- My Story -

 

This may sound familiar to some of you, to others it may not. We all have our story about how and when it happened, the things we noticed, the subtle or sudden changes. Many of you will relate to the medical procedures, the comments from the Doctors, the lack of understanding. Invariably we all go through much of the same things, alone when there are so many of us that share the same common thread.

In late August of 2010 I strained my lower back. It was minor, but it was at work so I saw my Doctor and he said it was a strain just as it felt. I declined medication and went on about my usual business. I had no idea what was about to ensue.

Two weeks later on September 15th I began dragging my left foot slightly, but more during the evening with some noticeable lower back pain mainly on the sides. So I decided to just take it easy and be careful about lifting and such. A few weeks later it was not improving, so I went back. This time I was given some Flexeril (Cyclobenzaprine) to relax the muscles. It was quiet strong I took it, slept heavily and felt ok, but the limp continued. It was more of an annoyance than anything else. So I just ignored it and continued to deal with it.

At Thanksgiving I noticed that I was having considerable difficulty stirring the gravy, some numbness and lack of dexterity in my left hand along with poor coordination in the left arm. This was new and I knew there was something wrong. I remained stoic and did not mention it to my spouse or to anyone else. I kept telling myself it would go away, but it did not and the limping progressively got worse over the next couple of months.

At the end of January the arm symptoms became constant. I could barely write my name. The limp became a consistent dragging of the left foot, worsening with activity.  The course of my treatment is chronicled below and set forth in two tables which illustrate my course of treatment both pre/post diagnostic testing by specialists.   Table 1 sets forth my course of treatment before testing by specialists.  Table 2 sets forth my course of treatment after diagnostic testing by specialists.  The results are and continue to be inconclusive and I am now on maintenance treatment.

DISORDER CHRONOLOGY

January 2011 -  Returned to the Doctor, prescription for Vicodin (hydrocodone) this time. I noticed that upon waking all of the symptoms were absent for about 30 steps. Then it would begin and continually worsen throughout the day.

February 2011 - Back to the Doctor again having some real issues with weakness.  Prescribed Percocet 5/250 (Oxycodone), still no effect.

March 24, 2011 - Consistent foot dragging on the left side, severe cramping in right leg, and extreme difficulty in ambulation. Stiffness in neck, sensation of dizziness and feeling of passing out when turning head hard to the right. 2 weeks off work, 10/325 Percocet has minor effect on pain, 12 day sliding dose of Prednisone (40/3 days, 30/3days) no effect. Unable to even stand up after work without resting for at least an hour or two.

March 31, 2011 - Restart Prednisone with no effect, still on heavy Percocet. Time for the Lumbar MRI

  • L5-S1 minimal patchy desiccation. Mild circumferential disc bulge with minimal osteophytosis and discogenic marrow signal change. No focal protrusion or spinal stenosis. Mild facet and ligamentum flavum degeneration. Mild right foraminal stenosis and borderline stennosis at the left foraminal entry zone. Prominence of the central canal of the distal spinal cord, recommend full spinal cord screening to evaluate for syrinx.

April 6, 2011 - Epidural steroid injection that was painful for a week and helped the limp for about 5 minutes after having a "Vasovagal Event" Translation: almost passed out and had to be given an ephedrine I.V. to keep me from hitting the floor. I saw the blood pressure reading of 80/60. I thought I was done for a second.

April 14, 2011 - Orthopedic Surgeon still on 10/325 Percocet. So his initial evaluation went something like this:

  • Unable to Tandem walk, obvious ataxic, stumbling gait. Poorly on Romberg test. Individual muscle shows good strength. Stand on heels/toes without weakness, some instability. Normocephalic, atraumatic, extraocular movements intact, neck supple, trachea midline,cranial nerves intact. 5/5 biceps, triceps, deltoid, wrist flexion, wrist extension and hand intrinsics bilaterally. Fully intact sensation in C5-T1 dermatomes. Negative Hoffman. Left side 4+ biceps, brachioradialis and triceps reflex with two beats of clonus. Suggestion of an inverted radial reflex on left. Right upper extremity: 2+ biceps, brachioradialis and triceps reflexes. No Clonus. Fairly slow thumb to finger alteration bilaterally. Appears clumsy and uncoordinated. Lower Extremity: 5/5 hip flexion, knee flexion, ankle dorsiflexion, ankle plantar flexion and extensor hallucis longus. Negative straight leg raise bilaterally. 4+ Patellar and achilles reflexes bilaterally, 2 beats of clonus. Negtive Babinski. Assessment:: Left upper extremity and bilateral lower extremity weakness and loss of coordination. Ataxic Gait, Left upper extremity and bilateral lower extremity hyperreflexia. Low back pain.

