Category Archives: PSP

Hyposmia (poor sense of smell) in PSP

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One thing clear to me from reading research articles over the last few years on PSP is that even though the Queen Square Brain Bank doesn’t have the number of brains that Mayo Jax has, QSBB does loads more research and publishes more based on what they do have.

This (mostly) British research is about hyposmia (poor sense of smell) in PSP.

Researchers gave 36 patients with PSP who had scored more than 18 on the MMSE the University of Pennsylvania Smell Identification Test (UPSIT). 140 patients with PD and 126 controls were also tested.

PSPers had worse sense of smell than controls but better sense of smell than those with PD.

“For patients with PSP, UPSIT scores correlated with MMSE but not disease duration…”

Though people may have PSP pathology in regions of the brain responsible for smell, their sense of smell may not be affected. “The brains of six of the patients with PSP were examined postmortem and all revealed neurofibrillary tangles and tau accumulation in the rhinencephalon, although only three had hyposmia.”

“Further prospective studies including patients with early PSP and PSP-P with postmortem confirmation might help clarify if smell tests could be useful when the differential diagnosis lies between PD and PSP.”

Robin

Research Article
Hyposmia in progressive supranuclear palsy

Movement Disorders, Published Online: 5 Mar 2010

ABSTRACT
Previous studies suggested that olfaction is normal in progressive supranuclear palsy (PSP). We applied the University of Pennsylvania Smell Identification Test (UPSIT) to 36 patients with PSP who scored more than 18 on the Mini Mental State Examination (MMSE), 140 patients with nondemented Parkinson’s disease (PD) and 126 controls. Mean UPSIT scores in PSP were lower than in controls (P < 0.001) but higher than in PD (P < 0.001) after adjusting for age, gender, and smoking history. For patients with PSP, UPSIT scores correlated with MMSE (r = 0.44, P = 0.006) but not disease duration (P = 0.6), motor subscale of the Unified Parkinson’s Disease Rating Scale (P = 0.2), or Frontal Assessment Battery (P = 0.5). The brains of six of the patients with PSP were examined postmortem and all revealed neurofibrillary tangles and tau accumulation in the rhinencephalon, although only three had hyposmia. Further prospective studies including patients with early PSP and PSP-P with postmortem confirmation might help clarify if smell tests could be useful when the differential diagnosis lies between PD and PSP.

Authors:
Laura Silveira-Moriyama, MD 1, Graham Hughes, MD 2, Alistair Church, MD 3, Hilary Ayling, MSc 4, David R. Williams, MD, PhD 1 5, Aviva Petrie, MSc, CStat 6, Janice Holton, MD, PhD 1 4, Tamas Revesz, MD 4, Ann Kingsbury, PhD 1, Huw R. Morris, MD, PhD 7, David J. Burn, MD 2, Andrew J. Lees, MD 1 4 *
1Reta Lila Weston Institute of Neurological Studies, UCL Institute of Neurology, London, United Kingdom
2Institute for Ageing and Health, Newcastle University, Newcastle Upon Tyne, United Kingdom
3Department of Neurology, Royal Gwent Hospital, Newport, Gwent, United Kingdom
4Queen Square Brain Bank, UCL Institute of Neurology, London, United Kingdom
5Department of Neurology, Faculty of Medicine (Neurosciences), Monash University (Alfred Hospital Campus), Melbourne, Australia
6Biostatistics Unit, UCL Eastman Dental Institute, London, United Kingdom
7Department of Neurology, Cardiff University School of Medicine, Cardiff University, Cardiff, United Kingdom

This abstract isn’t available yet on PubMed but is available on the publisher’s website.
http://www3.interscience.wiley.com/jour … 0/abstract

No “familial aggregation” in PSP, one old study

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I attended a PSP research symposium recently and there was quite a bit of discussion about the genetic risk for PSP, whether that risk was inherited, and whether first-degree relatives of those with autopsy-confirmed PSP have subtle neurological changes themselves.

This is a study published this summer on MSA and PSP. In France, from April 2000 to December 2003, 71 people who are first-degree relatives of those with MSA (not pathologically-confirmed MSA), 71 age-matched controls, 79 people who are first-degree relatives of those with PSP (not path-confirmed PSP), and 79 age-matched controls were studied to determine if the “first-degree relatives of patients with MSA or PSP were at increased risk of Parkinsonism or dementia.”

The researchers found:

* “No significant familial aggregation was found in PSP.”

