STIFF PERSON SYNDROME STUDY
RESULTS OF 33 PATIENTS copyright 2012




TERMS OF USE FOR THIS STUDY

This study is copyrighted and may not be used for personal or financial gain. It may not be published in any format without the author's permission. This study may not be published on any wesite, facebook account, my space account, or any internet form without the written authorization of the author.

However the author does hereby give permission for a patient to copy the study and give it to their physician in hope that it may help in obtaining a proper diagnosis for the patient. I also give permission for a physician to copy this study and use it for a reference work only and the physician can not reproduce the study for any personal or financial gain.



John C Crawford www.stiffpersonsyndrome.net

Stiff Person ( Stiffman ) Syndrome Study by John C Crawford
Copyright 2006-2012


Page 1


Acknowledgements

I want to thank the 33 respondents of this study. They have taught me so much. Without their cooperation this study would not have been possible. I would also like to recognize those who read the questionnaire but could not respond because the illness has incapacitated them to an extent they could not respond. Some were bedridden, others can no longer sit and type at the computer, and some cannot function well due to their level of medications. My prayers go out to all who are trying to hang in there until a suitable treatment or possible cure is found.


As for me personally, I want to acknowledge the grace and goodness of God, who daily comforts me, lights my path, and strengthens me. I feel without that inner strength He has provided, I would not have learned to enjoy what I do have in life, in spite of this illness. A relevant quote, “I asked God to give me all things so I can enjoy life; He replied, ‘I gave you life that you might enjoy all things’”

I must acknowledge the deep appreciation I have for my Neurologist, Dr Victor Maquera, and his staff for his/their in depth knowledge of Stiff Person (Stiffman) Syndrome, treatment regime, constant encouragement, and a much needed sense of humor. I thank them for their encouragement to do this study and their review of the finished work. Megan you are the greatest!


Page 2

Stiff Person ( Stiffman ) Syndrome Study by John C Crawford
Copyright 2006-2012

Date Completed Sept 1, 2006

Purpose: To verify or dispel the numerous claims made concerning this disease.
To provide health care professionals, information that will help them understand, diagnosis, and treat SPS more quickly than is currently being done.


Introduction

A search of available Internet Web Sites will provide a vast difference in the cause, how to diagnosis, what age group is affected, what the symptoms are, what gender is more affected, as well as the most confusing array of treatment methods currently available. Many are from personal opinions, single case studies, personal experiences, very old information, and just plain misleading information that can cause more harm than good.

I will not attempt to give definitions of the illness, symptoms of the illness, age groups and genders affected, or any other information currently reported by others. Only the actual information obtained from the respondents to this study will be used. For the benefit of those who are unfamiliar with Stiff Person (or Stiffman) Syndrome, I would refer you to other links on this web site.

The topics covered were not meant to portray a particular point of view, but to provide the most current information from people with the illness. Who they are, how long from symptom to diagnosis, to treatment, and what forms of treatment were started and what treatments are currently working for them.

There were 33 respondents to the questionnaire that was sent out to 70 or more people with the illness. All 33 respondents answered all questions. In some cases there are more than one answer given by some respondents
Since the questionnaire was not filled out in my presence, there were some answers that were not clearly definable. In these cases I took some liberty to interpret the answer as best I could. The number of questions like this was limited and had no major effect on the outcome of any given category.

No single topic was studied in depth. It is my sincere hope this study will generate enough interest for one or more of the topics to be studied in depth.

It is also my sincere desire this study may be used as a tool to help health care professionals to better understand the illness, diagnosis this illness sooner, and for many of their current thoughts about SPS to be verified or dispelled.

It is in no way to be interpreted, as, or used in place of, proper medical care and treatment. Nothing should replace prompt and proper treatment by a medical professional.

                                        The Study

The questions may not be in the same order in which they were asked on the questionnaire. This was done to more clearly see the flow of information on each topic.

