Referral of epileptic patients in North East Coast of West Malaysia an area with poor MRI coverage: an analysis.

Advances in neuroimaging techniques, particularly Magnetic Resonance Imaging (MRI), have proved invaluable in detecting structural brain lesions in patients with epilepsy in developed countries. In Malaysia, a few electroencephalography facilities available in rural district hospitals run by trained physician assistants have Internet connections to a government neurological center in Kuala Lumpur. These facilities are more commonly available than MRI machines, which require radiological expertise and helium replacement, which may problematic in Southeast Asian countries where radiologists are found in mainly big cities or towns. We conducted a cross-sectional study over a two year period begining January 2001 on rural patients, correlating EEG reports and MRI images with a clinical diagnosis of epilepsy to set guidelines for which rural patients need to be referred to a hospital with MRI facilities. The patients referred by different hospitals without neurological services were classified as having generalized, partial or unclassified seizures based on the International Classification of Epileptic Seizures proposed by the International League Against Epilepsy (ILAE). The clinical parameters studied were seizure type, seizure frequency, status epilepticus and duration of seizure. EEG reports were reviewed for localized and generalized abnormalities and epileptiform changes. Statistical analysis was performed using logistic regression and area under the curve. The association between clinical and radiological abnormalities was evaluated for sensitivity and specificity. Twenty-six males and 18 females were evaluated. The mean age was 20.7 +/- 13.3 years. Nineteen (43.2%) had generalized seizures, 22 (50.0%) had partial seizures and 3 (6.8%) presented with unclassified seizures. The EEG was abnormal in 30 patients (20 with generalized abnormalities and 10 localized abnormalities). The MRI was abnormal in 17 patients (38.6%); the abnormalities observed were cerebral atrophy (5), hippocampal sclerosis (4), infarct/gliosis (3), cortical dysgenesis (2) and tumors (2). One patient had an arachnoid cyst in the right occipital region. Of the 17 patients with an abnormal MRI, 14 had an abnormal EEG, this difference was not statistically significant. There was no significant associaton between epileptographic changes and MRI findings (p = 0.078). EEG findings were associated with MRI findings (p = 0.004). The association between an abnormal EEG and an abnormal MRI had a specificity of 82.4%, while epileptogenic changes had a specificity of 64.7% in relation to abnormal MRI findings. This meants that those patients in rural hospitals with abnormal EEGs should be referred to a neurology center for further workup and an MRI to detect causes with an epileptic focus.

Left ventricular thrombi in the presence of normal left ventricular function.

We report two cases of left ventricular thrombi identified by routine echocardiography in the presence of normal ventricular function to highlight the rarity and clinical significance of this condition. A 14-year-old boy, positive for anticardiolipin and antinuclear antibodies, was found to have a left ventricular thrombus. A 30-year-old male, who presented with a transient ischemic attack, was found to have hypereosinophilic syndrome and a mobile left ventricular thrombus. The thrombi disappeared in both patients after a few days of anticoagulant therapy without symptoms of embolization.

Coronary artery ectasia: angiographic, clinical profile and follow-up.

Out of 3200 coronary angiograms we reviewed, there were 144 cases of coronary ectasia--an incidence of 4.5 percent. Among these, 122 were associated with atherosclerotic coronary artery disease, i.e. coronary stenosis more than 50 percent (group A) and 22 not associated with coronary artery disease (group B). The patients in groups A and B were compared with age- and sex-matched patients (group C) (n=100) who had coronary artery disease alone without ectasia. The incidence of ectasia was not increased in patients with thoracoabdominal aortic aneurysm i.e. 2/154 (1.8%) or in patients with peripheral occlusive vascular disease i.e. 5/161 (3.1%). Ectasia was typed according to a modified version of the criteria proposed by Markis et al. Type II was the commonest, followed by type I, III and IV. Right coronary artery was the most commonly involved vessel by ectasia followed by left circumflex, left anterior descending artery and left main coronary artery. Diffuse ectasia was seen more frequently in right coronary artery and localised ectasia in left anterior descending artery. Patients in groups A and B had similar epidemiological characteristics, though more patients with ectasia alone (group B) had better left ventricular function and negative stress tests. The patients in group A had a similar incidence of previous myocardial infarction, coronary risk factor profile, treadmill exercise test status and severity of coronary artery disease when compared to group C. On a mean follow-up of 3+/-1.2 years, all the three groups had similar event rates.

