Background: Leflunomide is a disease-modifying, anti-rheumatic drug, whose active metabolite Teriflu-nomide is licensed drug therapy for reducing progression in Multiple sclerosis. Intravenous high dose Methyl-Prednisolone is an established treatment for acute relapses of multiple sclerosis that escalates reco-very. Intravenous methyl-prednisone also being used anecdotally for the prevention of relapses. Methods: Each patient after enrollment in the study received treatment for 1 year. A total of 30 patients, 15 patients in each group were taken. Patients fulfilling inclusion criteria and giving written informed con-sent were randomly divided into 2 groups (Group A and Group B) using a computer-generated random numbers table. Group A patients received Leflunomide 20mg with a loading dose of 3 tablets for 3 days and then once daily for one year. And group B patients received high-dose intravenous Methyl Prednisolone 1g monthly for a period of one year. Results: Methyl Prednisolone group, 5 (33.3%) had only 1(%) relapse and 10 (66.7%) cases had no relapse. In contrast, Leflunomide group, no relapse was recorded. The frequency of relapse was consequently statistically higher in Methyl Prednisolone group, p-value < 0.05. In respect of, a number of lesions on MRI; Methyl Prednisolone group demonstrated no change in 4 (26.67%) cases and 11 (73.33%) cases had lesions increased from baseline. Whereas, in the Leflunomide group the number of lesions significantly decreased (p-value < 0.05). Significant improvement in Expanded disability status was observed leflunomide group (p-value < 0.05). Conclusion: Leflunomide was more effective in preventing relapses and in decreasing disease activity on MRI, as compared with high dose monthly Methyl-prednisone in patients with Multiple Sclerosis.

Cerebral venous sinus thrombosis (CVST) is a rare cause of stroke that accounts for 0.5–1.0% of all strokes. Clinical presentation, predisposing factors, neuroimaging findings, and outcomes of CVST are extremely diverse, which causes a high index of suspicion in diagnosis. Therefore, early diagnosis of CVST is crucial for prompt treatment to prevent morbidity and mortality. Objective: The purpose of this prospective study is aimed at assessing the clinical characteristics, potential risk factors, and neuro-radiological features along with the topography of venous sinus involved in CVST patients in a tertiary care hospital, Lahore, Pakistan. Material and Methods: Consecutive patients enrolled in this study had a computed tomography (CT) scan, magnetic resonance imaging (MRI), and magnetic resonance venography (MRV) along with a clinical presentation to confirm the diagnosis of CVST. Categorical data were presented as percentages. Continuous variable and categorical data were compared (parenchymal lesions vs. non-parenchymal lesions) using the Student’s t-test and Chi-square test, respectively. Results: A total of 3261 patients with stroke were presented during the study period. Out of all patients, 53 confirmed patients with CVST (1.6%) were recruited; the predominant population was female (84.91%), having a male to female ratio of 1:4. Mean age of the cohort was 28.39 ± 7.19 years. Most frequent symptoms observed were headache (92.45%) followed by vomiting (75.47%), seizures (62.26%), papilledema (54.72%), visual impairment (41.51%), and altered consciousness disturbance (52.83%). The presumed risk factors associated with CVST were puerperium (52.83%), use of oral contraceptives (13.21%), antiphospholipid syndrome (7.55%), elevated serum levels of protein C and S (5.66%), and CNS infection (3.77%). On cranial CT scans, 50 patients (94.33%) showed abnormalities while 32 patients exhibited various parenchymal lesions. Seizures were more frequent in CVST patients with parenchymal lesions compared with subjects lacking parenchymal lesions. Seventy-two sinuses, either single or in combination, were involved in CVST patients, being more common in patients with parenchymal lesions than those without parenchymal lesions. The most frequent locations of CVST were the superior sagittal and transverse sinus. Conclusion: In short, non-contrast CT brain may be used as a first line investigation in suspected cases of CVST. Our study also demonstrates some regional differences in the clinical features, risk factors, and neuroimaging details of CVST as described by some other studies. Therefore, care must be taken while diagnosing and predicting the outcome of the CVST.

