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Background: Understanding the regional needs and available healthcare resources to treat Parkinson’s disease (PD) is essential to plan appropriate future priorities. The International Parkinson and Movement Disorder Society (MDS) Task Force for the Middle East was established to raise awareness and promote education across the region on PD and other movement disorders. Broadly, the task force encompasses the countries of the Middle East but has included North Africa and South Asia as well (MENASA). Objective: To create a list of needs and priorities in the advancement of PD in MENASA countries based on consensuses generated by the MDS task force for the Middle East. Methods: A Strengths Weaknesses-Opportunities-Threats (SWOT) analysis was conducted by the task force members to generate consensus about PD care this region. Results: Eight overarching principles emerged for the consensus statement on current needs: more movement disorders specialists, multidisciplinary care, accurate epidemiologic data, educational programs, availability of drugs, and availability of more advanced therapy, enhanced health care resources and infrastructure, and greater levels of awareness within the general population and among health care professionals. Conclusion: This pilot study sheds light on unmet needs for providing care to people with PD in the MENASA region. These data offer directions on priorities to increase awareness of PD, to develop better infrastructure for research and management of PD, to foster healthcare policy discussions for PD and to provide educational opportunities within these countries.

Most patients with autoimmune encephalitis do not present with well-described symptoms. Demographic data and information regarding co-morbidities could help in diagnosing the underlying disorder, but a definitive diagnosis is made by the result of autoimmune antibodies. Limbic encephalitis (LE), a variant of autoimmune encephalitis, is the inflammation of the limbic system of the brain. The disorder presents with the rapid development of confusion, working memory impairment, mood changes, and often seizures. LE could have paraneoplastic or non-paraneoplastic etiology. We present the case of a 15-year-old girl with seronegative LE, who presented with cognitive decline and seizures. This condition is rare, and therefore poses a great challenge in diagnosis at an early stage.

PurposeOrganizational culture is a determinant for quality improvement. This paper aims to assess organizational culture in a hospital setting, understand its relationship with perceptions about quality of care and identify areas for improvement.Design/methodology/approachThe paper is based on a cross‐sectional survey in a large clinical department that used two validated questionnaires. The first contained 20 items addressing perceptions of cultural typology (64 respondents). The second one assessed staff views on quality improvement implementation (48 faculty) in three domains: leadership, information and analysis and human resource utilization (employee satisfaction).FindingsAll four cultural types received scoring, from a mean of 17.5 (group), 13.7 (developmental), 31.2 (rational) to 37.2 (hierarchical). The latter was the dominant cultural type. Group (participatory) and developmental (open) culture types had significant positive correlation with optimistic perceptions about leadership (r=0.48 and 0.55 respectively, pr=−0.61, ppr=−0.55, pResearch limitations/implicationsIt is likely that, by adopting a participatory and open culture, staff views about organizational leadership will improve and employee satisfaction will be enhanced. This finding has implications for quality care implementation in other hospital settings.Originality/valueThe paper bridges an important gap in the literature by addressing the relationship between culture and quality care perceptions in a Pakistani hospital. As such a new and informative perspective is added.

Introduction:There are no studies from Pakistan that describe stroke presentation rates or factors associated with early or delayed presentation. This is important to know because current clinical protocols limit the use of recombinant tissue plasminogen activator (rtPA), the only available therapy for acute ischemic stroke, to a three-hour window from symptom onset.Methods:All patients aged 14 years or above with acute ischemic stroke of ≤ 48 hours duration were prospectively identified from the Aga Khan University Stroke Data Bank over a 22-month period ending May 2001.Results:269 ischemic stroke patients presented within 48 hours of stroke onset. 55 out of 269 (21%) presented within first three hours and 110 out of 269 (41%) within first six hours. Unawareness of treatment options (p p=0.005) were associated with delayed presentation. Small vessel occlusion or lacunar stroke in the TOAST (Trial of ORG 10172 in Acute Stroke Treatment) ischemic stroke subtype was associated with delayed presentation (p=0.047) and cardioembolic stroke was associated with earlier presentation (p=0.048). Stroke severity assessed with the National Institutes of Health Stroke Scale at a cut off score of ≥ 15 was not associated with earlier time to presentation at three hours (p=0.114) but there was some tendency at six hours (p=0.097).Conclusions:The rate of early stroke presentation in a Pakistani tertiary care facility is comparable to certain developed countries. To increase the proportion of patients who can benefit from thrombolytic therapy, programs need to be instituted to increase public awareness of treatment options for stroke and expedited referral by the primary care provider.