Saccular coronary aneurysms: angiographic and clinical profile and follow-up of 22 cases.

Saccular coronary aneurysms are defined as aneurysms with the transverse dimension at least 1.5 times the longitudinal dimension. Out of 3,200 coronary angiograms reviewed, there were 22 patients (20 males) with saccular coronary aneurysms (totalling 25 aneurysms). The morphology of the aneurysms, the extent and severity of associated coronary lesions, the clinical profile and follow-up data of these patients were analysed. Aneurysms were located in left main coronary artery 3 (12%), left anterior descending coronary artery 13 (52%), right coronary artery 5 (20%) and left circumflex 4 (16%). There were 5 large aneurysms (> 15 mm in diameter) (1 in left main coronary artery, 2 each in right coronary artery and left anterior descending coronary artery) averaging 32 mm in size. Fifteen aneurysms had significant coronary artery stenosis located proximal to it, supporting the theory of post-stenotic dilatation as the aetiology of aneurysm formation. Two patients had associated muscle bridges distal to the aneurysm; 20 had atherosclerotic coronary artery disease and one had coronary artery ectasia. Five patients were lost to follow-up, which ranged from 1 year to 19 years (mean 5.3 +/- 4.1 years). No patient had history suggestive of rupture of the aneurysm on follow-up. Two patients had myocardial infarction in the territory of the vessel with the aneurysm. Rest of the patients were in NYHA class I/II. One large right coronary artery aneurysm was subjected to aneurysmectomy due to symptoms of tricuspid valve obstruction. One left main coronary artery aneurysm measuring 12 mm, on follow-up of 19 years increased in size to 45 mm, in addition the patient developed a right coronary artery aneurysm. Coronary risk factor profiles in the 20 patients with atherosclerotic coronary artery disease and aneurysms were similar to age- and sex-matched control population with atherosclerotic coronary artery disease without aneurysms.

Clinical and angiographic profile and follow-up of myocardial bridges: a study of 21 cases.

Myocardial bridging describes an angiographic entity, which is any degree of systolic narrowing of a coronary artery observed in at least one angiographic projection. Among the cineangiograms of 3200 patients reviewed, there were 21 cases (19 males) of myocardial bridges--incidence of 0.6 percent. Of these, seven had hypertrophic cardiomyopathy, six had atherosclerotic coronary artery disease and remaining eight had no evidence of either. All 21 patients had myocardial bridges in proximal or mid left anterior descending coronary artery. In addition, one case of hypertrophic cardiomyopathy had whole posterior descending coronary artery under a myocardial bridge. Another case of hypertrophic cardiomyopathy had a short normal segment of 5 mm inside a long myocardial bridge of 35 mm (tandem myocardial bridges). The length of the bridges varied from 10 to 35 mm (mean 24.5 +/- 4.5 mm) and diameter stenosis during systole varied from 40-90 percent (mean 70 +/- 8%). Two patients had large saccular coronary aneurysms proximal to the muscle bridge. Four of the eight patients who had neither hypertrophic cardiomyopathy nor coronary artery disease presented with acute anterior wall myocardial infarction and three of them had regional wall motion abnormality of left descending territory. Of the six patients who had coronary artery disease, one had 60 percent left descending artery lesion and two had recanalized segments proximal to the bridge. Five of the above six patients had significant stenosis of other coronary vessels. Four patients were lost to follow-up (mean period 3.4 +/- 2 years). In the coronary artery disease group, one patient underwent coronary artery bypass graft surgery for 3-vessel disease including graft to left descending artery and one developed inferior wall myocardial infarction. The patients in the hypertrophic cardiomyopathy group and "no hypertrophic cardiomyopathy-no coronary artery disease" group were free of events at last follow-up. Long-term prognosis of isolated myocardial bridges appears to be excellent. Degree of systolic narrowing or length of myocardial bridge does not correlate with event rates on follow-up.

Bidirectional Glenn shunt: a step in the right direction.

Bidirectional Glenn (BDG) shunt was carried out in 14 patients (age range 1.5-22 years; mean 9.3 years) for a variety of congenital cyanotic heart diseases with decreased pulmonary blood flow. Postoperative cardiac catheterisation was carried out in 10 patients including successful balloon angioplasty of the shunt and LPA in one patient. There was a significant improvement in oxygen saturation and a drop in haematocrit level at follow-up. Doppler echocardiography studies in 13 patients revealed functioning shunts in all cases with low velocity continuous flow. Thus BDG is a useful palliative procedure and could be undertaken as the first stage of total cavopulmonary connection in high-risk Fontan groups where biventricular repair is not feasible.

