Pediatric emergency medicine trisk 2649 2649
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Acute dystonia resulting from exposure to antidopaminergic drugs is treated
with diphenhydramine (0.5 to 1 mg/kg/dose IV, orally [PO], or IM) or
benztropine (Cogentin; 1 to 2 mg/dose IM). Because the half-life of many of the
precipitating agents is fairly long, treatment should be continued for 24 to 48
hours.
Sydenham Chorea
Sydenham chorea, the most common form of acquired chorea seen in children,
occurs primarily between the ages of 3 and 13 years. Marked by involuntary
movements, coordination difficulties, and emotional lability, its onset may be
abrupt or insidious. Sydenham chorea is believed to be a poststreptococcal
disease and may occur months after the primary bacterial infection. It is one of
the major diagnostic criteria for rheumatic fever (see Chapter 86 Cardiac
Emergencies ).
The involuntary movements may be subtle at first and may be exacerbated by
stress. Initially, the movements classically affect the face and distal portion of the
upper extremities and consist of rapid, involuntary random jerks. This results in
the “milkmaid hand,” in which the child’s hand cannot maintain a uniform
strength while grasping the examiner’s hand. The involuntary movements
disappear during sleep. In some cases patients have predominantly unilateral
movements, hemichorea. There is usually associated muscular hypotonia and
marked coordination difficulties. Speech is often jerky.
Serologic evidence for preceding streptococcal infection is absent in up to 25%
of cases, and only one-third of patients have associated manifestations of
rheumatic fever at the time of diagnosis. In the absence of such confirmatory
evidence of a poststreptococcal cause, other disorders that may present with
chorea must be considered in the differential diagnosis. These include atypical
seizures, drug intoxication, choreoathetoid cerebral palsy, familial choreas, chorea
gravidarum, collagen vascular disease, Wilson disease, and Lyme disease.
Evaluation should include a hematologic profile, sedimentation rate, and
serologic tests performed for streptococcal infection. An ECG should also be
performed to look for evidence of rheumatic carditis (e.g., prolonged P-R
interval). If there is a question concerning diagnosis, further tests, such as MRI,
LP, and serologic evaluation for collagen vascular disease, might be helpful, but
are not usually necessary on an urgent basis. Patients should be presumptively
treated with chronic prophylactic antibiotics to prevent further beta-hemolytic
streptococcal infections; there is a considerable risk for reoccurrence of rheumatic
fever.
