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2017 Case 10

Case 10

K. Grenier1,2, A. Izenberg3, V. Kulasingam4,  R. John4, and J. Keith1

1Department of Anatomical Pathology, Sunnybrook Health Sciences Centre, 2McGill University Faculty of Medicine, 3Department of Medicine, Division of Neurology, Sunnybrook Health Sciences Centre, 4University Health Network, University of Toronto, Ontario, Canada

 

Clinical Summary:

This 73 year old male presented with a 3 year history of progressive  weakness and sensory symptoms. He intially developed a cold sensation in his right foot,which three months later had progressed to a tingling/burning sensation and pain in both of his feet followed by similar symptoms in his hands six months later.  Over the preceeding year and more rapidly over the preceeding weeks his sensory symptoms had spread proximally up to his knees and mid-forearms. He had also developed lower leg weakness, which was resulting in difficulty walking (he had recently begun using a walker and whellchair), and he noticed difficulty using his hands to hold objects or write. Neurological examination revealed decreased muscle bulk and weakness of his lower limbs (4+ proximally and very severe distally), weakness of his fingers (4), areflexia of the lower limbs, and decreased pin-prick sensation from his feet to lower thighs and from his hands to his forearms. Vibration sense was absent in his hands and feet. Plantar responses were flexor. Electrophysiologic studies showed a severe, length-dependent,sensorimotor axonal polyneuropathy. An extensive workup for metabolic, infectious, toxic/nutritional and paraproteinemic causes of neuropathy was negative. A lumbar puncture and MRI of the spine were also unrevealing.   He was suspected to have a non-systemic vascultic neuropathy, was started on empiric corticosteroids, and a sural nerve biopsy was obtained.

 

Materials submitted: A scanned H&E stained section of the left sural nerve biopsy.

Questions:      

  1. Diagnosis?
  2. What additional testing can confirm and sub-classify this diagnosis?