon medical emergency with significant neurological morbidity. The aim of the study was to identify factors that have a bearing on the final outcome. MATERIALS AND METHODS: Retrospective analysis of 37 consecutive cases of suicidal hanging admitted to our ICU from July 1996 - December 2002 was performed. Outcome at discharge was defined as good (complete neurological recovery) or poor (death or incomplete neurological recovery). Three clinical variables (at presentation) namely Glasgow Coma Scale (GCS) at presentation, time lapse (in hours) from the incident to arrival at our hospital and the presence of Hypotension (defined as a systolic blood pressure < or = 90 mm Hg) at admission were recorded and tested individually for an association with the outcome. Statistical analysis was done using the Odds ratio (OR +/- 95% Confidence Intervals) and Chi-square test of significance for categorical data. RESULTS: Patients with suicidal hanging constituted < 1% of ICU admissions (mean age 27 years). 34/37 survived giving a survival rate of 92%. Of those who survived, 31 patients (91%) had complete neurological recovery at the time of discharge from hospital. Among those who presented <4 hours of the incident (25 patients), 2 had an adverse outcome as compared to 4/10 patients who presented 4 hours or more after the incident (OR 7.6,95% CI 1.12-52.3). A low GCS (GCS <7) at presentation was also statistically associated with a poor outcome (OR 10.4, 95% CI 1.08-102.1). Hypotension was present in approximately 33% of patients (12/37) at presentation. Of these 12 patients, 3 had an adverse outcome as opposed to 3 patients among the remaining 25 (OR 1.6, 95% CI 0.41 - 14.47, NS). CONCLUSIONS: Suicidal hanging is an unusual medical emergency that is common among young individuals in developing countries. Our study indicates that a delayed presentation to a medical facility and a low GCS at presentation predict a poor outcome.

Rodenticide-induced hepatotoxicity.

Hepatotoxicity following acute poisoning with rodenticides has been infrequently reported in literature. To emphasize the fact that this form of clinical presentation is not unusual we are reporting two cases of rodenticide poisoning masquerading as severe hepatic dysfunction.

Role of the hypothalamic pituitary adrenal axis in the control of the response to stress and infection

The release of adrenocorticotropin (ACTH) from the corticotrophs is controlled principally by vasopressin and corticotropin-releasing hormone (CRH). Oxytocin may augment the release of ACTH under certain conditions, whereas atrial natriuretic peptide acts as a corticotropin release-inhibiting factor to inhibit ACTH release by direct action on the pituitary. Glucocorticoids act on their receptors within the hypothalamus and anterior pituitary gland to suppress the release of vasopressin and CRH and the release of ACTH in response to these neuropeptides. CRH neurons in the paraventricular nucleus also project to the cerebral cortex and subcortical regions and to the locus ceruleus (LC) in the brain stem. Cortical influences via the limbic system and possibly the LC augment CRH release during emotional stress, whereas peripheral input by pain and other sensory impulses to the LC causes stimulation of the noradrenergic neurons located there that project their axons to the CRH neurons stimulating them by alpha-adrenergic receptors. A muscarinic cholinergic receptor is interposed between the alpha-receptors and nitric oxidergic interneurons which release nitric oxide that activates CRH release by activation of cyclic guanosine monophosphate, cyclooxygenase, lipoxygenase and epoxygenase. Vasopressin release during stress may be similarly mediated. Vasopressin augments the release of CRH from the hypothalamus and also augments the action of CRH on the pituitary. CRH exerts a positive ultrashort loop feedback to stimulate its own release during stress, possibly by stimulating the LC noradrenergic neurons whose axons project to the paraventricular nucleus to augment the release of CRH.

The role of nitric oxide in reproduction

Nitric oxide (NO) plays a crucial role in reproduction at every level in the organism. In the brain, it activates the release of luteinizing hormone-releasing hormone (LHRH). The axons of the LHRH neurons project to the mating centers in the brain stem and by afferent pathways evoke the lordosis reflex in female rats. In males, there is activation of NOergic terminals that release NO in the corpora cavernosa penis to induce erection by generation of cyclic guanosine monophosphate (cGMP). NO also activates the release of LHRH which reaches the pituitary and activates the release of gonadotropins by activating neural NO synthase (nNOS) in the pituitary gland. In the gonad, NO plays an important role in inducing ovulation and in causing luteolysis, whereas in the reproductive tract, it relaxes uterine muscle via cGMP and constricts it via prostaglandins (PG).

Changes in level of certain serum constituents following sciatectomy in the common frog, Rana tigrina (Daud).

Effect of unilateral (one leg) and bilateral (two legs) sciatectomy was studied on certain serum constituents in the male frog over a period of 3 weeks. The level of creatine kinase, acid and alkaline phosphatases, urea, glucose and proteins increased initially following sciatectomy and decreased subsequently from day 14 onwards, the per cent change being more pronounced in case of bilaterally sciatectomized frogs. The present results indicate an increase in the rate of deamination of proteins in the liver, an impairment in the transportation processes across the cell membrane and an increase in cellular lysosomal activity on sciatectomy suggesting a change either in the amount or in the rate of various enzyme reactions. It is concluded that sciatectomy induces alterations in general metabolic activities and the functional state of the animal. Altered values of various serum constituents thus permit to speculate analysis of the factor that may be contributing to the atrophic processes and the wasting of the muscle fibres known to set in the denervated muscle.

Effect of phenobarbitone on pituitary gland of male albino mice.

The effects of phenobarbitone on gonadotrophs and lactotrophs of pituitary gland were studied in male albino mice which were administered phenobarbitone 50 mg/kg body weight intraperitoneally (ip) daily for 14 days. A group of control mice were administered saline ip. In the experimental group, there was significant reduction in the average cell population of FSH cells in the cephalomedian area (P < 0.001) and in the lateral lobe (P < 0.01). The average volume of both FSH and LH cells was also significantly reduced (P < 0.001). The changes in the gonadotrophs of the experimental group could be attributed to the negative feedback action of testosterone which is being secreted from hypertrophied Leydig cells of testis.