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Skull defect - Frontotemporal dementia sagging brain syndrome.

Wouter I Schievink1, Marcel M Maya2, Robin Babadjouni1

  • 1Department of Neurosurgery, Cedars-Sinai Medical Center, Los Angeles, California, USA.

Annals of Clinical and Translational Neurology
|December 15, 2024
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Summary

Revision cranioplasty can significantly improve symptoms in patients with Frontotemporal dementia (FTD) sagging brain syndrome, particularly those with a prior craniectomy history. This surgical approach offers a promising treatment option for this disabling condition.

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Area of Science:

  • Neurosurgery
  • Neurology

Background:

  • Frontotemporal dementia (FTD) sagging brain syndrome is a debilitating neurological condition.
  • Identifying the cause, often a cerebrospinal fluid leak, and effective treatments remain challenging.
  • A subset of patients with FTD sagging brain syndrome have a history of craniectomy/cranioplasty.

Purpose of the Study:

  • To investigate the efficacy of custom implant cranioplasty revision in patients with FTD sagging brain syndrome and a history of prior craniectomy.
  • To assess the impact of revision surgery on symptom severity and brain sagging.

Main Methods:

  • Retrospective review of 61 patients with FTD sagging brain syndrome.
  • Assessment of symptom severity using the SIH Disability Assessment Score (SIHDAS) before and after surgery.
  • Evaluation of brain sagging using pre- and post-operative MRI scans.

Main Results:

  • Eight patients (13.1%) had a history of craniectomy/cranioplasty.
  • Four patients underwent custom cranial implant surgery, with three experiencing prompt and remarkable symptom improvement.
  • One patient showed mild improvement, and MRI confirmed reduced brain sagging post-surgery.

Conclusions:

  • A significant proportion of FTD sagging brain syndrome patients have a history of supratentorial craniectomy/cranioplasty.
  • Revision cranioplasty using custom implants is a viable and effective treatment option for selected patients.
  • Consideration of revision cranioplasty should be part of the management strategy for FTD sagging brain syndrome.