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On one side, pregnancy is a bliss, a beautiful journey for most women while on other, it increases the risk of several diseases which may cause considerable morbidity and mortality in young women in the most productive period of their lives. Neurological emergencies in pregnancy often have grave prognosis and so, must be promptly diagnosed and treated. This article reviews the clinical features and management of some of the common severe neurological emergencies in pregnancy.

Marfan syndrome is an autosomal dominant systemic disorder of the connective tissue. Marfan syndrome affects most organs and tissues, especially the skeleton, lungs, eyes, heart, and the large blood vessels. Eye involvement may be in the form of retinal detachment which is a potentially dangerous manifestation for its sight threatening nature .We report a case where a 17 year old male developed sudden blindness due to spontaneous bilateral retinal detachment. Examination revealed features of Marfan syndrome and was stamped as a case of Marfan syndrome by Ghent criteria. The point to stress upon is that a young male developing spontaneous retinal detachment, a diagnosis of underlying Marfan syndrome should be kept in mind if appropriate clinical stigmata are present.

Introduction: Primary Intraventricular Haemorrhage (PIVH) is defined as bleeding in the ventricular system without a discernable parenchymal component or arising within 15 mm from the ventricular wall. PIVH is rare in clinical practice. Due to its rarity clinical and radiological factors affecting the outcome have not been widely studied. Aim: To evaluate clinical features, aetiology and predictors of outcome in PIVH. Materials and Methods: Forty patients with PIVH evaluated and treated at Bangur Institute of Neurosciences, Kolkata, West Bengal, India, from March 2015 to October 2016. PIVH was diagnosed as haemorrhage in the ventricles only, detected by Computed Tomography (CT) scan without evidence of intraparenchymal, Subarachnoid Haemorrhage (SAH), and Intraventricular Haemorrhage (IVH) associated with trauma. MR angiography was done in all patients as well as Digital Subtraction Angiography (DSA) was done in 34 patients. Categorical and continuous data were analysed using SPSS version 20.0.1. Results: A total of (n=16, 40%) patients were females and (n=24, 60%) were males. Headache was the most common mode of presentation (n=12, 30%). Hypertension was the most common predisposing factor (n=24, 60%) followed by Arterio Venous Malformations (AVMs) (n=10, 25%), Moyamoya Disease (MMD), (n=8, 20%), aneurysm (n=5, 12.5%), vaso occlusive disease (n=4, 10%). Seven patients developed hydrocephalus and died, while the rest 33 survived. Aetiology remained unidentified in 10 patients. Conclusion: Hypertension is an important predisposing factor in PIVH. Hydrocephalus is a common complication. There is a need for an appropriate work-up on patients with PIVH, as most cases are potentially treatable.

Background The intracerebral hemorrhage (ICH) score is well established as a reliable prognostic score in ICH, whereas recently, Acute Physiology and Chronic Health Evaluation II (APACHE II) has been observed to have a better discrimination in predicting mortality in primary pontine hemorrhage. Further, physiological parameters of APACHE II have been associated with outcome in ICH. This study is the first to observe a direct comparison between APACHE II and ICH scores in predicting 30-day mortality in spontaneous intracerebral hemorrhage (SICH). Materials and Methods This study was a prospective observational study where we compared the receiver operating characteristic (ROCs) of baseline ICH and APACHE II scores in patients with SICH for predicting 30-day mortality outcome. Results We observed that both APACHE II and ICH scores were good for predicting 30-day mortality with both having an area under the ROC curve of more than .8 (.831 [95% confidence interval {CI}, .740-.922; P .001] and .892 [95% CI, .757-.932; P .001], respectively). However, the ICH score was better discriminative (area under the curve AUC, .892 versus .831; P = .040) and better calibrated ( P = .037 versus P = .089, Hosmer–Lemeshow goodness-of-fit test for logistic regression) for the same. Both APACHE II and ICH scores had a sensitivity of 87% at cutoff values of 19 and 3, respectively; however, the ICH score had a better specificity (90% versus 76.5%). Conclusion The ICH score was observed to have a better discrimination and calibration for predicting 30-day mortality in SICH.

It is very rare for the primary presentation of a gastric malignancy to be with bone metastases. Also it is equally rare for MAHA to be a presenting feature of gastric adenocarcinoma as a paraneoplastic syndrome. This is a case report is 55years old female who presented with widespread vertebral deposits with diffuse bone marrow infiltration along with Coomb’s negative haemolytic anemia with thrombocytopenia and schistocytes in peripheral blood typical of MAHA. The combination of MAHA and bone infiltration in gastric adenocarcinoma is a very rare entity.

Kikuchi disease is a rare benign self limited lymphadenopathy. It is often associated with Systemic Lupus Erythematosus which can be diagnosed before; at the same time or after a diagnosis of Kikuchi disease is made. Again SLE is usually associated with APLA syndrome. Furthermore both SLE and Kikuchi disease can be complicated by HPS. We present a unique case in which Kikuchi disease and SLE were diagnosed together and disease course was complicated by Hemophagocytic syndrome (HPS). SLE was also associated with Anti Phospholipid Antibody Syndrome (APS) in this patient.

