Clinical profile and response to oral prednisolone in infantile spasm

To evaluate the clinical profile and response to oral prednisolone in infantile spasms. Case series. Neurology Department, The Children Hospital and Institute of Child Health, Multan, from July 2005 to June 2007. Fifty patients of infantile spasms were studied. Age, gender, age at onset of seizures, type of spasms [flexor, extensor or mixed], history of intrapartum asphyxia, developmental history, dysmorphic facial features, any hypopigmented/ hyperpigmented skin lesions, computed tomogram and electroencephalogram findings and response to oral prednisolone was noted. Data was analyzed statistically by SPSS 10. Descriptive statistics was used to find out frequencies and percentages of all above mentioned variables. Chi-square test was applied to determine the association between these variables and response to treatment. P-value of less than 0.05 was taken significant. Male to female ratio was 2.1:1. Mean age of babies was 6.5 +/- 3.35 months. Mean age at onset of seizures was 5.35 +/- 3.52 months. Flexor spasms was seen in 32 [64%], extensor spasms in 8 [16%] and mixed spasms in 10 babies [20%]. Symptomatic infantile spasms were noted in 48 [96%] babies while two babies [4%] were having cryptogenic infantile spasm. History of intrapartum asphyxia was noted in 54% of symptomatic cases. Favourable response to oral prednisolone was seen in 27 babies [54%]. Except male gender, none of the other variable reached the statistical significance for favourable response to treatment. Infantile spasms were found more common in males, flexor spasms were the commonest type noted. Symptomatic spasm was noted in 96% of cases and intrapartum asphyxia was the commonest cause of symptomatic group. Response to oral prednisolone was noted in more than half of cases of infantile spasms

Risk factors, presentations and outcome of the haemorrhagic disease of newborn

To determine the presentations, associated factors and acute outcome in the haemorrhagic disease of newborn. Cross-sectional analytical study. Paediatric Medicine Unit II, Nishtar Hospital, Multan, from June 2004 to May 2006. Fifty patients with haemorrhagic disease of newborn were studied. Age at onset of symptoms, gender, feeding pattern, place of delivery, site of bleeding and acute outcome of patients were noted. Chi-square test was applied to determine the significance of differences and relationship between variables and outcome. P-value of less than 0.05 was considered significant. The mean age at onset of symptoms was 51.65 +/- 39.49 days. Male to female ratio was 2.1:1 [p=0.047]. Late onset disease [8 days to 6 months of life] was noted in 32 [72%] babies [p=0.094]. Exclusive breastfeeding was noted in 45 [90%] babies [p <0.001]. Thirty babies [60%] were delivered at homes [p=0.025], 13 [26%] at private clinics and 7 [14%] at government hospitals. Intracranial haemorrhage was noted in 26 [52%] babies, skin bleeding in 09 [18%] babies, gastrointestinal in 08 [16%], bleeding from injection site in 04 [8%], hematuria in 02 [4%] and bleeding from umbilicus in 01 [2%] baby. Forty babies recovered, whereas death occurred in 10 babies. The cause of death was intracranial haemorrhage in all babies [p=0.059] and all were of late onset disease [p=0.088]. Haemorrhagic disease of newborn was common in male gender, breast-fed infants and spontaneous vaginal deliveries. Intracranial haemorrhage and late onset disease were the causes of mortality in all cases

Hypereosinophilic syndrome causing acute hemiplegia in childhood

Hypereosinophilic syndrome [HES] is a rare heterogeneous group of disorders, characterized by marked peripheral blood and tissue eosinophilia resulting in end organ damage. This case describes a six-year-old girl child who presented with sudden weakness of right half of body and fever. Computed tomogram of brain showed infarction of left internal capsule and basal ganglia. She had peripheral and bone marrow eosinophilia. No other cause was found for infarction

Rasmussen encephalitis

Rasmussen syndrome or Rasmussen encephalitis is a rare, progressive gray matter disease of childhood. A case of 4-year-old boy is reported here, who presented with recurrent episodes of status epilepticus of simple partial fits along with progressive left sided hemiplegia. EEG showed focal discharges from right hemisphere. Serial MRI brain showed progressive atrophy of right cerebral hemisphere with dilatation of ipsilateral ventricle