Attention deficits following ADEM ameliorated by guanfacine.

The authors report here the case of a patient with severe deficits in arousal and sustained attention, associated with hemispatial neglect. These impairments were secondary to acute disseminated encephalomyelitis, with bilateral involvement of the medial nuclei and pulvinar of the thalamus. Treatment with the noradrenergic agonist guanfacine, previously used for attention deficits in attention deficit/hyperactivity disorder and stroke, was associated with a significant amelioration of both the spatial and sustained attention impairments in neglect. Guanfacine may prove to be a useful tool in the treatment of disorders of attention associated with neurological conditions.

Central nervous system manifestations of Q fever responsive to steroids.

We report the clinical and radiological central nervous system manifestations of a 27-year-old man with Q fever who subsequently developed acute disseminated encephalomyelitis and showed a significant response to steroids. The patient presented with headache and fever and quickly progressed to develop acute respiratory failure and hepatitis. A prompt evaluation revealed positive serology for Q fever and doxycycline was initiated. Approximately 1 week into his illness he was noted to be profoundly weak. Neuroimaging with magnetic resonance imaging (MRI) revealed diffuse white matter T2/FLAIR hyperintensities, with evidence of restricted diffusion. He was given high-dose steroids for a presumed diagnosis of acute disseminated encephalomyelitis (ADEM) and within days he had both clinical and MRI improvement. In addition to well-described meningitis and encephalitis, Q fever may also be associated with diffuse CNS lesions that may be demyelinating inflammatory in pathophysiology, and therefore responsive to high-dose steroids.

Acute disseminated encephalomyelitis with bilateral thalamic necrosis.

Acute disseminated encephalomyelitis is a monophasic inflammatory demyelinating disease of the central nervous system involving the white matter, and to a lesser extent, the gray matter. Bilateral thalamic lesions have been reported in 12% of pediatric patients with acute disseminated encephalomyelitis. In most cases, there is a benign clinical course and complete resolution of the lesions. Here, we describe a case in which acute disseminated encephalomyelitis is associated with severe neurological deficits and bilateral thalamic necrosis. Necrosis should be considered in cases of acute disseminated encephalomyelitis with persistent severe neurological deficits. Its presence is a poor prognostic indicator.

Acute disseminated encephalomyelitis: an MRI/MRS longitudinal study.

A clinical and radiologic diagnosis of acute disseminated encephalomyelitis was made in two children: a 6-month-old female who presented with focal seizures and thalamic and cerebral white matter lesions, and a 4.5-year-old male who presented with tremor and dystonia and had bilateral basal ganglia lesions, without evidence of active brain infection. Serial clinical and laboratory evaluations were supplemented by neuroimaging including routine magnetic resonance imaging and (1)H magnetic resonance spectroscopy. They were treated symptomatically, without using steroids or intravenous immunoglobulin, and both children recovered. Single voxel (1)H magnetic resonance spectroscopy data were acquired from the involved areas and from normal-appearing white matter. Abnormalities in N-acetyl-aspartate, choline, and lactate peaks were evident during the symptomatic phase, and persistence of low N-acetyl-aspartate was observed during recovery. These spectroscopic findings are consistent with neuropathologic findings of neuronal dysfunction, cellular membrane turnover, cellular infiltration, and metabolic stress in the acute phase, and with neuronal loss in the chronic phase.