* “MSA cases reported Parkinsonism more often, but not dementia in their first-degree relatives than controls.”

Since the study was conducted so long ago, I’m not sure why it’s just being published now. The diagnostic criteria by which it was determined if the patients had MSA or PSP are somewhat dated.

A major shortcoming of the study is that the MSA and the PSP diagnoses given to first-degree relatives have not been confirmed upon brain autopsy. Also, those who participated in the study were not examined to confirm any reports they may have made of parkinsonism or dementia.

I’ve copied the abstract below.

Robin

Journal of Neurology. 2010 Aug;257(8):1388-93. Epub 2010 Jul 13.

Familial aggregation in atypical Parkinson’s disease: a case control study in multiple system atrophy and progressive supranuclear palsy.

Vidal JS, Vidailhet M, Derkinderen P, Tzourio C, Alpérovitch A.
Intramural Research Program, Laboratory of Epidemiology, Demography and Biometry, National Institute on Aging, National Institutes of Health, Bethesda, MD.

Abstract
Familial aggregation has been consistently found in PD, but it is unclear whether there is a familial aggregation in families of patients with multiple system atrophy (MSA) or progressive supranuclear palsy (PSP). MSA and PSP cases were recruited from a two-arm case control study. One control was matched to each case for age, gender and living area. Medical history of first-degree relatives was obtained through a face-to-face questionnaire. Age-specific cumulative incidence of Parkinsonism and dementia in first-degree relatives of cases and controls was compared for MSA and PSP separately. Seventy-one pairs for MSA and their controls and 79 pairs for PSP and their controls were included. No significant familial aggregation was found in PSP. MSA cases reported Parkinsonism more often, but not dementia in their first-degree relatives than controls. MSA patients, but not those with PSP, have Parkinsonism more often in their first-degree relatives than controls.

PMID: 20625759

Participation in a PSP public service announcement

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Some of you may want to participate in the PSP-related public service announcement.  This note was posted today to the PSP Forum by NY-based Sean Donnelly, whose mother died about a year ago with PSP.

Robin

———————————-

forum.psp.org/viewtopic.php?t=8351

Posted: 25 Feb 2010
Post subject: Participation in a PSP public service announcement.

Hello,

My name is Sean Donnelly. I am a designer in NYC and have been working with the Foundation for PSP to come up with ideas for public service announcements about the diseases. We are currently working on one and are in need of photos of patients. The gist of the spot involves showing “before and after” images to illustrate the path that PSP takes i.e “From vitality … to lethargy”. If anyone on this forum is interested in submitting images feel free to email me or write back here. I promise give any photos the love and respect they deserve, while trying to spread awareness about these horrible diseases. I also vow that the “quality” of the PSAs will be of the highest level.

This is also just the first many pieces of video I will be producing for the foundation. So if you are not able to participate this time there will be others.

Lastly this is a labor of love. My mother Patricia Donnelly passed away in April of 2009, due to PSP.

Thank you for your time.

 

Helpful Hints from Occupational Therapist (2-11-10 Webinar Notes)

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This post is of interest to everyone in our group even though the speaker’s topic refers to PSP and CBD only.

I’m not sure how many participated in last week’s (2-11-10) webinar put on by CurePSP with an occupational therapist.  Though the pace of the presentation was too fast (especially since I was trying to take notes), overall it contained useful information, particularly to newcomers.  The Q&A was helpful for its range of questions from webinar participants.  (A local support group member asked three questions!)  Some helpful resources were provided throughout the webinar and in a list at the end of the presentation.

My extensive notes are below.

Robin

—————————————————————————–

Helpful Hints from Occupational Therapy for PSP/CBD/Related Disorders:  How to Improve ADLs and Safety!
Webinar Host:  CurePSP
Speaker:  Christine Robertson, OTR/L, Occupational Therapist
February 11, 2010

Speaker’s Background:
CurePSP Medical Advisory Committee
Primary OT at the Dan Aaron Parkinson’s Rehabilitation Center in Philadelphia (at Penn Hospital)
LSVT BIG certified clinician
Contact info:  [email protected]
Goals of this webinar:
* describe how an OT can assist
* identify changes that lead to ADL dysfunction and decreased functional mobility
* provide adapted techniques
* suggest equipment

How can an OT help?
* assess functional mobility and ADL performance by talking to patient and family
* home safety assessments
* teach new and safer ways to perform activities
* train care-partners on how to safely assist
* recommend appropriate adaptive equipment for the home

