Arunodaya Gujjar

Neuromyelitis optica: an overview

Neuromyelitis optica (NMO) is a relapsing inflammatory disorder of the central nervous system that closely resembles multiple sclerosis. This review discusses recent understanding of NMO with reference to epidemiology, clinical spectrum, immunopathology, diagnostic evaluation, clinical course and management. Within the clinical spectrum of NMO, the classical form (with relapsing opticomyelitis) and limited forms (either recurrent myelitis or optic neuritis), as well as its association with other autoimmune disorders, have been recognised in recent years. Further, symptomatic or asymptomatic cerebral lesions may be present, and such brain lesions do not necessarily exclude the diagnosis. In the appropriate clinical context, the diagnosis is supported by longitudinally extensive myelitis on spinal MRI. Overwhelming evidence strongly indicates that aquaporin 4 antibody has a pathogenetic role in the development of NMO and serves as a useful diagnostic and prognostic marker. Detection of this autoantibody has led to the categorisation of NMO as an autoimmune channelopathy. NMO can be distinguished from multiple sclerosis by a combination of clinical, radiological and laboratory studies. However, the nosological position of Asian opticospinal multiple sclerosis is not settled. In NMO, acute attacks usually result in moderate to severe functional impairment, and, in the absence of a secondary progressive course (unlike relapsing–remitting multiple sclerosis), prevention of relapses is of crucial importance in the management of the disease. Indeed, relapse prevention with long-term immunosuppressive medication remains the cornerstone of therapy in this otherwise debilitating disease!

Extrapontine myelinolysis as presenting manifestation of adrenal failure: A case report

BACKGROUND Hyponatremia is a fairly common metabolic disorder. Hyponatremic myelinolysis is a relatively rare, life threatening complication with poorly understood pathophysiology, varied clinical manifestations and uncertain treatment. This case report highlights the range of clinical and imaging phenomena associated with hyponatremic myelinolysis. METHODS Case report. RESULT A middle aged lady presented with an acute delirious state, hypotension and severe hyponatremia on a background of skin hyper-pigmentation and weight loss. Her clinical course evolved to an akinetic-rigid state and later to parkinsonism. Extensive investigations for recognizing a primary neurologic disorder, including brain MRI and CSF analysis were normal, though she had disseminated miliary tuberculosis involving multiple organs. Brain MRI changes characteristic of extrapontine myelinolysis appeared two weeks after the onset of symptoms. The patient recovered completely over several weeks. CONCLUSION This case of hyponatremic extrapontine myelinolysis occurred as the presenting manifestation of adrenal failure secondary to disseminated tuberculosis. Extraponine myelinolysis is difficult to diagnose in the context of delayed brain MRI changes and can have a favorable outcome with modern management.

Reversible MRI changes in prolonged status epilepticus: A case report

ABSTRACT MRI is an essential investigation in epilepsy and status epilepticus (SE) for determining the underlying cause. However, SE or prolonged seizures may themselves induce changes in the MRI resulting in difficulty in interpretation. This case report illustrates a patient who recovered completely from prolonged generalized idiopathic SE lasting more than 2 weeks and had transient bilateral external capsule, fronto-temporal, and insular cortical MRI changes. The mechanism of such MRI changes and their implications for survival even after prolonged SE are discussed.

Full Outline of UnResponsiveness score and Glasgow Coma Scale in medical patients with altered sensorium: Interrater reliability and relation to outcome

PURPOSE Full Outline of UnResponsiveness, or FOUR score (FS), is a recently described scoring system for evaluation of altered sensorium. This study examined interrater reliability for FS and Glasgow Coma Scale (GCS) among medical patients with altered mental status and compared outcome predictability of GCS, FS, and Sequential Organ Failure Assessment score. PATIENTS AND METHODS Adult patients with altered mental status due to medical causes were rated by neurology consultants and internal medicine residents on FS and GCS. Interobserver reliability for GCS and FS was assessed using κ score. Relation with outcomes was explored using univariate and multivariate analyses. MAIN RESULTS Of the 100 patients (age, 62 ± 17 years), 60 had neurologic conditions; 26, metabolic encephalopathy; 9, infections; and 7, others. Thirty-nine patients died at 3 months. κ Scores ranged from 0.71 to 0.85 for GCS and from 0.71 to 0.95 for FS. On multivariate analysis, GCS was predictive of outcome at 3 months; FS was predictive of mortality. Area under the receiver operating characteristic curves suggested equivalent performance of both scoring systems. CONCLUSIONS Interrater reliability and outcome predictability for FS were comparable with those for GCS. This study supports the use of FS for evaluation of altered mental status in the medical wards.

Hydroxyurea or chronic exchange transfusions in patients with sickle cell disease: role of transcranial Doppler ultrasound in stroke prophylaxis.

Hydroxyurea is increasingly used in patients with sickle cell disease and acts by several underlying mechanisms. Its usage has been reported to eliminate transfusion needs in children with beta-thalassemia major. It has also been used in sickle cell disease patients on stroke prophylaxis with exchange transfusions who develop transfusional iron overload and is now being considered as a possible alternative to chronic transfusions for secondary stroke prophylaxis. Our case demonstrates a pitfall when using hydroxyurea without monitoring intracranial cerebral vessels for vasculopathic changes; presence of which can predict stroke risk and alert the need for chronic blood transfusions as primary stroke prophylaxis. Transcranial Doppler is a crucial investigation that can reveal elevated cerebral arterial flow velocities.

