Neuropediatrics
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A 16- year-old boy with long-standing severe Tourette syndrome (TS) and mental retardation, non-responsive to complex pharmocological and behavioural treatment was selected for bilateral deep brain stimulation (DBS) of the globus pallidus internus (GPi). Pre-operative and post-operative Yale Tourette syndrome scale (YTSS) scores and several other scores were used to quantify the effect of DBS up to one year follow-up. ⋯ This finding is in contrast to markedly improved TS of the only two adolescent TS patients in whom DBS has been performed so far. In this article we discuss possible reasons for the poor therapeutic effect of GPi-DBS in our patient contributing to the on-going debate on DBS inclusion criteria for adolescent TS patients.
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Case Reports
Fulminant sepsis-associated encephalopathy in two children: serial neuroimaging findings and clinical course.
We report on two children with sepsis-associated encephalopathy. They presented with fulminant neurological damage on clinical, neuroimaging, and neurophysiological findings. At onset, both went into deep coma after status epilepticus, resulting in near brain death. ⋯ MR imaging in the chronic phase revealed cracking lesions extending to the cerebral white matter, the cerebellum, and the brainstem. MR angiography showed diminished intracranial major arteries. These serial neuroradiological findings suggested severe brain damage resulting from fulminant elevation of intracranial pressure, which mimicked "brain death" or "respirator brain".
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Congenital infiltrating lipomatosis of the face (CILF) is a rare disorder, causing unilateral facial asymmetry characterized by enlargement of the cheek or chin. Hemimegalencephaly is a unique malformation characterized by enlargement of a cerebral hemisphere. The association of CILF and hemimegalencephaly has rarely been reported. We present a case of unilateral facial swelling in a 1.5-year-old boy in whom magnetic resonance imaging and computed tomography revealed CILF associated with unilateral megalencephaly.
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Case Reports
Central pontine and extrapontine myelinolysis in a pediatric patient following rapid correction of hypernatremia.
Central pontine and extrapontine myelinolysis are uncommon disorders characterized by distinctive clinical features and typical findings on neuroimaging. Only a few cases are reported in the pediatric age group. We describe the case of a leukemic, malnourished 14-year-old boy with a high serum sodium concentration that gradually increased to 170 mmol/L. ⋯ The patient was treated with steroids and intravenous immunoglobulins. He achieved an almost full neurological recovery and radiological improvement. The reported case demonstrates that central pontine and extrapontine myelinolysis can occur after excessively rapid correction of hypernatremia.
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Review Case Reports
Spontaneous intrauterine "ping-pong" fracture: review and case illustration.
We report a case of a closed outer-table parietal "ping-pong" skull fracture occurring in a 4 190-gram female infant born at 39 weeks and 5 days gestation after an uneventful Cesarean section (Apgar scores of 9 and 9 at one and five minutes). There was no maternal history of abdominal trauma during pregnancy and there were no complications or difficulties with Cesarean section delivery. Neurological examination was normal. ⋯ Spontaneous resolution did not occur by one month of age and the skull fracture was repaired with excellent cosmetic results. Rarely has a case of spontaneous intrauterine skull fracture been reported in an atraumatic Cesarean delivery. We believe this fracture resulted from a chronic in utero process without associated trauma as evidenced by deviation of the sagittal suture and sinus.