Causes of ADL dysfunction  [ADL = activities of daily living] * rigidity/stiffness of muscles and joints
* gait dysfunction (shuffling, freezing)
* narrow base of support
* slowness of movement/reaction time
* decreased coordination
* visual changes
* OH
* alien limb
* decreased safety awareness

Using a bed rail to help with getting in and out of bed
* bed rail fits between mattress and box-spring
* assists with rolling and moving up and down
* not covered by insurance plans
* order through catalogues
* recommended website where you can purchase a bed rail:  1800wheelchair.com

Getting out of bed:
* bend up knees
* roll completely on side
* grab the bed rail or sturdy chair
* allow legs to drop off the side of the bed…
* while you push yourself up sideways

Bedroom safety tips:
* make sure floor is clutter-free (shoes, electrical cords, etc)
* have easy reach to light switches or bedside table lamp or night lights
* consider bedside commode or urinal to decrease trips to bathroom if this is a problem
* bed rails to assist with getting in/out of bed
* satin sheets or pillowcase under bottom to improve bed mobility or transfers
* cordless phone or cell phone at bedside in case of emergency
* in cooler months, use heavier PJs and lighter or fewer blankets

Bathroom safety tips:
* remove throw rugs that slide.  (Only use rugs with nonskid surfaces on the back.)
* add grab bars where needed
* remove glass partitions in the shower/tub to make transfer in/out easier and safer
* add nonskid surface to floor of shower/tub (eg, rubber mat or nonskid strips/decals – her preference)
* use a shower bench/tub chair with a back to decrease risk of slipping, promote good posture and to conserve energy
* always sit as much as possible when bathing (eg, on shower bench or chair)
* install a hand-held shower head to eliminate all turns in the shower
* use a long-handled sponge to assist with safely bathing hard to reach areas
* use liquid soap or soap on a rope
* keep towels in close reach to dry as much as possible in a seated position or use a terry cloth robe to assist with absorbing moisture after bathing
* avoid extremely hot showers because this can cause dizziness/lightheadedness

Getting in and out of the bathtub safely:
* use tub bench or shower chair as this eliminates risk of losing balance, promotes good posture and conserves energy
* not covered by insurance plans
* order through catalogues
* recommended website where you can purchase a tub bench or shower chair:  1800wheelchair.com
* later comment by Janet Edmunson:  one of her support group members recommends a tub transfer chair from Dr. Leonard’s (phone 800/785-0880, drleonards.com)

Safety grab bars:
* purchase through medical supply companies, Home Depot, Lowe’s, etc.
* lots of grab bars don’t have a medical/rehab-look
* she’s not comfortable recommending suction grab bars

Getting up from the toilet:
* three-in-one commode can be used at bedside, over the toilet, or in the shower stall
* place just the frame over the toilet to raise seat height and have hand rails
* adjustable height makes sitting and standing easier and safer
* covered by Medicare and most insurance plans with MD’s prescription

Grooming tips to help with oral hygiene:
* tube squeezer.  Available from Sammons Preston (sammonspreston.com).  Easily squeezes tubes flat from the bottom up, making one-handed use possible.  Works with toothpaste, lotions, salves, silicones, and other household products.
* Touch N Brush Toothpaste Dispenser.  Available from Dr. Leonard’s (drleonards.com)
* Water Pick’s Oral irrigator:  helps fight gingivitis
* Floss picks:  pre-threaded; can be purchased many places (even grocery stores)
* consider switching to an electric tooth brush.  Crest toothbrush can cost as little as $10 at Walmart.

Grooming tips to help with shaving:
* consider switching to an electric razor.  This provides the patient with continued functional independence

Dressing safety tips:
* always dress as much as possible in a seated position to eliminate risk of falling/loss of balance.   It’s best to sit in a high firm back chair with armrests.  Stand only when necessary!
* always dress your affected/most difficult side first
* use a long-handled reacher to assist with lower body dressing (eg, putting pants on)
* sometimes use of a sock aid can help with putting socks on independently depending on the patient
* use a long-handled shoe horn to decrease effort of putting shoes on
* use elastic shoe laces.  Make shoes ready to be put on and removed without trying again.

