Short report 1UCL Institute of Cognitive Neuroscience, London, UK 2Imperial College London, London, UK 3UCL Institute of Neurology, London, UK Correspondence to Professor Masud Husain, Institute of Cognitive Neuroscience, 17 Queen Square, London, WC1N 3AR, UK; m.husain@ion.ucl.ac.uk Received 22 September 2009 Revised 20 January 2010 Accepted 22 January 2010 Published Online First 20 June 2010 This paper is freely available online under the BMJ Journals unlocked scheme, see http:// jnnp.bmj.com/site/about/ unlocked.xhtml 688 Attention deﬁcits following ADEM ameliorated by guanfacine Victoria Singh-Curry,1 Paresh Malhotra,1,2 Simon F Farmer,2,3 Masud Husain1,2,3 ABSTRACT 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. Acute disseminated encephalomyelitis (ADEM) is a relatively rare neuroinﬂammatory disorder asso- ciated with multifocal lesions, frequently preceded by a viral prodrome. It is usually a monophasic illness, with many patients recovering well, although up to half followed up long-term have been reported to have persistent neurological deﬁ- cits.1 MRI usually reveals asymmetrical subcortical white-matter involvement. However, the deep grey-matter nuclei, such as the thalamus and basal ganglia, may also be affected.2 There are few studies documenting the cognitive and neuropsychological sequelae of ADEM,3 4 with most focussing on motor disabilities. Here, we report a patient with thalamic lesions secondary to ADEM, causing persistent left-sided hemispatial neglect (failure to attend to stimuli in contralesional space) and difﬁculty sustaining attention. These deﬁcits were subsequently ameliorated by administration of the noradrenergic agonist guanfacine, a drug that has been shown to have positive effects in children with attention deﬁcit/hyperactivity disorder,5 as well as sustained and spatial attention deﬁcits in some patients with neglect secondary to stroke.6 CASE REPORT A 38-year-old male presented with a right-sided facial droop and hemiparesis following a 2-week prodrome of headache, fever, cough and right hemisensory symptoms. Soon after admission, he developed toniceclonic seizures, necessitating treatment with phenytoin, intubation and venti- lation. MRI revealed patchy signal changes in the thalamus, cerebellum, temporal and occipital lobes bilaterally, while MR angiogram revealed normal extra- and intracranial blood ﬂow. Cerebrospinal ﬂuid examination, vasculitic blood screen and transoesophageal echocardiogram were normal. Electroencephalography demonstrated features consistent with encephalopathy, and a diagnosis of ADEM was made. He subsequently received two courses of intravenous methylprednisolone, intra- venous immunoglobulin, plasma exchange and antibiotics. He was also anticoagulated for a deep venous thrombosis and required surgical treatment for an associated compartment syndrome. The patient remained in intensive care, due to persistent epileptiform activity, for 4 months, when seizures were stabilised on a regimen of levetiracetam 2 g, phenytoin 700 mg and prednis- olone 30 mg. He was then transferred to a rehabili- tation unit, at which time he had a tetraparesis, with predominant left-sided weakness. Neuropsychological testing also revealed signiﬁ- cant cognitive impairments, including left-sided neglect (with intact visual ﬁelds on confrontation), reduced arousal and difﬁculty sustaining attention. In addition, there were signiﬁcant impairments in verbal memory (chance performance on the short and easy Recognition Memory Test for verbal material and low average performance on the Wechsler Adult Intelligence ScaledRevised (WAIS- R) Digit Span subtest) and naming (5/15 correct on Graded Naming Test). There was also evidence of dysfunction on tests of executive function (concrete performance on Proverb Interpretation and perseveration on Single Letter Reading and the Similarities subtest of the WAIS-R). On the basis of educational and occupational background, he was estimated to have been functioning in at least the superior range premorbidly and had therefore suffered severe intellectual deterioration. Admission for reassessment occurred 2 years later, at which time anticonvulsant medication consisted of levetiracetam 750 mg and gabapentin 300 mg both twice daily (the total daily dose of levetiracetam was 1250 mg at 6 months’ follow-up, with a further reduction to 1000 mg 10 months later). Cranial nerve examination was normal, except for a mild left-sided facial weakness. Exam- ination of the limbs revealed a severe hemiparesis with increased tone on the left and a pyramidal distribution of weakness, worse distally. The limb reﬂexes were all brisk with bilaterally extensor plantar responses. There was severe left hemispatial neglect and impairments in sustained attention and arousal, with the patient spending 20 h a day in bed due to drowsiness. A decision was taken to trial the alpha-2-noradrenergic agonist guanfacine, with the hypothesis that this might improve these cognitive impairments. ASSESSMENT MEASURES MRI was repeated to determine the extent of lesions (ﬁgure 1A). Two standard bedside neuropsychological J Neurol Neurosurg Psychiatry 2011;82:688e690. doi:10.1136/jnnp.2009.195792