Establishing continuum in Transcranial Doppler characteristics of IIH, migraine and healthy controls- An exploratory study.

BACKGROUND: IIH is a severe form of headache that often has superimposed migraine and often it is very difficult to distinguish the two forms of headache. Intracranial hemodynamics is a relatively unexplored means of distinguishing between the two forms of headache. OBJECTIVES: We aimed to study intracranial flow dynamics using Transcranial Doppler in patients with IIH, migraine, and normal controls. MATERIALS AND METHODS: It was a hospital-based observational study that included 51 people with IIH, 87 people with migraine, and 101 healthy controls and all were subjected to TCD study after detailed clinical examination. RESULTS: Mean age of patients in three groups were similar with the mean age in IIH being 33.41 ± 10.75 (age in years ± SD). Vision loss was present in 66.67% of patients with IIH, and most common field defect was generalized constriction (27.5%). Neuroimaging was abnormal in 94.11% of patients of IIH with mean CSF pressure was 31.27±5.32 cm of water. Of all the TCD-measured velocities, mean flow velocity (MFV) showed a significant difference in all three groups with (p-value <0.001). The pulsatility index, both for middle cerebral arteries as well as ophthalmic arteries showed a significant difference in the three groups with the highest values in IIH patients (p-value<.001). The mean VMR in IIH (1.11±0.32) was lower than the mean VMR in migraine (1.34±0.43) as well as controls (1.49±0.46). CONCLUSION: TCD parameters like MFV and PI are useful parameters that show considerable variation and can be used to differentiate between IIH and migraine.

Endoscopic Ultrasound-Guided Transgastric Shunt Obliteration for Recurrent Hepatic Encephalopathy.

INTRODUCTION: Occlusion of spontaneous portosystemic shunts (SPSSs) in patients with cirrhosis may be required in recurrent or refractory hepatic encephalopathy. We describe a novel method for occlusion of SPSS using endoscopic ultrasound (EUS). METHODS: EUS-guided transgastric shunt obliteration was performed by injecting glue and coils directly into SPSS. RESULTS: EUS-guided transgastric shunt obliteration was performed for 7 patients in 9 sessions. Complete cessation of Doppler flow was achieved in 6/7 cases. Adequate clinical response was observed in 6/7 patients. No procedure-related severe adverse events were seen. DISCUSSION: This novel technique is a potentially effective and efficient method for shunt obliteration.

The Genetics of Ethambutol-Induced Optic Neuropathy: A Narrative Review.

Tuberculosis (TB) is a global health problem with the major brunt of disease occurring in developing countries. The cornerstone of treatment of TB is anti-tubercular therapy (ATT), which includes rifampicin, isoniazid, pyrazinamide and ethambutol. Because of emerging drug resistance, treatment failures, defaulters and increasing incidence of disseminated and extrapulmonary TB, the guidelines have been modified in some countries. Ethambutol is prescribed for longer times (in some cases >8 months) and hence the incidence of ethambutol-induced optic neuropathy (EtON) is expected to rise. The fundamental question which needs explanation is why only a small subset of patients on ethambutol are prone to develop loss of vision. This review focuses on available genetic studies which provide evidence that mitochondria are the likely substrates involved in the final pathway of reactive oxidative damage of the papillo-macular bundle. Genetic analysis of mitochondrial mutations encoding genes involved in oxidative phosphorylation pathways may help in isolating the subset of patients who are genetically susceptible. If the groups having high risk of developing EtON are recognised then prolonged duration of ethambutol treatment can be avoided in these susceptible individuals. A better understanding of the pathophysiology will also pave the way for the development of management strategies in this condition.

Oculomotor Cranial Neuropathies: Diagnosis and Management.

Ocular nerve palsies are among the most common cranial neuropathies in neurological practice. Nerves can get affected anywhere along their path from the brainstem to the orbit. There can be isolated involvement of multiple cranial nerves together. The etiologies differ according to the type of presentation. The steps toward the diagnosis need to be strategically planned and must be based on clinical localization. It is crucial to make proper localization to plan further investigations and thus treatment of the etiology. This review covers the approach toward the diagnosis, etiologies involved, and management of ocular cranial neuropathies.

A study on neurcognitive disorders and demographic profile of neurocysticercosis patients.

BACKGROUND: Neurocysticercosis is a common cerebral parasitic infestation, caused due to pork tapeworm infection the infestations risks parallels the socio-economic status, personal hygiene and education. The effect of NCC was assessed in neurocognition. OBJECTIVE: To study demographic characteristics and neurocognitive domains of patients with Neurocysticercosis. METHODS: Neurocysticercosis diagnosed patients by CT, MRI and LAMP tests. MMSE score was measured for assessment. RESULTS: MMSE score were reduced in majority of the patients. In attention was the most common deficit found. Repeat MMSE assessment done in 6 patients showed an improvement of scores post therapy. CONCLUSION: Cognitive involvement is common in NCC and is a major cause of morbidity.

