Pattern of congenital brain malformations at a referral hospital in Saudi Arabia: an MRI study

More than 2000 different congenital cerebral malformations have been described in the literature, for which several classification systems have been proposed. With the help of these classification systems, it is now possible, with neuroimaging, to time neuroembryologic events. Magnetic resonance imaging [MRI], in particular, is useful in studying these malformations. This study evaluated the pattern of congenital brain malformations in a university referral hospital setting. The records of all MRI brain examinations at our hospital over a period of 3 years for children younger than 15 years of age were reviewed. Cases of congenital cerebral malformations were analyzed by sex, age at presentation, type of congenital cerebral malformation, and other associated congenital cerebral malformations. Of the 808 MR examinations of different parts of the body for children in the study period, 719 [89%], on 581 patients, were of the brain. Eighty-six children [14.8%] were found to have single or multiple congenital brain malformations. In these children, 114 congenital brain malformations were identified, the commonest being cortical migration-al defects [25 patients, 22%], neural tube closure defects [22 patients, 19%], and corpus callosum dysgenesis [22 patients, 19%]. The least common was vascular malformation. Sixteen patients [18.6%] had more than one congenital brain malformation. Neural tube closer defects, cortical migrational abnormalities, and corpus callosum anomalies were the commonest congenital brain malformations, while vascular malformations were the least common. Most of the identified malformations demonstrated the usual pattern, but a few showed unusual patterns and associations

Stroke in Saudi children. Epidemiology, clinical features and risk factors

To describe the epidemiology and clinical features of stroke in a prospective and retrospective cohort of Saudi children and ascertain the causes, pathogenesis, and risk factors.The Retrospective Study Group [RSG] included children with stroke who were evaluated at the Division of Pediatric Neurology, or admitted to King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, Kingdom of Saudi Arabia during the period July 1992 to February 2001. The Prospective Study Group [PSG] included those seen between February 2001 and March 2003. During the combined study periods of 10 years and 7 months, 117 children [61 males and 56 females, aged one month-12 years] were evaluated; the majority [89%] of these were Saudis. The calculated annual hospital frequency rate of stroke was 27.1/100,000 of the pediatric [1 month - 12 years] population. The mean age at onset of the initial stroke in the 104 Saudi children was 27.1 months [SD = 39.3 months] and median was 6 months. Ischemic strokes accounted for the majority of cases [76%]. Large-vessel infarcts [LVI, 51.9%] were more common than small-vessel lacunar lesions [SVLL, 19.2%]. Five patients [4.8%] had combined LVI and SVLL. Intracranial hemorrhage was less common [18.2%], whereas sinovenous thrombosis was diagnosed in 6 [5.8%] patients. A major risk factor was identified in 94 of 104 [89.4%] Saudi children. Significantly more hematologic disorders and coagulopathies were identified in the PSG compared to the RSG [p=0.001], reflecting a better yield following introduction of more comprehensive hematologic and coagulation laboratory tests during the prospective study period. Hematologic disorders were the most common risk factor [46.2%], presumed perinatal ischemic cerebral injury was a risk factor in 23 children [22.1%] and infectious and inflammatory disorders of the circulatory system in 18 [17.3%]. Congenital and genetic cerebrovascular anomalies were the underlying cause in 7 patients [6.7%] and cardiac diseases in 6 [5.8%]. Six patients [5.8%] had moyamoya syndrome, which was associated with another disease in all of them. Inherited metabolic disorders [3.8%] included 3 children with Leigh syndrome and a 29-month-old girl with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes. Systemic vascular disease was a risk factor in 3 children [2.9%] including 2 who had hypernatremic dehydration; and post-traumatic arterial dissection was causative in 3 cases [2.9%]. Several patients had multiple risk factors, whereas no risk factor could be identified in 11 [10.6%]. Due to the high prevalence and importance of multiple risk factors, a comprehensive investigation, including hematologic, neuroimaging and metabolic studies should be considered in every child with stroke

