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Bickerstaff's brainstem encephalitis (BBE) is a rare disease diagnosed by specific clinical features such as 'progressive, relatively symmetric external ophthalmoplegia and ataxia by 4 weeks' and 'disturbance of consciousness or hyperreflexia' after the exclusion of other diseases involving the brain stem. Anti-ganglioside antibodies (GM, GD and GQ) in the serum or cerebrospinal fluid (CSF) are sometimes informative for the diagnosis of BBE because of the rarity of positive findings in other diagnositic methods: brain magnetic resonance imaging (MRI), routine CSF examination, motor nerve conduction study, and needle electromyography. We report a rare case of childhood BBE with elevated anti-GM1 antibodies in the serum, who had specific clinical symptoms such as a cranial polyneuropathy presenting as ophthalmoplegia, dysarthria, dysphagia, and facial weakness; progressive motor weakness; altered mental status; and ataxia. However, the brain MRI, routine CSF examination, nerve conduction studies, electromyography, somatosensory evoked potentials, and brainstem auditory evoked potentials were normal. BBE was suspected and the patient was successfully treated with intravenous immunoglobulins.

= Abstract = A 13-year-old girl with psoriasis of the elbow, trunk, and face suddenly developed a severe headache followed by left hemiparesis and facial palsy. Brain magnetic resonance imaging showed an acute infarction of the right temporofrontal lobe and basal ganglia on the T2- and diffusion-weighted images. Cerebral angiography showed pre-occlusive irregular scalloped stenosis (99%) in the proximal M1 segment of the right middle cerebral artery and a web-like stenosis at the supraclinoid portion of the right internal carotid artery (ICA) suggestive of a spontaneous intracranial ICA dissection. The patient was administered a low dose of dipyridamole, and a rehabilitation program was initiated. Headache, left motor weakness, and facial droop improved within a week. However, mild left facial palsy and reduced fine motor function of the left hand were still present after 3 weeks. We report a rare case of spontaneous intracranial ICA dissection in a child with psoriasis. (Korean J Pediatr 2009;52:1044-1047)

Purpose : Diamond-Blackfan anemia (DBA) is a rare heterogeneous genetic disorder of infancy and early childhood. It is characterized by red cell aplasia, congenital anomalies, and a predisposition to cancer. Corticosteroids and red cell transfusions are the mainstays of therapy. We describe our experience of 6 cases of DBA that were encountered over a period of 16 years. Methods : Medical records of 6 patients diagnosed to have DBA and admitted to the Chonnam National University Hospital between 1992 and 2008 were retrospectively reviewed. Results : Three patients were males. The age at diagnosis ranged from 3 to 18 months (median, 5.5 months). Heart defects were observed in 4, polydactyly in 2, and strabismus in 1 patient. The median number of transfusions was 3 (range, 2-8). All patients responded to initial treatment with steroids and had a hemoglobin level ≥9 g/dL with a median of 12.5 days (range, 7-22 days). Three patients are currently not receiving steroid therapy. A minimum dose of oral prednisolone (

A lung hernia, defined as the protrusion of pulmonary tissue and pleural membranes through a defect in the thoracic wall, is a rare event. It can be congenital or acquired, and cervical, thoracic, or diaphragmatic in location. We report the rare occurrence of a congenital atraumatic lung herniation through the azygoesophageal recess. An 8-month-old male infant, who was born at 35 weeks gestation, had a chronic cough. Chest radiography showed haziness at the right lower lobe of the lung (RLL). Chest computed tomography (CT) revealed herniation of the RLL through the azygoesophageal recess. If persistent unilateral haziness is observed on chest radiography, the possibility of lung herniation should be considered.