Subrata Chakrabarti

Cronkhite-Canada Syndrome (CCS)—A Rare Case Report

Cronkhite-Canada syndrome (CCS) is an extremely rare non-inherited condition characterized by gastrointestinal hamartomatous polyposis, alopecia, onychodystrophy, hyperpigmentation, weight loss and diarrhoea. The aetiology is probably autoimmune and diagnosis is based on history, physical examination, endoscopic findings of gastrointestinal polyposis, and histology. The disease is very rare; approximately 450 cases of CCS have been reported worldwide. The author reports a case of CCS in an elderly Indian male.

Amyotrophic lateral sclerosis as sole neurological manifestation of human immunodeficiency virus

Introduction: As more and more people with human immunodeficiency virus (HIV) live longer and enjoy healthier lives because of antiretroviral therapy, there are an increasing number of sexual transmissions of HIV. It is exceedingly important to assess the proportion of unsafe sexual practices among people living with HIV/AIDS (PLWHAs) and their disclosure status so that behavioral intervention can be designed optimally for them in order to curb secondary HIV transmission and potential reinfection with different or drug resistant strain of HIV. Aim: To assess sexual behavior and serostatus disclosure of PLWHAs attending antiretroviral therapy (ART) center in Vadodara. Settings and Design: The current cross-sectional study was carried out at ART center of a tertiary care hospital - Shree Sayaji General (SSG) Hospital, Vadodara after taking necessary permissions and approval from institutional review board (IRB). Materials and Methods: A semistructured questionnaire was used for interview with PLWHA who are on ART after taking written and informed consent. Data was entered and analyzed using Epi Info software. Results: A total of 175 PLWHAs were enrolled in this study. Forty-three percent of the PLWHAs practiced premarital sex, while 15% of them practiced extramarital sex (EMS). Nearly 90% of these sexual practices were unsafe. Fifty-eight percent of the PLWHAs continued these unsafe sexual practices even after HIV diagnosis. Nearly 20% of the PLWHAs did not receive any counseling regarding sexual behavior. Ninety-five percent of respondents had disclosed their serostatus to their spouse. Conclusions: Still a remarkable proportion of PLWHAs indulge in unsafe sexual practices and the most common reason encountered among them was desire for child. Although majority of the participants had disclosed their serostatus to their spouse, many respondents did not mention their serostatus to other sexual partners. There is a need to stress on the importance of safe sex among PLWHAs not only to prevent transmission to their partners, but also to help them avoid receiving potentially resistant HIV strains in case of seroconcordant couples. A need-based sexual behavior-related motivational counseling needs to be focused on.Introduction: This study was carried out among people living with human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS) receiving antiretroviral therapy (ART) in a tertiary health facility in Nigeria and it is aimed at determining practice of adherence among these patients. Materials and Methods: A total of 282 HIV positive patients receiving ART in Nnamdi Azikiwe University Teaching Hospital, Nnewi were selected by systematic random sampling technique and using a semi-structured, interviewer administered questionnaire, responses were elicited from them on knowledge and perception of ART and adherence. Practice of adherence was determined by self-report. Findings were analyzed using Statistical Package for the Social Sciences version 17 (IBM Corp, Chicago Illinois, USA). Results: Most of the respondents were females (66.3%) and their mean age of the respondents was 36.63 ΁ 8.56 years. Most of the respondents have good knowledge of ART and adherence, even though most of them (64.2%) thought that ART cures HIV/AIDS. Most patients still considered HIV/AIDS as a serious illness (78.0%). Only about half of them (50.7%) were of the opinion that ART reduces the risk of HIV transmission. However, the general perception of adherence was good. The mean level of adherence was 97.8%, however, about 86% of them attained optimal adherence level. Conclusion: The study revealed fairly good knowledge and perception of ART and adherence among the patients. The level of ART adherence was also high; however there is a need to address some gaps in knowledge identified as part of measures aimed at improving adherence in the long run.Context: Disclosing ones human immunodeficiency virus (HIV) status helps in reducing the spread of the disease. Intimate partners are encouraged to reveal their status to each other. Such strategies aid in determining discordance and also target proven interventions to the correct demographic. Objective: The objective was to determine the rates of discordance among HIV positive pregnant women and their partners attending Nnamdi Azikiwe University Teaching Hospital, Nnewi. Materials and Methods: Partners of HIV positive pregnant women who had been notified of their spouses status were invited, counseled, and those who accepted, were tested for HIV using the national testing algorithm. Pre- and post-test counseling was offered to all tested partners. Results: A total of 10,070 new patients booked for antenatal care during the study period. Eight thousand nine hundred and seventy-nine (89.2%) of them were counseled, with 8317 (92.6%) of them accepted to do the HIV test. Seven hundred and ninety-seven women tested positive giving a sero-prevalence of 9.6%. Although 84.4% (673) of the positive mothers stated their intent to notify their partners, only 21% (141) of the partners presented for counseling and testing. On the whole, 98.6% (139) of the partners who presented accepted the test with 45.3% (63) of them testing positive, giving a sero-discordance rate of 54.7%. Conclusion: The discordance rate among tested partners is high in our center and suboptimal counseling, and testing rates were observed in the male partners. Interventions aimed at increasing partner involvement should be devised and adapted for our region. This will yield more data, give a truer picture of discordance rates as well as the aid in better management of scarce resources in program implementation.Opportunistic fungal infections account for a significant amount of morbidity associated with human immunodeficiency virus (HIV) disease. Oral candidiasis is one of the earliest premonitory signs of HIV infection and its diagnosis may have grave prognostic implications for the eventual development of full blown acquired immunodeficiency syndrome (AIDS). It is considered as an important marker of immune suppression and may be the initial manifestation of the disease in about 10% of HIV-infected adults. Careful history taking and detailed examination of the patients oral cavity are important parts of the physical examination, and diagnosis requires appropriate investigative techniques. Early recognition, diagnosis, and treatment of HIV-associated oral lesions may reduce morbidity. This review is intended to provide information on clinical variants of oral candidiasis and management as dental care providers are likely to be among the first to recognize such manifestations.Motor neuron diseases (MND) are extremely rare neurological manifestations of human immunodeficiency virus (HIV). We report a case of a 33-year-old known HIV seropositive male who presented with progressive asymmetrical onset of weakness and wasting of both distal limbs along with dysphagia and difficulty in speech. Examination revealed significant atrophy with visible fasciculations in thenar and hypothenar areas of both hands and dorsum of both feet associated with brisk deep tendon reflexes and jaw jerks, but diminished gag reflexes. Electromyography revealed evidence of denervation pattern. Investigations for underlying causes of MND other than HIV were noncontributory. Administration of riluzole along with continuation of antiretroviral therapy brought significant relief to his limb weakness, but bulbar features continued to progress. The case report highlights the rare, but definite association between HIV and amyotrophic lateral sclerosis (ALS) and partial reversibility of disabling clinical features on highly active antiretroviral therapy, which mandates ruling out HIV in all cases of ALS.through implementation of option B+ would have various other benefits. The total fertility rate in Malawi is high, around five to six births per woman, which is unlikely to be much lower in HIV‑infected women. Soon after the breastfeeding period (median duration 23 months) many women become pregnant again. Thus, a stop‑start approach to ART administration is almost redundant. Many women present for antenatal care late in pregnancy—an estimated 50% are thought to attend after 28 weeks of gestation—and continuing prophylaxis with antiretroviral drugs would mean that the next pregnancy could be protected from conception. The stopping of ART after cessation of breastfeeding might lead to viral rebound, with the risk of transmission to a sexual partner or fetus being notably raised. In women in Zimbabwe even those with CD4 cell counts higher than 350 cells per μL had a risk of death around six times higher than that in noninfected women within 24 months post‑partum [2,3] , and early ART could reduce mortality by 50‑90%. [4] Prevention

