Ruptured Baker's Cyst in a 15-Year Boy.

Baker's cysts or popliteal cysts are common cystic lesions surrounding the knee joint. These are prevalent among the adult population but rare in children. These are asymptomatic in majority of cases, and are identified incidentally by the parent or physician. We report a case of 15-year boy who presented to the emergency room with symptoms of calf pain and swelling for two weeks. He was in good health previously, and had no underlying knee pathology. Prior to presenting in emergency department (ED), he was suspected as deep vein thrombosis (DVT), and referred for evaluation and management. During his evaluation for DVT, the diagnosis of ruptured Baker's cyst was made on ultrasound. This was further confirmed with magnetic resonance imaging (MRI) of knee joint. He responded to conservative management and was discharged home after reassurance.

The upsurge of SSPE--a reflection of national measles immunization status in Pakistan.

Subacute sclerosing panencephalitis (SSPE) is a rare disorder in the developed world. However, an upsurge has been seen lately in our part of the world owing to inadequate measles immunization coverage. At the midst of our struggle against polio, we are struggling with the war against other vaccine-preventable childhood illnesses like measles. The increasing numbers of SSPE that we reported over the past half decade suggest an underlying periodic measles epidemic in Pakistan. In addition, children are now presenting with SSPE in early childhood, warranting a relook, reinforcement and strengthening of primary immunization and mandatory two-dose measles vaccination for all children nationwide. Previously undertaken Measles Supplementary Immunization Activity were a failure in terms of providing the expected cover against measles in young children. Intensive surveillance and establishment of SSPE registers at the district level is essential for eradication of this easily preventable disorder. Unless timely efforts are made to achieve global immunization, SSPE is bound to add to the national disability burden.

Subacute sclerosing panencephalitis: clinical and demographic characteristics.

OBJECTIVE: To determine the clinical and demographic characteristics of children diagnosed with Subacute sclerosing panencephalitis (SSPE). STUDY DESIGN: Case series. PLACE AND DURATION OF STUDY: The Aga Khan University Hospital, Karachi, from January 2000 to June 2012. METHODOLOGY: A retrospective analysis was done, regarding medical charts of 43 children under the age of 16 years with a discharge diagnosis of SSPE. Demographic and clinical characteristics were recorded. RESULTS were expressed as percentages. RESULTS: Most of the 43 patients were male (72%). The average age at presentation was 8.7 years with average duration of symptoms being 100.6 days. History of measles was present in 17 patients (39.5%). All children had seizures at presentation and 65% had cognitive impairment. Most patients required poly therapy for control of seizures. Sodium valproate was the most commonly used anti-epileptic agent; Isoprinosine was tried in 22 (51%) patients. CSF for antimeasles antibodies was positive in approximately 86% of the 40 (93%) children. EEG showed burst suppression pattern in 36 (83.7%) cases. Forty-two patients (97.6%) were discharged home in a vegetative state. CONCLUSION: SSPE is progressive neurodegenerative disorder. It can be prevented by timely immunization against measles. Measles antibody in the CSF is diagnostic for SSPE and is helpful in early diagnosis. Most patients experience a gradual but progressive decline in motor and cognitive functions.

Early recovery in post Varicella transverse myelitis.

A 7 years old boy presenting with acute flaccid paralysis after Varicella zoster infection was diagnosed as having acute transverse myelitis on MRI. He recovered fully after treatment with intravenous corticosteroids and acyclovir. The occurrence of this condition during or following Varicella infection is uncommon. There are previously very few reported cases of post-Varicella acute transverse myelitis in which recovery started after 3 months of treatment. In this case complete recovery occurred in 2 weeks of treatment. This report emphasizes the need for Varicella zoster vaccine to prevent not only acute Varicella, but also its rare postinfectious neurologic sequelae.

Acute cerebellitis with hydrocephalus.

We report a case of an 8 years old child who presented with sudden onset of headache and vomiting. He had broad-based gait and intention tremors on admission. MRI brain revealed isointense signals on T1-weighted imaging and hyperintense signals on T2-weighted imaging. Cerebellar swelling was also identified with significant mass effect obliterating the fourth ventricle. CT head showed prominent third and lateral ventricles. He was treated with high dose corticosteroids and required an external ventricular drain (EVD) insertion. He made an uneventful recovery and suffered no neurologic deficit. The clinical and radiologic findings in this boy were consistent with cerebellitis complicated by hydrocephalus.

Adrenal insufficiency in a child with MELAS syndrome.

Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) are established subgroups of mitochondrial encephalomyopathy. m.3243A>G a common point mutation is detected in tRNA in majority of patients with MELAS phenotype whereas m.8344A>G point mutation in tRNA is observed, in MERRF phenotype. Adrenal insufficiency has not been reported in mitochondrial disease, except in Kearns-Sayre Syndrome (KSS), which is a mitochondrial deletion syndrome. We report an unusual presentation in a five year old boy who presented with clinical phenotype of MELAS and was found to have m.8344A>G mutation in tRNA. Addison disease was identified due to hyperpigmentation of lips and gums present from early childhood. This is the first report describing adrenal insufficiency in a child with MELAS phenotype.