Context The subject of economic migration among health care professionals has received intense attention. However, the aetiology of this migration has not been rigorously evaluated in Pakistan. Such knowledge can potentially influence health care and academic policies. Our current study proposes to quantify the relative contributions of various personal, professional and economic variables among final-year medical students in Karachi. Methods A self-administered structured questionnaire using a 10-point scale was developed and piloted among Karachi medical students. Additional open-ended questions were included to allow us to capture information not otherwise covered in the questionnaire. spss software was used for data entry and analysis. Results Over 95% of Aga Khan University (AKU) and over 65% of Baqai University (BU) final-year medical students intend to proceed abroad for their postgraduate training. The 2 most important factors behind this intent as pointed out by the students are poor salary structure (AKU mean score 8.94 ± 1.73, BU mean score 7.14 ± 2.6) and poor quality of training in the home country (AKU mean score 9.20 ± 1.20, BU mean score 8.68 ± 2.03). Other interesting factors captured through the open-ended questions were the poor work environment and lack of rigor in teaching of residents in domestic university hospitals. Over 50% of final-year medical students cited these issues as major reasons behind their decision to migrate.

Muscle stiffness in stiff-person syndrome (SPS) is produced by continuous, involuntary firing of motor units that is thought to be caused by an autoimmune mediated dysfunction of GABA-ergic inhibitory neurones. We have postulated that the loss of GABA-ergic inputs from spinal interneurones alone is insufficient to produce tonic firing of motor neurones and that excessive supraspinal excitation could also play a role. To determine whether SPS is associated with dysfunction in supraspinal GABA-ergic neurones, we assessed the excitability of the motor cortex with transcranial magnetic stimulation (TMS) in seven SPS patients and seven age-matched healthy volunteers. SPS patients had normal central motor conduction times, normal thresholds for motor evoked potentials (MEPs) in leg muscles, and a normal MEP stimulus versus response recruitment curve with increasing TMS intensities in resting hand and leg muscles. Cortical silent periods were shortened in leg muscles. Intracortical inhibition and excitation were assessed while recording from the abductor pollicis brevis, using a paired pulse TMS paradigm with subthreshold conditioning stimuli. Patients had decreased inhibition and markedly increased facilitation at short intervals. Using paired suprathreshold TMS, patients exhibited increased facilitation at 20- and 40-ms intervals. These results point to a hyperexcitability of the motor cortex in SPS, which could be explained by impairment of supraspinal GABA-ergic neurones, leading to an impaired balance between inhibitory and excitatory intracortical circuitry.

We assessed the cutaneous silent period (CSP) in 24 patients with Fabry disease with small-fiber sensory neuropathy and 12 normal subjects to test the hypothesis that small-diameter afferents are responsible for producing the CSP. Sensory nerve conduction studies and quantitative sensory testing for cold and vibration detection thresholds were also measured. Overall, Fabry patients had impaired thermal, but not vibration, detection thresholds, with greatest impairment in the feet. In the upper extremity, CSP latencies, duration, and suppression of electromyographic activity (EMG) did not differ. In the lower extremity, patients had reduced suppression of EMG during the CSP compared to normal controls. CSP durations exhibited a bimodal distribution in patients, including a subset of seven patients with durations shorter than all controls. This subset had profound loss of thermal sensation in the feet, but this was also true of some patients who had normal CSPs. Patients with shortened CSPs had modestly elevated vibration thresholds and reduced sensory potentials in comparison to patients with normal CSPs. Reduced CSPs in Fabry patients are associated with, but not entirely explained by, the severity of small-fiber neuropathy as measured by quantitative sensory testing. The possibility that large-diameter fibers provide a minor contribution to producing the CSP should be considered.

