Childhood primary angiitis of the central nervous system

To analyze the clinical course and magnetic resonance angiographic [MRA] abnormalities in children with primary angiitis of the central nervous system [cPACNS]. Cohort study. Neurosciences and Neuroradiology Department of the Children's Hospital, Lahore, from January 2009 to December 2010. The cohort comprised consecutive patients diagnosed as having cPACNS based on clinical findings and identification of arterial stenosis on magnetic resonance angiography [MRA] in the absence of an underlying condition that could cause these findings. The treatment protocol for ischaemic infarcts consisted of induction therapy with intravenous steroids pulses and intravenous immunoglobulin followed by maintenance therapy with azathioprine and low dose aspirin. When indicated, they were treated with anticoagulants at least for 4 weeks along with induction therapy. Patients were followed at a single centre and systemically assessed for clinical presentation, classification of disease as progressive or non-progressive, adverse effects of anticoagulants, aspirin, azathioprine and their hospital course. Sixty-eight children with medium-large vessel cPACNS [62% boys, 38% girls] with mean age of 8.5 +/- 3.5 years were enrolled in this study. Motor deficit [70%]; headache [64%] and fever [20%] were the commonest symptoms; whereas hemiparesis [60%]; seizures 55% [focal 35%, generalized 20%] and decreased conscious level [30%], were the commonest neurological findings. Neuroradiological findings were ischaemic strokes in 50 [73.5%], haemorrhagic strokes in 10 [14.7%] and ischaemic haemorrhagic lesions in 8 cases [11.8%]. Angiographically 51 [51/68, 75%] of the cohort had non-progressive [obliterative] and 17 [17/68, 25%] had evidence of progressive arteriopathy at the time of admission. No secondary haemorrhagic lesions were documented among infarcts strokes, which were treated with heparin and oral anticoagulants. Outcome was survival in 56 cases [81.5%] and death in 12 cases [18.5%]. All survivors were discharged on long-term oral aspirin; 15 of them were also commenced on azathioprine. Neurological findings among the 56 survivors were; normal 20%, minor disabilities in 25%, moderate disabilities in 20% and severe disabilities in 35%. The spectrum of cPACNS includes both progressive and non-progressive forms with significant morbidity and mortality. This treatment protocol of immunosuppressive therapy may improve long-term neurological outcome in children with medium-large vessel childhood primary angiitis of the CNS

Ventilator-associated pneumonia in children

To determine the frequency of Ventilator-Associated Pneumonia [VAP] and to identify the associated factors, causative organisms and outcome of VAP in children admitted to ICU. Cross-sectional, observational study. Medical ICU [MICU] of the Children/'s Hospital and Institute of Child Health, Lahore, from August 2008 to March 2009. All children admitted to MICU and requiring ventilation during the study period were included and monitored for any features suggestive of VAP. Partial septic screen was done in all suspected cases. VAP was labelled when any patient on the ventilator for more than 48 hours had at least 2 of the following features of nosocomial infection - fever > 101'F, TLC < 4000 or > 15000 per mm[3], neutrophils > 85%, CRP > 48 mg/L or new findings on chest examination suggestive of pneumonia' and radiological evidence of new or progressive and persistent infiltrates. Percentages were compared using chi-square test with the significance at p-value less than 0.05. Of the 93 children requiring mechanical ventilation during the study period, 16 developed VAP [17%]. Almost half [46%] were younger than 1 year with male to female ratio of 1.2:1. Children developing VAP required ventilation for 13.5 [+ 10.1] days compared to 7.7 [+ 5.5] days in those who did not develop VAP. The common organisms isolated were Pseudomonas, Klebsiella and E. coli. Factors associated with increased frequency of VAP included age less than 1 year, unplanned emergency intubation and use of continuous intravenous sedation. Features that strongly suggested underlying VAP included purulent tracheal secretions compared to increased secretions alone, CRP > 48 mg/L, positive radiological findings and positive tracheal aspirate culture. Overall mortality was 23% among the ventilated cohort. Thirty two percent of them had VAP compared to only 13% among those who survived to discharge [p = 0.03]. The frequency of VAP was 17% in this series. Factors significantly associated with VAP were age less than 1 year, unplanned intubation and continuous sedation. The important predictors of VAP included purulent tracheal secretions, high CRP and persistent new radiological findings

Clinical spectrum of infantile spasm at presentation

To determine the clinical and EEG findings in children with infantile spasms at their initial presentation to the Neurophysiology Department, Children's Hospital, Lahore, Pakistan. Observational study. The Neurophysiology Department, Children's Hospital, Lahore, Pakistan, from January 2008 to December 2010. Children aged