Page 3

Question 1: Gender
Answer: Males: 10 (30%)
Females: 23 (70%)


Question 2: Current Age
Answer: Females: Average Age: 52.7 Males: Average Age: 48.9
Youngest: 35 Youngest: 20
Oldest: 77 Oldest: 65
Age Group:
20 to 29---2
30 to 39---2
40 to 49---3
50 to 59---3 
60 to 69---2
70 to 79---1



Question 3: Age At Diagnosis:
Answer: Male: Average Age: 49.2 Female: Average Age: 42.9
Youngest: 33 Youngest: 8
Oldest: 69 Oldest: 58
Age Group:

      0 to 9---1
    10 to 19---1
    20 to 29---1
    30 to 39---6
    30 to 49---8
    40 to 49---8
    50 to 59   5
    60 to 69---1




Question 4: Age At First Symptoms:
Answer: Female: Average Age 40.6 Male: Average Age: 40.0
Youngest 23 Youngest 8
Oldest 53 Oldest 58 
Age Group: 0 to 9---1
                   10 to 19---1
                   20 to 29---1
                   30 to 39---4
                   40 to 49 ---2
                   50 to 59---6


Page 4

Questions 6 and 7 are answered in question 5.


Question 8: How many doctors did you see before receiving an accurate diagnosis?
Answer: Males: Averaged 4.6 doctors
Females: Averaged 4.9 doctors

Question 9: Do you have any other autoimmune diseases?

Answer: Yes---21 (64%) No---12 (36%)

Question 10: How many different types of autoimmune diseases, other than SPS, do you have?

Answer: 36% had none 12 patients had 0
64% had 1 or more 12 patients had 1
5 patients had 2
3 patients had 3
1 patients had 5

Diabetes accounted for 41%
Thyroid problems accounted for 18%
Fribromyalga accounted for 6%
Chronic Inflammatory Demyelinating Poly Neuropathy ( CIDP ) accounted for 6%
8 other autoimmune diseases accounted for the remaining 29%.

Question 11: Was your SPS diagnosis 1st, 2nd, or the 3rd or later:
Answer: 38% said 1st
43% said 2nd
19% said 3rd or later.

Question 12: Is there any history of autoimmune diseases in your family:
Answer: Yes---24 (73%) No---9 (27%)

Question 13: If yes, how many other family members have one or more autoimmune diseases?
Answer: Number of patients reporting :   4 6 5 5 1 1 1 0 0 1
Total Number of diseases in your family: 1 2 3 4 5 6 7 8 9 10


Family member: Mother Father Sister Brother Son Daughter Grandfather Grandmother
***
7 (1) 11 (1) 10 (1) 8 (1) 5 (1) 4 (1) 4 (1) 4 (1) 17

Number in ( ) Is the number Of diseases

*** The number of Aunts, Uncles, Cousins. Not immediate family members, which is what I wanted.


Question 14 & 15 are incorporated in question 13


Question 16: Does the doctor treating you have other SPS patients?
Answer: Yes---23 (70%) No---10 (30%)

Question 17: Was he/she aware of SPS before seeing you?
Answer: Yes---28 (85%) No---5 (15%)

Page 8

Question 48: How well did you respond to this treatment method?
Answer: Diazepam: Good---13 Bad---3              Prednisone: Good---0 Bad---1
Baclofen: Good---7 Bad---1                                Redomex (?): Good---1 Bad---0
IVIg: Good---6 Bad---0                                         Sinement (?): Good---0 Bad---1
Clonazepam: Good---2 Bad---0                          Klonopin: Good---1 Bad---0
Gabapentin: Good---0 Bad---2                            Zanaflex: Good---1 Bad---0

Question 49: Was this method of treatment successful in slowing the progress of the illness?
Answer: Diazepam: Y---10 N---6                       Prednisone: Y---0 N---1
Baclofen: Y---3 N---5                                         Redomex (?): Y---1 N---0
IVIg: Y---6 N---0                                                  Sinement (?): Y---0 N---1
Clonazepam: Y---2 N---0                                    Klonopin: Y---1 N---0
Gabapentin: Y---0 N ---2                                   Zanaflex: Y---1 N---0