Mimics of Optic Neuritis in Neuromyelitis Optica Spectrum Disorder: A Case Report.

The optic nerve can be involved in a myriad of pathologies underscoring the importance of ruling out both surgical as well as medical causes. Often, it is the temporal profile, clinical presentation and imaging that helps to establish the final diagnosis. Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory demyelinating disorder involving the optic nerves and the spinal cord, which if promptly and adequately managed, may yield gratifying outcomes. We report an unusual presentation of NMOSD, mimicking a compressive optic neuropathy. A comprehensive review of the history, extensive investigations including brain and spinal cord imaging, and positive anti-aquaporin 4 antibodies helped in the definitive management.

Infectious ophthalmoplegias.

Though infections account for a significant proportion of patients with ocular motor palsies, there is surprising paucity of literature on infectious ophthalmoplegias. Almost all types of infectious agents (bacteria, viruses, fungi and parasites) can lead to ocular motor palsies. The causative infectious agent can be diagnosed in most cases using an orderly stepwise approach. In this review we discuss how to approach a patient with ophthalmoplegia with main focus on infectious etiologies.

Magnetic resonance imaging (MRI) versus computed tomographic scan (CT scan) of brain in evaluation of suspected cavernous sinus syndrome.

BACKGROUND AND PURPOSE: The cavernous sinus is a unique region owing to anatomical factors and the pathologies affecting it. The diagnosis of cavernous sinus syndrome (CSS) predominantly relies on clinicoradiological correlation. We studied the utility of computed tomographic (CT) scan versus magnetic resonance imaging (MRI) in the diagnosis of CSS. METHODS: A prospective observational study was conducted in a tertiary care center in north India. All patients presenting with a clinical syndrome of cavernous sinus involvement with radiologically confirmed lesions were enrolled in the study. MRI and CT scan with cavernous sinus cuts were done and reviewed by experienced neuroradiologists for cavernous sinus lesions and compared with the final diagnosis. Sensitivity and specificity were calculated. RESULTS: We included 48 patients in our study. A final diagnosis was achieved in 41 out of 48 (85.6%) patients. Fungal infections (16 (33.3%)) constituted the commonest cause of CSS, followed by neoplastic involvement (13 (27.1%)) and Tolosa-Hunt syndrome (12 (25%)). Vascular involvement was seen in three (6.3%) patients. Other rare causes were seen in four (8.3%) patients. CT scan had an overall sensitivity of 14.6% in achieving a final diagnosis, whereas MRI had an overall sensitivity of 70.7%, with a statistically significant difference (p < 0.001). CONCLUSIONS: Although CT scan is a relatively cheap and accessible resource, its role in CSS diagnosis and management is limited because of poor yield. Hence, it is prudent to do an MRI as an initial investigation in cases of CSS.

Presence of allele CYP3A4*16 does not have any bearing on carbamazepine-induced adverse drug reactions in North Indian people with epilepsy.

OBJECTIVES: The objectives of this study were to determine the relationship between genetic polymorphisms in gene encodings for CYP3A4 and carbamazepine (CBZ)-induced dose-related side effects in North Indian people with epilepsy. PATIENTS AND METHODS: The current prospective study included 37 patients with CBZ-induced dose-related side effects and 102 patients who did not experience side effects while on CBZ. The genotyping for CYP3A4 allele (CYP3A4*16) was done using real-time polymerase chain reaction (RT-PCR) in Applied Biosystems 7500 RT-PCR System (USA). CBZ was administered in all patients at a dose varying from 15 to 20 mg/kg daily. RESULTS: Various demographic variables were comparable between the groups except that control of seizures was far better in controls. After testing, it was found that none of our patients had the presence of CYP3A4*16 allele. CONCLUSION: CYP3A4*16 allele is not represented significantly in North Indian people with CBZ-induced dose-related side effects.

Identification of Taenia solium DNA by PCR in blood and urine samples from a tertiary care center in North India.

