- Older RRMS patient with liver cirrhosis developed cognitive decline, gait disturbance and raised serum neurofilament light chain suggesting PIRA.
- Acute hepatic encephalopathy and MRI evidence of basal ganglia manganese deposition established acquired hepatocerebral degeneration, challenging the prior PIRA diagnosis.
- Careful assessment of comorbidities in older MS patients is essential; progressive worsening and high NfL may reflect alternative aetiologies if GFAP is normal.
Neurol Sci. 2026 Jul 31;47(8):670. doi: 10.1007/s10072-026-09284-x.
ABSTRACT
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.
PMID:42533190 | DOI:10.1007/s10072-026-09284-x
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