The Orthopedist tells me it is not your lower back and you seem to be falling all over yourself. That looks like it hurts he tells me. I am thinking maybe he should take some Percocet and try doing the tandem walk while scratching your armpit and the top of your head at the same time. At this point I am also thinking "Quack" He then tells me that he thinks it might be in my neck, which actually sounds reasonable to me at this time. I am holding on to my faith in the Doctors here. He schedules me for a Brain, Cervical and Thoracic MRI.  It sounds more serious now and he is obviously concerned. They offer me a cane on the way out and I politely decline.

April 15, 2011 - MRI. I ask how long it will take and they say about 4 hours. I tell them I'll be right back. Out to the car and take another Percocet. I was sleeping beautifully, but the guy kept waking me up "...ok one more on this one then....you ok?...) (Results from the Doctors are in green bold FYI) Results below:

  • Brain MRI Without Contrast:  Pineal mass or cyst is at the upper limits of normal is size (9-10mm) No evidence of Multiple Sclerosis or Chiari.

 

  • Cervical Spine:

Occiput to C2: No significant ventral extradural mass effect.

 

C2-3: Normal with desiccation, foramina normal in caliber.

C3-C4 Normal with desiccation. Mild circumferential bulge partially effaces CSF. Right moderate and left mild uncovertabral osteophytosis and associated disc contribute to right severe and left moderate foraminal narrowing.

 

C4-C5: Mild disc space narrowing with desiccation. Mild circumferential bulge partially effaces CSF. Moderate uncovertabral osteophytosis and associated disc contribute to bilateral severe foraminal narrowing.

 

C5-C6: Mild to moderate disc space narrowing with desiccation. Mild circumferential bulge partially effaces CSF. Caudad extension of right central disc material up to 5.6mm caudaad to the C6 superior endplate measures up to 2.7mm AP and partially effaces ventral CSF. Moderate uncovertabral osteophytosis and associated disc contribute to severe foraminal narrowing.

 

C6-C7: Normal height with desicccation. Mild increased convexity to the central through left foraminal aspect of the disc partially effaces CSF> Left mild uncovertabral osteophytosis and associated disc. Right moderate and left severe foraminal narrowing.

C7-T1/T2-T3: Normal disc space and foramina.

 

  • Thoracic:  Mild prominence to the central canal from the upper T6 level caudad. Maximal at T12 level where it measures 1 x 1.9mm in axial dimension. T11-12 Mild circumferential bulge partially effaces ventral CSF. Mid and Distal thoracic cord mild hydromyelia with no mass or compression identified.

April 18, 2011 – Orthopedic Surgeon.  Assessment is "it is not your neck, but you have this Pineal Cyst and you should really get that checked out." Now I am totally stressed out thinking Brain Tumor.  I am referred to a Neurologist whose office staff seemed completely incompetent when it came to scheduling me for an appointment. I found another neurologist that had great credentials and seemed quite competent. I went in to see him and he was quite genuine and sincere, which I appreciated. In the meantime, I was waiting to get into a Neurosurgeon that graduated both Harvard and Yale, one of the top 50 Doctors in the country. He would figure it out at the end of June when he could see me. So I continue with the Neurologist, Rheumatologist, my primary Doctor and wait for my referral to the University. They are all quite as helpless as I am.  I am still barely able to walk. If you have never had a spinal tap, I do not recommend it. It hurt like I cannot explain, mostly when they punctured the layers of the cord. There is no way to numb that deep, I do not care what they tell you. The SSEP was also physically taxing. It was 5 hours of being shocked with electrodes taped all over my body. This was worse than the first time, but I kept laughing every time they shocked me even though it was not very funny at the time.

May 2 through 31, 2011

  • EEG, EMG, VNG, TCD, Lumbar Puncture.

