For the Love of Music
By Alicia Harper
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published in the Boston Globe
Judging normal brain function in a neurologically impaired person is never an easy task. The case of Terri Schiavo illustrates this challenge in a dramatic way.
A Boston-area patient I first saw when she was 32 also proved the point. The woman had been born without the portions of her brain associated with thought and awareness -- a condition called hydranencephaly that's usually fatal prenatally or in infancy. But she had somehow survived, mainly through good custodial care, including being fed pureed foods by a caretaker all of her life. Her eyes were open wide, and she could move her head from side to side, and make groaning sounds, similar to Terri Schiavo. A quadriplegic, the woman was bedridden or strapped to a wheelchair for several hours each day. She weighed 77 pounds and was 4½ feet tall. She could swallow and breathe on her own.
The neurological evaluation revealed that the woman failed to blink in response to objects rapidly approaching her eye, indicating blindness; she had abnormal reflexes, and no clear sensitivity to touch. A bright light shone on one side of her slightly enlarged head revealed a bright red fluid-filled cranium. A subsequent MRI revealed the absence of cerebral hemispheres, with only small remnants of cerebral tissue, and a small brain stem.
The conclusion by the attending doctors was that this woman was unresponsive to sensory stimuli, devoid of any intellectual function, and in a persistent vegetative state.
To confirm these conclusions, she was referred to me for an objective, noninvasive evaluation of her brain's electrical activity and her response to sensory stimulation, including sound, sight and touch. In summary, I found that, although her eyes were open and moving from side to side with her head, there was no brain response to visual input. Similarly, mild electrical stimulation in the fingers and toes traveled up the spinal cord to the brain stem, but no farther in the absence of the cerebral hemispheres.
I was astounded, however, that when I turned on a child's music box in the room, I observed that this hydranencephalic patient turned toward the musical device and began to smile and make sounds, as if she were enjoying the experience. I then tested this observation several times and found a consistent response to sound stimulation. When I conducted a test of electrical activity in her brain stem, the portion of the brain that controls bodily functions like breathing, I was surprised to find that the neurons of the brain stem involved with hearing were normal.
Several more advanced electrophysiological brain measures showed that she had normal hearing response waves, reflecting neural activity in the higher brain stem. She was aware at some level of the sounds and people noises in her environment, and responded to these sounds with the appearance of joyfulness.
I immediately brought her other doctors back into the room, where they began to interact with her in a totally different manner, in some cases holding her hand and trying to speak with her, and treating her more like a normally functioning human being. I was so emotionally moved by her struggle for human definition through the single modality of hearing that I went down to a local electronics shop and bought her an audio cassette player, and some modern and classical music.
She continued to appear to enjoy the audio cassette player and her music until her death some years later.
This patient demonstrated the dilemma we face in determining whether people in an apparent persistent vegetative state, who by all objective measure have little or no function in the cerebral hemispheres, have any residual human capacity that would persuade us to sustain their lives, even by artificial means.
Her case was a reminder of how much we do not understand about the brain, and that even people in an apparent vegetative state may have ways of connecting to the world around them.
Dr. S. Allen Counter is professor of neurology and neurophysiology at Harvard Medical School and the Massachusetts General Hospital.