Equipment to assist with fine motor dressing difficulties:
* use a button hook to assist with fastening buttons on shorts and blouses
* use Velcro button aids to replace regular buttons
* both items available at sammonspreston.com

Tips for walking safety:
* think conscious movement!
* slow down and concentrate!
* do one thing as a time (eg, never reach and walk)
* keep your hands free
* never carry something
* to avoid shuffling, walk with the heel hitting down first, not the toes
* be aware of changes in the floor surface by scanning the environment
* avoid stepping back; instead, move sideways or turn
* do not pivot to turn; instead, try a U-turn or military turn
* always go in the same direction as your walker

Tips for preventing falls at home:
* scan your environment
* watch out for pets, papers, cords, etc
* avoid throw rugs without rubber backing
* remove throw rugs on carpet
* rearrange furniture to allow for more space
* keep stairwells and doorways clutter-free and well-lit
* install handrails in trouble spots

Functional mobility safety tips:
* do not over reach!  Get as close as possible to the object you are reaching for (eg, in cabinets, refrigerator, closet)
* always stand to the side of the refrigerator, dishwasher or stove when opening
* always lean on something stable (eg, the countertop) with one hand!
* place frequently used items in easy-to-reach places (between shoulder and waist height)
* consider using a microwave instead of the oven or stove
* be aware of water spills to decrease the risk of falling
* utilize counter space to slide dishes, pots and pans instead of lifting and carrying them
* if you are using a walker, use a walker bag/basket for safe item retrieval and transport
* arrange furniture in the home to facilitate safer mobility and to decrease obstacles

Movement technique to help reduce retropulsion (getting pulled backwards or falling backwards):
* stand to side of oven, dishwasher, fridge, and doors when opening them
* user “power stance” while performing activity
* steady self with one hand

Getting up from a chair:
* scoot to the edge of the chair
* separate feet to shoulder width
* position feet slightly behind the knees
* learn forward:  “nose over toes”
* push up from armrests
* firm chair is better than cushy couch

Common eating deficits due to decrease in vertical eye gaze:
* difficulty seeing food on the plate and difficulty guiding utensils to the mouth
* care-partner needs to encourage:  compensate for downward gaze impairments by cuing the person to move their head up and down during meals and by raising the height of the plate
* put elbows on the table.  Anchor elbow on the table.  Use arm as lever.
* bring food closer to the patient by increasing the height of their plate/bowl while supporting their elbow on the table
* build up the table surface with a bed tray, books, or a box.  Decrease spills and frustration.

Compensatory techniques for eating due to tremor:
* use a nonskid mat or Dycem (nonslip plastic mats) to prevent plates from sliding
* Asian soup spoon (with a deep bowl) or any deep spoon

Assistive devices for eating:
* plate guard aids with scooping and keeping food on the plate
* adapted utensils with built-up handles, rocker knife, pizza cutter, and weighted
* non-spillable cups/thermos to reduce risk of spills and burns
* clothing protectors.  Another option:  cloth napkin attached around the neck by alligator clips and a chain (often used in dentist offices).  Get different colors of cloth napkins to match shirts.

Handwriting compensation tips:
* consider the Pen Again for micrographia.  Available from penagain.com
* lined paper to provide visual guidelines or cues
* large grip pen.  Example:  Bic XXL
* experiment with different types of pens.  Examples:  Pen Again, felt tip, flair or fine point
* avoid cursive writing, if this is difficult; instead, print and or use block letters
* take time to stretch the hand, given hand rigidity
* write slowly, take your time, concentrate and visualize what you want to write
* support arm on table (elbow to hands)
* think big strokes!
* conscious movement is the key to success!
* use a computer, if able.  It’s a good form of exercise.  Be sure you have an ergonomic setup.

Reading:
* consciously move your head up and down when reading to compensate for lack of eye movement.  The care partner can remind the person to move his/her head up and down.
* book holders.  Available from Staples, Office Depot, sammonspreston.com
* audio books

Computer-accessibility options, with Windows 7 (as an example):
* make font larger, magnifier option, change contrast
* keyboard shortcuts or filter keys
* narrator or screen reader option
* voice recognition option.  (For older operating systems, consider Dragon Dictate software)
* search in “Help” for accessibility options

Telephone options:
* if having trouble dialing the telephone, consider a large button phone (available from Radio Shack) or a cell phone (using the voice dialer option)

Why exercise?
* has positive effects on sense of well being
* helps muscles to stay strong and joints to stay flexible, which can help improve function in ADLs, functional mobility, and balance
* examples:  stretching/flexibility; relaxation (eg, yoga, massage, tai chi, meditation); conditioning/aerobic (stationary/recumbent bike, swimming, dancing, chair aerobics, walking)
* speak to your MD to be sure you have clearance to exercise
* see a PT or OT who can give you an individualized program
* all exercise should be focused on extension, which is stretching and elongating.  Stay away from gripping or flexion exercise!

