Scars

scars

Splints

MRI's

Hi Prof Goel, I thought this little story line and picture time might be easier then emailing you a heap of photos.

Small Story: At the age of 12 I developed paresthesia and nystagmus, as well as complete loss of balance (going from pointe work at ballet to not being able to walk straight). After many doctors visits I got the diagnosis of Cerebellar Ataxia and migraines. This was left be till I was 18 and while falling apart (many dislocations) I saw a doctor (2004) about my continuing symptoms and pain in right arm who sent me to a neurologist. He gave me an MRI and found these.....

Chiari Malformation to the posterior arch of c2 (24mm)

and this

Syrinx running from c2-t10

He was very shocked and sent me a neurosurgeon who performed a posterior fossa decompression and laminectomy of c1 and c2 (2005). None of my symptoms abated and I got progressively worse. This is what I looked like post surgery.

Thinner syrinx (and spinal cord, syrinx had perforated the spinal cord) and as you can see there is a weird compression in the posterior area of the cerebellum. This caused them to go forward with decompression 2 (2006). This included further bony decompression of the occipital bone and a dura patch. 

A harsh recovery and still worsening of pain and symptoms. Post surgery I looked like this. 

Syrinx still stable and only partially collapsed. 

So I went for a few years in a state of limbo and pain. I went on disability and high doses of narcotics. I was also diagnosed with Ehlers Danlos (Hypermobile). As well as my central sleep apnea issues that developed immediately post surgery 2. I went from having no sleep conditions two weeks before surgery, to having them suggest a sleep test while in ICU days after surgery (wasn't done as we were still waiting for results of the previous study and I was unstable). Less then 6 months later a diagnosis of severe central sleep apnoea. While investigating the development of sleep apnoea over the years and getting no where (eventually to work as a sleep scientist).

In between all of this a mention of tethered cord finally got bought up by a doctor because my brainstem had herniated. MRI of my lumbar region didn't seem to show much.

I was also diagnosed with POTS and inappropriate sinus tachycardia. I obtained a standing MRI as well as other random MRI. I have been told the patch put in my second surgery in 2006 has adhered to the brain. Our plan is to go back in and use a different patch material and titanium mesh plate. 

Recumbent (2011)

Standing MRI (2011) - Bendy brainstem fun!

 Recumbent spine (2011)

Standing C-Spine 2011 - Syrinx still in situ - still c2-t10

Standing vs Laying Down

On the 15th of August 2012 I had a revision where the scar tissue was removed and the dura which was adhered to the brain was removed. Pericranium and a titanium mesh was used for the patch this time.

This is the MRI post op by 2 months. I am still having pain lying on the scar, and lots of other problems we are waiting and seeing because surgically things are doing what they should and my brain is moving properly. Pain increased despite increase in pain medication. I also had an intrathecal pump out in to help some with the pain. 

Intrathecal: Morphine, Clonadine, Ropivicaine

Journey of my C spine deterioration and brainstem kink

Perforation of spinal cord from syrinx seen on 'blip' on left side of syrinx. 

In 2018 I was diagnosed with intracranial pressure (21.5cmh2O) and MRI showed slit-like ventricles and partial empty sella. I failed medication wise and that was believed to be due to my Dysautonomia not tolerating the medications. A VP shunt was put in on the right side. 

2019 Under Dr Rao’s care I obtained some relief in symptoms while in a halo for the CCI/AAI so the craniocervical fusion was done from skull to C4. More spondylolisthesis found throughout C-Spine no imaging below checked with dynamic imaging or upright. 

Central sleep apnoea was cured but still inability to manage any decent periods upright without becoming very Ill requiring 20+ hour sleep and naps. 

In 2021 tethered cord surgery was done locally by Dr Ma for occult tethered cord. Helped lower leg symptoms but caused orthostatic hypertension (170/100). Never had blood pressure issues. Cardiologist said more likely autonomic dysreflexia. 

Spend most days lying in bed - had to give up PhD. Trying many medication for Dysautonomia and seeing physio and exercise physiologist to try and maintain some function as atrophy in right leg occurring. 

Recently (2022) right bar or fusion worked it’s was through skin leaving a hole that got infected. Requiring surgery to wash out MSSA infection and file down bar. Still on antibiotics nearly 1 yr later.  

Most recent MRI showing continued cord volume loss, irregular contour, cord atrophy and recently myelomalacia diagnosed in cervical spine. Cine MRI also suggesting brain adhered to mesh and irregular movement in cerebellum. 

Most uptodate imaging 

Australian doctors unsure what to do and have been advised to do my own research and speak to international specialists. Also diagnosed with mast cell activation disorder. Running out of options and doctors who aren’t confused by presentation. 

Comparison imaging of spinal alignment 

Recent chest xray

Pre and post fusion brainstem 

MRV/A

Symptoms:

• Nystagmus (vertical and horizontal) when upright for long periods. 
• Right side vocal cord paresis
• Myclonic Jerks
• Occipital headache (persistent)
• General malaise 
• Facial pain and tingling 
• Breast pain
• Paresthesia and increasing numbness and pain in both arms and legs. Right side dominant
• Right arm going cold
• Tachycardia
• Orthostatic Intolerance
• Balance Issues
• Impaired proprioception
• Visual Perception Issues
• Severe Chronic Pain (affecting almost all the body, some persistent and some transient)
• Bowel and Bladder issues including numbness in groin and bathrooming 1-2 times a week or diarrhoea 
• Irregular/absent period
• Digestion issues (slow motility)
• Loss of fine motor movements in right hand
• Subluxations and chronic pain in joints
• Lack of temperature senstation
• Drop Attacks
• Central Sleep Apnoea -cured by fusion
• Memory/Concentration Issues
• Dysphagia lower esophageal as per swallow study 
• Clumsiness/Issues walking/Feeling ground under feet
• Dizziness
• Excessive Hiccups or difficulty hiccuping 
• Chronic Fatigue
• Allergies
• Tinnitus
• Hyperactive reflexes
• Hypersomnia
• Increased gag reflex
• Nausea and vomiting (spent 3 months with pseudo morning sickness)
• Paresis
• Problems turning neck without feeling dizzy and faint (fallen)
• Fibromyalgia (trigger points hypersensitive)
• Cape like sensation from syrinx
• Dizziness
• Dysesthesia
• TMJ dislocation
• Husky scratchy voice - query Eagles? 
• Chronic subluxations and dislocations on minimal exertion (hips when sleeping)
• Weakness in arms
• Breathing difficulties
• Desensitised to temperature/difficulty maintaining appropriate body temp. Over heat very quickly 
• SIJ instability - query fusion offered 
• Hip pain 
• I think that is it, but may have missed stuff that comes and goes

My entire list of conditions are: Chiari Malformation 1.5, Syringomyelia c2-t10, Ehlers Danlos (Hypermobile type), Dysautonomia (postural orthostatic tachycardia), inappropriate sinus tachycardia, Hay Fever, depression, MCAS, Scoliosis, Asthma, Fibromyalgia, Keratosis Pilaris, folliculitis, TMJ dysfunction, Chronic fatigue syndrome, occult tethered cord, meylomalacia, cord volume loss, intracranial hypertension, low vitamins (B, C and D - had scurvy levels for C). Bulging disks and haemangioma, annular tears (many lumbar issues suddenly occurred). 

Surgeries: Removal of occipital bone (twice), laminectomy of c1 and c2. Trial, implant and repair of morphine pump, Nerve decompression left leg, adenoids/tonsils removed, removal or dura patch and replacement with new patch and titanium mesh, craniocervical fusion, VP shunt, surgical washout. 

Thanks again for your time. 

Kirstin