Abstract

Post-polio syndrome (PPS) develops in approximately 30% of polio survivors several decades after the acute attack of paralytic poliomyelitis. Some of these patients develop post-poliomyelitis muscular atrophy (PPMA) which is characterized by a slowly progressive muscle weakness. Due to its clinicopathological features, investigators have often studied PPS and PPMA in association with amyotrophic lateral sclerosis (ALS), the underlying hypothesis being an increased risk of developing ALS from a prior acute paralytic poliomyelitis. Various studies, however, have indicated that de novo ALS cases in patients with prior acute paralytic poliomyelitis are rare. Herein, we describe a rare case of a 75-year-old woman who at post-mortem examination presented a combination of a PPS with proven histopathological sporadic ALS features. Furthermore, neuropathology of this case also revealed several other histopathological findings reminiscent of a tauopathy, synucleinopathy and amyloid angiopathy and a large pituitary cyst. To our knowledge, this is the first reported case of PPS with clear pathological hallmarks of sporadic ALS, including ubiquitin-, TDP-43, phosphorylated TDP-43- and p62-positive inclusions, with accompanying features compatible with Alzheimer’s and Parkinson’s disease.

Author Details

Authors

• M. Casula¹
• K. Steentjes²
• E. Aronica¹
• B.M. van Geel²
• D. Troost¹

Departments

• ¹ Department of (Neuro) Pathology, Academic Medical Center, University of Amsterdam, and
• ² Department of Neurology, Medisch Centrum Alkmaar, The Netherlands

Address

D. Troost
Department Neuropathology
Academic Medical Center
Meibergdreef 9
1105 AZ Amsterdam, The Netherlands
Email: [email protected]

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