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Germinomas are highly immunogenic tumors eliciting a strong peri-tumoral immune response that can spillover into the surrounding healthy tissues. This phenomenon can also occur in intracranial germinomas, manifesting as secondary hypophysitis. Herein, we report a case of 12-year-old-girl presenting with polyuria and polydispsia. She had central diabetes insipidus (CDI) and panhypopituitarism. Imaging revealed a sellar-suprasellar mass with infundibular stalk thickening. Transphenoidal biopsy revealed epithelioid granulomas with immunostaining negative for germinomatous cells. Other causes of hypophysitis were ruled out. Accordingly, she was diagnosed as primary granulomatous hypophysitis and treated with high-dose corticosteroids. Three years later she again presented with headache, vomiting and diminution of vision. Imaging showed a heterogeneous, solid-cystic peripheral rim-enhancing lesion at the same location with involvement of hypothalamus, ependyma and pineal gland. Cerebrospinal fluid beta-human chorionic gonadotropin was markedly elevated, confirming the diagnosis of an intracranial germ cell tumor. She was started on chemotherapy; however, she succumbed to febrile neutropenia. We performed a literature search and found 18 anecdotal cases of secondary hypophysitis associated with intracranial germinomas. There was a slight male preponderance (male:female 5:4). Two-thirds of the cases were below 18 years of age. Polyuria was the most common presenting manifestation (83%). CDI and panhypopituitarism were seen in 89 and 78% cases, respectively. Imaging evidence of pituitary stalk thickening was seen in 12 cases (67%), while pituitary enlargement and/or sellar mass were reported in 11 cases (61%). Pineal involvement was extremely rare, being reported in only 1 case, implying the predilection of suprasellar (rather than pineal) germinomas in causing secondary hypophysitis. Histologically, 82% had lymphocytic hypophysitis, while 18% had granulomatous hypophysitis. Initially, the diagnosis of germinoma was missed in 60% of the cases who were wrongly treated with corticosteroids. To conclude, physicians should make it a dictum that all children and adolescents presenting with CDI and pituitary stalk thickening should be rigorously screened for an underlying intracranial germinoma before labeling them as primary hypophysitis. [ABSTRACT FROM AUTHOR]

Background: Imatinib, a tyrosine kinase inhibitor, causes growth failure in children with chronic myeloid leukemia probably by targeting the growth hormone (GH)/insulin like growth factor-1 (IGF-1) axis. We aim to explore the imatinib targets expression in pituitary adenomas and study the effect of imatinib on GH secretion in somatotropinoma cells and GH3 cell line. Materials and Methods: The expression pattern of imatinib's targets (c-kit, VEGF, and PDGFR-α/β) was studied using immunohistochemistry and immunoblotting 157 giant (≥4 cm) pituitary adenomas (121 non-functioning pituitary adenomas, 32 somatotropinomas, and four prolactinomas) and compared to normal pituitary (n = 4) obtained at autopsy. The effect imatinib on GH secretion, cell viability, immunohistochemistry, electron microscopy, and apoptosis was studied in primary culture of human somatotropinomas (n = 20) and in rat somato-mammotroph GH3 cell-line. A receptor tyrosine kinase array was applied to human samples to identify altered pathways. Results: Somatotropinomas showed significantly higher immunopositivity for c-kit and platelet-derived growth factor receptor-β (PDGFR-β; P < 0.009 and P < 0.001, respectively), while staining for platelet-derived growth factor receptor-α (PDGFR-α) and vascular endothelial growth factor (VEGF) revealed a weaker expression (P < 0.001) compared to normal pituitary. Imatinib inhibited GH secretion from both primary culture (P < 0.01) and GH3 cells (P < 0.001), while it did not affect cell viability and apoptosis. The receptor tyrosine kinase array showed that imatinib inhibits GH signaling via PDGFR-β pathway. Conclusion: Imatinib inhibits GH secretion in somatotropinoma cells without affecting cell viability and may be used as an adjunct therapy for treating GH secreting pituitary adenomas. [ABSTRACT FROM AUTHOR]