Question 50: Did this treatment method reverse the progress of the illness?
Answer: Diazepam: Y---8 N---8                        Prednisone: Y---0 N---1
Baclofen: Y---3 N---5                                         Redomex (?) Y---0 N---1
IVIg: Y---6 N---0                                                  Sinement (?) Y---0 N---1
Clonazepam: Y---1 N---1                                   Klonopin: Y---0 N---1
Gabapentin: Y---0 N---2                                    Zanaflex: Y---1 N---0


Question 51: Was a second method of treatment recommended?
Answer: Yes---23 (70%) No---10 (30%)

Question52: What was the second method of treatment?
Some responded with more than one answer.
Answer: IVIg---8,                     Clonazepam---1
Baclofen---8                            Diazepam---1
Neurontin---3                          Depakote---1
Lorozepam---2                         Imuran---1
Plasmapheresis---1                Celexia---1
Gabapentin---1

Question 53: Did you continue the first recommended treatment method while doing the second?
Answer: Yes---14 No---11 Did not apply to them---10

Question 54: How well did you respond after trying the second treatment method?
Answer: IVIg: Good---3 Bad---5              Depakote: Good---0 Bad---1
Baclofen: Good---4 Bad---4                     Imuran: Good---0 Bad---1
Neurontin: Good---2 Bad---1                   Celexa: Good---0 Bad---1
Lorozepam: Good---2 Bad---0                 Clonazepam: Good---0 Bad---1


Question 55: Was the second treatment method successful in slowing the progression of the illness?
Answer: Yes---8 (24%) No---15 (45%) Did not apply or could not tell---10 (31%)

Question 56: Was the second treatment method successful in reversing the progress of the illness?
Answer: Yes---5 (15%) No---17 (52%) Did not know or could not tell---11 (33%)

Page 9

Question 57: Did you experience any side effects from any of the treatment methods?
Answer: IVIg: 6-Headaches, nausea, flu symptoms
Baclofen-1 sickness
Other responses: drowsiness from meds10, Headaches-3, Concentration--2 Fatigue-1, Hives-1

Question 58: What is your current treatment method?
Some responded with more than one answer.
Answer: Diazepam---15           Zanaflex---2
Baclofen---11                            Keppra---2
IVIg---8                                      Neurontin---1
Depakote---5                            Imuran---1
Lorozepam---3                          Sinement (?)---1
Clonazepam---2                        Carbatrol---1

Question 59: Do you feel the current treatment method is working?
Answer: Yes---31 (94%) No---2 (6%)

Question 60: Did you experience pain with the illness?
Answer: Yes---27 (82%) No---6 (18%)

Question 61: What type of pain did you experience?
Some responded with more than one answer.
Answer: Muscle---12          Neck---2
Back---8                               Body tingle---1
Leg---7                                 Hips---1
Full Body---3                       Chest---1
Cramps---3

Question 62: Did your doctor prescribe anything for pain?
Answer: Yes---14 (43%) No---11 (33%) Did not respond---8 (24%)

Question 63: If yes, what medication did your doctor prescribe?
Answer: Neurontin---5                                    Norco---1
               Valium---3                                           Nemorous---1
               Topamax---2                                       Naproxen---1
                Vicodon---2                                        Tramadol---1
               Klonoplin---2                                      Oxycontin---1
               Oxycodine---                                      1 Percocet---1
               Gabapetin---1

Question 64: Did the medication relieve the pain?
Answer: Yes---12 (55%) No---3 (14%)
Partly---2 (9%) Did not notice any difference---5 (23%)

Question 66: Did you experience any anxiety with your SPS?
Answer: Yes---25 (76%) No---5, (15%) Not sure---3 (9%)

Question 67: Were you every treated for anxiety prior to your diagnosis of SPS?
Answer: Yes---8 (24%) No---25 (76%)



