Abstract

This 51 year old female nurse presented in Oct 2008 with acute onset of left leg weakness, progressing to mild left hand weakness and tingling of left face and arm.  On examination there were reduced fine finger movements of the left hand, mild left pronator drift and upgoing left toe, with subtle reduced pinprick over the left face.  CT brain showed a possible small hemorrhagic right thalamic infarct.  Symptoms had resolved on discharge.  In July 2009 and again in December 2010 she presented with heaviness and clumsiness of the left leg, and developed progressive inability to use the left hand.  By September 2011 she required a cane while walking.  She retired from nursing in 2016.  In December 2021 she was admitted to neurology for further workup.  She had a new dense left hemianopsia and left facial weakness, with markedly impaired left fine finger movements and alternating left hand movements.  There was marked spasticity in the left leg, with reduced touch in left hand and foot, brisk reflexes (left>right) and bilateral upgoing toes (left>right).  MRI showed progressive right frontoparietal atrophy, left hemisphere atrophy, right thalamic atrophy, atrophy in the right corticospinal tract (peduncle and pyramid) and atrophy of the posterior corpus callosum.  There were areas of right periventricular enhancement extending into adjacent white matter, corresponding to areas of dystrophic calcification on CT suggesting a deeply situated inflammatory process.  The periventricular region was biopsied.

Discussion points

1. Does the brain biopsy explain the progressive neurological syndrome?
2. What evidence supports the notion that wider axonal degeneration can accompany localized pathology of the sort seen here?