Background: Patients with acromegaly have 2-3 times the expected mortality rates primarily due to cardiovascular risks. Echocardiographic studies showing improvement of cardiac function following transsphenoidal surgery (TSS) are limited.Materials and Methods: All patients with acromegaly underwent preoperative echocardiography and tissue Doppler (Philips i33, 3D ECHO) for assessment of cardiac indices. In the prospective group of patients, echocardiography was repeated after 6 months of surgery. In the retrospective group of patients, echocardiography was performed for left ventricular diastolic dysfunction and ejection fraction. Biochemical cure was confirmed at least after 6 months by glucose-suppressed plasma growth hormone (GH) concentrations (GH-OGTT) of less than 0.4 ng/ml, random GH of less than 1 ng/ml, and normal age-corrected insulin-like growth factor (IGF-1) values.Results: In the prospective group (38 patients), there was a significant decrease in the left ventricular mass (LVM) and LVM index (LVMI) in patients who were cured as well as in patients with postoperative growth hormone (GH) 1-5 ng/ml. In the prospective group, LVMI completely normalized in 2 and a new-onset deterioration was detected in 1 patient (who was not cured) and improved in 8 others. Left ventricular systolic function was abnormal at baseline in 18 (47.3%) patients, which normalized in 11 (61.1%) patients postoperatively, and in 7 patients, it improved significantly although it did not normalize completely. There was also a significant improvement in the left ventricular ejection fraction (P = 0.01). Post TSS, in patients with GH-OGTT >5 ng/ml, there was no significant decrease in the LVM, LVMI and ejection fraction (EF). In the retrospective group, 62 patients were analyzed for a change in the EF with a mean follow-up of 20.3 months. There was a significant improvement in the left ventricular EF in patients who were cured (P < 0.001).Conclusion: Reduction in growth hormone levels and insulin-like growth factor type 1 can decrease the LVM and LVMI, which directly or indirectly contributes to the improvement in diastolic as well as systolic function and probably mortality. [ABSTRACT FROM AUTHOR]

Background: To evaluate the feasibility, safety and efficacy of dose fractionated gamma knife radiosurgery (DFGKRS) on a daily schedule beyond the linear quadratic (LQ) model, for large volume arteriovenous malformations (AVMs).Material and Methods: Between 2012-16, 14 patients of large AVMs (median volume 26.5 cc) unsuitable for surgery or embolization were treated in 2-3 of DFGKRS sessions. The Leksell G frame was kept in situ during the whole procedure. 86% (n = 12) patients had radiologic evidence of bleed, and 43% (n = 6) had presented with a history of seizures. 57% (n = 8) patients received a daily treatment for 3 days and 43% (n = 6) were on an alternate day (2 fractions) regimen. The marginal dose was split into 2 or 3 fractions of the ideal prescription dose of a single fraction of 23-25 Gy.Results: The median follow up period was 35.6 months (8-57 months). In the three-fraction scheme, the marginal dose ranged from 8.9-11.5 Gy, while in the two-fraction scheme, the marginal dose ranged from 11.3-15 Gy at 50% per fraction. Headache (43%, n = 6) was the most common early postoperative complication, which was controlled with short course steroids. Follow up evaluation of at least three years was achieved in seven patients, who have shown complete nidus obliteration in 43% patients while the obliteration has been in the range of 50-99% in rest of the patients. Overall, there was a 67.8% reduction in the AVM volume at 3 years. Nidus obliteration at 3 years showed a significant rank order correlation with the cumulative prescription dose (p 0.95, P value 0.01), with attainment of near-total (more than 95%) obliteration rates beyond 29 Gy of the cumulative prescription dose. No patient receiving a cumulative prescription dose of less than 31 Gy had any severe adverse reaction. In co-variate adjusted ordinal regression, only the cumulative prescription dose had a significant correlation with common terminology criteria for adverse events (CTCAE) severity (P value 0.04), independent of age, AVM volume, number of fractions and volume of brain receiving atleast 8 Gy of radiation.Conclusion: DFGKRS is feasible for large AVMs with a fair nidus obliteration rate and acceptable toxicity. Cumulative prescription dose seems to be the most significant independent predictor for outcome following DFGKRS with 29-30 Gy resulting in a fair nidus obliteration with least adverse events. [ABSTRACT FROM AUTHOR]

