Alexander disease: management

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Alexander disease: management. Contrast enhancing lesions, Ventricular garlands. Fully referenced neurology checklist.

Magnetic resonance imaging (MRI): features

  • Contrast enhancing lesions
  • Ventricular garlands
  • Pial signal changes: along the brainstem
  • Eye-spot signs: in the anterior medulla
  • Angel wings appearance: in the middle medulla
  • Atrophy of medulla and cervical cord
  • Ventricular garlands

Magnetic resonance imaging (MRI): sites of enhancing lesions

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Cerebrospinal fluid (CSF) analysis

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Other investigations

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Treatment

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References

  1. Pareyson D, Fancellu R, Mariotti C, et al. Adult-onset Alexander disease: a series of eleven unrelated cases with review of the literature. Brain 2008; 131:2321-2331.
  2. Namekawa M, Takiyama Y, Honda J, Shimakazi H, Sakoe K, Nakano I. Adult-onset Alexander disease with typical "tadpole" brainstorm atrophy and unusual bilateral basal ganglia involvement: a case report and review of the literature. BMC Neurology 2010; 10:21.
  3. Kang YR, Nam TS, Kim JM, et al. Older adult-onset Alexander disease with atypical clinicoradiological features: a case report. Front Neurol 2023; 14:1139047.
  4. Oliveira DA, Araújo LC, Paiva ARB, Melo ES. Adult-onset Alexander disease with brainstem and cervical cord enhancing lesions. Pract Neurol 2023 (Online ahead of print).
  5. Gopal N, Gupta V, Williams LN, Sandhu SJS. Teaching NeuroImages: neuroimaging in adult-onset Alexander disease. Neurology 2021; 96:e814-e815.
  6. And 2 more. Subscribe to see the full list

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