Hematologic risk factors for stroke in Saudi children

To explore the hematologic risk factors for stroke in a cohort of Saudi children. We evaluated children at the Division of Pediatric Neurology at King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, during the periods July 1992 to February 2001 [retrospective study] and February 2001 to March 2003 [prospective study]. Investigations for suspected cases included neuroimaging, transcranial Doppler [TCD] for cases of sickle cell disease [SCO], and Duplex scan. Hemostatic assays included coagulation screening tests, tests of thrombin generation and fibrinolysis, coagulation inhibitors, and activated protein C resistance. During the study period, 104 Saudi children [aged one month to 12 years] with stroke were seen. The mean age of the cohort was 27.1 months [SD = 39.3 months] and median was 6 months. Ischemic strokes accounted for the majority of cases [76%]. A major risk factor was identified in 93 of 104 cases of stroke [89.4%]. Hematologic disorders were the most common [46.2%], followed by prothrombic disorders [31.7%]; microcytic hypochromic anemia [26%]; sickle cell disease [SCD], or SC beta-thalassemia, [11.5%], and factor IX deficiency [2.9%]. Raised anticardiolipin antibodies [13/49, 26.5%] was the most frequent abnormality. Deficiencies of the natural anticoagulants [protein S, protein C and antithrombin III] were as follows: protein S [15/70, 21.4%]; protein C [15/70, 21.4%] and combined deficiency of 2 or more inhibitors [9/70, 12.9%]. Activated protein C resistance has not been detected. Contrary to the findings of previous studies from Saudi Arabia, SCD is a common risk factor and is severe, as it resulted in multiple strokes. Moyamoya syndrome was diagnosed in 2 patients with SCD, one of whom had revascularization surgery [encephaloduroarteriosynangiosis]. Assessment of children with SCD at risk of stroke was helped by the introduction of TCD followed by neuroimaging, using MRI and magnetic resonance angiography. The study strongly highlights the importance of prothrombotic disorders and the severe phenotype of SCD as risk factors for stroke in Saudi children.

Perinatal stroke in Saudi children. Clinical features and risk factors

To describe the clinical features and presentations of perinatal stroke in a prospective and retrospective cohort of Saudi children and ascertain the risk factors. Patients with perinatal stroke were identified from within a cohort of 104 Saudi children who were evaluated at the Division of Pediatric Neurology at King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, Saudi Arabia from July 1992 to February 2001 [retrospective study] and February 2001 to March 2003 [prospective study]. Neuroimaging for suspected cases of stroke consisted of cranial CT, MRI, or both. During the study period, 23 [22%] of 104 children [aged one month to 12 years] were diagnosed to have had perinatal stroke. The male:female ratio was 1.6:1. Ten [67%] of the 15 children who had unilateral ischemic involvement had their lesion in the left hemisphere. The presentation of the ischemic result was within 24-72 hours of life in 13 [57%] patients, and in 6 children [26%], motor impairment was recognized at or after the age of 4 months. Nine children [39%] had seizures at presentation. Pregnancy, labour, and delivery risk factors were ascertained in 18 [78%] cases. The most common of these included emergency cesarean section in 5 cases, and instrumental delivery in another 5. Screening for prothrombotic risk factors detected abnormalities in 6 [26%] patients on at least one test carried out between 2 months and 9 years of age. Four children [17%] had low protein C, which was associated with low protein S and raised anticardiolipin antibodies [ACA] in one patient, and low antithrombin III in another. Low protein S was detected in a 42-month-old boy. The abnormality in the sixth child was confined to raised ACA. The present study highlights the non-specific features by which stroke presents during the neonatal period. The data are in keeping with the potential role for inherited and acquired thrombophilia as being the underlying cause. However, the high prevalence of additional acquired antenatal and perinatal risk factors support a multifactorial disorder

Infectious and inflammatory disorders of the circulatory system as risk factors for stroke in Saudi childern

