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Pediatrics

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A 6-year-old fully immunized girl developed a fever of 39°C followed by weakness in her lower extremities and right upper extremity with numbness up to the midthoracic level. Cerebrospinal fluid (CSF) examination showed mild protein and cell elevation with no bacteria on Gram's stain. The most likely diagnosis

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(A) transverse myelitis

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(B) tick-borne paralysis

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(C) poliomyelitis

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(D) botulism

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(E) vascular malformation

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(A) The acute onset of symptoms in this child is most likely related to acute transverse myelitis. This diagnosis is supported by the occurrence of a rapid asymmetric neurological deficit and a sensory level suggesting cross-sectional involvement of the spinal cord. Magnetic resonance imaging (MRI) of the spinal cord would be the procedure of choice to confirm the diagnosis. It may show evidence of cord swelling at the level of demyelination and rule out any acute cord compression in the epidural space. Transverse myelitis is an acute demyelination of the spinal cord that may progress over hours or days. It may be associated with optic neuritis in Devic disease and uncommonly with multiple sclerosis in childhood. The mean age of onset is 9 years. The level of demyelination is usually thoracic. The motor deficit is commonly asymmetric, and maximum weakness is reached within 48 hours. Recovery begins after 6 days. Fifty percent of patients make a full recovery, 10% have no recovery, and 40% recover incompletely. Relapsing myelitis may occur. Corticosteroids remain the most common treatment despite the absence of controlled studies. Poliomyelitis may cause asymmetric weakness, but the presence of a sensory level excludes this diagnosis. Tick-bite paralysis is unlikely to be the diagnosis because of the presence of fever and the abnormal CSF. The absence of ophthalmoplegia, the presence of fever, and abnormal CSF results are against the diagnosis of botulism. Finally, a spinal cord vascular malformation is unlikely to be the diagnosis because of the presence of fever and the absence of red blood cells in the CSF. In this case, respiratory failure is the major concern because of the involvement of the thoracic spinal cord. Vital capacity should be measured multiple times per day. Pulmonary emboli are a rare complication during acute paralysis. Hypotension and sphincter incontinence, like other autonomic instabilities, are usually not life-threatening. However, cardiac rhythm and blood pressure should be monitored closely. (Fenichel, 264–265)

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Which of the following distinguish newborn benign nocturnal myoclonus from seizure?

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(A) It occurs solely during sleep.

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(B) It is activated by a stimulus.

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(C) It may be accompanied by an abnormal electroencephalogram (EEG).

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