Ajay Panwar

Adult Presentation of Dyke- Davidoff-Masson Syndrome: A Case Report

Dyke-Davidoff-Masson syndrome (DDMS) is a rare disease which is clinically characterized by hemiparesis, seizures, facial asymmetry, and mental retardation. The classical radiological findings are cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses. This disease is a rare entity, and it mainly presents in childhood. Adult presentation of DDMS is unusual and has been rarely reported in the medical literature. Key Messages: DDMS is a rare disease of childhood. However, it should be kept in mind as a diagnostic possibility in an adult who presents with a long duration of progressive hemiparesis with seizures and mental retardation. Cerebral hemiatrophy, calvarial thickening, and hyperpneumatization of the frontal sinuses are diagnostic for this illness on brain imaging.

Neuromyelitis Optica Spectrum Disorder with Tumefactive Demyelination mimicking Multiple Sclerosis: A Rare Case.

Neuromyelitis optica spectrum disorder (NMOSD) is a diverse condition which not only encompasses isolated longitudinally extensive transverse myelitis (LETM) and optic neuritis but also includes area postrema syndrome, acute brainstem syndrome, symptomatic narcolepsy or acute diencephalic clinical syndrome, and symptomatic cerebral syndrome. Imaging may reveal periependymal lesions surrounding the ventricular system or involvement of corticospinal tracts, area postrema, diencephalon, and corpus callosum. Rarely, there may be hemispheric tumefactive lesions that enhance in a “Cloud-like” fashion on gadolinium injection unlike in tumefactive multiple sclerosis where there is incomplete ring enhancement. Here, we present a case of aquaporin-4 positive relapsing NMOSD who presented to us with recurrent episodes of paraparesis with LETM and tumefactive lesions of brain on imaging, which enhanced in an incomplete ring like pattern resembling multiple sclerosis.

25-Hydroxy Vitamin D, Vitamin D Receptor and Toll-like Receptor 2 Polymorphisms in Spinal Tuberculosis: A Case-Control Study.

AbstractVitamin D deficiency and vitamin D receptor (VDR) gene abnormalities confer susceptibility to tuberculosis. Toll-like receptors (TLRs), such asTLR-2, are also important mediators of inflammatory response against Mycobacterium tuberculosis. We evaluated serum vitamin D, and VDR and TLR-2 gene polymorphisms in patients with spinal tuberculosis.This study comprised of 3 groups: spinal tuberculosis, pulmonary tuberculosis, and controls (each with 106 subjects). Enzyme-linked immunosorbent assay was used to measure vitamin D levels, and polymerase chain reaction-sequencing method was used to analyze VDR and TLR-2 gene polymorphisms. Patients were followed up for 6 months.Vitamin D deficiency was significantly more prevalent in patients with spinal tuberculosis (P < 0.001) and pulmonary tuberculosis (P = 0.011), versus controls. The heterozygous and mutant genotypes of VDR TaqI gene were significantly associated with spinal tuberculosis (P < 0.001; odds ratio [OR] 4.74 [2.45–9.18]) and pulmonary tuberculosis (P < 0.001; OR 3.52 [1.80–6.88]) when compared with controls. The heterozygous and mutant variants of VDR ApaI gene were significantly more common in patients with spinal tuberculosis in comparison with patients with pulmonary tuberculosis (P < 0.001; OR 2.90 [1.65–5.10]) and controls (P < 0.001; OR 6.56 [3.41–12.61]). We did not observe any significantly different results for TLR-2 gene polymorphisms. Vitamin D deficiency, VDR, and TLR-2 polymorphisms did not affect the 6-month disability.Vitamin D deficiency and VDR gene polymorphisms are significantly more prevalent in people with pulmonary and spinal tuberculosis. They may, in isolation or collectively, confer susceptibility to pulmonary and spinal tuberculosis.

