Pituitary Dysfunction in Idiopathic Intracranial Hypertension: An Analysis of 80 Patients.

BACKGROUND: Empty sella is a commonly described imaging entity in patients with idiopathic intracranial hypertension (IIH). Though menstrual and hormonal disturbances have been associated with IIH, available literature lacks systematic analysis of pituitary hormonal disturbances in IIH. More so, the contribution of empty sella in causing pituitary hormonal abnormalities in patients of IIH has not been described. We carried out this study to systematically assess the pituitary hormonal abnormalities in patients with IIH and its relation to empty sella. METHODS: Eighty treatment naïve patients of IIH were recruited as per a predefined criterion. Magnetic resonance imaging (MRI) brain with detailed sella imaging and pituitary hormonal profile were done in all patients. RESULTS: Partial empty sella was seen in 55 patients (68.8%). Hormonal abnormalities were detected in 30 patients (37.5%), reduced cortisol levels in 20%, raised prolactin levels in 13.8%, low thyroid-stimulating hormone (TSH) levels in 3.8%, hypogonadism in 1.25%, and elevated levels of gonadotropins were found in 6.25% of participants. Hormonal disturbances were independent and were not associated with the presence of empty sella (p = 0.493). CONCLUSION: Hormonal abnormalities were observed in 37.5% patients with IIH. These abnormalities did not correlate with the presence or absence of empty sella. Pituitary dysfunction appears to be subclinical in IIH and responds to intracranial pressure reduction, not requiring specific hormonal therapies.

Effectiveness of therapeutic plasma exchange in case of rare neurological disorder Isaacs syndrome.

Isaacs syndrome is a disease characterized by nerve hyperexcitability and pseudomyotonia and treated with immunomodulatory and symptomatic therapy approaches. Here, we report a case of anti-(leucine-rich glioma-inactivated 1) antibody-positive patient diagnosed as Isaacs syndrome and accomplished a nearly complete response to only four sessions of therapeutic plasma exchange (TPE). Our experience suggests that TPE along with other immunomodulatory agents may be beneficial and well-tolerated approach in patient with Isaacs syndrome.

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.

Sensitivity and specificity of neuroimaging signs in patients with idiopathic intracranial hypertension.

BACKGROUND: The primary role of neuroimaging in idiopathic intracranial hypertension (IIH) is to exclude secondary causes of raised intracranial pressure. Recently, a few imaging markers have been described which may suggest diagnosis of IIH in atypical cases. We carried out this study to assess the prevalence and accuracy of these neuroimaging signs in predicting the diagnosis of IIH. METHODS: Eighty treatment-naive patients with IIH and 30 controls were recruited as per a predefined criterion. Magnetic resonance imaging (MRI) brain with detailed sella imaging was done in all patients. RESULTS: The most common abnormality noted was optic nerve tortuosity in 82.5% of patients, followed by posterior scleral flattening in 80%, perioptic subarachnoid space (SAS) dilatation in 73.8% and partial empty sella in 68.8% of patients. The presence of optic nerve tortuosity was the most sensitive sign on neuroimaging, though the highest specificity was seen for posterior scleral flattening and perioptic SAS dilatation. The presence of more than three neuroimaging features correlated with severity of vision loss. CONCLUSION: In suggestive clinical scenarios, posterior scleral flattening, perioptic SAS dilatation and optic nerve tortuosity are highly sensitive and specific signs in IIH. This study also highlights the utility of MRI as a valuable tool for prognosis of visual outcome in patients with IIH.

Subacute sclerosing panencephalitis and brain stem involvement: a rare combination.

Subacute sclerosing panencephalitis (SSPE) is a progressive lethal neurological inflammatory disease due to persistent, wild measles virus infection in the central nervous system that is seen most frequently in children and young adolescents. Atypical presentations are seen in up to 10% of cases. Most frequently and severely affected region in the brain is the parieto-occipital region of the brain. Less commonly involved organs are the cerebellum, basal ganglia and corpus callosum. Brainstem involvement is rare and usually occurs when other areas of brain are involved along with it. Here, we describe an unusual male patient of 15 years age, having SSPE with MRI of brain showing extensive involvement of brainstem with no significant involvement of other cortical structures of the brain. It is very rarely described in SSPE, but one should be vigilant about such involvement of brainstem and cerebellum, and SSPE should not be missed when brainstem hyperintensities are seen in MRI brain with or without other region of the brain to avoid misdiagnosis.

Carbamazepine versus levetiracetam in epilepsy due to neurocysticercosis.

BACKGROUND: The choice of antiepileptic drug (AED) in newly diagnosed neurocysticercosis (NCC) patients with epilepsy continues to be arbitrary. We compared efficacy and side effect profile of levetiracetam (LEV) and carbamazepine (CBZ) for the treatment of seizures in newly diagnosed patients with NCC. PATIENTS AND METHODS: This was an open-labeled randomized comparative monotherapy study including newly diagnosed drug naïve patients of NCC (n = 99) presenting with seizures who were randomized in 1:1 ratio using computed generated numbers. All patients were followed up for at least six months after start of treatment. The primary outcome measure was seizure control over six months following start of AEDs. RESULTS: Fifteen (15.2%) patients [CBZ- 4(8.2%); LEV- 11(22%)] developed recurrence of seizures. A trend (p = 0.09) was found toward better control of seizures in CBZ compared to LEV. Two (4%) patients in LEV group and 17 (34.6%) patients in CBZ group developed drug-related minor side effects (p < 0.0001). Three patients in CBZ group needed discontinuation of therapy due to skin rash. Eleven patients who relapsed while on LEV did not have any recurrence of seizures after switching over to CBZ. Out of 3 patients who relapsed while receiving CBZ and were changed to LEV, two developed seizures during follow-up. CONCLUSION: CBZ and LEV could be used as alternatives in newly diagnosed patients of NCC at the behest of minor side effects in the CBZ group.