To report on the role of infectious and inflammatory disorders as risk factors for stroke in a prospective and retrospective cohort of Saudi children. Children, who presented with stroke, were evaluated at the Division of Pediatric Neurology or admitted to King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, Kingdom of Saudi Arabia during the periods July 1992 to February 2001 [retrospective study] and February 2001 to March 2003 [prospective study]. Investigations for suspected cases included hemostatic assays, microbiological and serological tests. Neuroimaging included cranial CT, MRI, magnetic resonance angiography [MRA], magnetic resonance venography [MRV] and single photon emission computed tomography [SPECT] brain scan. Of the 104 Saudi children with stroke, seen during the combined study periods of 10 years and 7 months, infectious and inflammatory disorders of the circulatory system were the identified risk factor in 18 [17.3%]. Five children had stroke following acute bacterial meningitis at ages ranging between 5-21 months. The causative organism was identified in 3 of them and consisted of Haemophilus influenzae [in a 5-month-old girl], Streptococcus pneumoniae [in a 21-month-old girl complicated by subdural empyema and sinovenous thrombosis], and Staphylococcus aureus in a 6-month-old boy who had an underlying chronic granulomatous disease. Unspecified meningitis/meningoencephalitis affected 4 patients, whereas 3 children had an underlying congenital infection as a cause for their stroke. Two of the latter 3 children were diagnosed to have congenital toxoplasmosis, and the third had congenital rubella syndrome. Two girls had stroke following septicemia at ages of one and 2 months. Neurobrucellosis caused stroke in 2 boys at the ages of 4 1/2 and 4 years. In both patients, neuroimaging revealed lacunar and other infarcts involving mainly the deep cerebral nuclei, secondary to occlusion of small penetrating end arteries. Two patients presented with cerebrovascular disease following systemic lupus erythematosus. These were a 12-year-old girl and a 5-year-old boy. Several of the infectious diseases that caused stroke in this cohort of Saudi children are potentially preventable through childhood immunization programs or other maternity health programs. In particular, immunogenic conjugate vaccines against the 3 most common organisms causing acute bacterial meningitis [Haemophilus influenzae type b, Neisseria meningitidis and defined serotypes of Streptococcus pneumoniae] are needed to protect the young [<2 years] who are mostly affected.

Congenital and genetic cerebrovascular anomalies as risk factors for stroke in Saudi children

To explore the role of and report on congenital and genetic cerebrovascular anomalies as risk factors for stroke in a prospective and retrospective cohort of Saudi children. Children with stroke were evaluated at the Division of Pediatric Neurology [DPN], or were seen as inpatients in the Pediatric Wards at King Khalid University Hospital [KKUH], Riyadh, Kingdom of Saudi Arabia during the periods July 1992 to February 2001 [retrospective study] and February 2001 to March 2003 [prospective study]. Stroke work-up for each suspected case included hemostatic assays, serological, biochemical and neurophysiological tests. Neuroimaging modalities included routine skull X-rays, CT, MRI, magnetic resonance angiography [MRA] and conventional cerebral angiography. Of 104 children with stroke, congenital and genetic cerebrovascular anomalies were the underlying risk factor in 7 [6.7%]. The patients were evaluated at the DPN at a mean age of 66 months [range = 8 months to 11 years, median = 6 years]; and they had stroke at a mean age of 48 months [range = 2 months to 10 years, median = 8 months]. Four patients had stroke in association with neurocutaneous syndromes. Two had Sturge-Weber syndrome [SWS], one had Klippel-Trenaunay syndrome associated with SWS, and the fourth had neurofibromatosis type 1. Two patients had intracranial hemorrhage secondary to ruptured aneurysm. A girl [aged 9 years and 4 months] had left posterior cerebral artery aneurysm. She was diagnosed to have autosomal dominant polycystic kidney disease following renal ultrasonography. She died 5 months later despite surgical intervention [clipping of aneurysm]. The second child was an 8-month-old boy who presented with subarachnoid and intraventricular hemorrhage [IVH] following ruptured anterior communicating artery aneurysm. He recovered with no residual symptoms following successful clipping of the aneurysm. Arteriovenous malformation [AVM] caused IVH in a 7-year-old boy who reported to hospital 5 hours after onset of headache, vomiting, drowsiness, and dizziness. Following drainage of the IVH and stabilization of the patient, the AVM was successfully embolized 6 weeks later. As a group, congenital and genetic cerebrovascular anomalies constitute a significant risk factor for stroke in Saudi children. Recognition of these diseases is important since some are treatable and because other family members may be at risk.