Hand exercise:
* extension exercise/stretching can sometimes help with decreasing rigidity and cramping

Conclusion: 
Remember the importance of activity modification and being open to change.  This can help to improve your function, safety and independence.

Resources:

AOTA – American Occupational Therapy Association
301-652-2682 or www.aota.org
[Robin’s note:  AOTA does NOT have a directory of OTs]

APTA – American Physical Therapy Association
800-999-2782 or www.apta.com

LSVT® BIG
888-438-5788 or www.lsvtglobal.com

Certified Aging-In-Place Specialist (CAPS)
Certified specialist through The National Association of Home Builders
800-368-5242 or www.nahb.org (CAPS Directory)

Pennsylvania’s Initiative on Assistive Technology (PIAT)
http://disabilities.temple.edu/programs/assistive/piat/

RESNA ­ Rehabilitation Engineering & Assistive Technology Society of North America
www.resna.org

National Assistive Technology Technical Assistance Partnership (NATTAP)
Phone:  703/524-6686    Fax: 703/524-6630

[Robin’s note:  here’s a link to the NATTAP state contact list — http://www.resnaprojects.org/nattap/at/statecontacts.html
In California, the program is called CATS, California Assistive Technology Systems.  See www.atnet.org]

Contact Local Outpatient Centers
Rehab Centers associated with Hospitals (particularly non-sports oriented centers)
Ask if they have therapists who have worked with PD, PSP, MSA, CBGD or those who deal with neurological and balance problems

National Parkinson’s Foundation
800-327-4545 or www.parkinson.org

We Move
www.wemove.org
Find a Doctor

Questions & Answers:  All answers were given by Christine Robertson, unless indicated.  I’ve re-organized them along topic lines.

GETTING THERAPY

Q:  If someone’s MD has never recommended PT or OT, should the patient or family ask for a referral?

A:  Yes, the patient or family should ask for a referral.  Many MDs are not aware of what OTs do.  Even in the hospital where I work, some general practitioners are not aware of what OTs can do.

Ask for “prescription for OT evaluation and treatment.”

Q:  What about LSVT?

A:  Go to the LSVT website — lsvtglobal.com — to find a clinician near you.

Q:  Does Medicare or insurance cover LSVT BIG?

A:  I believe so.  Talk to the office manager associated with an LSVT BIG clinician to find out about insurance.  Find an LSVT BIG clinician at lsvtglobal.com.

Comment by Janet Edmunson, CurePSP Board Chair:  LSVT LOUD is for speech.  LSVT BIG treats movement, not speech.  LSVT BIG applies the sample principles of LOUD to improving motor skills.

Q:  Can a program comparable to LSVT LOUD be done at home?  We live in a rural area and can’t find an LSVT LOUD practitioner.

A:  LSVT LOUD includes homework and four appointments per week.

Maybe make one LSVT LOUD consult appointment and learn what can be done at home.  Many therapists do one-time appointments, teaching the patient and family as much as possible.  Follow up with a less-experienced therapist (OT, PT) in your area after the one-time appointment.

Comment by Laura Purcell Verdun, SLP:  Check out LSVTglobal.com for a DVD you can purchase.

Comment by Janet Edmunson:  Would training at home via web-cam be an option?

SAFETY

Q:  Biggest obstacle is going to the bathroom.  We are using a catheter but is there something else you can suggest?

A:  More conservative (and non-invasive) is better.  How about just a urinal?  How about a bedside commode?  This requires some bed mobility and the ability to transfer.

Comment from a webinar participant:  recommend the SuperPole
[See:  www.healthcraftproducts.com/superbar.htm ]

EXERCISE AND WALKING

Q:  Any suggestions on exercises to help patients move?

A:  Without seeing the patient, it’s hard to suggest something.  It’s best to see a patient when prescribing exercising.  It’s best to have a PT or OT consult with a therapist who knows neurological disorders.  We don’t have one set of suggestions we hand to everyone.  Programs are designed around a person’s abilities.

It’s nice to find an exercise group with all neurological patients.  An instructor can give adaptations.  Socializing is great!