OBJECTIVE To determine the frequency of low and high anti-AChR (acetylcholine receptor) antibody titers and to evaluate their relationship with clinical severity in myasthenia gravis. STUDY DESIGN Cross-sectional, observational study. PLACE AND DURATION OF STUDY Department of Neurology, King Edward Medical University/Mayo Hospital, Lahore from April 2017 to March 2018. METHODOLOGY Fifty-six seropositive patients, aged between 18-75 years, were included. A blood sample was obtained from each patient to assess for the anti-AChR antibody titers by enzyme-linked immunosorbent assay (ELISA) technique and classified as low (0.4-<50 nmol/L) and high AChR antibody titers (>50 nmol/L). Clinical severity was graded according to the Osserman's classification. RESULTS Out of 56 patients, 51.79% (n=29) were males and 48.21% (n=27) were females, and mean age was 32.73 +8.48 years. Mean anti-AChR antibody titer was found 40.45 + 13.54; 60.71% (n=34) had low and 39.29% (n=22) had high titers. Upon grading the severity, 1.79% (n=1) had grade I, 25% (n=14) had grade IIa, 26.79% (n=15) had grade IIb, 37.5% (n=21) had grade III, and 8.93% (n=5) had grade IV. These grades were significantly associated with high/low titers of anti-AChR antibody (p<0.001) but no significant association was found with age and gender (p=0.39 and 0.19 respectively). CONCLUSION Serum concentration of anti-AChR antibodies has significant association with the clinical severity in myasthenia gravis. Key Words: Anti-acetylcholine receptor antibody, Myasthenia gravis, Neuromuscular junction diseases.

Neurology still remains one of the most underserved specialties of medicine in Pakistan with roughly one neurologist per million people. Movement disorders (MD) are neurological problems that interfere with patient's motor abilities and diagnosis is typically clinical. In this review, we describe a practical approach to common MD emergencies that may be encountered by a non-neurologist physician, emphasizing on formulating a working diagnosis and their immediate management. Movement disorder emergencies can be classified based on MD phenomenology and we will provide a brief overview of dystonia including acute dystonic reaction, PAID syndrome and dystonic storm; chorea, myoclonus including serotonin syndrome and startle disease; and rigidity including neuroleptic malignant syndrome and malignant hyperthermia.

April 24, 2018April 10, 2018Free AccessFeasibility of Online Resident Education in Pakistan with international collaborations: Implementing Blended learning (P3.023)Danish Bhatti, Nadir Syed, Athar Javed, Muhammad Saleem Ilyas, John Bertoni, and Arsalan AhmadAuthors Info & AffiliationsApril 10, 2018 issue90 (15_supplement)https://doi.org/10.1212/WNL.90.15_supplement.P3.023 Letters to the Editor

April 23, 2018April 10, 2018Free AccessCharacteristics of Patients with Parkinson Disease in Pakistan: Prospective Multicenter Cross-sectional Study (P2.068)Wagma Shahzad, Saba Aslam, Kiran Waqar, Muslim Lakhiar, Nabeel Syed, Farheen Niazi, Safia Bano, … Show All … , Waqas Arshad, Manzoor Lakhair, Athar Javed, Nadir Syed, Arsalan Ahmad, Zikria Saleem, John Bertoni, and Danish Bhatti Show FewerAuthors Info & AffiliationsApril 10, 2018 issue90 (15_supplement)https://doi.org/10.1212/WNL.90.15_supplement.P2.068 Letters to the Editor

