Sheehan syndrome associated with pancytopenia - A rare entity.

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.

Amyotropic lateral sclerosis as a para-neoplastic manifestation of gastric adenocarcinoma - A rare case report.

Motor neuron diseases (MND) have been reported as a rare paraneoplastic syndrome of a systemic neoplasm. Amyotrophic 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 neuropathy 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.

An interesting case of tuberous sclerosis without cutaneous manifestations presenting with only neurological features.

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.

Multiple myeloma presenting as isolated sixth cranial nerve palsy.

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.

Isolated oculomotor nerve palsy as initial presentation of tuberculous meningitis.

Tuberculous meningitis (TB meningitis) is a subacute or chronic meningitis with diversemanifestations. Isolatedoculomotor nerve palsy as initial manifestation is rare, which often makes early diagnosis difficult. The present case report demonstrates a patient with TB meningitis, who presented with isolated left oculomotor nerve palsy. A 40 year-old male presented with gradual onset left side ptosis. Neurologicalexamination revealed isolated left oculomotor nerve palsy. Brain magnetic resonance imagingand cerebral angiography showed no diagnostic finding. Cerebrospinal fluid (CSF) study and the polymerase chain reaction(PCR) on CSF confirmedthe diagnosis of TB meningitis. Thisreport is to highlight the fact that TB meningitis should be kept in differential diagnosis for isolated third cranial nerve palsy especially in endemic countries like India.

Epidermolytic hyperkeratosis: a rare case.

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.

Krabbe's disease in a 28 year old adult - A rare case report .

Krabbe 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 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.

Predictors of dengue mortality in a tertiary care hospital at Kolkata: A cross sectional study.

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. Aims & Objective: The present study was done to evaluate the magnitude of dengue death and its predictor factors in a tertiary care hospital of Kolkata. Material and 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 > 6 mg/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 < 0.05). Conclusion: The laboratory parameters could predict the dengue mortality, so proper emphasis should be given to address the situation.