EMG:  Carpal tunnel and ulnar neuropathy.

Lumbar Puncture: WBC 1 H, RBC 1, Glucose 64, Protein 38, IgG synthesis rate <0.0, IgG Index 0.44(normal), Oligoclonal Bands negative. Alb CSF 24, IgG CSF 1.7, Alb Serum 4390 f, Alb index 5.5, IgG Alb ratio CSF 0.07 L, IgG Serum 708 L. Gram Stain no WBC, No Organisms, Culture result Negative.

Blood Tests: ESR 5, ANA Negative, Normal/Negative: B12, Methylmalonic Acid, RPR,

VDRL:  Requested for positive elimination of tertiary syphilis not performed at this time.

SSA/SSB, ceruloplasmin, Copper, CBC, CPK, AST, Creatinine, CRP, Alkaline Phosphatase, BUN, ALT, TSH.  All normal or5 negative.

June 21, 2011 – Appointment with Neurosurgeon to finally find out what is wrong. I go to the Neurosurgeon and the first thing he said was "What did they say about the Pineal Cyst?" I knew this guy was on to something. So he schedules me another MRI on the Brain with contrast.

  • June 30 through August 2, 2011

Condition unchanged, peak of exhaustion at end of day, nearly unable to stand, profuse stiffness in neck at times and pain in lower back, primarily left sacral and L5-S1 area, hunching at shoulders, sporadic difficulty getting out of chairs (feels like extreme weakness). Resting (lying prone on back with head elevated) for 15 minutes to one hour relieves symptoms similar to waking.  Approximately 30 steps and limping begins, arm problem is consistent at this point. Tendency to stumble backwards once motion is initiated in that direction.

  • MRI Brain: Unremarkable, pineal cyst 9mm, no hydrocephalous , normal pons, normal parasagittal height to middle of peduncle, no infarct or geographic perfusion abnormality.
  • MRI Thoracic: Similar to previous.
  • July 22, 2011 - SSEP: Similar to EMG findings.

I am referred to a brain surgeon at a major hospital. This guy was a sour grape for sure. I felt like he was paying me and I was just wasting his time. He said it's not the cyst, I don't know what's wrong with you, but either you need to start all over instead of walking in here with a book of what you've had done, or it could be in your head. At this point I got irritated and told him we're in a Hospital, there has to be someone I can see. So he refers me to yet another Neurologist across the hall who in turn ends up being quite a colorful character. So after about an hour of getting checked out he says "I think you might have possible MSA" Let's put you on some medicine and see what happens.  I am thinking after all this there is no way a pill is going to fix this. Off I go with the prescription and another 4 hour MRI on the brain scheduled for my Sunday. I Google MSA in the car - Life expectancy is 3 to 7 years!  I get the Sinemet as prescribed and 2 days later I am walking fine.

  • July 27, 2011 - Pineal Cyst ruled out, MSA Suspected after exam. No autonomic symptoms or facial involvement of any type at any time.

Gait Abnormality and coordination problems present at exam.

BP Lying 5 minutes: 126/85 HR 72

Standing 1-2 minutes: 110/80 HR 84

Standing 3-5 minutes: 110/80 HR 84

Limbs normal, some subtle ridgity in lower left limb noted.

Reflexs:  Biceps: 2; Knees: 2+; Wrists: 2; Ankles: 2

Plantar (Babinski): Down

Asymmetry of Gait:  Without actual focal weakness or foot drop, reflexes not pathologic, non pathologic drop on blood pressure when standing, no tremor

All other aspects normal

 

  • July 28, 2011 - Initiated dose of Sinemet 25/100 3 times daily.
  • July 29, 2011 - Modest Improvement in walking.
  • July 30, 2011 - Limping and dragging of foot alleviated, modest improvement in dexterity of left hand, range of motion in left relatively unchanged. Sporadic Radiculopathy through extent of left tricep continues as well as numbness in left thumb and small finger. Slight twitching of left index and small finger, arms extended, palms inverted. (this condition was present from 4-11 to current date.)

 

  • August 2, 2011

Reflexes:  Biceps: 1; Knees: 1;

Diagnosed as unusual presentation of Parkinson's.