Leptospirosis and dengue coinfection: Report of three cases with review of literature

Leptospirosis is a zoonosis having a worldwide prevalence and has recently emerged as a major public health problem, especially in tropical and subtropical regions. Likewise, dengue is one of the major endemic health problems in the Indian subcontinent. It is a mosquito-borne arboviral infection causing considerable morbidity and mortality. Epidemiologically, mixed infections of dengue and leptospirosis are possible because similar environmental conditions are needed for the transmission of both these infections. Still, their coinfection is rarely reported in medical literature. Here, we report three such cases of dengue and leptospirosis coinfection, encountered in clinical practice during the monsoon season at Kolkata, West Bengal, India.

Irreversible Hemichorea–Hemiballism in a Case of Nonketotic Hyperglycemia Presenting as the Initial Manifestation of Diabetes Mellitus

Background Hemichorea–hemiballism (HCHB) is a hyperkinetic movement disorder with features of both chorea and ballism occurring on the same side. Case report We present a case of HCHB due to nonketotic hyperglycemia (NKH) that was the initial presentation of diabetes and was irreversible clinically even after 6 months of optimal blood sugar control. Discussion Although HCHB due to hyperglycemia is a potentially reversible condition in the majority of patients, prolonged uncontrolled hyperglycemia may cause ischemic insult and persistent symptoms. Hyperglycemia should always be kept in the list of differentials while dealing with patients who are newly diagnosed with HCHB.

Effect of CPAP Therapy in Improving Daytime Sleepiness in Indian Patients with Moderate and Severe OSA

INTRODUCTION Obstructive Sleep Apnoea (OSA) is a highly prevalent disease and a major public health issue in India. Excessive daytime sleepiness is an almost ubiquitous symptom of OSA. Epworth Sleepiness Scale (ESS) score is a validated objective score to measure the degree of daytime sleepiness. Continuous Positive Airway Pressure (CPAP) therapy has been established as the gold standard treatment modality for OSA patients. A few Indian studies have reported the effectiveness of CPAP therapy in improving ESS scores after 1st month of CPAP use. AIM To observe both, short-term (one month) and long-term (three month) effects of CPAP therapy on ESS scores in moderate to severe OSA patients. MATERIALS AND METHODS The patients complaining of excessive day-time sleepiness, snoring and choking episodes during sleep, consecutively presenting to medicine OPD over a period of 2 years, were subjected to Polysomnography (PSG). Seventy-three patients with apnoea-hypopnea index (AHI) ≥15 were categorised as having moderate to severe forms of OSA (moderate OSA with AHI=15-30 and severe OSA with AHI >30), and were scheduled for an initial trial of CPAP therapy. Forty-seven patients reported good tolerance to CPAP therapy after a trial period of 2 weeks and comprised the final study group. ESS scores in these patients were recorded at the baseline, and after 1st and 3rd month of CPAP therapy, and statistically analysed for significance. RESULTS Mean ESS score at the baseline among moderate and severe OSA patients were 13.67±2.29 and 16.56 ±1.87, respectively. ESS score in both these subgroups improved significantly to 11.63±3.79, p=0.022, CI (0.3293-4.0106)} and 14.13 ±3.74, p < 0.001, CI (1.2991-4.5408), respectively after one month of CPAP therapy. Likewise, mean ESS scores among moderate and severe OSA patients improved significantly to 9.84 ±2.97, p = 0.022, CI (0.3293-4.0106) and 12.29 ±3.97, p <0.001, CI (2.9414-6.1385), respectively after three months of CPAP therapy. CONCLUSION The result of the present study shows that CPAP therapy is significantly effective in improving ESS scores in Indian patients having moderate to severe OSA. Benefits in daytime sleepiness were observed after short-term as well as long-term use of CPAP therapy.

Moyamoya disease involving anterior and posterior circulation

A 6‐year‐old female presented with sudden‐onset right‐sided hemiparesis with motor aphasia for the past 7 days. Magnetic resonance imaging of the brain [Figure 1a] revealed acute infarct in the left parietooccipital region. Magnetic resonance angiogram (MRA) [Figure 1b] and computed tomography angiogram (CTA) [Figure 2] showed narrowing of bilateral supraclinoid internal carotid arteries (ICAs), nonvisualization of bilateral middle cerebral arteries and distal part of posterior cerebral arteries (PCAs) along with extensive collateralization of vessels, creating a “puff of smoke” appearance, diagnostic of moyamoya disease.

