The diagnosis of ALS is largely a diagnosis of exclusion. There is no blood test or imaging study that confirms the diagnosis. The work up includes extensive blood work, imaging studies and nerve conduction tests to rule out other diseases that may cause the symptoms. Only in the absence of another cause is the presumptive diagnosis of ALS made.

 

The workup included MRIs of my brain and spine. The MRI of my brain revealed an “incidental” finding that I had a brain tumor called an Acoustic Neuroma. An Acoustic Neuroma is a benign brain tumor arising from the nerves that innervate the balance component of the inner ear. Unfortunately, it was thought to be unrelated to ALS and just another thing to deal with. I was having only mild symptoms of intermittent tinnitus and a feeling of fullness in my right ear, and any therapeutic intervention was deferred. I had something more ominous to face with the diagnosis of ALS.

 

I was fortunate to have the slower progressing limb-onset predominant form of ALS. That gave my Acoustic Neuroma time to grow. Over the next 18 months, the symptoms became constant and more intense. I had another MRI which documented growth and the decision was made to treat it with minimally invasive Stereotactic Radiosurgery Gamma Knife, which was performed on June 27, 2013. There was no thought of definitive surgery by me or anyone else because I was living with the fatal diagnosis of ALS. 

 

[The Gamma Knife Stereotactic Radiosurgery procedure involves screwing a rigid headframe to the patient’s skull, a brief MRI to localize the target within the headframe, Precise computations to determine the treatment plan (amount of radiation to give each point within the target), securing the headframe within the collimator (a hemispherical device with 192 apertures all focused to a point), and exposing the target to a precise amount of radiation from 192 Cobalt 60 sources.]

 

The Gamma Knife was deemed a success and I suffered no complications. The auditory symptoms stabilized; the feeling of fullness abated, allowing the Acoustic Neuroma to recede from my consciousness. For the next 14 months, I was caught in the relentless grip of this progressive disease culminating in a hip fracture resulting from a fall. A Hidden Blessing, I would start learning about yoga during the recovery. My Acoustic Neuroma symptoms remained in the background until mid-2015 when my yoga practice achieved an adequate degree of intensity. The most strenuous parts of the practice would provoke auditory hallucinations, an increased feeling of fullness, a headache, and lightheadedness. 

 

In spring of 2016, the symptoms would increase to pre-syncopal levels, where I would have to stop the practice to prevent myself from passing out. These events would be accompanied by an intense headache and loud auditory hallucinations of pinging and high frequency whining. I got another MRI which showed minimal tumor growth but discovered that my blood pressure was greatly elevated. I obtained an appointment with Dr. John Y.K. Lee, a Neurosurgeon at Penn, but by the time of my appointment my elevated blood pressure was treated, and the symptoms had returned to baseline. Obviously, he didn’t want to operate.

 

My proposed mechanism of action as to why my practice would provoke an increase in symptoms is based on my knowledge of factors influencing intercranial pressure. 

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A little anatomy lesson: The brain is enclosed in a rigid “box”, our skull. The only other components in the skull besides the brain tissue are blood in the arteries and veins and spinal fluid which the brain “floats” in. These three components are in a delicate balance. When one component increases in volume, this necessitates a decrease in volume of one or both of the other components to maintain normal intercranial pressure. Gradual changes are more easily compensated for than rapid ones. Also, there are limits to the amount of compensation possible. 

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My Theory of why the symptoms would increase during my practice:  The Acoustic Neuroma occupied space within the skull requiring changes in the blood and spinal fluid compartments to maintain normal intercranial pressure and my symptoms were stable at rest. These changes used up some of the compensation available rendering the delicate balance more sensitive to acute changes in the other components. When I practice, I activate the core musculature. The muscles of my core were the greatest beneficiaries of rehabilitation of my physical body and are the strongest they have ever been. When I engage the core, the pressure in my intrabdominal cavity increases. This pressure is transferred to all the contents of the abdomen including the Great Vessels (Aorta and Vena Cava). This, in turn, raises the pressure in the Aorta (minimal effect) and Vena Cava (major effect). This elevation of Central Venous Pressure is transmitted to the veins within the skull causing a sudden increase in the volume of blood imposed on an already compromised balance of intracranial components. This would result in a spike in Intracranial Pressure and trigger the symptoms I was experiencing. These symptoms would color my practice until late 2017 when I would be forced to make a decision… either take definitive action to treat the Acoustic Neuroma or stop my yoga practice. 

 

Things changed drastically in November 2017. The following is an entry in my Yoga Journal written on November 22:

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“Acoustic Neuroma Symptoms abruptly changed starting 11/15. My right upper lip tingled for a few days before the entire right side of my face went numb. Temperature sensation is absent, pain is blunted, and touch is intact. Don’t think I have any weakness, but my right eye feels weird. Cornea is numb. The right side of my teeth and tongue are numb. The density of numbness has increased. Have an increased sense of fullness and fleeting pain in my right ear. If I push my yoga, the sense of auditory distortion and lightheadedness grows stronger.”

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MRI showed the volume of tumor had more than tripled since April 2016. The tumor was trapping the Trigeminal Cranial nerve (C.N.5) against the brainstem and causing the right side of my face to go numb. The Acoustic Neuroma is a benign tumor and would likely have its growth arrested by another Gamma Knife. The Trigeminal nerve is the sensory nerve to the face and a numb face is not fatal. I had conservative options to mitigate the tumor growth but would not prevent the yoga practice symptoms I was having. So, the decision to seek definitive relief through invasive surgery would be based entirely on my desire to continue my yoga practice. I had a tough decision to make.

 

I brought my quandary to my Neurologists in Miami, Drs. Bradley and Verma. While they cautioned about the risks of major surgery in a patient with ALS, they recommended that if I was entertaining the surgical option, I should see Dr. Jacques Morcos. Dr. Morcos is a Professor of Neurosurgery at The University of Miami with expertise in Skull Base surgery and extensive experience in resection of large Acoustic Neuromas like mine. I made the decision that my yoga practice was responsible for my breath, attitude and constitutional sense of well-being, and life without yoga would be to commit to dying. The risk was worth taking and I set about to convince Dr. Morcos and the Anesthesia Department that I was a good surgical risk. I underwent the 6 hour surgery on January 8, 2018. The recovery was difficult, but at a month post-op, I knew that my conviction was right!

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