A ten-year post-surgical assessment of pulmonary hypertension in adults with patent ductus arteriosus.

The long-term follow-up was analysed for 25 patients above the age of 12 years who had patent ductus arteriosus with severe pulmonary hypertension and had undergone surgical closure. There were 11 males and 14 females (age range 12-27 years; mean 18.6 years). There were 2 early and 2 late deaths. All the 21 survivors were followed up at regular intervals for 8-10 years. They were assessed by haemodynamic study: 13 of the 21 survivors underwent a study 6 months to 1 year after surgery and another 4 patients, 8 years after surgery; 7 patients of the 13 had a second study 8-10 years after surgery. The pulmonary artery systolic and mean pressures regressed to 50% of the preoperative level in the first study for 15 patients, and in the second study 2 patients had further drop in pulmonary artery pressures; however, the pressure levels never reached normal values. The pulmonary vascular resistance did not show a correspondingly significant fall. In conclusion, the ten-year follow-up shows that the patients have benefited by the surgery though the pressures have not come down to normal levels, and that they have to be followed up closely to assess the natural history of residual pulmonary hypertension and pulmonary vascular disease and its effect on their quality of life.

Blade and balloon atrial septostomy for creation of an adequate interatrial communication.

During a 7 year period from 1984 to 1991, 100 infants underwent either balloon atrial septostomy (BAS) (n = 92) or blade septostomy (BLS) (n = 8). The indication was complete transposition of the great arteries in all the patients. The mean age in the BAS group was 1.8 +/- 1.5 months while that in the infants requiring BLS due to a thick septum was 3.03 +/- 2.29 months. The degree of improvement in arterial oxygen saturation in both groups was satisfactory -27.16 +/- 14.06% in the BAS group vs 23.5 +/- 12.18% in the BLS group. There were no procedure related deaths in the BLS group, through only monoplane fluoroscopy was used in 6/8 patients. Three patients however died following balloon septostomy. We conclude that BLS is a safe and effective alternative to surgical septostomy when performed with due care. Two dimensional echocardiography during BAS enhances the speed and safety of the procedure and helps to identify patients who may require BLS due to a thick interatrial septum.

Primary pulmonary hypertension: natural history and prognostic factors.

A retrospective cohort study was carried out in 61 patients (30 males, 31 females, age 24.6 +/- 11.8 years) with primary pulmonary hypertension diagnosed by strict clinical and hemodynamic criteria, to obtain an understanding of the natural history and prognostic markers. While 15 patients were alive, 46 patients (76%) had expired during the follow up period. Two, five and ten years survivals were 48%, 32% and 12% respectively. Median survival duration from time of diagnosis was 22 months. The survivors had significantly higher age of onset, cardiac index and significantly lower right atrial mean pressure, right ventricular end diastolic pressure, cardiothoracic ratio from chest rontgenogram and calculated pulmonary vascular resistance as compared to non survivors. While pulmonary artery systolic pressure was not significantly different, pulmonary artery diastolic and pulmonary artery mean pressures were significantly lower in survivors than in non-survivors. Lower New York Heart Association class, right atrial mean pressure < or = 7 mm Hg, right ventricular end diastolic pressure < or = 10 mmHg, cardiac index > 2.5 L/min/m2, pulmonary arterial oxygen saturation > 60%, were associated with significantly longer survival. The degree of pulmonary arterial hypertension had an indirect prognostic effect through the above parameters. Vasodilator therapy did not significantly alter the outcome of patients with primary pulmonary hypertension.

Thrombolytic therapy for prosthetic cardiac valve thrombosis.

