Moyamoya disease: A clinical spectrum, literature review and case series from a tertiary care hospital in Pakistan

<p>Abstract</p> <p>Background</p> <p>Moyamoya is a rare cerebrovascular disease of unknown etiology. The data on moyamoya disease from Pakistan is sparse. We report a case series of 13 patients who presented with moyamoya disease to a tertiary care hospital in Pakistan with a national referral base.</p> <p>Methods</p> <p>We conducted a retrospective review of thirteen patients who presented to The Aga Khan University and diagnosed with "Moyamoya Disease" during the period 1988 – 2006. These patients were identified from existing hospital database via ICD-9 codes. A predesigned questionnaire containing information about clinical presentation, management and neuroimaging was administered to all identified patients.</p> <p>Results</p> <p>There were seven males and six females. Mean age at presentation was 16.5 years and a female predominance was found in the pediatric age group (n = 10, 71.4%). Stroke (n = 11, 84.2%) was the most common presentation with motor deficit being the universal cortical symptom. Fever was a common symptom in the lower age groups (n = 4, 51.7%). Cerebral Angiography and Magnetic Resonance Angiography showed bilateral involvement of the vessels in eleven patients while unilateral in two. Subarachnoid and interventricular haemorrhage appeared in 2(15.4%) adults. Twelve (92.3%) patients were discharged as independent with minor deficits regardless of therapeutic modality. Only three (23.0%) patients underwent surgery whereas the remaining were managed conservatively.</p> <p>Conclusion</p> <p>Physicians when dealing with childhood strokes and characteristic deficits in adult population should consider Moyamoya disease.</p

Cerebral venous thrombosis: a descriptive multicenter study of patients in Pakistan and Middle East

Background and Purpose: The natural history, causative factors, and outcomes of patients with cerebral venous thrombosis from Asia and Middle East have not been well described. This descriptive multicenter study describes the results for cerebral venous thrombosis patients in South Asia and the Middle East. Methods: The retrospective and prospective data of patients with radiologically confirmed cerebral venous thrombosis were collected from 4 centers located in Pakistan and United Arab Emirates. The demographic, clinical, radiological, and outcome data were recorded and analyzed. Primary outcome was death or dependency (modified Rankin score \u3e2) at the time of hospital discharge. Results: This study included 109 patients with cerebral venous thrombosis; the presenting features most commonly being observed were headache (81%), focal motor deficits (45%), seizures (39%), and mental status changes (37%). Important predisposing factors included systemic and central nervous system infection (18%), postpartum state (17%), hyperhomocystinemia (9%), genetic thrombophilia (5%), and oral contraceptive pill use (3%). Ninety-six (67%) patients received therapeutic anticoagulation. Seven patients died and 43 had poor outcome at discharge. Focal motor deficits (OR, 2.93; 95% CI, 1.2–7.5; P=0.018) and hemorrhagic infarctions (OR, 2.81; 95% CI, 1.04–7.85; P=0.041) were independent predictors of unfavorable outcome at discharge. Hemorrhagic infarction was the most significant factor of long-term unfavorable outcome (OR, 5.87; 95% CI, 1.49–23.02; P=0.011). Conclusions: Infections and postpartum state were the most common predisposing factors for cerebral venous thrombosis in this cohort. Most patients (67%) were treated with anticoagulation therapy. Almost 50% of patients were dead or disabled at discharge

Utilization and outcome of thrombolytic therapy for acute stroke in Pakistan

Our objective was to identify the outcome of Patients treated with tissue plasminogen activator (r-tPa) in Pakistan and compare these data to available regional and international data. The charts of all Patients treated with r-tPa for acute stroke at two centers in Pakistan (Aga Khan University, Karachi and Liaquat National hospital, Karachi) were retrospectively reviewed. We identified 21 Patients. The utilization of tPa at Aga Khan Hospital was 18/1,185 Patients (1.5%) from 2005 to 2007 while at Liaquat National Hospital was 3/575 Patients (0.52%) over 1 year (2007). Mean time interval between stroke onset and start of r-tPa infusion was 169 min. Three Patients (14%) developed fatal hemorrhage and two (10%) developed non-fatal hemorrhage. Four Patients (19%) died. We identified protocol violations in seven Patients (33%). In conclusion, the utilization of thrombolytic therapy is low and complication rate is high. Interventions for training physicians are needed