  • August 6 through August 9, 2011

Continued ability to walk normal, dexterity similar or improved in left fingers, arm condition same. Finger twitching only present when resting fingers on keyboard typing. Mild cramping in left gastrocnemius (anterior), involuntary movement in left tendons on foot and toes (clenching of toes, lateral tapping of foot at ankle typically during 2-4 hours during sinemet ON time) Slight anxiety Energy levels at or above normal, able to spend day walking and working as normal.

  • September 6, 2011 - Follow up with Neurosurgeon to evaluate neck and arm problems.

 

  • September 22, 2011 - Follow up with neurologist and movement disorder specialist.

 

  • October 18, 2011 - Referral to UW Neurology Movement disorders Suggested Parkinson's, but unsure.

 

  • November 2011 – March 2012

3 Cervical Steroid injections, severe work restrictions imposed which helps with overall well being.

  • Current course of treatment:

Sinemet 25/100 ½ to 4 times daily depending on activity. Rigidity in left arm and neck in and Diskynesia left foot presents at 2 to 3 hours for a duration of ½ hour to 1 ½ hours. Regardless of initial dose. Alleviated by ingesting protein or adding a dose of Sinemet. Can actually take Sinemet just as needed for walking. Usually taken to help with movement and coordination in left arm.

Any period of rest results in symptoms disappearing for a short period, can last up to 4 hours. Exertion (walking, lifting) intensifies symptoms. Some days there is no requirement for Sinemet based on activity.

DAT scan 5/2012 – No Obvious activity seen in Putamen of both hemispheres, radioactive tracer activity confined to caudate nuclei. Activity of striatum with respect to background reduced.

This remains Parkinson's diagnosed by committee.

Subsequent blood tests (4/2012) and exams have revealed low Vitamin D content (31.5) and elevated WBC (14.5 then 13.1 two weeks following) RBR negative.

Trial of Ropinerole for 2 days terminated due to intoxicating effects. Azilect prescribed and not taken. Sinemet remains tolerable and effective with the exception of the left foot Diskynesia.

Symptoms have remained unchanged since July 2011.

My symptoms remain consistent, not better or worse. Some days I take no medication at all. I saw yet another Neurologist that strongly believed I have Dopa-Responsive Dystonia which quite frankly fits the bill. My regular neurologist tends to disagree. I did have an expert opinion on the DAT scan and he called it unremarkable. I don't have any evidence of brain damage and lack a great number of common Parkinson's symptoms.

I remain hopeful and any of you should as well. I just remain diligent and persistent driving the Doctors crazy. If they had the information and the answers I wouldn't be on their cases. This is what I hope to achieve with the database. We all deserve to be well and feel good everyday. It's none of our fault, but there must be a common thread, a cause, something identifiable amongst all of us that can point a finger in the right direction and lead us to the cure. There is an Answer, we are just left to find it.

TABLE 1

Table 1: Symptoms and Treatment BEFORE Diagnostic Testing by Specialists

Date

Symptom

M.D.

Diagnosis

Rx and Treatment

Dosage

15 Aug 2010

Lower back discomfort

GP

Lower Back Strain

Limit lifting activities

Rest

N/A

15 Sept 2010

Began dragging left foot slightly, but more during the evening, with some noticeable lower back pain mainly on the sides.

 

GP

Lower Back Strain

Limit lifting activities

Rx: Flexeril

Limit lifting activities

 

10 mg

24 Nov 2010

Thanksgiving – New symptom; Difficulty stirring gravy, some numbness and lack of dexterity in left hand along with poor coordination in left arm; Symptoms progressively worse over next 2 months.

 

 

 

 

 

31 Jan 2011

Symptoms constant; use of left hand impaired.

 

 

 

 

 

31 Jan 2011

Constant limp; dragging of the left foot, worsening with activity; symptoms absent upon waking.

 

 

 

Rx: Vicodin

3/325mg

Feb 2011

Issues with weakness

GP

 

Rx: Percocet

250 mg

24 Mar 2011

Constant dragging of left foot, severe leg cramps in right leg, extreme difficulty in ambulation; stiffness in neck; dizziness; feeling of passing out when turning head to right; Unable to stand after work without resting for  2 hours.

