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Basal ganglia necrosis: a ‘best-fit’ approach
  1. Mihaela Boca1,
  2. Katie Lloyd2,
  3. Marcus Likeman3,
  4. Philip Jardine4,
  5. Alan Whone5
  1. 1Bristol Brain Centre, Southmead Hospital, Bristol, UK
  2. 2Faculty of Medicine and Dentistry, University of Bristol, Bristol, UK
  3. 3Department of Neuroradiology, Southmead Hospital, Bristol, UK
  4. 4Institute of Child Health, Bristol Royal Hospital for Children, Bristol, UK
  5. 5Department of Clinical Neurosciences, University of Bristol, Bristol, UK
  1. Correspondence to Dr Mihaela Boca, Bristol Brain Centre, Southmead Hospital, Bristol BS10 5NB, UK; mihaela.boca{at}gmail.com

Abstract

A previously well 16-year-old boy developed a rapid-onset hypokinetic syndrome, coupled with a radiological appearance of extensive and highly symmetrical basal ganglia and white matter change. The diagnostic process was challenging and we systematically considered potential causes. After excluding common causes of this clinico-radiological picture, we considered common disorders with this unusual radiological picture and vice versa, before finally concluding that this was a rare presentation of a rare disease. We considered the broad categories of: metabolic; toxic; infective; inflammatory, postinfective and immune-mediated; neoplastic; paraneoplastic and heredodegenerative. Long-term follow-up gave insight into the nature of the insult, confirming the monophasic course. During recovery, and following presumed secondary aberrant reinnervation, his disorder evolved from predominantly hypokinetic to hyperkinetic. Here, we explore the process of finding a ‘best-fit’ diagnosis: in this case, acute necrotising encephalopathy.

  • MRI
  • MOVEMENT DISORDERS
  • BASAL GANGLIA NECROSIS

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    Phil E M Smith Geraint N Fuller