Background: Extraskeletal mesenchymal chondrosarcoma (ESMCS) is an unusual pathologic variant of chondrosarcoma. There are no specific clinicoradiographic parameters to distinguish it from other intracranial pathologies. The diagnosis can be established only on the basis of histopathology, which may pose significant challenges in certain unusual locations. Purpose: In this case, we discuss the pitfalls in diagnosis, management, and major characteristics of ESMCS with a review of current literature. Methods and Results: A 47-year-old female patient presented with a jugular foramen tumor which was misdiagnosed as glomus jugulare tumor for which she received primary gamma knife radiosurgery at 2 instances. But unfortunately, the patient died because of the increase in size of the lesion associated with necrosis. Autopsy analysis revealed a highly cellular tumor, rich in sarcomatous cells, and well-differentiated cartilages consistent with ESMCS. Conclusion: A definite preoperative diagnosis of ESMCS is crucial though difficult especially when located at complex sites like jugular foramen and clinicoradiological mimicry. The most crucial step in the management of ESMCS is accurate diagnosis with critical evaluation of clinical, radiological, and histopathological parameters and realization of highly variable clinical course of the disease. [ABSTRACT FROM AUTHOR]

Esophageal perforation is a dreaded complication of anterior cervical spinal surgery. A 52-year-old diabetic man had undergone a surgery for a C6-C7 disc prolapse and developed spiking fever with chills and rigor on the 7th postoperative day. No cause could be found out but a CT scan of thorax done in the course of investigations revealed pneumomediastimum. The patient succumbed on the 10th day after surgery. Autopsy revealed the cause of death to be mediastinitis following iatrogenic esophageal perforation. A second patient, 53 years of age, following surgery for C5-C6 disc prolapse and developed intractable dysphagia. Later, fever and purulent discharge from the wound prompted an MRI showing prevertebral collection extending to the superior mediastinum. Presuming only wound infection, debridement and implant removal was done. However persistent serous discharge from the wound revealed an esophageal injury. Late diagnosis precluded primary repair. With conservative treatment, the fistula finally closed after 42 days. Postoperative dysphagia, a common complaint following surgery, may not always be present in cases of esophageal injury. A high index of suspicion is required for diagnosing and initiating treatment for esophageal perforation before complications set in. [ABSTRACT FROM AUTHOR]

Objective: To study the visual outcome after surgery for pituitary adenomas with visual deficits.Materials and Methods: All patients with pituitary adenoma, who presented from 2003-2014 in a tertiary care institute, were included in the study. Surgical outcome was measured in terms of difference in visual acuity, visual fields and optic fundus parameters documented before surgery, immediate post-operatively and at the third, and twelfth months following surgery.Results: At the initial presentation, visual involvement was seen in 87.2% patients. One year after surgery, 93.2% patients having abnormal vision had improvement in visual acuity and visual fields; whereas visual parameters were static in 5.2%. Visual deterioration occurred only in 1.3% patients. Moreover, five-percent of those who did not even have perception of light at presentation experienced significant improvement in vision after surgery. The shorter the duration of visual symptoms, the more was the percentage of patients having faster recovery in the early postoperative period.Conclusion: Post-operative visual outcome was directly proportional to the pre-operative visual acuity. Though the visual outcome was good in the long run irrespective of the duration of symptoms, the speed of recovery was proportional to the duration of visual deficits. However, presence of long-standing visual symptoms should not deter us to subject the patient to surgery. Even patients who are completely visually impaired for years should be subjected to surgery as early as feasible. [ABSTRACT FROM AUTHOR]

The article presents abstracts on medical topics including the research that disproves the claim that alcohol consumption reduces the risk of stroke, the issue of confidentiality between patient and caregiver, and the study that proves the effectiveness of chlorhexidine in disinfecting.