Cardiac diseases as a risk factor for stroke in Saudi children

To ascertain the role of cardiac diseases as a risk factor for stroke in a cohort of Saudi children who were evaluated in a retrospective and prospective study. Children with cardiac diseases were identified from within a cohort of 104 Saudi children who presented with stroke. They were seen as inpatients in the Pediatric Wards or evaluated at the Outpatient Clinics of the Division of Pediatric Neurology [DPN], and the Division of Pediatric Cardiology at King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia during the periods July 1992 to February 2001 [retrospective study] and February 2001 to March 2003 [prospective study]. A comprehensive form for clinical, neuroimaging, neurophysiological and laboratory data retrieval was designed and completed for each patient. Cardiac evaluation included 12-lead ECG and serial echocardiograms. Cardiac catheterization and 24-hour ambulatory ECG [Holter] were conducted on clinical discretion. Cardiac diseases were the underlying risk factor for stroke in 6 [5.8%] of the 104 children [aged one month to 12 years]. The patients [4 males and 2 females] were evaluated at the DPN at a mean age of 5.3 years [range = 1 - 8 years; median 6.5 years]. Onset of stroke was at a mean age of 34 months [range = 4 months - 8 years; median = 30 months]. Five patients had stroke in association with congenital heart disease [CHD], whereas the sixth had restrictive cardiomyopathy. The identified CHD consisted of membranous ventricular septal defect in a 5-year-old boy who had moyamoya syndrome and sickle cell beta - thalassemia, asymptomatic patent ductus arteriosus [PDA] in a 17-month-old girl, atrioventricular canal defect and PDA in an 8-year-old boy who also had Down syndrome, partial anomalous pulmonary venous drainage in a one-year-old boy, and Tetralogy of Fallot in an 8-year-old boy. The latter patient developed hemiparesis secondary to a septic embolus, which evolved into brain abscess involving the right fronto-parietal region. This was successfully managed surgically. The sixth patient was an 8 1/2 -year-old girl who had hemiparesis and complex partial seizure in association with restrictive cardiomyopathy. Serial echocardiograms depicted resolution of the cardiac abnormalities within 5 years and subsequent normal findings. Cardiac diseases, as a group, constitute a significant risk factor for stroke in Saudi children. Early diagnosis of these diseases is important to prevent further recurrences of stroke, and because some of them are potentially curable.

Moyamoya syndrome as a risk factor for stroke in Saudi children. Novel and usual associations

