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Mov Disord Clin Pract. 2016 Jan 18;3(4):382-388. doi: 10.1002/mdc3.12301. eCollection 2016.

Clinical Heterogeneity in Cerebral Hemiatrophy Syndromes.

Movement disorders clinical practice

Eva Reiter, Beatrice Heim, Christoph Scherfler, Christoph Mueller, Michael Nocker, Jean-Pierre Ndayisaba, Wolfgang Loescher, Klaus Seppi, Andrew J Lees, Thomas Warner, Werner Poewe, Gregor K Wenning, Atbin Djamshidian

Affiliations

  1. Department of Neurology Innsbruck Medical University Innsbruck Austria.
  2. Department of Molecular Neuroscience and Reta Lila Weston Institute for Neurological Studies University of London London United Kingdom.

PMID: 30713929 PMCID: PMC6353474 DOI: 10.1002/mdc3.12301

Abstract

BACKGROUND: Cerebral hemiatrophy syndromes can present with variable neurological symptoms. In childhood epilepsy, mental retardation and neuropsychiatric disorders are common while in adults movement disorders, such as highly asymmetric parkinsonism or hemidystonia as well as neuropsychiatric problems have been reported.

METHODS: Here, we present three adult patients with features that expand the clinical spectrum and give an overview of the most common clinical signs associated with this rare condition.

RESULTS: All three patients had prominent neuropsychiatric symptoms such as mood swings and increased irritability. Furthermore, one patient developed hemichorea which can be a rare presentation of cerebral hemiatrophy.

CONCLUSIONS: Cerebral hemiatrophy syndromes are a heterogeneous group of disorders that may also present with neuropsychiatric symptoms or hemichorea.

Keywords: apomorphine; cerebral hemiatrophy syndrome; hemichorea; imaging; neuropsychiatric symptoms

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