Objective: To identify common clinical features, sociodemographic characteristics and laboratory parameters of diagnosed cases of Subacute Sclerosing Panencephalitis.Design: Cross sectional descriptive type of study.Setting: Department of Paediatrics, Dhaka Medical College Hospital.Study period: January 2006 to December 2008.Subjects: Twenty clinically diagnosed patients of SSPE.Results: The mean age at presentation was 8 years. Male: Female ratio was 19:1. Most of the patients came from lower socio-economic group (70%). Forty five percent had history of primary measles infection and seventy percent were vaccinated against measles. Most common presenting features were fall to ground (95%), cognitive decline (85%), myoclonic seizures (80%), altered speech (70%), gait disturbance (60%), personality changes (55%), dysphagia (50%) and less commonly blindness (20%). EEG showed abnormal findings in 100% of patients who underwent this test. Measles specific IgG antibody in CSF was positive in 90% cases. Neuroimaging findings were abnormal in 43% cases.Conclusion: The diagnosis of SSPE should be considered in children presenting with deteriorating milestones of development especially cognition and behavior, fall to ground along with myoclonic jerks in an endemic country for measles infection. Investigations like CSF and serum antibody to measles virus and characteristic EEG changes may help further in the diagnosis. Key words: SSPE; Myoclonic jerks; Milestones regression; Measles antibody; EEG. DOI: 10.3329/jdmc.v17i2.6586J Dhaka Med Coll. 2008; 17(2) : 72-77

Ataxia Telangiectasia (AT) is a rare, neurodegenerative disease that affects many parts of the body and causes severe disability is characterized by progressive Cerebellar ataxia, oculocutaneous telangiectasia, and recurrent respiratory and sinus infections. AT is caused by a defect in the ATM gene, which is responsible for recognizing and correcting errors in duplicating DNA when cells divide, and in destroying the cells when the errors can not be corrected. A 10 years old girl who is the first issue of non consanguineous parents completely immunized according to EPI schedule was admitted to Dhaka Medical College Hospital, Dhaka, on 26.01.2010 with the complaints of generalized weakness and difficulty to walk from the 6th year. The patient has one younger sister of 7 years old who has such complaints. On examination the patient was found mildly anemic with congestions in both eyeballs that is radiating from the both corners to limbus. The patient was conscious and cooperative. Cranial nerves were intact. There were hypotonia which was more marked in lower limbs with diminished jerks and flexor plantar reflexes. Gait was wide based and ataxic. She was diagnosed as a case of AT. This case is presented as academic interest. Key words: Ataxia Telangiectasia (AT); neurodegenerative disease. DOI: 10.3329/jdmc.v19i1.6258 J Dhaka Med Coll. 2010; 19(1) : 69-71.

Objectives: The objective of this study was to observe the outcome of patients treated with penicillamine. Design: Intervention type of study Setting: Department of Paediatrics, Dhaka Medical College Hospital Study period: January 2007 to December 2008. Study subjects: Sixteen diagnosed cases of Wilson's disease as per inclusion criteria. Intervention: D-penicillamine was started in a low dose, which was titrated gradually. The clinical and biochemical parameters were evaluated to look for the response to treatment. Results: A total of 16 cases were included. Among them 12 were male and 4 were female. The mean (± SD) of age of the patients was 10 (± 2.34) years. Consanguinity between parents was present in 44% (n=7). The hepatic and neurological variety of WD were 56 % (n=9) and 44% (n=7) respectively. The K-F ring was present in 75% (n=12/16) of WD cases. The excretion of 24 hrs urinary copper was steadily increased from discharge till second follow-up in response with increasing dose of penicillamine, thereafter the value was declining gradually till final follow-up at 1 year. Regarding outcome, 7 patients improved of which 4 were in hepatic and 3 in neurological group, 3 of hepatic WD expired and 2 developed neurological manifestations. One patients developed proteinuria while penicillamine treatment .About half of patients with WD were improved. Adequate cupriuresis occurred at three months. All the symptoms and biochemical markers WD improved gradually. No significant side effect was seen. Key words: Wilson's disease; penicillamine; urinary copper. DOI: 10.3329/jdmc.v18i1.6304 J Dhaka Med Coll. 2009; 18(1) : 37-43

Two boys, one was 7 years and the other was 3 years 7 months old, were admitted into Dhaka Medical College Hospital one year apart with almost similar complaints of intractable partial seizure, progressive hemiparesis and cognitive deterioration following an episode of encephalitis. Developmental milestones was age appropriate till occurrence of encephalitis, thereafter started deteriorating with progressive deterioration of cognition, behavior, learning, memory and speech. Different anticonvulsants were tried in optimal doses, yet seizure remained uncontrolled. After admission CSF study, EEG and brain imaging was done. EEG showed unilateral slow waves and MRI revealed unilateral cortical atrophy with hyper intense signals in T2 and FLAIR. According to European consensus statement both cases were diagnosed as Rasmussen's Encephalitis (RE). Key words: Rasmussen's Encephalitis; hemispheric atrophy; seizure. DOI: 10.3329/jdmc.v18i2.6283 J Dhaka Med Coll. 2009; 18(2) :178- 182