Manoj Kumar Goyal

Levetiracetam versus phenytoin in management of status epilepticus

The purpose of this study was to compare safety and efficacy of intravenous (IV) levetiracetam (LEV) with IV phenytoin (PHT) in management of status epilepticus (SE). The second-line treatment of SE is limited to a few drugs available in an IV formulation such as PHT, fosphenytoin and valproate. The relative lack of serious side effects and favourable pharmacokinetics of LEV made it a promising option in management of SE. Randomized trials comparing relative efficacy of second-line agents are remarkably lacking. In this study, consecutive patients of SE (n=44) were randomized to receive either IV PHT (20mg/kg) or IV LEV (20mg/kg). The primary end point was successful clinical termination of seizure activity within 30min after the beginning of the drug infusion. Secondary end points included recurrence of seizures within 24 hours, drug related adverse effects, neurological outcome at discharge, need for ventilatory assistance, and mortality during hospitalization. Both LEV and PHT were equally effective with regard to primary and secondary outcome measures. PHT achieved control of SE in 15 (68.2%) patients compared to LEV in 13 (59.1%; p=0.53). Both the groups showed comparable results with respect to recurrence of seizures within 24 hours (p=0.34), outcome at discharge as assessed by functional independence measure (p=0.68), need of ventilatory assistance (p=0.47) and death (p=1). From this study it can be concluded that LEV may be an attractive and effective alternative to PHT in management of SE.

Status epilepticus severity score (STESS): A useful tool to predict outcome of status epilepticus

OBJECTIVE The treatment protocols for status epilepticus (SE) range from small doses of intravenous benzodiazepines to induction of coma. The pros and cons of more aggressive treatment regimen remain debatable. The importance of an index need not be overemphasized which can predict outcome of SE and guide the intensity of treatment. We tried to evaluate utility of one such index Status epilepticus severity score (STESS). METHODS 44 consecutive patients of SE were enrolled in the study. STESS results were compared with various outcome measures: (a) mortality, (b) final neurological outcome at discharge as defined by functional independence measure (FIM) (good outcome: FIM score 5-7; bad outcome: FIM score 1-4), (c) control of SE within 1h of start of treatment and (d) need for coma induction. RESULTS A higher STESS score correlated significantly with poor neurological outcome at discharge (p=0.0001), need for coma induction (p=0.0001) and lack of response to treatment within 1h (p=0.001). A STESS of <3 was found to have a negative predictive value of 96.9% for mortality, 96.7% for poor neurological outcome at discharge and 96.7% for need of coma induction, while a STESS of <2 had negative predictive value of 100% for mortality, coma induction and poor neurological outcome at discharge. CONCLUSION STESS can reliably predict the outcome of status epilepticus. Further studies on STESS based treatment approach may help in designing better therapeutic regimens for SE.

Functional convergence spasm

Convergence spasm (CS) means intermittent episodes of convergence, miosis and accommodation with disconjugate gaze mimicking abducens palsy. The organic causes range from metabolic to host of neurological and ophthalmic diseases that we describe. It was first described as a presentation of psychogenic disorders by von Graefe as early as in 1856. Nonetheless, patients exhibiting this sign are often subjected to plethora of unnecessary, sophisticated and invasive diagnostic procedures. Such functional cases were treated with either cycloplegic/placebo eye drop or amytal abreaction. Though epidemiological studies suggest that conversion disorder is equally prevalent in industrialized nations and developing countries, a few cases of functional CS are reported from West including Asia, that to, decade(s) before and none from India, to the best of our knowledge. We illustrate a case of functional CS with photograph after consent from patient and its successful treatment.

The Diagnostic Dilemma of Neurolymphomatosis.

Neurolymphomatosis (NL) defined as infiltration of the central nervous system or the peripheral nervous system (PNS) by malignant lymphoma cells is a rare clinical entity. However, the increasing use of fluorodeoxyglucose positron-emission tomography (FDG-PET) and magnetic resonance imaging in evaluating PNS disorders is resulting in; this condition being recognized more frequently. Here; we report five NL patients and review the current literature. We report five patients with non-Hodgkins lymphoma (NHL) and NL, all of whom were men aged 47–69 years. The clinical presentation varied from symmetrical peripheral neuropathy to mononeuropathy. Peripheral neuropathy was the presenting manifestation of a systemic lymphoma in two patients (40%). Neuroimaging as well as whole-body FDG-PET helped in determining the correct diagnosis in all of the patients. NL is an unusual presentation of NHL resulting from infiltration of the PNS by malignant lymphomatous cells. While evaluating peripheral neuropathy, a high degree of suspicion of NL is required since the presenting symptoms vary, conventional radiology has only modest sensitivity, and a pathological diagnosis is often difficult. FDG-PET helps in the early diagnosis and treatment of this condition.

A motor neuron disease.

