Ramachandiran Nandhagopal

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!

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.

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.

Acute Drug Overdose: Clinical Profile, Etiologic Spectrum and Determinants of Duration of Intensive Medical Treatment

OBJECTIVES Acute drug overdosing is an important cause of organ dysfunction and metabolic derangements and the patients often require intensive care. This study aims to determine the clinical pattern of severe drug overdose as well as the factors influencing the duration of intensive care METHODS The clinical characteristics and course of consecutive adult patients admitted with a diagnosis of acute drug poisoning in the ICU of a tertiary hospital in Oman from January 2007 to December 2008 were reviewed retrospectively from the electronic case records. RESULTS Acute drug poisoning (n=29) constituted 3.9% of admissions to the ICU. Mean age was 29.38±7.9 years. They were brought in by their relatives (72%) or the state services (24%). Accidental poisoning was noted in 21 patients (72%) and suicidal overdosing in 6 (21%). The commonest drug was an opioid (65.5%). Glasgow Coma Scale score of ≤8 was recorded in 18 (62.1%). Sixty two percent of patients required mechanical ventilation. The prominent complications were hypotension in 9 (31%), pulmonary in 19 (65.5%), hepatic in 18 (62.1%) and renal in 12 (41.4%) patients. The major electrolytes abnormalities were low bicarbonate in 11 (37.9%), hyponatremia in 5 (17.2%) and hypokalemia in 4 (13.8%). Patients stayed in the ICU for 1 to 20 days (median-2 days). Factors associated with a longer ICU stay included hypotension upon arrival (p=0.048) and the need for mechanical ventilation on the first (p=0.001) and second (p=0.001) days of hospitalization. There was no mortality. CONCLUSION Early and prompt intensive medical therapy in acute drug poisoning can favorably influence the outcome. In addition, the presence of hypotension and requirement of mechanical ventilation on the first two days of hospitalization were responsible for prolonged ICU stay.

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.

Idiopathic intracranial hypertension presenting as postpartum headache

Postpartum headache is described as headache and neck or shoulder pain during the first 6 weeks after delivery. Common causes of headache in the puerperium are migraine headache and tension headache; other causes include pre-eclampsia/eclampsia, post-dural puncture headache, cortical vein thrombosis, subarachnoid hemorrhage, posterior reversible leukoencephalopathy syndrome, brain tumor, cerebral ischemia, meningitis, and so forth. Idiopathic intracranial hypertension (IIH) is a rare cause of postpartum headache. It is usually associated with papilledema, headache, and elevated intracranial pressure without any focal neurologic abnormality in an otherwise healthy person. It is more commonly seen in obese women of reproductive age group, but rare during pregnancy and postpartum. We present a case of IIH who presented to us 18 days after cesarean section with severe headache and was successfully managed.

Predictive Value of Short-Term EEG Recording in Critically ill Adult Patients

ABSTRACT We assessed the EEG patterns and their prognostic significance in critically ill adult patients with encephalopathy, by digital EEGs lasting up to 1 hour. Of the 110 patients (age: 43.8 ± 19.4 years, male:female:1.6:1) studied, 32% had hypoxic ischemic encephalopathy (HIE), 17% severe infections, and 14.5% stroke. Observed EEG patterns were diffuse slowing (41%), low-voltage cerebral activity (LVCA, 18%), nonconvulsive status epilepticus (NCSE, 13.6%), and periodic abnormalities (9.1%). LVCA, age, Glasgow Coma Score (GCS) < 8, HIE, and modified Hockaday’s EEG grades of IV and V were associated with poor outcome (p < 0.005) at hospital discharge; generalized slowing was associated with a relatively good outcome (p = 0.003). On multivariate analysis, factors independently predictive of mortality were LVCA, older age, and poor GCS. In conclusion, LVCA and generalized background slowing were common EEG patterns among critically ill intensive care unit (ICU) patients with encephalopathy of varied etiologies. While LVCA was associated with a poor outcome, generalized background slowing predicted better prognosis. Conventional short-duration, bedside EEG studies could aid in the recognition of electrographic patterns of prognostic importance in facilities where continuous EEG monitoring is lacking.

Encephalopathy mimicking non-convulsive status epilepticus

Primary hyperammonemic encephalopathy due to urea cycle disorders (UCD) typically manifests with episodic unresponsiveness and this clinical entity is not often included in the differential diagnosis of presumed non-convulsive status epilepticus (NCSE). However, this diagnostic consideration has therapeutic implications. In this report, we document the therapeutic importance of elucidating the specific cause of hyperammonemic encephalopathy that closely mimicked NCSE through 2 unique illustrative cases.

Proximal muscle weakness and skin rash

A 34 year old woman presented with a 9 day history of progressive proximal bilateral limb weakness and mild dysphagia with fluids. On examination, she had a peri-orbital rash (fig 1). Neurological findings included moderately severe (grade 3-4/5) bilateral proximal limb and neck flexor weakness with preserved deep tendon reflexes and sensation. Fig. 1 Peri-orbital rash Her laboratory test findings were Fat suppressed magnetic resonance imaging (MRI) of the thigh muscles showed abnormal signals (hyperintensities) of the lateral, medial, and anterior compartments (fig 2). Fig. 2 Magnetic resonance imaging (MRI) (fat suppressed short tau inversion recovery image) of the thigh muscles. (Left) Coronal section, (right) axial section Needle electromyography of the proximal arm and leg muscles showed fibrillation, positive sharp waves, and early recruitment of volitional motor unit potentials. ### 1. What is the diagnosis? Dermatomyositis—an inflammatory disorder involving the skin and muscles. Figure 1 shows dusky bilateral erythematous macular rashes in the slightly swollen peri-orbital regions. This is a heliotrope rash and is characteristic of dermatomyositis.12 The patient’s proximal limb weakness, mild dysphagia, preserved deep tendon reflexes, elevated creatine kinase, MRI findings (fig 3), and needle electromyography findings are also suggestive of myopathy.34 Fig. 3 Magnetic resonance imaging (MRI) (fat suppressed short tau inversion recovery image) of the thigh muscles. (Left, A) Coronal section, (B, right) axial section. Hyperintensities (stars) …

Prevalence of Sleep-Disordered Breathing During Routine Electroencephalogram (EEG): A Hospital-Based Descriptive Study

Abstract Sleep-disordered breathing (SDB) in adults is a common condition that is associated with a range of medical problems including hypertension, cardiovascular complications, and increase of seizure frequency in susceptible individuals. Polysomnography (PSG) is considered the gold standard measure in the diagnosis of SDB. This is an observational study on the frequency of SDB in adult patients referred for routine EEG. We found that routine EEG was capable of detecting moderate to severe symptoms of SDB in 14% of adult patients (95% confidence interval = 8.1–19.9%). The state of sleep during a routine EEG recording could help in assessing a SDB pattern and could provide an opportunity for further diagnostic sleep consultation if the patient has not previously reported problems with sleep or if SDB was not considered by the referring physician. This study underscores the need for a practice approach to ensure that patients suffering from SDB are properly referred to a sleep specialist. In the context of this report, some training and experience in PSG can be an added advantage for EEG technologists in the detection of SDB.