Wegener’s granulomatosis or granulomatosis with polyangiitis is a necrotizing vasculitis affecting both arterioles and venules. The disease is characterized by the classical triad involving acute inflammation of the upper and lower respiratory tracts with renal involvement. However, the disease pathology can affect any organ system. This case presents Wegener’s granulomatosis presenting with facial nerve palsy as the first manifestation of the disease, which is rarely reported in medical literature.

Myotonic dystrophy (MD) or Steinert's disease is a rare cause of chronic diarrhoea and anal incontinence. We present the case of a 37 year old man who presented with chronic diarrhoea and faecal incontinence as the initial symptoms to our outpatient clinic and was later diagnosed with MD. In the presence of chronic diarrhoea and faecal incontinence with muscle weakness, neuromuscular disorders such as myotonic dystrophy should be considered in the differential diagnosis.

Erasmus syndrome is a rare entity in which systemic sclerosis develops following exposure to silica with or without silicosis. Few case reports are available in literature. We report here a case of Erasmus syndrome in a 42-year-old manual labourer. The patient presented with arthralgia, Raynoud’s phenomenon, skin tightening and microstomia along with features of Interstitial Lung Disease (ILD) and pulmonary arterial hypertension. Evidence of Interstitial Lung Disease (ILD) with mediastinal lymphadenopathy as well as pulmonary arterial hypertension with vascular reactivity was found in appropriate investigations. Serological markers of systemic sclerosis were strongly positive. After a diagnosis of Erasmus syndrome was made, a combination of drugs including Prednisone, Cyclophosphamide and Nifedipine was instituted this led to moderate improvement in his symptoms over 6 months.

Metronidazole can produce neurological complications although it is not a common scenario. We present a case where a patient developed features of encephalopathy following prolonged metronidazole intake. Magnetic resonance imaging (MRI) brain showed abnormal signal intensity involving both dentate nuclei of cerebellum and splenium of corpus callosum. The diagnosis of metronidazole toxicity was made by the MRI findings and supported clinically.

Enteric fever (or typhoid) is a common infectious disease worldwide, especially in developing countries like India. We report a case of a 27 year old patient who presented with fever and abdominal pain and was eventually diagnosed as having enteric fever complicated by acute pancreatitis. Enteric fever presenting as acute pancreatitis is a rare entity and must be considered in endemic areas like India, in patients presenting with fever and concomitant severe abdominal pain as any delay in timely institution of treatment can prove fatal. There are very few case reports in the world which have depicted this unusual association.

Wilson's disease can manifest as neurological disorder without hepatic involvement. However, first presentation as status epilepticus is extremely rare. Herein, we report a case where a 21-year-old male presented with status epilepticus. Clinical background, biochemical tests and typical magnetic resonance imaging findings in the form of Face of giant Panda sign confirmed the diagnosis of Wilson's disease.

Interstitial lung disease (ILD) is regarded as an unusual manifestation of systemic lupus erythematosus (SLE). ILD in SLE patients usually develop in long-standing disease course and follows a chronic course. Isolated presentation of ILD in SLE without another involvement is quite striking. We report such a unique case where patient′s first presentation in SLE was ILD.

Motor neuron diseases(MND) have been reported as a rare paraneoplastic syndrome (PNS) of a systemic neoplasm.Amyotropic Lateral Sclerosis as a paraneoplastic manifestation of gastric carcinoma is even rarer. We present a patient with amyotrophic lateral sclerosis (ALS) in association with adenocarcinoma of stomach. A 48year old man presented with a four months history of progressive dysphagia, spastic dysarthria and marked fasciculation in his atrophic tongue. Gag reflexes were diminished bilaterally. There was significant atrophy in thenar and hypothenar areas of both hands and dorsum of both feet.Electromyography result was compatible with diffuse motor neuronopathy with active denervation.MRI brain showed classical findings of ALS. Upper GI endoscopic study showed ulcerated mucosa in body of stomach. Histological biopsy of stomach confirmed the presence of adenocarcinoma. The importance of considering a paraneoplastic syndrome in a patient with presentation of ALS is that it can lead to searching for underlying neoplasm before its apparent signs and symptoms develop and a scope to initiate treatment for primary carcinoma. Again treating the underlying neoplasm may halt or even resolve the neurologic signs and symptoms.

Multiple myeloma with isolated third nerve palsy as the presenting complaint is a rare entity. We report a case of a 30-year-old man who developed sudden onset left-sided complete abducens nerve palsy without pain. Cranial and orbital magnetic resonance imaging and cerebrospinal fluid examination demonstrated no abnormalities. He was diagnosed as having IgG type multiple myeloma, based on the immunological and pathological investigation. After instituting chemotherapy, palsy resolved within 3 weeks. Etiology is not established beyond doubt but probably palsy was caused by nerve ischemia due to hyperviscosity of the serum.