Guillain-Barre Syndrome Following Falciparum Malaria:A Rare Association -

Malarial parasite as a cause of Guillain-Barre syndrome (GBS) is very rare and there are only a very few case reports in literature. Here we report a case of acute flaccid quadriparesis with generalised areflexia following malaria caused by P.falciparum.An adult male developed acute lower motor neuron type of quadriparesis following an episode of microscopically proven Plasmodium falciparum malaria. Clinical examination, cerebrospinal fluid analysis, and nerve conduction studies confirmed the diagnosis of GBS, depicting the rare association of GBS following falciparum malaria

A curious case of Fever and hyperpigmentation.

Megaloblastic anaemia as a cause of pyrexia is a rare entity. Similarly, hyperpigmentation of skin has rarely been reported as the presenting manifestation of folate and/or vitamin B12 deficiency. The author reports the case of a patient who presented with fever and hyperpigmentation and was diagnosed to have megaloblastic anaemia secondary to vitamin B12 and folate deficiency after other infective, inflammatory/autoimmune, endocrine causes of pyrexia and hyperpigmentation were excluded by appropriate investigations. The patient responded remarkably well to the treatment with vitamin B12 and folic acid supplementation. Although presentation of megaloblastic anaemia as isolated fever or hyperpigmentation are noted in literature, simultaneous fever and hyperpigmentation as its initial presentation is exceedingly rare.

Erasmus Syndrome in a 42-Year-Old Male: A Rare Case Report.

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, Raynouds 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.

Enteric Fever Presenting as Acute Pancreatitis

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.

First presentation of systemic lupus erythematosus as interstitial lung disease: A unique scenario

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.

Acute Myocardial Infarction Following Viper Bite: A Rare Scenario

Acute myocardial infarction (AMI) is a rare complication of snake bite with few reported cases in literature. The author reports a case of a 32-year-old male who developed an anterior wall AMI several hours after a Russell′s viper bite. The diagnosis of myocardial infarction was confirmed by a characteristic retrosternal chest pain, typical electrocardiographic changes, and elevated Troponin I levels. The patient had no risk factors for coronary artery disease and the coronary arteries were normal on cardiac catheterization. The importance of the case report is to highlight upon the fact that physicians, especially from developing countries like India should be aware of this potentially life-threatening complication of viper bite and take immediate steps for its recognition and management.

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.

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 (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.