GL: clinical condition in summary


     Patient GL, whose results are reported here was followed by the Doctor Yves Lamarre at the Hôtel Dieu hospital in Montreal, Quebec, Canada. She was 27 years old when suffering in 1975 from a first episode of acute polyneuropathy with a complete paralysis including the respiratory muscles. A diagnostic of Guillain-Barré was made. She left the hospital three months later. It took three more months for complete functional recovery that allowed her to meet again her normal professional and familial activities, and she even gave birth to a child. 
      A second episode of extensive sensory polyneuropathy occurred suddenly four years later (April 1979). The neuronopathy selectively affected  the large myelinated sensory fibers; There was no paralysis but only a transient muscular weakness that vanished after two months. In contrast the severe sensory impairment affecting her whole body below the nose remained unchanged over the years until now.

      GL has a complete loss of touch, vibration, pressure and kinesthetic senses and no tendon reflexes in the four limbs, the trunk being more moderately affected. Pain and temperature sensations are present, indicating a selective impairment of the large diameter peripheral sensory myelinated fibers. The motor fibers have not been affected as shown by electrophysiological tests: Motor nerve conduction velocities and needle EMC investigation of the muscles of the arms were normal. H-reflex were absent in the legs. No sensory potential could be recorded in the hands or the feet and no cortical response could be evoked by electrical stimulation of the peripheral nerves of either arm. The results of a sural nerve biopsy, published in Robert Forget thesis (see figure below) revealed a severe demyelination affecting particularly the large fibers: the density of the myelinated fibers, 2,496 fibers/mm2, was much lower than normal values (more than 6,000 fibers/mm2 according to Ochoa & Mair (1969)). The percentage of myelinated fibers larger than 9µm in diameter was very small (0.31%) as compared to normal values (more than 18%).

                           
                             

      No sign of recovery has been observed since 1979 in periodical control tests made since. Functionally, GL remains largely confined to a wheelchair since her ataxic gait resulted in frequent falls even with continuous visual monitoring of her legs and the use of a walker. Daily manual tasks gradually improved but have to be performed under constant visual guidance and a heavy dose of attention.


Bibliography

Ochoa J., Mair W.G.P. (1969) The normal sural nerve in man. 
Acta Neuropathol (Berl)
 13:197-216

Charron L., Peyronnard J.M., Marchand L. (1980) Sensory neuropathy associated with primary biliary cirrhosis
Arch Neurol 37:84-87 


Cooke, J.D., Brown, S., Forget, R., and Lamarre, Y.
 (1985). Initial agonist burst duration changes with movement amplitude in a deafferented patient. 
Exp.Brain Res. 60:184-187. 

Olausson, H., Lamarre, Y., Backlund, H., Morin, C., Wallin, B.G., Starck, G., Ekholm, S., Strigo, I., Worsley, K., Vallbo, A.B. and Bushnell, M.C. (2002) Unmyelinated tactile afferents signal touch and project to insular cortex
Nature Neuroscience. 5 n° 9 900-904


Videos
 
VIDEOS, in FRENCH, but images speak by themselves.
 
Le magazine de la santé, France 5. Rudy Bancquart & Alexandre Delaval.
Deafferented Ginette's daily life (5 min.). Mercredi 12 Juin 2013. 
Deafferented Ginette's experiments @ the Institute of Movement Sciences, Marseille (5 min.). Jeudi 13 Juin 2013.
 
Proprioception & Motor Control is a video, lasting 21 minutes with French audio, highlighting the roles of proprioception and vision for the control of our movements. 
 

VIDEO: In ENGLISH
The Man Who Lost His Body is a health documentary film about an English patient who was suddenly deprived of proprioception at the age of 19. The 48 minutes long video is part of BBC's Horizon series (1998), and was directed by Chris Rawlence and produced by Emma Crichton Miller. More info in Jonathan Cole's Pride and a Daily Marathon (1991, The MIT Press).
 


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