IMPORTANCE Allgrove syndrome is a rare autosomal recessive disorder characterised by achalasia, alacrima, adrenal insufficiency, autonomic dysfunction and amyotrophy. The syndrome has been described in childhood and adult presentation, as in our case, is very rare. There is a considerable delay in diagnosis due to lack of awareness about the syndrome. OBSERVATIONS We report a single case of a 36 year old man who was initially diagnosed and treated for achalasia cardia in our institute 14 years before. After 8 years he presented again with weakness and wasting predominantly distally. He had tongue fasciculations, brisk reflexes and extensor plantar. After supportive electrophysiological studies he was diagnosed as Amyotrophic lateral sclerosis. After 5 years he presented with generalised fatigue without any significant worsening of his neurological status. On reevaluation he had alacrimia, autonomic dysfunction and mild ACTH resistance. CONCLUSIONS AND RELEVANCE Allgrove syndrome may be an underdiagnosed cause of multisystem neurological disease due to the heterogeneous clinical presentation as well as for ignorance of clinician about the syndrome. Based on our case, we also believe that there does exist a subgroup of patients who follow a less severe and chronic course. Recognition of syndrome allows for treatment of autonomic dysfunction, adrenal insufficiency and dysphagia.

Neurocysticercosis: An uncommon cause of drug-refractory epilepsy in North Indian population.

Being a common cause of epilepsy in endemic areas, neurocysticercosis (NCC) is expected to account for a sizable proportion of patients with drug‐refractory epilepsy (DRE) as well. However, data regarding prevalence of DRE in NCC are sparse. This study aimed to determine the prevalence of DRE as well as identification of clinical and radiologic factors that lead to DRE in patients with NCC.

‘Face of giant panda’: a rare imaging sign in Wilson’s disease

A 26-year-old girl presented with fever and jaundice at 17 years of age followed by generalized tonic clonic seizures, right hand postural tremor, reduced school performance, inappropriate laughter and slowness of activities 2 years later. She also reported dystonic posturing of the right upper and lower limb with drooling of saliva. She had a positive Kayser–Fleischer …

Parkinson syndrome: A precise localization for abducens palsy

BACKGROUND Unilateral isolated abducens palsy can occur due to any lesion from pons to orbit. The precise localization is made through the associated neurological signs. Parkinson syndrome is a symptom complex of unilateral abducens palsy with ipsilateral postganglionic Horner syndrome localizing the lesion to posterior cavernous sinus. RESULTS We describe here a 55 year old lady who presented with headache and diplopia for 3 months. On examination she had right lateral rectus palsy and postganglionic Horner syndrome. No other neurological deficit was present. MRI brain and MRA of intracranial vessels showed aneurysm of the right cavernous internal carotid artery which was confirmed on cerebral angiography. Endovascular coiling was advised but refused by the patient and she was treated symptomatically CONCLUSIONS Parkinson syndrome gives precise localization to unilateral abducens palsy and hence is a valuable clinical pearl.

Clinical and radiological predictors of outcome in tubercular meningitis: A prospective study of 209 patients

OBJECTIVES The predictors of poor outcome in tuberculous meningitis (TBM) remain to be delineated. We determined role of various clinical, radiological and cerebrospinal fluid (CSF) parameters in prediction of outcome in TBM. PATIENTS AND METHODS Current study was a prospective observational study including 209 patients of TBM. All patients underwent detailed evaluation including Gadolinium enhanced Magnetic resonance imaging (GdMRI) of brain as well as tests to detect evidence of tuberculosis elsewhere in body. They also underwent GdMRI at three and nine month follow up. All patients received treatment as per standard guidelines. RESULTS Mean age was 30.4±13.8years. 139 (66.5%) patients had definite TBM while 70 (34.5%) had highly probable TBM. 53 (25.4%) patients died. On univariate analysis, longer duration of illness, altered sensorium, stage III TBM, hydrocephalus and exudates correlated with poor outcome. On multivariate analysis presence of hydrocephalus (p=0.003; OR=3.2; 95% CI=1.5-6.7) and stage III TBM (p<0.0001; OR=8.7; 95% CI=3.7-20.2) correlated with higher risk of mortality. In addition, there was significant positive association between presence of hydrocephalus (p=0.05; OR=2.2; 95% CI=0.97-5.1), stage III TBM (p<0.0001; OR=28; 95% CI=4.9-158) and presence of altered sensorium (p=0.05; OR=22; 95% CI=0.99-4.8) with either death or survival with severe disability. CONCLUSIONS It is possible to prognosticate TBM using a combination of clinical and radiological. The duration of illness (65.9±92days) before diagnosis of TBM continues to be unacceptably long and this stresses on need to educate primary care physicians about TBM. Future studies where intensity and duration of treatment is guided by these cues may help in sorting out some of the most difficult questions in TBM, namely duration of antitubercular therapy as well as dose and duration of steroid therapy etc.

‘Hot cross bun’ sign

Multi system atrophy (MSA) is a sporadic neurodegenerative disorder with an onset above the age of 50 years; characterized by autonomic, pyramidal, parkinsonian or cerebellar features and pathologically by the presence of glial cytoplasmic inclusions. Amongst the clinical subtypes, 80% present with predominant parkinsonian features (MSA-P), rest with predominant ataxia (MSA-C).1 Here, we present two cases of MSA-C with typical Magnetic Resonance Imaging (MRI) findings of ‘hot cross bun’ (HCB) sign. A 46-year man presented with insidious onset ataxic gait, hypophonic slow speech …