Postpartum pituitary necrosis, or Sheehan\'s syndrome, is the infarction of the pituitary gland during the peripartum period. Although hyponatremia and normocytic, normochromic anaemia are documented features of this condition, pancytopenia is an extremely rare manifestation of this condition. We report a case where a patient presented with severe hyponatremia along with pancytopenia in the background of Sheehan\'s syndrome.

Tuberous Sclerosis Complex (TSC) is a neurocutaneous disorder characterised by hamartoma formation in multiple organs, particularly the skin, brain, eye, kidney and heart. It is rare for TSC to manifest as intractable seizures without cutaneous manifestations. Targeted history elicitation along with appropriate systemic examination backed by proper investigations in high index of suspicion is mandatory to diagnose a case of TSC in such scenario. We report such a case where a 27 year old male was diagnosed with TSC following admission with refractory seizures.

Krabbe”s disease (KD) or Globoid cell leukodystrophy (GLD) is a disorder involving the white matter of both the central and peripheral nervous systems. It is caused by congenital deficiency of a lysosomal enzyme, galactosylceramidase I, which is responsible for hydrolyzing the galactolipids in myelin. The disease is extremely rare occurring only one in 100,000 to one in 200,000 live births. The disease is classically of infantile origin but it can also occur in older children as well as adults. Adult variant is the rarest type. We hereby report a 34 year old male patient with progressively increasing ataxia, dysarthria. Typical MRI brain changes and diminished leucocyte galactocerebrosidase (GALC) enzyme levels clinched the diagnosis of Krabbe”s disease . There are very few cases of KD reported from India. This case report is to stress upon the fact that this rare entity should not be missed in an appropriate clinical setting.

A 6 month old girl presented with generalized hyperkeratosis, most marked over the flexures since birth. On the basis of the clinical& histopathologic findings, she was diagnosed as a case of epidermolytic hyperkeratosis .She was treated with retinoid therapy. Epidermolytic hyperkeratosis (EHK) is an uncommon form childhood keratinizing disorder. Early in life it is associated with generalized blistering and erythroderma. Later on, it produces rippled type of hyperkeratosis. The purpose of this report is to highlight the features of this rare case.

Objective: Unplanned Caesarean section (CS) constitutes a very stressful event, and is associated with traumatic birth experience. Especially feelings of helplessness, poor information and communication are reported as distressing factors in unplanned Caesarean birth. Addressing these stressors by offering timely patient education and counselling, might be a way to improve sense of control and reduce anxiety. This study compared anxiety and sense of control between women who had a planned caesarean section (PCS) and patients who had an unplanned/secondary caesarean section (SCS). Methods: The sample consisted of 129 women (PCS=66; SCS=63). It was hypothesized that the PCS group benefitted more from patient education than the SCS group leading to lower levels of anxiety and a higher sense of control. Possible confounding factors were controlled for (example, previous child birth experiences and the patient’s locus of control). Anxiety and sense of control were measured by Visual Analogue Scales intraoperatively and one day postoperatively. The Control Preferences Scale and the Multidimensional Health Locus of Control assessed the discrepancy between desired - perceived control and the locus of control, respectively. By means of a checklist it was registered whether subjects had an adequate comprehension of the patient education provided. Multiple regression analyses were used to analyze the data. Results: Intraoperative sense of control during SCS was scored significantly lower as compared to PCS. A higher sense of control was associated with significantly lower anxiety scores in both groups. Furthermore, a more internal locus of control was related to a greater discrepancy between desired and perceived control. Additional patient education in the PCS condition seemed to increase the sense of control. Conclusion: Patient education about the Caesarean procedure might benefit the psychological well-being of the mothers as it lowers anxiety scores indirectly via a heightened sense of control.

Background: Dengue fever, the most common arbo viral illness transmitted worldwide. Dengue infection in adolescents and adults is also a potential hazard in endemic areas, especially South-east Asia. Objectives: The present study was done to evaluate the magnitude of dengue death and its predictor factors in a tertiary care hospital of Kolkata. Materials & Methods: This observational cross sectional study was conducted at a tertiary care centre of Kolkata over 1 year period with 77 patients with a diagnosis of dengue fever based on the serology positive for dengue IgM with fulfilling the inclusion and exclusion criteria. The data were collected on predesigned questionnaire which include gender, clinical findings, complications and laboratory test.Baseline laboratory tests obtained were complete blood count (CBC), partial thromboplastin time (PTT), serum glutamine pyruvate transaminase (SGPT),SGOT, bilirubin, urea, creatinine and IgM. SPSS 16.0 version was used for statistical analysis. Results: Dengue mortality rate was 28.57% in this study. Among 5 lab parameters chosen i.e.leukopenia, bilirubin>6mg/dl, thrombocytopenia, altered PT/APTT, enzyme>4 fold rise,raised urea/cr ; On multivariate logistic regression analysis hyperbilirubinemia(>6 mg/dl)was statistically significant predictor for mortality(as p value