To report on moyamoya syndrome [MMS] as a risk factor for stroke in a prospective and retrospective cohort of Saudi children. The usual and novel associations of MMS in this cohort will also be described. Children with stroke were evaluated at the Division of Pediatric Neurology at King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, Kingdom of Saudi Arabia during the periods July 1992 to February 2001 [retrospective study] and February 2001 to March 2003 [prospective study]. Investigations for suspected cases included hemostatic assays, biochemical, and serological tests. Neuroimaging included CT, MRI, magnetic resonance angiography [MRA], single photon computerized tomography [SPECT] brain scan and conventional cerebral angiography. Moyamoya syndrome was the underlying risk factor for stroke in 6 [5.8%] of the 104 children [aged one month to 12 years]. They were 4 females and 2 males. Their first cerebral ischemic event occurred at a mean age of 45 months [median = 44 months, range 17-66 months]. In all 6 cases, MMS was associated with an underlying hematologic abnormality or other diseases. Protein C deficiency was identified in one girl and protein S deficiency in another. Two patients had respectively, sickle cell disease [SCD] and sickle cell-B-thalassemia [S beta-thalassemia], which had been associated in the latter with membranous ventricular septal defect. Adams-Oliver syndrome [AOS, OMIM 100300] was associated with MMS in an 18-month-old girl. A 4-year-old boy had wrinkly skin syndrome [WSS, OMIM 278250] phenotype. The association of MMS and protein C deficiency was first reported in this cohort of patients, whereas the association of the syndrome with WWS and AOS has not, hitherto, been described. The 3 patients who had MMS associated with protein C deficiency, SCD, and AOS underwent successful revascularization surgery in the form of encephaloduroarteriosynangiosis. Moyamoya syndrome constitutes an important risk factor of stroke in Saudi children. Comprehensive clinical evaluation and investigations, including screening for thrombophilia and neuroimaging studies, are required for the primary diagnosis of the disease and for unraveling other diseases associated with MMS. This will help in managing these patients and in guiding genetic counseling for their families.

Stroke due to mitochondrial disorders in Saudi children

To report on the clinical and biochemical features of patients who presented with stroke due to mitochondrial disorders amongst a prospective and retrospective cohort of Saudi children. Children, who presented with stroke, were evaluated at the Division of Pediatric Neurology, or admitted to King Khalid University Hospital, College of Medicine, King Saud University, Riyadh, Kingdom of Saudi Arabia, during the periods July 1992 to February 2001 [retrospective study] and February 2001 to March 2003 [prospective study]. Open muscle biopsies were obtained from patients suspected to have mitochondrial disorders, and examined using conventional histological and histochemical techniques. Biochemical, molecular pathological investigations, or both, of muscle could be arranged for only some of the patients. Mitochondrial disorders were the underlying risk factor for stroke in 4 [3.8%] of 104 children [aged one month to 12 years]. Three patients [one male and 2 females] had Leigh syndrome [LS] and one had mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes [MELAS]. At the time of stroke, the 3 children with LS were 11 months, 15 months, and 7 years old. They presented with psychomotor regression and seizures. Muscle histology and histochemistry showed mild non-specific changes but no ragged red fibers. Biochemical analysis of muscle [in one patient] revealed deficiency of pyruvate dehydrogenase complex. Analysis of mitochondrial DNA [mtDNA], [the other 2 patients] was negative for the 2 point mutations [T-G and T-C] at nucleotide position 8993, and for two T-C point mutations [at positions 8851 and 9176 of the ATPase 6 gene] that have been described in patients with LS. The girl with MELAS syndrome presented with a stroke-like episode at the age of 29 months and had focal brain lesions in the medial aspect of the left occipital and temporal lobes, and in the posteromedial aspect of the left thalamus, which resolved within 7 weeks. She had raised cerebrospinal fluid lactate but no ragged red fibers on muscle histochemistry. Biochemical assay of muscle homogenate showed reduction in respiratory chain complexes I, III and IV. Mutation screening of mtDNA at nucleotides 3243 [tRNA Leu[UUR] and 8344 [tRNA Lys] was negative. Mitochondrial disorders constitute a risk factor for stroke in Saudi children. However, demanding and highly specialized investigations are needed to confirm the diagnosis. These are better performed at supraregional centers where facilities for clinical, biochemical and molecular work-up are available

Stroke from systemic vascular disorders in Saudi children. the devastating role of hypernatremic dehydration

Systemic vascular disorders, leading to childhood stroke, include volume depletion or systemic hypotension and hypernatremic dehydration. We describe 3 cases of stroke following systemic vascular disorders. These were diagnosed during a prospective and retrospective study on childhood stroke, which included 104 patients. Post-gastroenteritis hypernatremic dehydration is an important, potentially preventable, cause of stroke in Saudi children

Stroke from cervicocephalic arterial dissection in Saudi children

Cervicocephalic arterial dissection [CCAD] is an important, but rarely recognized, cause of stroke in children. We describe 3 cases of CCAD who were diagnosed during a study on childhood stroke which included 104 patients. A high index of suspicion and targeted investigations are needed for the diagnosis and management of CCAD in childhood.