Page 10

Question 68: When did you first notice the anxiety?
Answer: Before start of symptoms---4
Start of symptoms---14
6 mo. to 1 yr from start of symptoms---7
1 yr to 2 yr from start of symptoms---1
Never or not sure---8

Question 69: Did the anxiety increase over time?
Answer: Yes---24 (73%) No---4 (12%) Do not know---5 (15%)

Question 70: What things cause your anxieties?
Some responded with more than one answer.
Answer: Stairs---13                    Crowds---3
Open areas---13                         Lack of body support---1
Crossing street---11                  Bathtub---1
Fear of driving---8
Fear of falls---6
People to close---3

Question 71: Has your doctor prescribed anything for your anxiety?
Answer: Yes---15 (45%) No---18 (55%)

Question 72: If yes, what medication was prescribed?
Answer: Diazepam---4                 Anti-depressant---1
Zanax---2                                       Zoloft---1
Celexa---2                                     Anti-anxiety seritonin---1
Elavil---1                                       Lorazepam---1
Remuron---1                                 Lexapro---1


Question 73: Do you feel it has reduced your anxiety?
Answer: Yes---14 (42%) No---4 (12%) Not sure---15 (46%)

Question 74: If your symptoms of SPS improved, did your anxiety level reduce?
Answer: Yes---18 (55%) No---3 (9%) Not sure---12 (36%)

Question 75: Have you tried any form of anxiety therapy besides medication?
Answer: Yes---20 (61%) No---13 (39%)

Question 76: If yes, please describe.
Some responded with more than one answer.
Answer: Meditation---7                  Prayer---2
Deep breathing---6                        Positive thinking---1
Self hypnosis---6                            Massage therapy---1
Yoga---6 Laughing---1
Bio feedback---3

Question 77: Do you feel it is helping you?
Answer: Yes---15 (45%)       No---4 (12%)      Not sure---14 (43%)



Page 11

Question 78: Have you experience any depression with your SPS?
Answer: Yes---17 (45%) No---18 (55%) Question 79: Has your doctor prescribed any anti- depressant medication?
Answer: Yes---11 (33%)                   No---22 (67%)

Question 80: What medication or medications were prescribed?
Some responded with more than one answer.Answer: Effexra---4 Cymbalta---1 Remoron---3 Pexeva---1 Tofamil---3 Paxil---1 Aurorix---1 Lexapro---1 Wellbutrin---1

Question 81: Of those answering yes: Do you feel it is helping you?
Answer: Yes---4 (36%),        No---7 (64%)












Question 5: What country do you reside:
Answer: United States---22
Canada---4
Europe---4
Australia---3


No geographical information was asked to ascertain the possibility of “clusters”
of SPS patients.




Page 5

Question 18: Before you received a diagnosis of SPS, did any doctor suggest a psychological examination?
Answer: Male: Yes---2 (20%)            No--- 8 (80%)
Female: Yes---11 (48%)                    No---12 (52%
)

Question 19: Did any doctor suggest your problems might be caused by anxiety?
Answer: Male: Yes---2 (20%)              No---8 (80%)
Female: Yes---11 (48%)                      No---12 (52%)

Question 20: Did you experience falls before your diagnosis of SPS, but after the start of symptoms?
Answer: Yes---23 (70%)                     No---10 (30%)

Question 21: Did the falls increase until you started to receive medical treatment for SPS?
Answer: Yes---23 (70%)                     No---10 (30%)

Question 22: Did the falls decrease in frequency after you started medical treatment for SPS?
Answer: Yes---20 (61%)                      No---13 (39%)

Question 23: What symptoms did you first notice before you sought medical help?
Answer: Back & leg pain---7       Walking problems---2        Reduced flexibility---1  Stiff legs---7                   
Full body stiffness---5      
Falling---4      Abdomen problems---2     Facial paralysis---1
 Eyesight---Weak hands---2           Arm stiffness---1

