[show abstract][hide abstract] ABSTRACT: Importance: Allgrove syndrome is a rare autosomal recessive disorder characterised by achalasia, alacrima, adrenal insufficiency, autonomic dysfunction and amyotrophy. The syndrome has been described in childhood and adult presentation, as in our case, is very rare. There is a considerable delay in diagnosis due to lack of awareness about the syndrome. Observations: We report a single case of a 36year old man who was initially diagnosed and treated for achalasia cardia in our institute 14years before. After 8years he presented again with weakness and wasting predominantly distally. He had tongue fasciculations, brisk reflexes and extensor plantar. After supportive electrophysiological studies he was diagnosed as Amyotrophic lateral sclerosis. After 5years he presented with generalised fatigue without any significant worsening of his neurological status. On reevaluation he had alacrimia, autonomic dysfunction and mild ACTH resistance. Conclusions and relevance: Allgrove syndrome may be an underdiagnosed cause of multisystem neurological disease due to the heterogeneous clinical presentation as well as for ignorance of clinician about the syndrome. Based on our case, we also believe that there does exist a subgroup of patients who follow a less severe and chronic course. Recognition of syndrome allows for treatment of autonomic dysfunction, adrenal insufficiency and dysphagia.
Journal of the Neurological Sciences 10/2013; · 2.24 Impact Factor
[show abstract][hide abstract] ABSTRACT: A 33-year-old man with bipolar affective disorder presented with acute onset of rigidity, tremors, and confusion. He had been taking oral lithium (1,200 mg daily) for the past 2 months. Baseline blood, EEG, and CSF analyses were normal. Serum lithium level was 0.67 mEq/L (0.3-1.3). Gadolinium MRI brain showed multiple bilateral symmetric T2-weighted hyperintensities (figure, A-C). On suspicion of lithium-induced neurotoxicity, lithium was replaced with valproate. At 1-month follow-up, his extrapyramidal symptoms had resolved completely, with significant resolution noted on cranial MRI (figure, D-F). Paradoxical lithium neurotoxicity occurs at therapeutic or low serum levels due to lithium-induced toxic demyelination.(1-3.)
[show abstract][hide abstract] ABSTRACT: The present study was intended to explore the dynamics of viral and host factors determining the outcome of Japanese encephalitis viral infection. 223 patients with acute encephalitic syndrome, 126 with febrile illness suspected of JE and 79 apparently healthy individuals as control were enrolled. Elevated levels of TNF-α and IL-6 in encephalitis patients and IFN-γ in febrile JE patients without encephalitis were observed. A cutoff value of >55pg/ml of TNF-α and >370pg/ml of IL-6 in CSF was found as poor prognostic marker. Th1 shift (IFN-γ/IL-4: >1) was observed in encephalitis patients.
Journal of neuroimmunology 08/2013; · 2.84 Impact Factor
[show abstract][hide abstract] ABSTRACT: Brucellosis remains an important public health problem especially in the underdeveloped countries as well as the Middle East. It may be a "master mimic" leading on to grave diagnostic dilemmas. Chronic neurobrucellosis is seldom associated with signs and symptoms of toxaemia due to multi organ dysfunction. We report the case of a 23-year-old man who presented with fever of 8weeks associated with ascending weakness of lower limbs and sensorineural type of hearing loss of 6weeks duration with prominent systemic findings in the form of hepatosplenomegaly and significant loss of weight. He responded well to treatment with combination antimicrobials with clinicoradiological resolution after 6months of therapy. Even though a rare complication of brucellosis, neurobrucellosis causes significant morbidity if not promptly recognised and treated. Favourable outcomes can be achieved in neurobrucellosis with appropriate protracted polymicrobial antibiotic therapy as was illustrated in our patient.
Journal of the neurological sciences 06/2013; · 2.32 Impact Factor
[show abstract][hide abstract] ABSTRACT: Central nervous system (CNS) infections caused by non tuberculosis Mycobacteria (NTM) are believed to be rare. In developing countries, these are also likely to be missed either due to lack of awareness or lack of facilities to diagnose them. We are reporting M. avium CNS infections in 13.6% (12/114) of the HIV patients visiting our tertiary care centre in North India. This is one of the largest series of M. avium CNS infection from a developing country. Our experience highlights the need to look for M. avium infection in HIV patients presenting with CNS infection and shows that molecular techniques like PCR are a beneficial tool in diagnosing them.