Childhood epilepsy

To determine the community-based prevalence of childhood epilepsy and its treatment gap in rural and urban population in Punjab, Pakistan. Cross-sectional study. Gujranwala District, from March to June 2007. Ten out of 52 Union Councils of District Gujranwala, Pakistan, were randomly selected. Field officers, specifically trained for screening children with active epilepsy, performed a door-to-door survey in the selected Union Councils using area vaccinators as key informants. Final confirmation of active epilepsy and treatment details were ascertained by a qualified paediatrician and a paediatric neurologist, where required. Treatment gap was defined as relative [when treated inappropriately] and complete [complete lack of treatment]. Among a total, under 16 years population of 92254, prevalence of childhood epilepsy was found to be 7.0/1000 [n=643] with similar distribution between urban and rural residents. Up to 66% [n=424] patients were being managed by an unqualified person including paramedics and faith healers. Treatment gap was found in 88% [n=566] patients. Childhood epilepsy is common in both urban and rural areas of District Gujranwala. Area vaccinators may be incorporated into screening and referral program to bridge the treatment gap utilizing minimum available resources

Predictors of intractable childhood epilepsy

To determine the prognosis of seizures in epileptic children and identify early predictors of intractable childhood epilepsy. Case-control study. The Epilepsy Centre of the Children's Hospital Lahore, from February 2005 to April 2007. All children [aged 1 month to 16 years] with idiopathic or cryptogenic epilepsy who were treated and followed at the centre during the study period were included. The patients who had marked seizures even after two years of adequate treatment were labeled as intractable epileptics [cases]. Children who had no seizure for more than one year at last follow-up visit were the controls. Adequate treatment was described as using at least three anti-epileptic agents either alone or in combination with proper compliance and dosage. Records of these patients were reviewed to identify the variables that may be associated with seizure intractability. Of 442 epileptic children, 325 [74%] intractable and 117 [26%] control epileptics were included in the study. Male gender [OR=3.92], seizures onset in infancy [OR=5.27], >/= 10 seizures before starting treatment [OR=3.76], myoclonic seizures [OR=1.37], neonatal seizures [OR=3.69], abnormal EEG [OR=7.28] and cryptogenic epilepsy [OR=9.69] and head trauma [OR=4.07] were the factors associated with intractable epilepsy. Seizure onset between 5-7 years of age, idiopathic epilepsy, and absence seizures were associated with favourable prognosis in childhood epilepsy. Intractable childhood epilepsy is expected if certain risk factors such as type, age of onset, gender and cause of epilepsy are found. Early referral of such patients to the specialized centres is recommended for prompt and optimal management

Neurofibromatosis type II: a rare neurocutaneous syndrome

Neurocutaneous syndromes are heterogeneous group of disorders with abnormalities of central as well as peripheral nervous system. Neurofibromatosis type II [NF-II] is an autosomal dominant neurocutaneous syndrome rarely diagnosed in pediatric population. Diagnosis is based on clinical history and radioimmaging. We present a 14 years old boy with headache and decreased hearing, who turned to be a case of neurofibromatosis type II

Diagnostic role of EEG in children having unprovoked seizures, experience at the neurophysiology department, The children's hospital, Lahore

Although advances in imaging technology offer ever-increasing diagnostic accuracy, the electroencephalogram [EEG] retains its importance and is the cornerstone for the diagnosis and treatment of epilepsy. A cross-sectional descriptive study was conducted at Neurophysiology Department of The Children's Hospital, Lahore. Our aims were to find out the different types of epilepsies diagnosed on EEG in children referred for interictal EEG with recent clinical diagnosis of epilepsy. Out of 645 children referred for EEG after seizures, 415 [64%] were males and 230 [36%] were females, 21% had their first seizure before one year of age, 54% of them were between the ages of 1-5 years and 46% of them were more than 5 years of age. We found normal interictal EEGs in 54% whereas 46% children had abnormal EEGs. So in addition to supporting the diagnosis of epilepsy, EEG has significant potential to classify epileptic seizures

Early predictors of intractable epilepsy in children: a case control study

Case control, Hospital based. Site: Epilepsy Centre, The Children's Hospital, Lahore-Pakistan. From 1st Sept 05 to 30th Nov 06 Knowing the prognosis of epilepsy in children would undoubtly influence the treatment strategy. All the children, age 1 year to 16 years receiving anti-epileptic drugs for their idiopathic or cryptogenic epilepsy were the study population. After six months of commencement of treatment various factors associated with well control the ["controls"] and intractability the ["cases"] were analyzed and a univariate comparison was done between various factors for these two groups. Over a period of 14 months, 520 children, 33% the "control" and 67% the "cases" among these patients were enrolled for this study. Statistically, male gender history of birth asphyxia, initial seizure type, past head trauma, previous CNS infection and family history of epilepsy are not the risk factors, whereas seizures starting in infancy, seizures episodes >10 before commencing treatment, neonatal seizures and neurological deficit present at presentation were the risk factors for intractable epilepsy. Myoclonic seizures, status epilepticus before starting treatment and infantile spasm were seen in the "case" group only. We conclude children having risk factors for intractable epilepsy have poor prognosis and recommend that such children should be referred to epilepsy centre as soon as possible