The transient suppression of muscle contraction during the cutaneous silent period (CSP) could be produced either through postsynaptic inhibition of motoneurons or through presynaptic inhibition of the excitatory inputs to motoneurons that sustain voluntary contraction. We sought to delineate the mechanisms underlying the CSP in hand muscles by measuring changes in H-reflexes and motor-evoked potentials (MEPs) produced by transcranial magnetic stimulation (TMS) during the CSP in 10 healthy volunteers. H-reflexes and MEPs both measure the excitability of the motoneuron pool and activate similar subpopulations of motoneurons through different pathways. Inhibition of H-reflexes and MEPs of similar size was maximal at the midpoint of the CSP and gradually returned to baseline. The similar time course of recovery suggests that the H-reflex and MEP are affected by inhibition at a common site, most likely postsynaptic inhibition of the motoneurons.

Spontaneous Intracranial Hypotension is a syndrome involving reduced intracranial pressure secondary to a dural tear which occurs mostly due to connective tissue disorders such as Marfans Syndrome, and Ehler Danlos Syndrome. Patients with dural ectasias leading to CSF leakage into the subdural or epidural space classically present with orthostatic headaches and cranial nerve deficits mostly seen in cranial nerves V-VIII. Diagnosis of SIH is confirmed with the aid of neuroimaging modalities of which Cranial MR imaging is most widely used. SIH can be treated conservatively or with epidural blood patches which are now widely being used to repair dural tears, and their effectiveness is being recognized. Recently epidural injection of fibrin glue has also been used which has been found to be effective in certain patients.

Objective:Our main objective was to use videoconferencing as a primary means to: a) assist in launching an epilepsy surgery program in Pakistan; 2) participate in case conferences on complex epilepsy patients in each country.Methods:Extensive testing using both point to point and bridged integrated service digital network (ISDN) and internet protocol (IP) connections was carried out using bandwidths of 384-768 kilobits per second (kbps). Videoconferences between sites were arranged two to three weeks in advance and connections were tested a day prior to the scheduled conference. Sharing of PowerPoint presentations, neuroimaging and video-EEG was available to all sites. Discussions centered on patients with medically refractory epilepsy.Results:Between July 2006 and June 2008, 17 sessions were booked. Five of these conferences bridged in specialists from West Virginia University. Most successful connections occurred using IP point to point calls or a bridge connecting end points through IP at 512 kbps. We conducted three surgeries for medically refractory temporal lobe epilepsy in Pakistan. At follow-up in January 2009, two patients have been seizure free and one had two breakthrough seizures after sudden unsupervised discontinuation of Levetiracetam.Conclusion:Our international tele-epilepsy collaboration has proven feasible and valuable to all participants. Our experience suggests considerable thought and preparation are needed before a teleconference to ensure its success. We provide a recipe to set-up similar telemedicine collaborations. Considerations include time zone differences, equipment type, interoperability between endpoints, connection capabilities, bandwidth availability, and backup plans for unsuccessful connections. Telemedicine can facilitate epilepsy care around the world, identifying with the concept of a “Global Health Village”.

Background Intracranial stenosis is the most common cause of stroke among Asians. It has a poor prognosis with a high rate of recurrence. No effective medical or surgical treatment modality has been developed for the treatment of stroke due to intracranial stenosis. We aim to identify risk factors and biomarkers for intracranial stenosis and to develop techniques such as use of transcranial doppler to help diagnose intracranial stenosis in a cost-effective manner. Methods/Design The Karachi Intracranial Stenosis Study (KISS) is a prospective, observational, case-control study to describe the clinical features and determine the risk factors of patients with stroke due to intracranial stenosis and compare them to those with stroke due to other etiologies as well as to unaffected individuals. We plan to recruit 200 patients with stroke due to intracranial stenosis and two control groups each of 150 matched individuals. The first set of controls will include patients with ischemic stroke that is due to other atherosclerotic mechanisms specifically lacunar and cardioembolic strokes. The second group will consist of stroke free individuals. Standardized interviews will be conducted to determine demographic, medical, social, and behavioral variables along with baseline medications. Mandatory procedures for inclusion in the study are clinical confirmation of stroke by a healthcare professional within 72 hours of onset, 12 lead electrocardiogram, and neuroimaging. In addition, lipid profile, serum glucose, creatinine and HbA1C will be measured in all participants. Ancillary tests will include carotid ultrasound, transcranial doppler and magnetic resonance or computed tomography angiogram to rule out concurrent carotid disease. Echocardiogram and other additional investigations will be performed at these centers at the discretion of the regional physicians. Discussion The results of this study will help inform locally relevant clinical guidelines and effective public health and individual interventions.