AIDS research and human retroviruses 02/2013; · 2.18 Impact Factor
[show abstract][hide abstract] ABSTRACT: Racemose cysticercosis is a less frequent presentation of neurocysticercosis (NCC). It's presentation and management is quite different from cerebral parenchymal NCC. Diagnosis of racemose cysticercosis is based on the combination of clinical, epidemiologic, radiographic, and immunologic information. Compared with cysticercus cellulose, which most commonly presents as seizures, racemose NCC due to its extraaxial location presents with raised intracranial pressure and meningitis, and frequently requires neurosurgical intervention. Dementia as a sole presenting feature of NCC is rare. We report a case of racemose NCC with dementia as the presenting manifestation. The outcome of dementia patients with NCC seems favorable in most cases therefore a high index of suspicion for NCC should be kept especially in endemic areas.
Annals of Indian Academy of Neurology 01/2013; 16(1):88-90. · 0.93 Impact Factor
[show abstract][hide abstract] ABSTRACT: Marchiafava-Bignami disease (MBD) is a rare complication of chronic alcoholism. Its clinical diagnosis has considerably changed during recent times, with MRI of the brain paving way for in life diagnosis. We believe that physicians need to have a high index of suspicion, because acute onset MBD is not always fatal and complete recovery is possible, provided the diagnosis is made early and treated appropriately. We report a case of MBD who was diagnosed early in the disease course with subsequent clinical and radiological recovery on institution of appropriate treatment.
[show abstract][hide abstract] ABSTRACT: Autism is a neurodevelopmental disorder, with a multifactorial etiology, characterized by severe abnormalities in communications, social awareness and skills, and the presence of restrictive and stereotyped patterns of behaviors. It is traditionally considered a "static" encephalopathic disorder without any specific cure and few effective biomedical interventions. There are various factors which are involved in the etiopathogenesis of autism or autism spectrum disorder (ASD) such as impaired immune responses, neuroinflammation, abnormal neurotransmission, oxidative stress, mitochondrial dysfunction, environmental toxins and stressors. The autism is often associated with a number of genetic disorders such as fragile X syndrome, tuberous sclerosis, epilepsy and Down syndrome. The recent approaches to autism treatment included various non-pharmacological and pharmacological therapy such as food supplementation, detoxification, treatment of neuroinflammation, immunologic treatments and psychotropic medications, which are found to be effective in treating various behavioral symptoms of autism. In current practice, there is no curative treatment for autism but the recommended treatment for autism involves educational therapies: speech therapy, sensory integration therapy, auditory therapy. There are classes of different pharmacological agents which are found to be effective in improving behavioral symptoms of ASD such as neurotransmitter reuptake inhibitors (fluoxetine), tricyclic antidepressants (imipramine), anticonvulsants (lamotrigine), atypical antipsychotics (clozapine), acetylcholinesterase inhibitors (rivastigmine), etc. New classes of drugs with novel mechanisms of action should be there so that this disorder will become less prevalent in the future.
[show abstract][hide abstract] ABSTRACT: Purpose: To assess the association between epilepsy and exposure to the parasites, Toxocara canis and Taenia solium in a slum-community in India. Methods: A door-to-door survey to determine the prevalence of epilepsy was carried out by trained field workers. For every case, one age- and gender-matched control was selected from the same community. Serologic evaluation was carried out to detect antibodies against T. canis and T. solium. Key Findings: The crude prevalence of active epilepsy was 7.2 per 1,000. We enrolled 114 people with active epilepsy and 114 controls. The prevalence of antibodies to T. canis was similar in people with active epilepsy (4.7%; 5 of 106 people) and in controls (5.7%; 6 of 106 people). The prevalence of antibodies to T. solium was 25.5% (27 of 106) in people with active epilepsy, significantly higher than in controls (12.3%; 13 of 106 cases; p = 0.02). Adjusted conditional (fixed-effects) logistic regression estimated an odds ratio of 2.8 (95% confidence interval 1.2-6.8) for detection of T. solium antibodies. Nineteen people with active epilepsy demonstrated evidence of neurocysticercosis (NCC) on magnetic resonance imaging (MRI), including 7 (36.5%) with solitary cysticercus granuloma. Significance: Our findings do not support an association between epilepsy and exposure to T. canis in the community studied. A significant association between T. solium exposure and epilepsy was observed. Of those with active epilepsy and evidence of NCC on MRI, a large proportion demonstrated solitary cysticercus granuloma.