Question 24: What parts of your body were first affected?
Some responded with more than one answer.
Answer: Back---10       Abdomen---2
Legs---17 Arms---2
Full body---5 Feet---2
Hips---2 Hands---1
Face---1 Neck---1


Question 25: What parts of your body are currently affected?
Some responded with more than one answer

Answer: Legs---17      
Back---10   Total body---9     Hips---6   Arms---6         Neck---5            Abdomen---2
 Feet---2      Hands---1


Question 26: Do you experience spasm type episodes at the present time?
Answer: Yes---31 (94%)          No---2 (6%)

Question 26A: How long where your episodes lasting when you were first diagnosed with SPS?
Answer:
1 min to 30 min—8     30 min to 1 hr---6      1 hr to 2 hr---3      2 hr or more---1    Varied---15

Page 6

Question 27: How long do your episodes last now?

Answer:
1 min to 30 min---15
30 min to 1 hr---8
1 hr or more ---4
Varied---
6

Question 28: Has any of your episodes caused joint dislocation or broken bones (not including from falls)?
Answer: Yes---5 (15%)                    No---27 (85%)

Question 29: If yes, explain.
Answer:
Shoulder dislocation---3,
Knee damage---1
Hip dislocation---1


Question 30: Is this still happening
Answer: Yes---3 (9%)         No---30 (91%)

Question 31: If no, please explain, i.e.; medication, new form of treatment, etc
Answer: Medication: 3

Question 32: Do you have a startle effect that sets off a spasm episode?
Answer: Yes---28 (85%)        No---5 (15%)

Question 33: Do you know what triggers the startle effect?
Answer: Yes---28 (85%)       No---5 (15%)

Question 33A: List the triggers.
Some responded with more than one answer.
Answer: Sound---16
Touch---9
Crowds---4
Excitement---3
Anxiety---2
Fear of falls---2


Question 34: Do you have more than one trigger?
Answer: Yes---25 (76%)      No---8 (24%)

Question 35: If yes, what is the dominant trigger?
Answer: Sound---12
Touch---7
Excitement---3
Anxiety---2
Crowds---2
Shower---1


Page 7

Question 36: Do you think weather affects you illness? )

Answer: Yes---8 (85%) No---5 (15%Question 37:
Please explain, i.e.; barometric pressure change, heat, cold, etc

Some res
ponded with more than one answer.
Answer: Barometric change---15
Cold temp---12
Rain---8
Hot Temp---7
Snow---7

Question 38: Has a nerve conductivity test been administered?
Answer: Yes---21 (64%)    No or don’t remember---12 (36%)

Question 39: Did your doctor already suspect SPS?
Answer: Yes---14 (42%)              No or don’t know 19---(58%)

Question 40: What were the results?
Answer: Poor nerve conduction---9
Poly neuropathy---1
Diabetic neuropathy---1
Negative or don’t know---22

Question 41: Has an EMG test been administered?
Answer: Yes---25 (76%)       No---8 (24%)

Question 42: Did your doctor already suspect SPS?
Answer: Yes---21 (64%)       No---12 (36%)

Question 43: What were the results?
Answer: Nerves firing continuously---18
Inconclusive---3
Not sure or don’t know---12

Question 44: Was an Anti-Gad test administered?
Answer: Yes---30 (91%           ) No---1 (3%)              Don’t remember---2 (7%)

Question 45: What were the results?
Answer: Positive---25 (76%)             Negative---6 (18%              Inconclusive or don’t know---2 (6%)

Question 46: What was your final diagnosis?
Answer:
Stiff Person Syndrome---32          Scleroderma---1

Question 47: What was the first method of treatment your doctor recommended?
Some responded with more than one answer.
Answer: Diazepam---16    
Baclofen---8      IVIg---6    Clonazepam---2    Gabapentin---2      Prednisone---1
Sinement (?)---1    Klonopin---1    Zanaflex---1      Redomex (?)---1