Background: Growth hormone (GH) levels following oral glucose tolerance test (OGTT) at 12 weeks or later after surgery have been accepted as the most reliable parameter for defining remission and/or cure in patients with acromegaly. However, the role of random GH in predicting remission in the immediate postoperative period using modern criteria is not known. This study was undertaken to evaluate the role of random GH levels in first 5 postoperative days as an early predictive tool for long-term remission of patients with acromegaly following transsphenoidal pituitary surgery (TSS).Patients and Methods: Seventy-five consecutive acromegaly patients with at least three postoperative OGTT values at 3, 6, and 12 months of follow-up were included in the study. GH levels were measured just before surgery, in the immediate postoperative period, at 6 h and on day 1 to day 5 after surgery. Remission was defined as normal age-specific insulin-like growth factor-1 and either basal fasting GH <1 ng/ml or a nadir GH following OGTT <0 .4 ng/ml at 3 months of surgery.Results: Of the 75 patients with acromegaly who underwent TSS, long-term remission was achieved in 42 (56%) patients. GH values ≤1.55 ng/ml at 6 h of surgery showed the highest predictive power for long-term remission, with a sensitivity of 81.2% and a specificity of 83.3%. The duration of disease and tumor volume had no effect on the 6 h GH value-related prediction of cure.Conclusion: Early postoperative GH values may be used to predict long-term cure. A value of ≤1.5 ng/ml at 6 h following surgery may predict long-term cure in two-thirds of the patients with acromegaly who undergo TSS. [ABSTRACT FROM AUTHOR]

Aims: We present retrospective analysis of patients of glioblastoma multiforme (GBM) and discuss clinical characteristics, various treatment protocols, survival outcomes, and prognostic factors influencing survival. Materials and Methods: From January 2002 to June 2009, 439 patients of GBM were registered in our department. The median age of patients was 50 years, 66.1% were males, and 75% underwent complete or near-total excision. We evaluated those 360 patients who received radiotherapy (RT). Radiotherapy schedule was selected depending upon pre-RT Karnofsky Performance Status (KPS). Patients with KPS <70 (Group I, n = 48) were planned for RT dose of 30-35 Gy in 10-15 fractions, and patients with KPS >70 (Group II, n = 312) were planned for 60 Gy in 30 fractions. In group I, six patients and in group II, 89 patients received some form of chemotherapy (lomustine or temozolomide). Statistical Analysis Used: Statistical analysis was done using Statistical Package for Social Sciences, version 12.0. Overall survival (OS) was calculated using Kaplan-Meier method, and prognostic factors were determined by log rank test. The Cox proportional hazards model was used for multivariate analysis. Results: The median follow-up was 7.53 months. The median and 2-year survival rates were 6.33 months and 2.24% for group I and 7.97 months and 8.21% for group II patients, respectively (P = 0.001). In multivariate analysis, site of tumor (central vs. others; P = 0.006), location of tumor (parietal lobe vs. others; P = 0.003), RT dose (<60 Gy vs. 60 Gy; P = 0.0001), and use of some form of chemotherapy (P = 0.0001) were independent prognostic factors for survival. Conclusions: In patients with GBM, OS and prognosis remains dismal. Whenever possible, we should use concurrent and/or adjuvant chemotherapy to maximize the benefits of post-operative radiotherapy. Patients with poor performance status may be considered for hypofractionated RT schedules, which have similar median survival rates as conventional RT. [ABSTRACT FROM AUTHOR]