BACKGROUND: NCC is a neglected zoonotic disease with high endemicity and disease burden. Neurocysticercosis is a frequent cause of seizures in endemic countries. Early diagnosis and therapy helps in reducing morbidity and DALYs (daily adjusted life years) lost. Definite diagnosis still relies on neuroimaging identification of scolex or by histopathological examination. Molecular method such as PCR (Polymerase Chain Reaction) is an emerging modality to supplement or complement these Gold standard methods. AIM: To determine the utility of PCR in detecting Taenia Solium DNA in NCC patients. METHODS: A total of 100 blood samples of cases of NCC and 50 control blood samples, 58 urine samples of NCC cases and 24 control samples were collected. Repetitive element PTsol9 of the Taenia Solium was targeted. RESULTS: The sensitivity and specificity of PCR in blood samples was 57% and 94%, while sensitivity and specificity in urine samples was 64% and 87%. CONCLUSION: PCR assay can be used as an adjunct for the diagnosis of NCC especially in ambiguous cases, this is relatively rapid and non-invasive diagnostic modality.

Newer magnetic resonance imaging techniques in neurocysticercosis.

INTRODUCTION: The definitive diagnosis of neurocysticercosis continues to be challenging. We evaluate the role of newer magnetic resonance imaging techniques including constructive interference in steady state, susceptibility-weighted imaging, arterial spin labelling and magnetic resonance spectroscopy in the diagnosis of neurocysticercosis. AIMS AND OBJECTIVES: To study the utility of newer magnetic resonance imaging sequences in the diagnosis of neurocysticercosis. PATIENTS AND METHODS: Eighty-five consecutive patients with neurocysticercosis attending a tertiary care hospital and teaching centre in northern India were included in the study. The diagnosis of neurocysticercosis was made by the Del Brutto criteria. All patients received treatment according to standard guidelines and were followed at 3-month intervals. The following magnetic resonance sequences were performed at baseline: T1 and T2-weighted axial sequences; T2 fluid-attenuated inversion recovery axial sequences; diffusion-weighted imaging; susceptibility-weighted imaging; pre and post-contrast T1-weighted imaging; heavily T2-weighted thin sections (constructive interference in steady state); arterial spin labelling (n = 19); and magnetic resonance spectroscopy (n = 24). RESULTS: The mean (±SD) age was 29.4 ± 12.9 years and 76.5% were men. Seizures were the commonest symptom (89.4%) followed by headache (24.3%), encephalitis (9.4%) and raised intracranial pressure (9.4%). Scolex could be visualised in 43.7%, 55.5% and 61.2% of neurocysticercosis patients using conventional, susceptibility-weighted angiography and constructive interference in steady state imaging sequences, respectively. Susceptibility-weighted angiography and constructive interference in steady state images resulted in significantly higher (P < 0.01) visualisation of scolex compared to conventional sequences. CONCLUSION: Newer magnetic resonance imaging modalities have a lot of promise for improving the radiological diagnosis of neurocysticercosis.

Use of rituximab in muscle-specific tyrosine kinase antibody-positive myasthenia gravis: Preliminary observations from a tertiary care center in Northern India.

OBJECTIVES: Approximately 10%-15% of patients with myasthenia gravis (MG) are refractory to standard treatment. A sizable chunk of these patients is due to muscle-specific tyrosine kinase (MuSK) antibody-positive MG which often runs a severe course with frequent relapses and poor response to conventional treatment. We report six patients with refractory MuSK-positive MG who responded well to the treatment with rituximab. PATIENTS AND METHODS: In this prospective institute-based observational study, we report six MuSK antibody-positive MG patients, who did not achieve remission with standard treatment and were later started on rituximab infusion. RESULTS: There was a significant clinical improvement in all patients after starting rituximab. CONCLUSION: Rituximab is an effective immunomodulatory therapy in MuSK antibody-positive MG patients who are not responding to the standard treatment.

Opsoclonus Myoclonus Syndrome Due to an Ovarian Teratoma: A Case Report and Review of Literature.

The importance of ovarian teratoma as a cause of opsoclonus myoclonus ataxia syndrome (OMAS) and other paraneoplastic syndromes continues to be underestimated. A strong suspicion and appropriate diagnosis remain keys to successful outcome of paraneoplastic OMAS with ovarian teratoma. We report a 14-year-old girl with paraneoplastic OMAS in association with an ovarian teratoma who improved completely following resection of tumour as well as immunotherapy and review the literature briefly.

Tuberculomas in "Critical" Locations of the Visual Pathway-A Masquerader.

Tuberculosis never stops fascinating physicians. The burden of tuberculosis is a major cause of disability. While drugs are often blamed when patients develop new focal deficits on treatment, the deterioration in these patients can have a myriad of causes including the development of new tuberculomas. Recognizing the critically located tuberculomas as potential causes of vision loss is indispensable. Here, we report two patients with strategic tuberculomas leading to vision loss. In one patient, the cause of visual morbidity was compression of the optic pathways; in the other patient, cortical vision loss was noted due to strategic location of tuberculomas.