Pathophysiology and management of spontaneous intracranial hypotension--a review.

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.

Experiences from an international tele-epilepsy collaboration.

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".

Culture and quality care perceptions in a Pakistani hospital.

PURPOSE: Organizational 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/APPROACH: The 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). FINDINGS: All 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, p < 0.00). Hierarchical (bureaucratic) culture was significantly negatively correlated with domains; leadership (r = -0.61,p < 0.00), information and analysis (-0.50, p < 0.00) and employee satisfaction (r = -0.55, p < 0.00). Responses reveal a need for leadership to better utilize suggestions for improving quality of care, strengthening the process of information analysis and encouraging reward and recognition for employees. RESEARCH LIMITATIONS/IMPLICATIONS: It 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/VALUE: The 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.

The Karachi intracranial stenosis study (KISS) Protocol: an urban multicenter case-control investigation reporting the clinical, radiologic and biochemical associations of intracranial stenosis in Pakistan.

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.

Early ischemic stroke presentation in Pakistan.

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 < or = 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 < 0.001) and inappropriate diagnosis and field triage (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 > or = 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.

Hypotension at presentation is an indicator of poor prognosis in acute intracerebral haemorrhage.

OBJECTIVE: To identify frequency of hypotension in a large cohort of patients with intracerebral haemorrhage and its prognostic significance. METHODS: 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 (DBP < 70 mmHg), normotensive group (DBP; 71-90 mmHg) and hypertensive group (DBP > 90 mmHg). RESULTS: 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). CONCLUSION: 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.

Diagnostic yield of transthoracic echocardiography for stroke patients in a developing country.

OBJECTIVE: To assess the utility and cost effectiveness of transthoracic echocardiography (TTE) as part of stroke workup in a developing country. METHODS: All patients over the age of 14 years with acute stroke were prospectively enrolled in Aga Khan University stroke databank from August 1999 to May 2001 (22 month period). All patients were evaluated by a consultant neurologist and underwent standard stroke work up such as neuroimaging, TTE, carotid doppler sonography and blood tests e.g. serum glucose, lipid profile, blood urea nitrogen and serum creatinine. For the purpose of this study, patients with ischemic stroke were identified and their echocardiographic data was retrieved and analyzed. TOAST criteria was used to identify findings indicating cardioembolic stroke. RESULTS: Ischaemic stroke was identified in 393 (76%) patients. Of these 278 (71%) patients underwent transthoracic echocardiography as part of stroke workup. Although 199 (73%) patients had abnormal echocardiogram; only 43 (15.5%) had findings suggestive of possible cardioembolism as defined by the TOAST criteria. CONCLUSION: Tranthoracic echocardiography is a valid diagnostic modality for stroke workup. However there is a need to further investigate the specific indications which can be used to prioritize patients for undergoing echocardiography to minimize cost.

Surgical training programs in Pakistan.

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 ways of tackling rural urban disparities in health delivery.

Reasons for migration among medical students from Karachi.

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.

Prognostic indicators in cerebral venous sinus thrombosis.

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.

Characteristics of patients with Guillain Barre Syndrome at a tertiary care centre in Pakistan, 1995-2003.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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%). CONCLUSION: 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.

Familial homocystinuria.

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.