Background: The microanatomy features of cerebral arteries may be variable and may be different in different ethnic groups. Aim: To study the anterior cerebral artery (ACA) anatomy in North-West Indian cadavers. Materials and Methods: Microanatomy features of the ACA were studied in 15 formalin fixed human cadaveric brains under microscope. The outer diameter, length, and number of perforating branches with respective anomalies were measured for each of the following vessels: ACA (proximal A1 segment to distal A2 segment), anterior communicating artery (ACoA), Recurrent artery of Heubner (RAH), and callosomarginal artery and photographed for documentation. Results: The mean length and external diameter of right and left A1 segment was 12.09 mm and 12.0 mm and 2.32 mm and 2.36 mm respectively. Narrowing, duplication, and median ACA were seen in 6.6%, 3.3% and 6.6% of the vessels respectively. Complex ACoA type was seen in 40% cadavers. RAH originated at an average point of 0.2 mm distal to ACoA, but in one cadaver it arose 5 mm proximal to ACoA. Double RAH was found in 26.6%. The course of RAH in relation to A1 was superiorly in 60%, in anteriorly 30% and posteriorly in 10% of cadavers. The orbitofrontal artery (OFA) and frontopolar artery (FPA) arose from A2 in 83.3% to 40% respectively. The mean distance of OFA and FPA from ACoA was 4.17 mm and 8.5 mm respectively. After giving rise to central, callosal and cortical branches, pericallosal artery terminated near the splenium of the corpus callosum or on the precuneus as the inferomedial parietal artery. Conclusion: Knowledge of the microvascular anatomy is indispensable and it is mandatory to be aware of the possible variations in the anomalies to minimize morbidity. [ABSTRACT FROM AUTHOR]

Background: Moyamoya disease is a rare, progressive cerebrovascular disorder caused by intracranial stenosis of the circle of Willis, resulting in successive ischemic events. Computed tomography (CT) and magnetic resonance imaging (MRI) play a major role in diagnosis. Objective: The aim of the study was to describe the spectrum of findings on brain SPECT in patients with Moyamoya disease and to compare the findings with other investigations. Materials and Methods: Tc99m-ECD SPECT scans of seventeen patients (7 children and 10 adults) were analysed to study the brain perfusion. Results: Features of Moyamoya disease were detected on DSA in 11 patients, CTA in one, MR angiography in one patient. Brain perfusion SPECT analysis showed unilateral perfusion defects in 11 patients, normal perfusion in 2 and bilateral defects in 4 patients. No perfusion defects despite bilateral vascular changes were noted in one patient. Cerebral infarcts were detected on MRI unilaterally in three subjects while multiple infarcts were identified in one. Tc99m-ECD Brain SPECT showed perfusion defects that were more extensive compared to those detected on MRI. Post acetazolamide studies for assessment of cerebrovascular reserve were done in three patients. Two of them showed good cerebrovascular reserve (>1). Follow-up studies post-surgical procedures (Myo-dura synangiosis) done in two patients showed partial resolution of perfusion defects in the involved areas. Conclusion: Brain perfusion scintigraphy is an important adjunct in evaluation of patients with Moyamoya disease yielding information about the direct end results of the pathology in the vessels and also prognostic information. [ABSTRACT FROM AUTHOR]

Subarachnoid hemorrhage (SAH) is a significant health care problem. One of the major determinants of outcome following surgery of intracranial aneurysms is development of intracranial infarcts. All patients underwent clipping for aneurysms in one year in the department of neurosurgery, PGIMER, Chandigarh were studied. Data regarding age, sex, date of ictus, date of admission, any co-morbidity, clinical grades at presentation, CT findings, infarcts, intraoperative rupture, and clinical status in the postoperative period were recorded. Outcome at discharge was assessed by Glasgow outcome scale (GOS). First, 174 patients were included in the study. Radiological cerebral infarctions occurred in 69 patients (39%). The most frequent location of infarct was deep perforator infarct followed by ACA territory infarct. 69.58% of patients developed infarct on the same side of aneurysm and 20.28% of patients developed infarct on opposite side, whereas 11% developed bilateral infarcts. Infarcts that occur early after surgery may be related to surgical factors whereas the late infarcts were probably as results of delayed ischemic deficits. Anatomical distribution of infarcts also showed two different patterns, infarcts limited to one vascular territory (more commonly seen in early onset infarcts) or multiple, cortical, bilateral infarcts (more commonly seen in late onset infarct). Patients with poor H&H grade, higher Fisher's grade, intraoperative rupture and prolonged temporarory clipping had more chances of developing an intracranial infarct. [ABSTRACT FROM AUTHOR]