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.

Rare case of bilateral carotid artery dissection presenting with Foix-Chavany-Marie syndrome.

Carotid artery dissection is one of the most common causes of ischaemic stroke in young and middle-aged population. We report a case of bilateral carotid artery dissection presenting with opercular syndrome or Foix-Chavany-Marie syndrome. This 46-year-old obese and hypertensive man with a history of fall from bike 1 week prior, presented with sudden onset of anarthria, dysphagia and deviation of angle of mouth. His speech and dysphagia gradually improved over 10 days to normal, but he developed pseudobulbar affect and difficulty in calculations 4 weeks later. MRI showed acute infarcts in bilateral operculum. CT angiography showed dissection in bilateral cervical ICAs. He was managed conservatively with oral anticoagulation, given for 6 months. Probability of dissection must be considered in patients with a history of trauma or falls developing focal neurological deficits. To our knowledge, this is the first case report of bilateral ICA dissection presenting with Foix-Chavany-Marie syndrome.

Non-Arteritic Anterior Ischaemic Optic Neuropathy - Myths and Misconceptions: Experience from a Tertiary Care Centre in North India.

We prospectively evaluated 22 consecutive patients (24 eyes) suffering from non-arteritic anterior ischaemic optic neuropathy (NAION) at a tertiary care centre in Northern India. The mean age was 51.7 years. Visual loss on awakening was noticed only in three (12.5%) eyes. Six (27.3%) patients had headache while three (9.7%) patients had retro-orbital pain at the onset of visual loss. Peripapillary haemorrhages were seen in two (8.3%) eyes. At follow up, only one eye showed improvement in visual acuity. The results of our study suggest that NAION may not always present with the classic clinical picture. Accordingly, a high index of suspicion remains the key to correct diagnosis.

Atypical neurological manifestations of dengue fever: a case series and mini review.

BACKGROUND: In this mini review, we discuss some of the atypical neurological manifestations of dengue virus and attempt to bring them to attention to highlight the neurotropic property of the dengue virus. METHODS: Cases were chosen from retrospective hospital and outpatient records of all patients seropositive for dengue who attended the neurology referral. Seven patients have been chosen as illustrative examples of dengue-associated neurological involvement. We discuss the various central and peripheral nervous system involvement of patients and discuss the relevant findings in them. CONCLUSION: Through this case series, we wish to highlight that the dengue virus can affect the nervous system at various targets, using multiple mechanisms of pathogenesis to generate a plethora of presentations. Hence, it is vital to be aware of its presentations to be able to diagnose dengue and treat it accordingly.

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.

Systemic lupus erythematosus with autoimmune neurological manifestations in a carrier of chronic granulomatous disease - a rare presentation.

Chronic granulomatous disease (CGD) is an uncommon genetic immunodeficiency disorder affecting neutrophil function, characterized by recurrent bacterial and fungal infections. X-linked carriers of CGD have an increased risk of autoimmune disorders, in particular lupus like disorders. We describe the case of a 37 years old female carrier of X-linked CGD, who presented with clinical features and serology consistent with a definite diagnosis of Systemic lupus erythematosus (SLE), with rare immune mediated neurological manifestations including secondary central nervous system (CNS) vasculitis and Longitudinally extensive transverse myelitis (LETM), responsive to immunomodulation. These neurological manifestations have not been described previously in carriers of CGD. We recommend early diagnosis of these immune mechanisms, especially in X-linked carriers of CGD, and appropriate immunomodulation in order to improve life expectancy and improve neurological outcome.

18FDG-PET is sensitive tool for detection of extracranial tuberculous foci in central nervous system tuberculosis - Preliminary observations from a tertiary care center in northern India.

PURPOSE: To study the role of 18FDG- PET (Flourodeoxyglucose positron emission tomography) in a) determining the extent of cranial and extra-cranial disease and b) diagnosis as well as prognosis of CNS TB (central nervous system tuberculosis) including TBM (tuberculous meningitis). PATIENTS AND METHODS: This prospective observational study (n = 70) was carried out at a tertiary care institute in Northern India from 1.1.2017 to 30.6.2018. Diagnosis of TBM was made according to modified Ahuja's criteria. All patients were evaluated in detail and treated as per standard guidelines. All patients underwent 18FDG-PET scanning of brain and whole body at baseline. RESULTS: Mean age was 35.2 ±â€¯14.8 years. There were 37 men. Majority of patients (n = 47; 67.1%) were below 40 years of age. 43 (61.4%) patients were in stage II TBM. The mean duration of illness was 77 ±â€¯101.9 days. Majority of patients presented with fever (94.3%), headache (90%) and vomiting (84.3%). MRI was abnormal at baseline in 67 (95.7%) of patients, most common abnormalities being meningeal enhancement (68.6%) and tuberculomas (57.1%). PET was abnormal in 66 (95.7%) of patients. All these patients had either lung lesions (n = 62, 88.6%) or lymphadenopathy (n = 61; 87.1%). 18FDG-PET revealed evidence of brain lesions in 52 (74.3%) patients. It revealed vertebral involvement in 19 (27.1%) and genitourinary lesions in 9 (12.9%) patients. PET evidence of lymphadenopathy correlated significantly (p = .04) with good outcome in CNS TB. Conclusion 18FDG-PET does seem to have a promising role in initial evaluation of patients with CNS TB.