 

 

 

Rx: Percoset

Rx: Prednisone

12 day sliding dose

(40/3 days, 30/3days)

Two weeks off work

325 mg

40 mg

30 mg

31 Mar 2011

Symptoms Continuing

GP

 

Rx:  Percoset

RX: Pednisone

12 day sliding dose

(40/3 days, 30/3days)

325 mg

40 mg

30 mg

 

 

TABLE 2

Table 2: Symptoms and Treatment AFTER Diagnostic Testing by Specialist

Date

Symptom

M.D.

Diagnosis

Test Administered

Rx

Test Result

31 Mar 2011

Constant dragging of left foot, severe leg cramps in right leg, extreme difficulty in ambulation; stiffness in neck; dizziness; feeling of passing out when turning head to right; Unable to stand after work without resting for  2 hours.

NEUR

Assessment: Inconclusive

 

Lumbar MRI

L5-S1

Schedule full spinal cord screening to evaluate for syrinx.

L5-S1 minimal patchy desiccation. Mild circumferential disc bulge with minimal osteophytosis and discogenic marrow signal change. No focal protrusion or spinal stenosis. Mild facet and ligamentum flavum degeneration. Mild right foraminal stenosis and borderline stennosis at the left foraminal entry zone. Prominence of the central canal of the distal spinal cord.

 

6 Apr 2011

Symptoms Continuing

NEUR

Assessment: Inconclusive

 

Epidural Steroid Injection

 

Rx: Percoset

 

325 mg

 

14 Apr 2011

Symptoms Continuing

ORTHO SURG

Assessment: Left upper extremity and bilateral lower extremity weakness and loss of coordination. Ataxic Gait, Left upper extremity and bilateral lower extremity hyperreflexia. Low back pain.

Tandem Walk; Romberg Test; Hoffman Test

Rx: Percoset

Schedule

Brain MRI Without Contrast, Cervical and Thoracic MRI.

 

325 mg

 

15 Apr 2011

Symptoms Continuing

ORTHO SURG

 

Assessment: 

 

 

MRI

 

 

18 Apr 2011

Symptoms Continuing

ORTHO SURG

Assessment: Pineal Cyst

Office Consult

Schedule EEG, EMG, VNG, TCD, Lumbar Puncture

 

 

2 May 2011 thru

31 May 2011

Symptoms Continuing

 

Assessment:

Ulnar Neuropathy

EEG, EMG, VNG, TCD, Lumbar Puncture.

Blood Tests:

VDRL requested not performed.

 

 

Results Unremarkable

21 June 2011

Symptoms Continuing

NEURO

Assessment:

Office Consult

 

Schedule SSEP

 

Continuing

22 July 2011

Symptoms Continuing

BRAIN SURG

Assessment:

Pineal Cyst Normal

Office Consult

 

Results Unremarkable

 

 

27 July 2011

Symptoms Continuing

NEURO

Assessment:

Possible MSA

Office Consult

 

Pineal Cyst Ruled Out

MSA suspected after exam

 

28 July 2011

Symptoms Continuing

 

 

 

RX:  Sinemet

100 mg/3 x day

 

29 July 2011

Symptoms Continuing

 

 

 

 

 

 

30 July 2011

Symptoms Continuing

 

 

 

 

 

 

2 Aug 2011

Symptoms Continuing

 

 

 

 

 

 

6 Aug 2011 thru

9 Aug 2011

 

Symptoms Continuing

 

 

 

 

 

6 Sept 2011

Symptoms Continuing

NEUR

Assessment:

Office Consult

 

Evaluate Neck and Arm problems

 

22 Sept 2011

Symptoms Continuing

NEUR

Assessment:

Parkinson’s

Office Consult

 

Meet with Movement Order Specialist to evaluate movement disorder; Referred to UW Neurology Movement Disorders

 

18 Oct 2011

Symptoms Continuing

NEUR

Assessment:

Office Consult

 

Possibly Parkinsonism

 

Nov 2011 thru

Mar 2012

Symptoms Continuing

 

 

 

Rx:  Sinemet

100 mg

3 Cervical Steroid Injections; Severe work restrictions

 

May 2012

Symptoms Continuing

NEUR

Assessment:

Unremarkable

DAT SCAN

Blood Tests

 

No obvious activity

Low Vitamin D

Elevated WBC

Trial of Ropinerole terminated due to side effects.

 

Current

Symptoms Continuing