Background: Near-Death experience (NDE) following a severe head injury, critical illness, coma, and suicidal attempt has been reported. Purpose: Purpose of study was to examine why a few patients report NDE after survival, do cultural and socio-demographic factors may play a role? Methods: The details of 3 cases of patients who reported near-death experience (NDE), is presented here. Several theories regarding the reasons, of the various components of the experiences, are discussed with a brief review of literature. Results: All the three patients report the Out of body experience OBE. All the three patients reported to remember initially the events that took place during this time, but after some time all three patients could not recall exactly the events that had happened. Conclusion: Whether these are only hallucinations or a proof of `after life' will remain debatable until more data is communicated. [ABSTRACT FROM AUTHOR]

McCune-Albright syndrome (MAS) is characterized by a triad of poly/monoostotic fibrous dysplasia, café-au-lait macules and hyperfunctioning endocrinopathies. Association of MAS with GH excess is rare, and in most of the instances somatotropinoma has not been documented. Treatment of patients of MAS with acromegaly is difficult because of thickened calvarium and dysplastic skull bone. We report a 17-year-old girl, who presented with cranio-facial fibrous dysplasia, café-au-lait macules and also had acromegaly due to pituitary macroadenoma, and treated with gamma knife radiosurgery. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented which discusses the case of a nine-year-old boy with abnormal head movements, poor height gains, and poor scholastic performance of three years duration who was diagnosed with third ventricular cyst with tactile hallucinations and bobble head movements.

The authors report a 36-year-old male who presented with headache and hypopituitarism, and MRI revealed a ring enhancing lesion with pituitary stalk thickening. During follow-up, he presented with recurrent pyogenic meningitis with persistence of the lesion, therefore a diagnosis of pituitary abscess was considered. He underwent trans-sphenoidal surgery (TSS) with evacuation of pus and received antibiotic treatment for the same. After this he remarkably improved and had no recurrence of symptoms. He is on levothyroxine, glucocorticoids and testosterone replacement therapy for his respective hormone deficits. [ABSTRACT FROM AUTHOR]

Competing Interests: There are no conflicts of interest.

A 38-year-old man presented with excessive height gain and progressive enlargement of the extremities since childhood. This was compounded by lower limb deformities over the past 5 years. On examination, his height was 196 cm, he had macroglossia, acral enlargement, seborrhoea, hyperhidrosis-suggesting acrogigantism. He had facial asymmetry, wind-swept deformity of lower limbs and a café-au-lait macule over his trunk. Investigations revealed normal-sized pituitary gland with dysplastic cranial bones. Isotope bone scintigraphy was suggestive of polyostotic fibrous dysplasia. A diagnosis of McCune-Albright syndrome was made and trans-sphenoidal hypophysectomy was undertaken. He had persistent hypophosphataemia. Tubular reabsorption of phosphate adjusted for glomerular filtration rate was low and serum FGF-23 level was high. Ga-DOTATATE scintigraphy showed somatostatin-receptor expression in all the dysplastic lesions. FGF-23 produced by the bony lesions could counteract the phosphate-retaining effect of GH excess resulting in hypophosphataemia, which further worsened following hypophysectomy., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

A 27-year-old woman presented with anorexia, weight loss and psychiatric symptoms for the past 4 years. She did not have history of headache, visual disturbances or symptoms of raised intracranial pressure. She was sarcopenic with body mass index of 10.16 kg/m 2 Her systemic examination was normal except for temporal hemianopia suggesting a sellar/suprasellar lesion. Hormonal evaluation revealed pan-hypopituitarism with central diabetes insipidus. Subsequent neuroimaging revealed sellar-suprasellar mass lesion with intense contrast enhancement and leptomeningeal metastases. Cerebrospinal fluid analysis showed elevated β human chorionic gonadotropin and the presence of syncytiotrophoblast germ cells. Histopathology from the mass lesion confirmed the diagnosis of germinoma. Immunohistochemistry of the tumour tissue was positive for c-kit and placental alkaline phosphatise. She received a combination of chemotherapy with craniospinal irradiation. Significant weight loss in a young woman may not always be an eating disorder like anorexia nervosa albeit more common than germ cell tumour., Competing Interests: Conflicts of Interest: None declared., (2016 BMJ Publishing Group Ltd.)

