Pontine tubercular abscess: A rare presentation of CNS tuberculosis masquerading as glioma in a child.

Tuberculous brain abscess (TBA) in a child was initially misdiagnosed as glioma. Two craniotomies, abscess drainage, and anti-tubercular therapy led to recovery. Pontine TBA, though rare and atypical, can have better outcome with timely intervention.

A Comparison of the Intracerebral Hemorrhage Score and the Acute Physiology and Chronic Health Evaluation II Score for 30-Day Mortality Prediction in Spontaneous Intracerebral Hemorrhage.

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.

Dystonia, myoclonus, and encephalopathy in a single patient: A rare association of moyamoya.

Moyamoya disease (MMD) is a rare neurovascular disorder which pathologically, is a chronic cerebrovasculopathy. It is characterized by stenosis of the internal carotid artery (ICA) and the main branches within the circle of Willis with consecutive development of collateral vessels. There are a few cases in the literature which have described movement disorders as a manifestation of MMD; however these have been uncommonly reported in cases of moyamoya syndrome (MMS). We present a 10-year-old boy with dystonia, myoclonus and encephalopathy like features. These features in association with moyamoya, are rarely described.

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.

Clinical and Neuroradiological Spectrum of Metronidazole Induced Encephalopathy: Our Experience and the Review of Literature.

Metronidazole is an antimicrobial agent mainly used in the treatment of several protozoal and anaerobic infections, additionally, is often used in hepatic encephalopathy and Crohn disease. Apart from peripheral neuropathy, metronidazole can also cause symptoms of central nervous system dysfunction like ataxic gait, dysarthria, seizures, and encephalopathy which may result from both short term and chronic use of this drug and is collectively termed as "metronidazole induced encephalopathy"(MIE). Neuroimaging forms the backbone in clinching the diagnosis of this uncommon entity, especially in cases where there is high index of suspicion of intoxication. Although typical sites of involvement include cerebellum, brain stem and corpus callosum, however, lesions of other sites have also been reported. Once diagnosed, resolution of findings on Magnetic Resonance Imaging (MRI) of the Brain along with clinical improvement remains the mainstay of monitoring. Here we review the key clinical features and MRI findings of MIE as reported in medical literature. We also analyze implication of use of this drug in special situations like hepatic encephalopathy and brain abscess and discuss our experience regarding this entity.

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.

"Reverse Tigroid" Pattern in Pachygyria: A Novel Finding.

Pachygyria is considered a subtype of lissencephaly which, in turn, is a spectrum of disorders caused by abnormal neuronal migration. Clinical presentation in this disorder may be varied including microcephaly, developmental delay, facial dysmorphism, seizures, and mental retardation. Magnetic resonance imaging (MRI) of brain identifies the exact nature and extent of the disease and helps in delineating further plan of management. A Tigroid pattern on axial MRI scan and leopard pattern on a sagittal plane has been classically reported in disorders of myelin formation such as metachromatic leukodystrophy and Pelizaeus-Merzbacher disease. We present here a case of pachygyria who presented to us with some atypical features including "tigroid-like stripes" and "leopard-like pattern" on MRI brain which has not been reported in the medical literature previously.

Japanese Encephalitis Complicated with Obstructive Hydrocephalus.

Japanese Encephalitis (JE), caused by Japanese encephalitis virus (JEV), a flavi-virus, is the most significant aetiology of arboviral encephalitis worldwide. It has resulted in epidemics of encephalitis in the Indian subcontinent. Here, we report a case of 36-year-old female who presented with a short history of fever and headache followed by altered sensorium. Funduscopic examination revealed Papilloedema. Pyogenic or viral meningoencephalitis along with complicated malaria were kept as initial differential diagnosis. Magnetic Resonance Imaging (MRI) of brain revealed involvement of posterior limb of internal capsule and bilateral thalami in the form of haemorrhagic encephalitis along with obstructive hydrocephalus. Cerebro Spinal Fluid (CSF) serology (IgM ELISA) showed JE as the causative agent. Despite extensive literature search, we could not find a case of JE reported with hydrocephalus as a complication. This case highlights the typical and atypical features of JE including imaging findings and exemplifies the way, how diversely JE can present and would thus help in preparing management paradigms accordingly.

Sjögren-Larsson syndrome: a rare disease of the skin and central nervous system.

Sjögren-Larsson syndrome is a recessively inherited disease caused by a deficiency of fatty aldehyde dehydrogenase with presenting features of congenital ichthyosis, spastic diplegia or tetraplegia, and mental retardation. The basic pathogenic mechanism is deficiency of fatty aldehyde dehydrogenase, which may lead to an accumulation of long-chain fatty alcohols hampering cell membrane integrity, which further disrupts the barrier function of skin and white matter of the brain. MRI of the brain shows diffuse symmetrical white matter hyperintensities on T2-weighted sequences. Although there is no definitive cure for Sjögren-Larsson syndrome, most patients survive until adulthood and management involves therapies directed towards controlling specific problems. We present a case of Sjögren-Larsson syndrome with classical clinical and MRI features, including a few distinctly atypical characteristics in various attributes.

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

UNLABELLED: 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.

Reply #2 to: Glycemic Choreoballism.

In Response To: Lee D, Ahn, TB. Glycemic choreoballism. Tremor Other Hyperkinet Mov. 2016; 6. doi: 10.7916/D8QJ7HNF Original Article: Roy U, Das SK, Mukherjee A, et al. Irreversible hemichoreahemiballism in a case of nonketotic hyperglycemia presenting as the initial manifestation of diabetes mellitus. Tremor Other Hyperkinet Mov. 2016; 6. doi: 10.7916/D8QZ2B3F.

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.

Van der Knaap disease: a rare disease with atypical features.

Megalencephalic leukoencephalopathy with subcortical cysts (MLC), or Van der Knaap disease, is a rare autosomal recessive disorder. It is characterised by macrocephaly that either presents at birth or develops during infancy. It occurs more commonly in some ethnicities where consanguinity is common, such as in the Agrawal community in India. This disease typically presents with a history of delayed motor milestones in affected children. MRI findings including leukodystrophy and subcortical cysts are hallmarks of the disease and yield the diagnostic clue in most cases. Several cases of Van der Knaap disease with classical features have been reported in the literature. We present a case of Van der Knaap disease with classical MRI features, including a few distinctly atypical characteristics in its epidemiological, clinical and electrophysiological attributes.