Abstract
We present the case of a South African man who presented with lower limb weakness and spasticity beginning at the age of 62 years on 2007. He was diagnosed with lower limb onset of Amyotrophic Lateral Sclerosis following which he emigrated to Australia
First seen in 2012 into his fifth year of disease at age 67 years, he had features of global upper and lower motor neuron dysfunction confirmed neurophysiologically, with no abnormality on neurimaging. Of interest he had poliomyelitis as an infant, symptomatically only affecting his left leg, requiring a caliper until the age of eleven, followed by normal neurological function.
His presentation clinically was consistent with all-limb amyotrophic lateral sclerosis.
There was no respiratory or swallowing impairment. His subsequent course was very slowly progressive over the next ten years. His dysarthria and spastic paraparesis progressed to quadraparesis by 2022, although he could still transfer and walk short distances. There was no significant respiratory involvement.
Following a fall at home in 2023, the patient sustained significant chest injury with a flail segment and respiratory failure. A conservative approach was adopted and the patient deceased aged 78 years.
Neuropathological examination revealed Upper and Lower motor Neuron loss due to post polio syndrome and cerebral congophilic angiopathy and widespread aging-related tau astrogliopathy (ARTAG).
The literature and pathology of post polio syndrome, together with its relevance to Amyotrophic Lateral Sclerosis presentations will be presented, highlighting the heterogeneity of the causes and pathogenesis of motor neuron diseases.