[show abstract][hide abstract] ABSTRACT: M. tuberculosis (MTB) is an important cause of morbidity and mortality in India. However, in recent years, the significance of nontuberculous mycobacteria (NTM) has also increased after the onset of HIV/ AIDS epidemic. Meningitis due to M. avium is rare, but often responsible for fatal outcome as high as 60% of cases. Literature regarding the true prevalence of dual infection with MTB and M. avium is scanty and most of these cases are treated as drug resistant cases. We report here two cases of dual infection due to MTB and M. avium which had a fatal outcome. The paper highlights an urgent need for use of multiplex polymerase chain reaction in routine diagnostic laboratories. This would help in the diagnosis of co-infections due to NTMs, especially M. avium with MTB, in patients with HIV/AIDS and therefore assist in better patient management and outcome of the disease.
Journal of Medical Microbiology 08/2012; · 2.30 Impact Factor
[show abstract][hide abstract] ABSTRACT: More than three billion populations are living under the threat of Japanese encephalitis in South East Asian (SEA) countries including India. The pathogenesis of this disease is not clearly understood and is probably attributed to genomic variations in viral strains as well as the host genetic makeup. The present study is to determine the role of polymorphism of TNF-alpha promoter regions at positions -238G/A, -308G/A, -857C/T and -863C/A in the severity of Japanese encephalitis patients.
Total of 142 patients including 66 encephalitis case (IgM/RT-PCR positive), 16 fever cases (IgM positive) without encephalitis and 60 apparently healthy individuals (IgG positive) were included in the study. Polymerase chain reaction restriction fragment length polymorphism (PCR-RFLP) using site specific restriction enzymes were implemented for polymorphism study of TNF alpha promoter.
Following the analysis of the digestion patterns of four polymorphic sites of the TNF-alpha promoter region, a significant association was observed between the allele -308A and -863C with the patients of Japanese encephalitis.
TNF-alpha 308 G/A has been shown to be associated with elevated TNF-alpha transcriptional activity. On the other hand, polymorphism at position -863C/A in the promoter region has been reported to be associated with reduced TNF-alpha promoter activity and lower plasma TNF levels. As per the literature search, this is the first study to identify the role of TNF-alpha promoter in JE infection. Our results show that subjects with -308A and -863C alleles are more vulnerable to the severe form of JE infection.
[show abstract][hide abstract] ABSTRACT: To determine the effect of administration of a short course of prednisolone on seizure and radiologic outcome in patients with solitary cysticercus granuloma (SCG).
One hundred forty-eight subjects presenting with new-onset seizures (<15 days duration) and with SCG demonstrated on imaging studies were randomly allocated to either treatment with prednisolone (40-60 mg/day for 2 weeks) or placebo in addition to standard antiepileptic drug therapy. The subjects were followed up for seizure recurrence for 9 months. Repeat computed tomography (CT, at 3 months) and magnetic resonance imaging (MRI, at 6 months) to evaluate resolution and calcification of the lesion.
There was no difference in the proportion of subjects with seizure recurrence during the follow-up period in the treatment (n = 16, 21.9%) and control (n = 19, 25.33%) groups (p = 0.7). However, generalized seizures occurred in a significantly lesser proportion of subjects in the treatment group (n = 3, 15.79%) in comparison to the control group (n = 12, 60.00%) (p = 0.015). There were no significant differences in the proportion of subjects with complete resolution of the SCG on repeat CT at 3 months [treatment group (27, 46.7%) and control group (23, 39.8%); p = 0.453] and repeat MRI at 6 months [treatment group (28, 46.7%) and control group (21, 38.9%); p = 0.402].
The administration of a short course of prednisolone does not offer significant improvement in seizure control, although a benefit in terms of reducing the likelihood of generalized seizures is possible. Furthermore, it does not improve the chances of resolution of the SCG on follow-up imaging studies.
[show abstract][hide abstract] ABSTRACT: Risk of cerebrovascular disease is increased in patients with HIV infection; however, cerebral venous sinus thrombosis is not commonly reported in this condition. We report a case of young man with intracranial hemorrhage whose radiologic investigations revealed thrombosis of the left transverse and sigmoid sinuses extending into the left internal jugular vein. On further investigations, he was found to be positive for HIV-1, and his procoagulant workup showed protein S deficiency. He was started on anticoagulant therapy, after which he showed gradual recovery. This case highlights a rarely encountered presentation of HIV infection as cerebral venous sinus thrombosis.
The American journal of emergency medicine 03/2011; 30(3):517.e1-3. · 1.54 Impact Factor
[show abstract][hide abstract] ABSTRACT: A 24-year-old male presented with features of progressively worsening spastic quadriparesis of 5 years' duration with similar milder features in the younger brother. His neuroradiological investigations revealed diffuse thickening of posterior longitudinal ligament, ligamentum flavum, and duramater in the cervical spine causing severe canal stenosis with secondary ischemic cord changes. As both brothers had dysmorphic facial features, further work-up suggested the diagnosis of a rare familial form of myelopathy due to mucopolysaccharidosis VI.