To identify frequency of hypotension in a large cohort of patients with intracerebral haemorrhage and its prognostic significance.We retrospectively reviewed medical records of 920 patients with spontaneous intracerebral haemorrhage (ICH). Patients were divided in three groups based on Diastolic blood pressure (DBP); hypotensive group (DBP70 mmHg), normotensive group (DBP; 71-90 mmHg) and hypertensive group (DBP90 mmHg).Of the total patients with ICH, 7% (64) presented with hypotension, 13% (120) were normotensive and 80% (736) were hypertensive. In the hypotensive group, 37% (24) patients died as compared to 25%(30) in normotensive group and 25% (182) in hypertensive group (p = 0.03). Hypotension at presentation, thalamic and lobar haemorrhages were predictors of poor outcome. Patients with diastolic BP of less than 70 were significantly more likely to die than with DBP 71-90 (OR = 1.9, 95% CI; 1.1-2.9, p = 0.03). This relationship was still significant after adjusting for age, sex, history of presentation, coma at presentation and location of haemorrhage (OR = 1.45, 95% CI; 1.0-2.2, p = 0.045).Our findings suggest that hypotension at presentation is a predictor of poor outcome in patients with ICH. Patients with diastolic blood pressure less than 70 are more likely to have a fatal outcome as compared to those with normal blood pressure.

This paper traces the history and describes the status of surgical training in Pakistan. A key revelation is that excellent surgeons are produced through systems which on formal review might appear to lack standards. Personal characteristics of residents modify outcomes in high volume surgical training units; and consequent variation in quality of outputs is noted. Attention needs to be given to (i) develop new educational systems which are not prolonged costly and cumbersome, and which produce the adequate number, types and spread of highly skilled and cognitively developed empathic surgeons for the country; (ii) the improvement of the health systems which currently impede the development of surgeons and (iii) novel wars of tackling rural urban disparities in health delivery.

The subject of economic migration among health care professionals has received intense attention. However, the aetiology of this migration has not been rigorously evaluated in Pakistan. Such knowledge can potentially influence health care and academic policies. Our current study proposes to quantify the relative contributions of various personal, professional and economic variables among final-year medical students in Karachi.A self-administered structured questionnaire using a 10-point scale was developed and piloted among Karachi medical students. Additional open-ended questions were included to allow us to capture information not otherwise covered in the questionnaire. SPSS software was used for data entry and analysis.Over 95% of Aga Khan University (AKU) and over 65% of Baqai University (BU) final-year medical students intend to proceed abroad for their postgraduate training. The 2 most important factors behind this intent as pointed out by the students are poor salary structure (AKU mean score 8.94 +/- 1.73, BU mean score 7.14 +/- 2.6) and poor quality of training in the home country (AKU mean score 9.20 +/- 1.20, BU mean score 8.68 +/- 2.03). Other interesting factors captured through the open-ended questions were the poor work environment and lack of rigor in teaching of residents in domestic university hospitals. Over 50% of final-year medical students cited these issues as major reasons behind their decision to migrate.

Cerebral venous sinus thrombosis (CVT) can affect all age groups, particularly women of childbearing age. Overall prognosis for survival and functional independence is better than it was believed. Mortality usually ranges from 6-15% and transtentorial herniation is the major cause of death. Approximately 80% of patients are functionally independent in the long term. Altered mental status and cerebral haemorrhage at presentation are the strongest predictors of death and disability. Patients with CVT related to pregnancy and puerperium generally do better than patients with other causes. Septic CVT carries a worse prognosis than aseptic CVT and of the latter, patients with syndrome of isolated intracranial hypertension have a better prognosis than those with focal deficits or encephalopathy. Anticoagulation is believed to improve outcome in CVT although robust data are lacking. Epilepsy, headaches, visual loss, pyramidal deficits and cognitive impairment are some of the long term sequelae. The risk of recurrence of CVT is low, particularly after the first 12 months of the first episode.

