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<p>A 63-year-old patient with relapsing-remitting multiple sclerosis (MS) and comorbid liver cirrhosis presented with progressive cognitive decline, gait disturbance and elevated serum neurofilament light chain (NfL), suggesting disease progression independent of relapse activity (PIRA). Development of acute hepatic encephalopathy led to reconsideration of the cause of previous clinical worsening and MRI revealed progressive basal ganglia manganese accumulation, establishing the diagnosis of acquired hepatocerebral degeneration, retrospectively challenging the PIRA diagnosis. Careful evaluation of comorbidities is warranted in older MS patients, as progressive worsening and high NfL may indicate alternative etiologies, particularly if GFAP (glial fibrillary acidic protein) stays within normal range.</p>

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Comorbidities in multiple sclerosis: acquired hepatocerebral degeneration mimicking disease progression

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年7月31日

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  • Maximilian Einsiedler,
  • Alexandros Polymeris,
  • Nikolaos Raptis,
  • Anika Beyer,
  • Denise Becker,
  • Lisette Krassenburg,
  • Isabelle Panne,
  • Mustafa Ahmed Mahmutoglu,
  • Johanna Maria Lieb,
  • Stephan Rüegg,
  • Tobias Derfuss,
  • Jens Kuhle

摘要

A 63-year-old patient with relapsing-remitting multiple sclerosis (MS) and comorbid liver cirrhosis presented with progressive cognitive decline, gait disturbance and elevated serum neurofilament light chain (NfL), suggesting disease progression independent of relapse activity (PIRA). Development of acute hepatic encephalopathy led to reconsideration of the cause of previous clinical worsening and MRI revealed progressive basal ganglia manganese accumulation, establishing the diagnosis of acquired hepatocerebral degeneration, retrospectively challenging the PIRA diagnosis. Careful evaluation of comorbidities is warranted in older MS patients, as progressive worsening and high NfL may indicate alternative etiologies, particularly if GFAP (glial fibrillary acidic protein) stays within normal range.