Fluid-fluid level in Langerhans cell histiocytosis of the skull

We present a case of solitary eosinophilic granuloma in the skull of a 6-year-old Saudi boy. This osteolytic lesion has fluid-fluid level on CT and MRI. We are presenting a rare radiological finding of eosinophilic granuloma

Role of diffusion-weighted MRI in neuro-behcet disease

We report the findings on serial diffusion-weighted MRI in a 29-year-old male with neuro-Beh‡et's disease. Initial T2-weighted and fluid-attenuated inversion recovery images showed a hyperintense lesion in the brain stem. The lesion showed slight hyperintensity on diffusion-weighted images with no evidence of diffusion restriction on apparent diffusion coefficient maps. A follow up study after 7 months showed complete resolution of the brain stem lesion. Our findings indicate that diffusion-weighted imaging is a useful tool to differentiate acute exacerbation of neuro-Behcet's disease from acute infarction, and therefore it helps in selecting the appropriate therapy

criteria and analysis of good multiple choice questions in a health professional setting

Assessment of health workers as students and professionals has a profound impact on their learning and is an essential safety valve before certification. It is used for their training, their placement, their certification, and their promotion. The multiple choice question MCQ type of tests represents one of the most important examination tools that is commonly used in this assessment. The MCQs can be reliable, valid, and cost-effective in assessing medical knowledge. This paper portrays the different purposes of assessment in the medical field. The paper discusses in detail the criteria of a good assessment tool. Interpretation of MCQ test results is the final section of the paper

Imaging findings of neuro-Behcet disease

To study the neuroimaging findings of neuro-Behcet's disease [NBD] in Saudi patients and to discuss the radiological differential diagnosis. The clinical data and radiological findings on CT, MRI and cerebral angiography of 16 patients with NBD attending King Khalid University Hospital, Riyadh, Kingdom of Saudi Arabia, from January 1990 to February 2003 were reviewed. Out of 16 patients with NBD, 11 patients [68.75%] had cerebral venous thrombosis predominantly involving superior sagittal sinus causing intracranial hypertension, while 5 patients [31.25%] had symptoms and signs related to brain parenchymal involvement predominantly affecting brainstem. Pattern of distribution of brain parenchymal lesions in NBD might help to differentiate it from other vasculitides as well as from demyelinating disease such as multiple sclerosis. Cerebral venous thrombosis is a common manifestation of NBD

radiological manifestations of childhood lipoid pneumonia in Saudi Arabia: literature review and report of two new cases

Two cases of lipoid pneumonia with a positive history of fat aspiration were described. Plain films showed multilobar persistent consolidation and air bronchogram. One case showed mass like lesion in right upper lobe. The diagnosis has been made with the help of clinical history and radiological findings. This was confirmed with lung biopsy in one case. Review of literature published from Saudi Arabia has been discussed. Lipoid pneumonia can result from aspiration of variety of materials including milk, mineral or vegetable oil, animal fat, and oily nasal drops. There is low incidence of lipoid pneumonia in pediatric age group in developed countries. However, in Saudi Arabia the condition continues to be a problem because of traditional practices of administration of oily substances to children in some areas of the kingdom in the believe that oil is good for blocked nose, irregular bowel habits and general well being. Sometimes, in fact, infants and children are forcibly fed such substances. The diagnosis of lipoid pneumonia is usually delayed unless parents are directly questioned about oil feeding practice. The condition is difficult to differentiate from infectious pneumonia both clinically and radiologically. The aim of this paper is to review the radiological findings in the reported cases of lipoid pneumonia in Saudi Arabia and to discuss the possible explanations of these findings with report of 2 new cases