Neuroleptic Malignant Syndrome (NMS) is a medical entity that has received little attention in the clinical settings in Pakistan. The aim of our study was to review the predisposing factors, outcomes and characteristics of in-patients diagnosed with NMS.We performed a retrospective chart review of all cases (age15 years) at a tertiary care center in Karachi between January 01, 1990 and November 30, 2001, diagnosed using ICD 10 coding. Data was collected using a standardized data entry form and statistical analysis was performed using Epi Info 6, Version 6.02.There were a total of 20 patients diagnosed with NMS (11 male and 9 female) in our study with a mean age of 46.6 +/- 15.9 years. Haloperidol was the most frequently responsible neuroleptic. Of the 18 patients on a neuroleptic, most developed NMS after 8 weeks of therapy. There were 5 mortalities all of which were associated with septic shock. Fourteen patients recovered completely from the episode and did not have any neurologic sequelae.NMS is an important preventable clinical entity. Early diagnosis and judicious use of antipsychotics is warranted to prevent mortality and heightened morbidity.

To study the clinical presentation, hospital course and outcome of patients admitted with Guillain Barre Syndrome (GBS) to a tertiary care hospital in Karachi, Pakistan.The charts of patients conforming to International Classification of Diseases (ICD) code 9.0, for GBS, from September 1995 to January 2003 were reviewed. Clinical data was recorded on a standardized questionnaire, which included patients' age, sex, antecedent events, neurological signs and symptoms and ventilation requirement. The hospital course was analyzed, including nosocomial infections, therapy given and the functional status of patients, using the Rankin scale (0-6). Standard SPSS 11.5 software (Windows) was used for data analysis.Thirty-four cases of GBS were admitted to the hospital during the study period, with an age range of 3 to 70 years. The mean age for disease onset was 35.2 years for female patients, compared to 30 years for males; the male/female ratio was 1.6:1.Gastrointestinal infections (12/22, 54.6%) were the most common antecedent event, followed by upper respiratory tract infections (9/22, 40.9%) and skin lesions (1/22, 4.5%). Most patients developed GBS within one month of the preceding infection. Cranial nerve abnormalities (30/34, 88.2%), autonomic dysfunction (21/34, 61.8%) and respiratory failure requiring intubation (19/34, 55.9%) were also common. The median Rankin score of patients at admission, and at 30 and 60 days thereafter was 5, 4 and 3.5 respectively. The in-patient mortality was 1 of 34 (2.4%).We found that GBS occurred at all ages and was slightly more common in males. Majority of patients had an antecedent history of infection and had severe disease on presentation. The patients were treated with either plasmapheresis or intravenous immunoglobulins and there was no significant difference in outcome in the two groups. Despite severe persistent disability, in-hospital mortality was low.

Two cases of siblings diagnosed as cases of familial homocystinuria are reported. Both the cases have classical presentation of familial homocystinuria including history of dislocation of lens of the right eye. Brother had history of psychomotor retardation while sister had a significant history of deep vein thrombosis. Levels of plasma homocysteine were elevated and urinary homocysteine was positive in both the cases.

Kleine-Levin Syndrome (KLS) is a rare disorder of uncertain etiology, characterized by recurring episodes of undue sleepiness lasting for days. We report a case of young female presenting with episodes of undue sleep along with hypersexuality and excessive food intake, who improved significantly on lithium and valproate. KLS should be considered in young patients, who present with episodes of undue somnolence.

