Journal of child neurology
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A 10-year-old girl presented with progressive proximal limb muscle weakness without facial, ocular, or bulbar muscle involvement. There was no fatigability or diurnal fluctuation in symptoms. Her weakness worsened with febrile illnesses and recovered with accruing disabilities over a few weeks. ⋯ A significant decrement on repetitive nerve stimulation test and positive response to therapeutic neostigmine challenge test confirmed the diagnosis of limb-girdle myasthenia. She responded well to corticosteroids and thymectomy, demonstrating a likely autoimmune etiology. This case highlights the long-term fluctuations in a case of myasthenia gravis and the need for a high index of suspicion for myasthenia in children presenting with unexplained muscle weakness, even in the absence of typical features such as fatigability, diurnal fluctuation, and oculobulbar weakness.
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The authors describe the clinical features and management of lateral sinus thrombosis associated with mastoiditis and otitis media in children. Of 475 patients with mastoiditis and otitis media, 13 (2.7%) had lateral sinus thrombosis identified by magnetic resonance imaging/magnetic resonance venography (n = 11) and angiography (n = 2). Clinical features included headache, vomiting, fever, diplopia, papilledema, sixth nerve palsy, seventh nerve palsy, and unilateral cerebellar ataxia. ⋯ Magnetic resonance imaging/magnetic resonance venography should be obtained in any child with otitis media having features of raised intracranial pressure and/or focal neurodeficits to rule out lateral sinus thrombosis. Antibiotics and mastoidectomy are essential in management. A hypercoagulable state may predispose to lateral sinus thrombosis.
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Review Case Reports
Spontaneous intracranial hypotension in childhood: a case report and review of the literature.
Spontaneous intracranial hypotension results from 1 or more spontaneous spinal cerebrospinal fluid leaks, and generally presents with severe and persisting orthostatic headache. Diagnosis can be difficult as spontaneous intracranial hypotension is very rare in childhood and has a wide spectrum of clinical features and neuroimaging findings. Lumbar autologous epidural blood patch can be helpful for confirmation of diagnosis and symptom relief. We report a 15-year-old female with spontaneous intracranial hypotension who experienced immediate resolution of her symptoms following lumbar autologous epidural blood patch on 2 occasions, and review the literature on this well-recognized but probably underdiagnosed headache syndrome in childhood.
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Clinical Trial
Electrographic seizures during therapeutic hypothermia for neonatal hypoxic-ischemic encephalopathy.
Electrographic seizures are common in neonates with hypoxic-ischemic encephalopathy, but detailed data are not available regarding seizure incidence during therapeutic hypothermia. The objective of this prospective study was to determine the incidence and timing of electrographic seizures in term neonates undergoing whole-body therapeutic hypothermia for hypoxic-ischemic encephalopathy as detected by conventional full-array electroencephalography for 72 hours of therapeutic hypothermia and 24 hours of normothermia. Clinical and electroencephalography data were collected from 26 consecutive neonates. ⋯ Electrographic seizures occurred in 17 of 26 (65%) patients. Seizures were entirely nonconvulsive in 8 of 17 (47%), status epilepticus occurred in 4 of 17 (23%), and seizure onset was in the first 48 hours in 13 of 17 (76%) patients. Electrographic seizures were common, were often nonconvulsive, and had onset over a broad range of times in the first days of life.