During 1992-93 12 patients (5 males, 7 females) with thrombosed prosthetic cardiac valves were treated with streptokinase on 13 occasions (one patient with prosthetic tricuspid valve had two thrombotic episodes). Their age ranged from 14 to 52 years (median 39). Two valves were in aortic position, six in mitral and four in tricuspid position. Eight were Bjork-Shiley prosthesis, three were Medtronic Hall valves and one was a St. Jude Valve. Timing of prosthetic valve thrombosis ranged from 3 months to 12 years after valve replacement surgery. Duration of symptoms due to valve thrombosis ranged from 1-4 months with tricuspid valve thrombosis and 1-14 days with left sided valve thrombosis. Five were in functional class II and four each were in functional class III and class IV. All patients were evaluated by echo Doppler and cine fluoroscopy. Loading dose of streptokinase was 2.5 lakh units in 4 patients and 1 lakh units in 9 patients. Maintenance infusion was at 1000 units/Kg/hour in 11 patients and 1 lakh units/hour in 2 patients. Duration of streptokinase infusion ranged from 3 hr to 38 hr. Thrombolytic therapy was successful (clinical, echo Doppler and fluoroscopy) in 12 out of 13 cases (92%). It was unsuccessful in a patient with valve at tricuspid position in whom infusion had to be stopped after 24 hour due to bleeding gums. One patient developed intracerebral bleed and expired. In conclusion streptokinase therapy is useful for prosthetic cardiac valve thrombosis.

A correlative study of the functional class and hemodynamic status following intracardiac repair of tetralogy of Fallot.

An attempt was made to correlate the NYHA Functional class with the hemodynamic status of 196 patients at an average of 21.2 months following the intracardiac repair of tetralogy of Fallot. 171 patients were in Functional class I (87.2%), 9 in class III (4.5%), 5 in class II (2.5%) and 11 in class IV (5.6%). Right and left heart catheterisation and cardiac angiography revealed the hemodynamic status of these patients to be excellent in 92 (46.4%), good in 37 (18.8%), satisfactory in 6 (3.0%) and unsatisfactory in 61 (31.6%). Whereas all patients in Functional class II, III and IV had unsatisfactory hemodynamic findings, patients in Functional Class I were hemodynamically heterogeneous and included excellent (53.8%), good (21.6%), satisfactory (3.5%) and unsatisfactory (21%) groups. The surgical technique did not seem to determine the functional status. While early reoperation is advisable for patients with functional disability, caution is necessary in considering reoperation for patients in Functional class I who have hemodynamic findings which are classified as unsatisfactory.

Assessment of severity of aortic stenosis by continuous wave Doppler echocardiography.

Fifty consecutive patients with aortic stenosis were evaluated by continuous wave Doppler echocardiography for assessment of the transaortic gradient. The Doppler derived gradients were compared with the gradients measured at cardiac catheterisation. Excellent correlation was found between the Doppler and catheterisation findings for the maximum instantaneous gradient (r = 0.92) and the mean systolic gradient (r = 0.84). The maximum, midsystolic and late systolic Doppler gradients also showed a good correlation with the peak to peak catheter gradient. The maximum Doppler velocity however, showed overestimation of the peak to peak gradient in the presence of mild aortic stenosis (predictive accuracy 86%). The midsystolic Doppler velocity showed the highest predictive accuracy (94%) for the detection of severe aortic stenosis. No case of severe aortic stenosis was missed by Doppler using either the maximum or midsystolic Doppler velocity. These findings indicate that continuous wave Doppler ultrasound provides a reliable estimate of the gradient in patients with aortic stenosis.

Small volume plasma exchange in Guillain-Barre syndrome: experience in 25 patients.

The impact of small volume plasma exchange (PE) on the treatment of Guillain-Barre syndrome (GBS) was studied by comparing 25 patients treated with PE since 1982 with 25 historic controls treated without PE prior to 1982. Small volume PE was done by removing 10-15 ml plasma/kg body weight daily till the progression of the disease was arrested or recovery started. The PE group started recovering earlier (median 3 days, compared to 17.5 days in controls, 2P = 0.01), attained better clinical grades at the end of the 1st and 3rd months (2P = 0.001), and took much shorter time to recover by one clinical grade (median 15 days, compared to 53 days in controls, 2P = 0.01). The median duration of ventilation among the surviving patients was shorter in the PE group (8 days compared to 24.5 days, 2P = 0.10) and total number of complications was less in the PE group (15 events compared to 22 in the controls, 2P = 0.05). Three months after the onset of neuropathy, 13/25 controls were still bed bound, whereas only 4/25 in the PE group remained in that grade (2P = 0.02). There was no significant difference in the mortality rate in two groups (2P = 0.09), but the difference was significant in the subgroup of patients who were ventilated (2P = 0.02).(ABSTRACT TRUNCATED AT 250 WORDS)

Fatal outcome of group-G streptococcal meningitis (a case report).

An adult woman developed meningitis caused by Group-G streptococci. The organism was successfully isolated both from blood and cerebrospinal fluid of the patient. The woman succumbed to infection despite an appropriate antibiotic therapy.