The International journal of neuroscience 02/2011; 121(6):337-40. · 0.86 Impact Factor
[show abstract][hide abstract] ABSTRACT: A cerebrovascular accident, or stroke, is defined as the abrupt onset of a neurological deficit, which can be due to ischemia. Cerebral ischemia is caused by a reduction in blood flow that thereby decreases cerebral metabolism. Chronic cerebral hypoperfusion leads to irreversible brain damage and plays an important role in the development of certain types of dementia. Vinpocetine, chemically known as ethyl apovincaminate, is a vinca alkaloid that exhibits cerebral blood-flow enhancing and neuroprotective effects. Non-clinical and clinical studies have suggested multiple mechanisms responsible for the beneficial neuroprotective effects of vinpocetine. As no significant side effects related to vinpocetine treatment have been reported, it is considered to be safe for long-term use. This vasoactive alkaloid is widely marketed as a supplement for vasodilation and as a nootropic for the improvement of memory. The present review focuses on studies investigating the role of vinpocetine in cerebrovascular diseases.
[show abstract][hide abstract] ABSTRACT: Solitary cysticercus granuloma (SCG) is one of the most common forms of presentation of neurocysticercosis (NCC). The diagnostic workup and management approach to this condition remain uncertain and controversial.
To review evidence and develop a consensus approach to the diagnosis and treatment of SCG.
A multidisciplinary expert group meeting was convened in order to review and discuss various aspects of management of patients with SCG. Evidence reviewed was classified and a consensus was evolved according to standard protocols.
SCG is commonly recognized on CT as an enhancing lesion measuring <20 mm. Further evaluation with MRI does not add much information. The use of antihelminthic agents (specifically, albendazole in combination with corticosteroids) and corticosteroids alone have been shown to improve radiologic resolution and seizure outcome in patients with SCG. However, the sizes of the effects are modest. By convention, all patients with SCG presenting with seizures are initiated on antiepileptic drugs (AEDs). Available evidence suggests that withdrawal of AEDs after complete resolution of the SCG is safe. There is a high risk of seizure relapse after AED withdrawal in patients with calcific residue following resolution of the SCG. The duration of AED prophylaxis in these individuals is unclear.
It is desirable to have large, multicenter trials with sufficiently long follow-up, comparing outcomes with the use of antihelminthics with or without corticosteroids and corticosteroids alone in order to dissect out the benefits accrued due to each of these classes of drugs.
[show abstract][hide abstract] ABSTRACT: To evaluate the association between diabetic cystopathy (DC) and neuropathy (autonomic and peripheral) in patients with diabetes mellitus (DM) presenting with lower urinary tract symptoms (LUTS).
Men with DM who presented with bothersome LUTS were enrolled from January 2008 to June 2009. Their demographic and clinical profiles were noted. Multichannel urodynamic studies were performed using the Solar Silver digital urodynamic apparatus. Hand and foot sympathetic skin responses, and motor and sensory nerve-conduction velocity studies were performed using the Meditronic electromyographic/evoked potentials system.
A total of 52 men (mean age 61.3 ± 12.1 years, DM duration 11.0 ± 7.5 years) completed the study protocol. Of these 52 men, abnormal sympathetic skin responses, motor and sensory nerve-conduction velocity studies, and combined neuropathy (all 3 tests abnormal) were noted in 80.7% 57.7%, 57.7%, and 51.9%, respectively. Urodynamic studies showed impaired first sensation (>250 mL), increased capacity (>600 mL), detrusor underactivity, detrusor overactivity, high postvoid residual urine volume (more than one third of capacity), and bladder outlet obstruction (Abrams-Griffiths number >40) in 23.1%, 25.0%, 78.8%, 38.5%, 65.4%, and 28.8% of the men, respectively. Both sensory and motor DC correlated with abnormal motor and sensory nerve-conduction velocity studies (P = .015 and P = .005, respectively). Only motor DC correlated with abnormal sympathetic skin responses (P = .015). The correlations were stronger in the presence of combined neuropathy (sensory DC, P = .005; motor DC, P = .0001).
Men with DM and LUTS can present with varied urodynamic findings, apart from the classic sensory or motor cystopathy. A large proportion of these patients will have electrophysiologic evidence of neuropathy, and electrophysiologic evidence of neuropathy can moderately predict the presence of cystopathy.