Background: Extra-pituitary birth defect (EPBD) in children with congenital hypopituitarism is largely unknown., Objective: The study aims to evaluate the incidence and pattern of EPBD in children with congenital hypopituitarism and to evaluate whether it can serve as a clue to diagnose this condition., Patients and Methods: Retrospective analysis of hospital record of patients of short stature due to various etiology from which patients with congenital hypopituitarism with age ≥18 years were recruited for the analysis. Clinical, hormonal, radiological and ocular electrophysiological studies were done in all patients and all EPBD were noted., Results: Twenty seven patients (79%) had multiple pituitary hormone deficiency (MPHD) of which growth hormone was universal followed by gonadotropin (62%), TSH (59%), ACTH (44%) and prolactin (12%). Nineteen patients (56%) had multiple EPBD in various combinations. Twenty three ocular abnormalities were present in 12 patients (35%). Nine patients (26%) had other associated EPBD along with ocular abnormalities while 3 had ocular abnormalities without any other associated birth defect. Skeletal defects were present in 10 patients (29.5%). On the contrary, 5 patients in the EPBD group had total 15 visual defects. The most common abnormality of the visual system were abnormal visual evoke response (VER, 18%), followed by strabismus (15%), visual acuity (VA, 12%), electroretinogram (ERG) and electrooculogram (EOG) 8% each and visual field defect 6%. There was a trend towards early age at presentation with EPBD., Conclusions: Presence of EPBD in a short child is a sensitive marker to diagnose congenital hypopituitarism. Subtle abnormalities of visual pathway without absent septum pellucidum or midline brain defects were common., (© Journal of the Association of Physicians of India 2011.)

Aim: The diagnosis and treatment of acromegaly, a rare and possibly curable disease, has undergone a paradigm shift in the past few decades. Our aim was to study the changing trends in clinical presentation, management and outcome of the disease in the last fifteen years., Methodology: 271 consecutive patients with acromegaly treated at the Departments of Endocrinology and Neurosurgery, PGIMER, Chandigarh, between 2000 and 2014, were included in the study. Clinical and hormonal profiles, comorbidities, treatment modalities, outcome and mortality data were evaluated. The cure rate was assessed according to the present consensus criteria., Results: The gender distribution was equal with the mean age (±SD) of 37.1 ± 12.3 years at diagnosis. The average lag period to diagnosis was 4.7 ± 4.2 years. The most common presenting manifestations were acral enlargement and headache followed by visual deficits. The overall mortality rate was 5%, with the perioperative mortality being 1.5%. The most prevalent comorbidities in our series were hypertension (17.7%), diabetes mellitus (16.2%), arthropathy (11.8%) and obstructive sleep apnea (10.3%). Overall, 2 patients in our series suffered from extra-pituitary neoplasms and 12 patients had apoplexy as the presenting manifestation. As per the present consensus criteria, cure rate in our series was 28.5%. The cure rate was only 7.9% when many surgeons were operating. It increased to 25.5% when surgeries were being performed by one surgeon exclusively; and, when a sub-specialty clinic exclusively for pituitary diseases was set up, the cure rates improved upto 56%., Conclusion: Acromegaly has wide-ranging manifestations from acral enlargement to altered sensorium; incidental diagnosis was not prevalent in our series. Majority of the cases were due to the presence of a pituitary macroadenoma. Better cure rate can be achieved only when a dedicated group of multi-disciplinary team is involved.

