Locked in syndrome

I saw a national news report last week about Martin Pistorius. At age 12 he came home sick from school and quickly grew worse, lapsing into a coma that lasted for some 3 years. The doctors were not sure what was going on but given his condition, did not expect him to live. They also told the family he could not see, hear or feel anything. Diagnostic details are lacking. Then he slowly ‘woke’ up—he was conscious but could not move or control movements like eyeblinks, so no one knew he was present. After some time a therapist noticed some “glimmer” in his eyes and this led eventually to the discovery that he was conscious, just “locked in”. Over more time he recovered some motor control over eye blinks and even his arms/hands, so that he could then use a computer to communicate. He eventually became a computer programmer and married a friend of his sister’s and wrote a book about his experience, Ghost Boy.

I am curious about this and have put his book on my reading list. His MEMBRAIN essentially shut down, all channels closed, nothing in or out, yet his brain maintained his vegetative functions so he continued living, and then the channels in (at least vision and hearing) came back and then in a very limited way some channels out returned. I went online to find out more but there is not much out there. I did find reference to ‘locked in syndrome’, a rare and usually fatal condition believed to be from some brain infection, but Martin Pistorius weathered this viral storm and came back. Wow!

Many questions now arise. His connectome seems to have survived essentially intact; he remembers events and people except when he was in the deepest coma and he retains his identity, but is there some amnesia for some events? He seems quite able to learn and grow. How can this be? His muscles atrophied and neurons also do not do well without stimulation but his brain, despite its comatose level of functioning, kept on. I wonder what his pre- and post IQ were (but probably no record). When he was emerging from the coma, was he sentient but unconscious for awhile, as in highway hypnosis or the claustrum study reported here a month or two back? Did he dream during any of this time? Diurnal/sleep cycles? Did he perceive without any emotional response at some time? Did he experience frustration arising from intentions being formulated but without the capability of implementation? Questions about what caused this or how he weathered it are probably pretty unanswerable at this point but still remain.

What I can figure out is that part of his brainstem was affected, most certainly the pons which enervates the brain for arousal and conscious processing. Maybe also the thalamus which acts as a processing center between the brainstem/lower midbrain perceptual input and the cortex was also affected. Other brainstem centers, e.g., medulla, concerned with vegetative functions certainly were relatively unaffected.

pons

The hypothalamus was probably unaffected as it is involved in maintaining vegetative homeostasis. As he recovered, the pons and thalamus came back to function, but anterior motor systems remain impaired either through damage to the basal ganglia which coordinate movements or motor neuron damage similar to ALS, a sort of high level atrophy.

Basal_Ganglia_and_Related_Structures

Finally he has recovered some motor control over his head, eyes, arms and hands. I recently learned through my reading of Joseph’s Neuroscience text that two different systems serve high level sensorimotor functions. One system works to maintain the body’s position in space and then to guide movement through the environment. Mr. Pistorius can navigate as he moves in his wheelchair, so his movement disorder is more similar to motor neuron syndrome such as ALS. The second system works to perceive objects that are within reach and then to guide his arms and hands through touch and vision as they perform skilled movements. Fortunately this system and the lower level motor neurons for his hands/arms seem mostly intact.

So a devastating brain illness affecting MEMBRAIN functions but keeping the soma alive though with muscle atrophy, and then recovery of the connectome holding the identity and psychology of one person, such as personality, memory, etc. and partial recovery of motoric abilities, fine motor better than much gross motor functions. He and his family deal with tragedy and then triumph through a perceptive therapist and computer technology permitting him to communicate from the interior of his MEMBRAIN. His book should be a very interesting read.

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