Pontine Infarct Presenting with Atypical Dental Pain: A Case Report

Orofacial pain’ most commonly occurs due to dental causes like caries, gingivitis or periodontitis. Other common causes of ‘orofacial pain’ are sinusitis, temporomandibular joint(TMJ) dysfunction, otitis externa, tension headache and migraine. In some patients, the etiology of ‘orofacial pain’ remains undetected despite optimal evaluation. A few patients in the practice of clinical dentistry presents with dental pain without any identifiable dental etiology. Such patients are classified under the category of ‘atypical odontalgia’. ‘Atypical odontalgia’ is reported to be prevalent in 2.1% of the individuals. ‘Atypical orofacial pain’ and ‘atypical odontalgia’ can result from the neurological diseases like multiple sclerosis, trigeminal neuralgia and herpes infection. Trigeminal neuralgia has been frequently documented as a cause of ‘atypical orofacial pain’ and ‘atypical odontalgia’. There are a few isolated case reports of acute pontine stroke resulting in ‘atypical orofacial pain’ and ‘atypical odontalgia’. However, pontine stroke as a cause of atypical odontalgia is limited to only a few cases, hence prevalence is not established. This case is one, where a patient presented with acute onset atypical dental pain with no identifiable dental etiology, further diagnosed as an acute pontine infarct on neuroimaging. A 40 years old male presented with acute onset, diffuse teeth pain on right side. Dental examination was normal. Magnetic resonance imaging(MRI) of the brain had an acute infarct in right pons near the trigeminal root entry zone(REZ). Pontine infarct presenting with dental pain as a manifestation of trigeminal neuropathy, has rarely been reported previously. This stresses on the importance of neuroradiology in evaluation of atypical cases of dental pain.

Isolated vision loss and disappearing lesions as the manifestation of subacute sclerosing panencephalitis

Sir, Subacute sclerosing panencephalitis (SSPE) is a devastating disease of central nervous system (CNS) caused by persistent mutant measles virus infection. Although it is a rare disease in developed nations, it is of great concern in developing countries. Patients present with progressive cognitive decline and myoclonus.[1] Visual manifestations are present in 10-50% of patients but they usually occur concurrently with above neurological findings. Rarely visual symptoms may precede neurological manifestations.[2] Here we report a case of SSPE that had presentation in the form of acute vision loss and remained undiagnosed for 2 years until he developed typical manifestations of SSPE.

Acute Ischemic Stroke Thrombolysis with Tenecteplase: An Institutional Experience from South India

Objective: Outcome assessment of intravenous (IV) thrombolysis with tenecteplase in acute ischemic stroke. Materials and Methods: We consecutively enrolled acute ischemic stroke patients who underwent IV thrombolysis with tenecteplase from October 2016 to May 2017. Primary clinical efficacy outcome was defined as an improvement in the National Institute of Health Stroke Scale (NIHSS) score of ≥4 points at 24 h (h). Secondary clinical efficacy outcome was the favorable outcome on modified Rankin scale at 90 days defined as a score of 0 or 1. The safety endpoints were death rate at 90 days and symptomatic intracranial hemorrhage (SICH). Results: Mean NIHSS scores at baseline and 24 h were 13 (±3.81) and 9.29 (±5.74), respectively, the difference being statistically significant (P = 0.016). In this study, nine patients (64%) met the primary clinical efficacy outcome and eleven (78.5%) patients met the secondary clinical efficacy outcome. Only 1 (7%) patient developed SICH and additionally, aspiration pneumonia with subsequent death. Conclusion: This study confirms the efficacy and safety of tenecteplase for stroke thrombolysis in our clinical setting. Tenecteplase appears to be a suitable option for stroke thrombolysis in resource-limited settings, considering its cost-effectiveness, and ease of administration.