OBJECTIVE:To determine the frequency of metabolic syndrome and its components in patients with carpal tunnel syndrome.STUDY DESIGN:Case-series.PLACE AND DURATION OF STUDY:Department of Neurology, Mayo Hospital, Lahore, from January to June 2012.METHODOLOGY:Seventy-five (64 females and 11 males) patients with clinically diagnosed and electrodiagnostically confirmed carpal tunnel syndrome were inducted. Their waist circumference, blood pressure, fasting blood glucose, fasting triglycerides and high density lipoprotein cholesterol levels were recorded. Patients were categorized having metabolic syndrome according to Adult Treatment Panel III criteria, if any 3 were present out of hypertension, elevated fasting triglycerides, reduced high density lipoprotein cholesterol, elevated fasting blood glucose, and elevated waist circumference.RESULTS:Mean age of the patients was 42.04 ±9.31 years, mean waist circumference was 95.32 ±9.03 cm, mean systolic blood pressure was 134.13 ±13.72 mmHg, mean diastolic blood pressure was 89.13 ±8.83 mmHg, mean fasting blood glucose was 94.35 ±21.81 mg/dl, mean fasting triglycerides was 177.48 ±48.69 mg/dl, and mean high density lipoprotein cholesterol was 41.95 ±11.17 mg/dl. Metabolic syndrome was found in 54 (72%) patients including 9 (16.7%) males and 45 (83.3%) females. Out of 75 patients, 54 (72%) had elevated waist circumference, 52 (69.3%) had elevated blood pressure, 19 (25.3%) had elevated fasting blood glucose, 53 (70.6%) had elevated fasting triglycerides and 54 (72%) had reduced high density lipoprotein cholesterol. Highest frequency of metabolic syndrome was found in age range of 40 - 49 years in both genders.CONCLUSION:Metabolic syndrome is frequently found in the patients with carpal tunnel syndrome.

A 30-year-old woman presented with progressive history of acroamputations of both hands and feet since early childhood. Because of her insensitivity to pain, she had repeated trauma, recurrent burns, and self-mutilating behavior, ie, tongue biting, self–tooth extraction, and frequent scratching of the nasal septum, eventually leading to its complete erosion. The patient had recurrent episodes of unexplained fever, heat intolerance, especially during summer, anhidrosis, and hypolacrimia. Her parents were related by first-degree consanguinity. She had 4 living siblings, with similar complaints in her elder sister. There was history of infant death due to recurrent high-grade fever in 6 of her siblings. The patient had a depressed nose, corneal opacity in the left eye, an eroded tip of the tongue, multiple missing teeth (Figure 1, A and B), and acroamputations in both hands and feet with plantar ulcers (Figure 2, A and B). She had sluggishly reactive pupils, diminished facial and oral sensation, and loss of pain and temperature sensation. Autonomic dysfunction was documented by abnormal results of isometric handgrip, cold pressor, starch-iodine sweat, Schirmer, and skin wrinkle testing and abnormal 30:15 ratio and E/I ratio on electrocardiography. Radiographs of the hands and feet revealed loss of multiple phalanges and osteolytic defects (Figure 3, A and B). Nerve conduction studies found normal motor function with absent sensory nerve action potentials. A smear test was negative for Mycobacterium leprae.Figure 2A, Acroamputations of both hands. B, Acroamputations of both feet and plantar ulcers.View Large Image Figure ViewerDownload (PPT)Figure 3A, Radiograph of both hands showing loss of multiple phalanges. B, Radiograph of both feet showing osteolytic defects, marked porosis, and bony destructions.View Large Image Figure ViewerDownload (PPT) Congenital insensitivity to pain with anhidrosis is an autosomal recessive disorder caused by lack of small myelinated and unmyelinated fibers of the peripheral nerves causing absence of pain and temperature sensation. Symptoms begin early in infancy and lead to multiple injuries, fractures, osteomyelitis, self-mutilation, and acroamputation.1Axelrod F.B. Gold-von Simson G. Hereditary sensory and autonomic neuropathies: types II, III and IV.Orphanet J Rare Dis. 2007; 2: 39Crossref PubMed Scopus (141) Google Scholar Defects in thermoregulation and anhidrosis lead to episodic hyperthermia, which may be associated with seizures. Up to 20% of patients die from hyperpyrexia by age 3. Mild to moderate mental retardation is often present. Tearing is preserved, and fungiform papillae are present on the tongue.2Rosemberg S. Marie S.K. Kliemann S. Congenital insensitivity to pain with anhidrosis (hereditary sensory and autonomic neuropathy type IV).Pediatr Neurol. 1994; 11: 50-56Abstract Full Text PDF PubMed Scopus (164) Google Scholar