The twelfth nerve

As a neurosurgeon, I’ve often found that the human body, in all its intricate glory, sometimes presents us with puzzles that even Sherlock Homes would struggle to solve. And then there are those cases that aren’t so much puzzles as they are… cruel cosmic jokes.

Sorab, a Parsi man in his late 60s, whose wit was as sharp as his perfectly pressed suits, was a retired English literature professor with a booming laugh and an uncanny ability to quote Shakespeare. “Doctor,” he declared, his voice a little like Sean Connery, “my tongue seems to be a little muffled.” His wife, Rati, a woman whose patience was clearly forged in the crucible of living with a literary genius, chimed in. “He can’t properly enunciate his sonnets anymore, Doctor. And his attempts at whistling now sound more like sounds made by a dying pigeon,” she said in jest.

I asked him to pull out his tongue. “It feels like a lazy slug, rather than the agile instrument of eloquence it once was,” Sorab quipped, attempting to stick it out, only for it to deviate slightly to one side. I could clearly see fasciculations – tiny, involuntary muscle twitches, like a bag of worms wriggling under the skin. “This is a problem with your hypoglossal nerve, the twelfth cranial nerve,” I told him. “Its job is to be the maestro of your tongue. It dictates every nuanced movement, from articulating the most complex polysyllabic words to helping you savour a perfect meal. It’s the last of your 12 cranial nerves.”

“I once read a book called Doctors by Eric Segal, which contained a mnemonic to remember the 12 cranial nerves used by medical students at Harvard – Olfactory, Optic, Oculomotor, Trochlear, Trigeminal, Abducens, Facial, Auditory, Glossopharyngeal, Vagas, Accessory, Hypoglossal,” he belted out without hesitation. “Wow – and do you remember the mnemonic?” I prodded, knowing it would be naughty. “O O O To Touch And Feel A Girl’s Vagina – Ah Heaven!” he said with a cocky smile. His wife’s jaw dropped.

My mind immediately considered the usual suspects for an isolated hypoglossal nerve palsy. Perhaps a small tumour near the brainstem, pressing on the nerve as it exited. Or it could be a vascular lesion, a tiny stroke, infections, even inflammatory conditions… the list was exhaustive. But then, I asked him to remove his shirt and saw muscles twitch over his arm as well. The MRI, as expected, was unremarkable, but the EMG test confirmed my suspicion.

“ALS or amyotrophic lateral sclerosis,” I explained to Sorab and Rati, choosing my words with the care of a poet selecting the perfect metaphor, “is a rather cruel trick of nature. It is a progressive neurodegenerative disease that attacks the nerve cells in your brain and spinal cord – the very cells that control voluntary muscle movement.” I watched their faces trying to comprehend this, the flicker of fear in her eyes, the brave, slightly forced smile on his.

“Unfortunately,” I continued, “it is often diagnosed by exclusion, by ruling out everything else. And the initial symptoms can be incredibly varied – it might start with weakness in a limb, difficulty speaking, or, as in your case, a problem with the tongue.”

Over the next few months, the picture, sadly, became clearer. The weakness in his tongue slowly, inexorably, began to spread. His speech became more slurred. Swallowing became challenging. His once booming laugh was now a mere whisper. “It’s like my body is gradually forgetting how to perform the most basic of human functions,” he confided in me one day, his voice thick with a mixture of frustration and weariness. “Soon, I fear, I’ll be more of a statue than a scholar.”

ALS is a brutal disease. Despite the tremendous scientific advancements made over the last century, there is no real cure. We have medications that can modestly slow its progression, supportive therapies like speech and physical therapy, and assistive devices to maintain quality of life for as long as possible. But the trajectory, for now, remains heartbreakingly clear. “It’s a marathon against time,” I told him, “and we’ll run it with you, every step of the way.”

He faced his diagnosis with a blend of dark humour and profound resignation. “Well,” he mused, “at least I’m in good company. I hear Stephen Hawking knew a thing or two about this. And Lou Gehrig, the baseball legend! Though, I must admit, I prefer intellectual challenges to hitting a fastball.” He even joked about a new “ALS diet” consisting solely of pureed Shakespeare.

Over the next few years, I watched Sorab, a man of such vibrant intellect and spirit, gradually succumb to the relentless grip of ALS. His initial struggles with his tongue progressed to weakness in his limbs, then in his respiratory muscles. The man who once filled a room with his booming voice now communicated through an eye-gaze device, meticulously spelling out words one agonizing blink at a time. The physical struggle was immense, a daily battle for breath, for movement, for communication.

During one particularly poignant visit, I found him lying in bed, frail but still with that spark in his eyes. He had painstakingly spelled out a message for me: “Doctor… my body may be… a silent… fortress… but my mind… still quotes… Hamlet.” He then managed a weak, lopsided smile. Sorab passed away peacefully in his sleep, surrounded by his beloved Rati and his treasured books. His passing, like all patients lost to this relentless disease, was a stark reminder of the frontiers we still need to conquer in neuroscience.

But even in death, Sorab left an indelible mark. He showed me that when the body betrays you, the mind, if nurtured, can remain a vibrant, unyielding beacon. And sometimes, the most profound lessons are learned not from what we can fix, but from what we are yet to understand. His legacy, I believe, is not just in the sonnets he taught but in the quiet, powerful poetry of his fight.

“Don’t forget to teach the mnemonic to your students,” were his last words to me.

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