To identify the factors that predispose to ischemic versus hemorrhagic stroke in hypertensive patients.Cohort study.The study was conducted at the Aga Khan University Hospital (AKUH), Karachi, from August 1999 to May 2001.All the hypertensive patients, who were registered in AKUH acute stroke outcome data base, over a period of 22 months, were identified and from this cohort the patients with first ever stroke were selected. The data regarding demographics, stroke type (ischemic vs. hemorrhagic), pre-existing medical problems, laboratory and radiological investigations was recorded and analyzed.Five hundred and nineteen patients with either ischemic stroke or parenchymal hemorrhage were registered over a period of 22 months. Three hundred and forty-eight patients (67%) had hypertension and of these, 250 had first ever stroke at the time of admission. Presence of diabetes mellitus (OR: 3.76; CI:1.67-8.46) and ischemic heart disease (OR: 6.97; CI:1.57-30.98) were found to be independent predictors of ischemic strokes.Presence of diabetes mellitus and ischemic heart disease predict ischemic stroke in a patient with hypertension.

We prospectively evaluated thalidomide-induced neuropathy using electrodiagnostic studies. Sixty-seven men with metastatic androgen-independent prostate cancer in an open-label trial of oral thalidomide underwent neurologic examinations and nerve conduction studies (NCS) prior to and at 3-month intervals during treatment. NCS included recording of sensory nerve action potentials (SNAPs) from median, radial, ulnar, and sural nerves. SNAP amplitudes for each nerve were expressed as the percentage of its baseline, and the mean of the four was termed the SNAP index. A 40% decline in the SNAP index was considered clinically significant. Thalidomide was discontinued in 55 patients for lack of therapeutic response. Of 67 patients initially enrolled, 24 remained on thalidomide for 3 months, 8 remained at 6 months, and 3 remained at 9 months. Six patients developed neuropathy. Clinical symptoms and a decline in the SNAP index occurred concurrently. Older age and cumulative dose were possible contributing factors. Neuropathy may thus be a common complication of thalidomide in older patients. The SNAP index can be used to monitor peripheral neuropathy, but not for early detection.

Background Autonomic nervous system (ANS) dysfunction is present in up to one third of patients with tetanus. The prognostic value of ANS dysfunction is known in severe tetanus but its value is not well established in mild to moderate tetanus. Methods Medical records of all patients admitted with tetanus at two academic tertiary care centers in Karachi, Pakistan were reviewed. The demographic, clinical and laboratory data was recorded and analyzed. ANS dysfunction was defined as presence of labile or persistent hypertension or hypotension and sinus tachycardia, tachyarrythmia or bradycardia on EKG. Patients were divided into two groups based on presence of ANS dysfunction (ANS group and non ANS group). Tetanus severity was classified on the basis of Ablett criteria. Results Ninety six (64 males; 32 females) patients were admitted with the diagnosis over a period of 10 years. ANS group had 31 (32%) patients while non ANS group comprised of 65 (68%) patients. Both groups matched for age, gender, symptom severity, use of tetanus immunoglobulin and antibiotics. Twelve patients in ANS group had mild to moderate tetanus (Ablett I and II) and 19 patients had severe/very severe tetanus (Ablett III and IV). Fifteen (50%) patients in ANS group required ventilation as compared to 28 (45%) in non-ANS group (p = 0.09). Fourteen (47%) patients died in ANS group as compared to 10 (15%) in non ANS group (p= 0.002). Out of those 14 patients died in ANS group, six patients had mild to moderate tetanus and eight patients had severe/ very severe tetanus. Major cause of death was cardiac arrhythmias (13/14; 93%) in ANS group and respiratory arrest (7/10; 70%) in non ANS group. Ten (33%) patients had complete recovery in ANS group while in non ANS group 35(48%) patients had complete recovery (p= 0.05). Conclusions ANS dysfunction was present in one third of our tetanus population. 40% patients with ANS dysfunction had only mild to moderate tetanus. ANS dysfunction, irrespective of the need of mechanical ventilation or severity of tetanus, predicted poor outcome.

Background We describe the case of a 38 year old male with Marfan syndrome who presented with orthostatic headaches and seizures. Case Presentation The patient was diagnosed with Spontaneous Intracranial Hypotension secondary to CSF leaks, objectively demonstrated by MR Myelogram with intrathecal contrast. Epidural autologus blood patch was administered at the leakage site leading to significant improvement. Conclusion Our literature search shows that this is the second reported case of a Marfan patient presenting with symptomatic spontaneous CSF leaks along with tonsillar herniation.