Frequency of Obstructive Sleep Apnea Syndrome Among Patients with Epilepsy Attending a Tertiary Neurology Clinic

OBJECTIVES Epilepsy is a common neurological disorder with a median lifetime prevalence of 14 per 1000 subjects. Sleep disorders could influence epileptic seizure. The most common sleep disorder is obstructive sleep apnea syndrome (OSAS) which occurs in 2% of adult women and 4% of adult men in the general population. The aim of this study is to estimate the frequency of OSAS among patients with epilepsy and to study the seizure characteristics among those patients with co-morbid OSAS. METHODS Patients with a confirmed diagnosis of epilepsy who attended the Sultan Qaboos University Hospital neurology clinic were recruited for the study between June 2011 and April 2012. Patients were screened for OSAS by direct interview using the validated Arabic version of the Berlin questionnaire. Patients identified as high-risk underwent polysomnography. RESULTS A total of 100 patients with epilepsy (55 men and 45 women) were screened for OSAS. Generalized and focal seizure was found in 67% of male and 27% of female patients. Six percent of the participants had epilepsy of undetermined type. Only 9% of the sample was found to have high risk of OSAS based on the Berlin questionnaire. No significant correlation was found between risk of OSAS, type of epilepsy, and anti-epileptic drugs. CONCLUSION The risk of OSAS was marginally greater in patients with epilepsy compared to the general population with the overall prevalence of 9%.

Varicella Zoster Virus Pneumonitis and Brainstem Encephalitis Without Skin Rash in an Immunocompetent Adult

Varicella zoster virus (VZV) pneumonitis and brainstem encephalitis developed in an immunocompetent adult without rash. Chest computed tomography exhibited nodularity; lung biopsy revealed multinucleated giant cells, Cowdry A inclusions, VZV antigen, and DNA. Varicella zoster virus central nervous system disease was verified by cerebrospinal fluid (CSF) anti-VZV IgG antibody with reduced serum/CSF ratios.

Intravenous levetiracetam vs phenytoin for status epilepticus and cluster seizures: A prospective, randomized study

PURPOSE Status Epilepticus (SE) is a common medical emergency carrying a high morbidity and mortality. Levetiracetam (LEV) is a novel anticonvulsant effective against varied seizures. Few prospective studies have addressed its use in SE. We aimed to examine the efficacy of intravenous LEV in controlling SE and cluster attacks of seizures (CS), in comparison with IV phenytoin (DPH), using a prospective, randomized study design. METHOD Adult patients with SE or CS, following an initial dose of IV benzodiazepine to control ongoing seizure, were randomized to receive either medication. Rates of seizure control over 24h, adverse effects and outcomes were compared. A logistic regression model was used to identify outcome predictors. RESULTS 52 patients with SE and 63 with CS received either LEV or DPH. In the SE group, LEV was effective in18/22(82%) and DPH in 22/30(73.3%) patients in controlling seizures. Among patients with CS, LEV was effective in 31/38(81.6%) and DPH in 20/25(80%). With the use of LEV, DPH or both, SE and CS were controlled among 92% and 96% of patients respectively. Adverse events included hypotension (in 2 on DPH) and transient agitation (2 on LEV). CONCLUSIONS IV Levetiracetam controls status epilepticus or cluster seizures with an efficacy comparable to that of phenytoin. Use of these two agents consecutively may control >90% of all such conditions without resort to anaesthetic agents. Further studies should explore its efficacy in larger cohorts of epileptic emergencies.

Eating epilepsy in Oman: A case series and report on the efficacy of temporal lobectomy

Eating epilepsy (EE), where seizures are triggered by eating, is rare and has not been reported in the Gulf region. In EE, the ictal semiology includes partial or generalised seizures. Focal brain changes on imaging, if present, are often confined to the temporal lobe or perisylvian region. Therapeutic options, especially in those patients who are refractory to pharmacotherapy, have not been well-established. We report a series of five patients with EE from Oman, a country located in the eastern part of the Arabian Gulf region, and highlight the usefulness of temporal lobectomy in one patient who had medically-intractable EE. Surgical intervention could be considered as a potential therapeutic option in carefully selected patients with medically-intractable seizures.

Transcranial Doppler Ultrasonography in Sickle Cell Disease: A Study in Omani Patients

Changes on Transcranial Doppler (TCD) ultrasonography have been proposed as significant predictors of cerebrovascular complications in sickle cell disease (SCD). However, consensus with regards to the TCD criteria to recognize abnormalities in cerebral vasculature is lacking. We studied the TCD characteristics of cerebral arteries among Omani patients with SCD and correlated them with cerebrovascular events. TCD was performed through the temporal and suboccipital windows using a 2 MHz probe (DWL). Thirty-three of 59 patients (56%) with SCD had neurological symptoms including stroke—12 (20%) and epilepsy—7. Fifteen patients (25%) had significant TCD abnormalities including: markedly increased velocities—11 (3 with stroke); turbulent flow—2; and reversal of flow—2. No patient had a time averaged maximum mean velocity of >200 cm/s in anterior circulation. On applying a modified definition of “abnormal TCD” to anterior and posterior circulation studies, increased TCD velocities in posterior circulation correlated with history of stroke (P<0.05). TCD velocities in the 18 adult patients ( older than 15 y) were significantly lower than in children. Logistic regression analysis revealed abnormal TCD in the left posterior cerebral artery to be an independent predictor of stroke in this cohort (P=0.035).