Paroxysmal autonomic instability with dystonia after pneumococcal meningoencephalitis

Abstract

Streptococcus pneumoniae is a common cause of bacterial meningitis, frequently resulting in severe neurological impairment. A seven-month-old child presenting with Streptococcus pneumoniae meningoencephalitis developed right basal ganglia and hypothalamic infarctions. Daily episodes of agitation, hypertension, tachycardia, diaphoresis, hyperthermia, and decerebrate posturing were observed. The diagnosis of paroxysmal autonomic instability with dystonia was established. The patient responded to clonidine, baclofen, and benzodiazepines. Although this entity has been reported in association with traumatic brain injury, and as a sequel to some nervous system infections, this is the first case, to our knowledge, associated with pneumococcal meningoencephalitis.

Figure 1

(a), (b) Brain magnetic resonance imaging one day after admission. (a) Axial apparent diffusion coefficient (ADC) map shows large areas of cortical and subcortical restricted diffusion in the frontal areas bilaterally, left parietal and left temporal regions, suggestive of encephalitis. (b) Fluid attenuated inversion recovery (FLAIR) sequence showing normal brain tissue.

Figure 2

Enhanced brain computed tomography scan nine days after admission. Axial image through the basal ganglia shows hypodensities involving the right head of the caudate and anterior aspect of the putamen (thick white arrow) as well as the right hypothalamus (thin white arrow) representing subacute infarcts. In addition, there is development of bilateral hemispheric hygromas.

Figure 3

Nonenhanced brain computed tomography scan eight weeks after admission. Axial image shows significant nonobstructive hydrocephalus resulting in compression of cerebral parenchyma. There are hypodensities involving the periventricular regions and the frontal lobes bilaterally with associated cystic encephalomalacia, more on the right.