The effectiveness and short-term safety of recombinant human GH (r-hGH) in acromegaly patients with GH deficiency (GHD) after treatment are not well established. The study includes ten subjects with acromegaly who had GHD treated with r-hGH for 6 months. Control groups consisted of ten age-, gender-, and BMI-matched healthy subjects and ten active acromegaly patients who were treatment naïve. Body composition, quality of life (QoL), muscle strength, lipid profile, and cardiovascular risk factors were assessed in all subjects at baseline, and the same parameters were reassessed after 6 months of therapy with r-hGH in acromegaly with GHD. Repeat magnetic resonance imaging of the sella was performed in treated subjects. Optical colonoscopy was done and biopsies were taken from multiple sites for proliferation indices (Ki67). The median duration of GHD was 17.8 months and dose of r-hGH administered was 5.7±1.5 μg/kg per day. There was improvement in bone mineral content (P=0.01), bone mineral density (P=0.04), muscle strength (P<0.001), total cholesterol (P=0.003), high-density cholesterol (P<0.001), and QoL - score (P=0.005), and reduction in low-density cholesterol (P=0.003) and triglyceride (P=0.004) after treatment. There was no change in lean body mass, total body fat, hsCRP, lipoprotein (a), and fibrinogen levels. There was a modest increase in plasminogen activator inhibitor 1 (P=0.002), but it was lower compared with healthy controls and treatment naïve acromegalics (P=0.007). Six month-r-hGH therapy improves body composition, atherogenic lipid profile, QoL, and muscle strength in GHD patients who had acromegaly. Long-term prospective studies are needed to evaluate the effect of r-hGH therapy in these patients., (© 2015 The authors.)

The coexistence of a pituitary neoplasm and pheochromocytoma is a rare condition, which may be another undefined variant of Multiple endocrine neoplasia (MEN) syndrome. Moreover, the coexistence of pituitary macroadenoma and multiple paragangliomas is more uncommon and only few authors have reported these findings. We are reporting the use of Ga DOTATATE PET/CT in a rare case of coexisting pituitary macroadenoma and multiple paragangliomas.

Internal carotid artery (ICA) bifurcation aneurysms are relatively uncommon and frequently rupture at a younger age compared to other intracranial aneurysms. We have treated a total of 999 patients for intracranial aneurysms, of whom 89 (8.9%) had ICA bifurcation aneurysms, and 42 of the 89 patients were 30 years of age or younger. The present study analyzed the clinical records of 70 patients with ICA bifurcation aneurysms treated from mid 1997 to mid 2003. Multiple aneurysms were present in 15 patients. Digital subtraction angiography films were studied in 55 patients to identify vasospasm and aneurysm projection. The aneurysm projected superiorly in most of these patients (37/55, 67.3%). We preferred to minimize frontal lobe retraction, so widely opened the sylvian fissure to approach the ICA bifurcation and aneurysm neck. Elective temporary clipping was employed before the final dissection and permanent clip application. Vasospasm was present in 24 (43.6%) of 55 patients. Forty-eight (68.6%) of the 70 patients had good outcome, 14 (20%) had poor outcome, and eight (11.4%) died. Patients with ICA bifurcation aneurysms tend to bleed at a much younger age compared to those with other intracranial aneurysms. Wide opening of the sylvian fissure and elective temporary clipping of the ICA reduces the risk of intraoperative rupture and perforator injury. Mortality was mainly due to poor clinical grade and intraoperative premature aneurysm rupture.

The authors report a case of primary brainstem tethering, a component of a spinal dysraphic state, occurring in a 13-year-old girl. This patient also had associated hydrocephalus, a low-lying tethered cord, and a syrinx in her conus medullaris. The significance of imaging surveys of the craniospinal region and head in a case involving a low-lying tethered cord is highlighted. The insertion of a ventriculoperitoneal shunt, leading to traction on the tethered brainstem, proved nearly fatal for this patient. In cases involving a multiple-level tethered cord, the sequence of detethering should be decided on with caution. It seems prudent to detether the vital neural structure first, so as to minimize the traction force on that structure. All four previously reported cases of secondary tethered brainstem and cerebellar vermis in the literature are reviewed and compared with the present case of primary brainstem tethering. The literature describing brainstem dysfunction caused by ventricular drainage is also reviewed.