Q:  What is range of motion exercising?

A:  Some of the LSVT BIG exercises are ROM exercises.  Big movements.  Stretching.  Even seated exercises can be helpful.  A pulley system can be used.  Yoga is helpful.  Using the full range of a joint.

She refers the Wii system to lots of people, including the Wii Fit Plus.  Have to be careful with the balance programs!  The recommendations must be patient-specific.

Q:  What about virtual reality, Wii, and Interactive Metronome?

A:  We don’t use the metronome at our clinic.  Some studies say it works by helping the patient keep a beat when he/she is walking, for example.  Some people say it works well for them.

Rather than buying a metronome, have someone clap or smacking a table while the patient is walking.  This will help determine if the metronome might work for you.

Q:  Do you have experience with a body-weight support treadmill?

A:  Our center doesn’t have one so I can’t make any comments on how effective it is.  The person is in a harness and on a treadmill.

Comment by Lesley Smith, PT ([email protected]), a webinar participant:  Has experience with the body-weight support treadmill.  Email her with questions.

Q:  Any tips for battling the stutter step?

A:  This might refer to festination.  Stutter steps are small, tiny, quick steps.  These are often on the toes.  To combat this:  take your time, start yourself with feet spread, heel first.

EQUIPMENT

Q:  Any recommendations for neck support systems?

A:  This is more of a PT question.  There is something called a “head nester” that Heather Cianci sometimes recommends.  This works for some, not all.

Q:  Why doesn’t insurance cover bathroom equipment?

A:  I don’t know.  You would think that with insurance covering these items, they would save money in the long-run because people would fall less.

Comment by Janet Edmunson:  Many insurance policies do have durable medical equipment (DME) coverage.  The shower chair for her late husband Charles was covered.  There’s a limit.  Ask!

POSITIONING

Q:  My husband complains nightly about his left arm bothering him.

A:  Look at bed-positioning.  Is the person sleeping always on that arm?  Can you rest the arm on top of a pillow?  Is this the side most-affected by the disease process?  Consult with an MD to determine if this is an orthopedic problem, nerve problem, etc.

Comment by Janet Edmunson:  A primary care physician diagnosed her late husband Charles as having a frozen shoulder.  The MD recommended range-of-motion exercise and stretching.

Q:  How can we keep a patient upright in a wheelchair?

A:  Add a wedge in the seat.  Use the appropriate seat cushion.  Put a strap across the chest.  Seated exercise:  bring arms behind body; bring shoulders back; bring head back.  If patient can do this on his/her own, the care partner can assist with this exercise.  Work on re-alignment of the body as the disease process may be flexing the body forward.

OTHER

Q:  What affect does a paraffin bath have on a hand that is frozen or contracted?

A:  Hand therapists use this for moist heat for 15-20 minutes before passing stretching the joints.

It depends on how contracted the hand is.  And just dipping the hand in bath feels good but won’t have much impact.

Q:  Are there any supplements to help with mobility?

A:  Ask your neurologist.  In particular, ask if there are any drug trials involving supplements.

Q:  What is FMC?

A:  Fine motor coordination.

Q:  Would hospice early on in the progression of the disease be of value?

A:  I don’t believe so.  As soon as you receive a diagnosis, it’s better to go to rehab.  Therapists can teach the patients early on as to how to do things.

To get hospice, you have to be pretty progressed.

Clinical aspects in frequent fallers with PSP

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This is fascinating German research comparing frequent fallers and infrequent fallers with a clinical diagnosis of PSP. The authors state:

“It is concluded that the occurrence of falls in PSP seems strongly associated with the deterioration of bulbar function, but not relevantly with typical parkinsonian features like rigidity and bradykinesia. The decreased ability to deal with distraction of attention under a dual-task situation points to a relevant impact of cortical and subcortical dysfunction on the frequency of falls.”

“Frequent fallers also showed an increased probability of an altered walking pattern with shortened step lengths and increased cadence under a dual-task situation.”

Frequent falling was not associated with disease duration.

Of the 26 cases studied, “13 patients suffered from Richardson’s syndrome (11 of them were frequent fallers, 2 were infrequent fallers), and 9 suffered from PSP-Parkinsonism (4 were frequent fallers, and 5 were infrequent fallers).” (One of the clinical characteristics of Richardson’s syndrome is unexplained falls as a primary symptom.) Four patients could not be assigned to a sub-type.

The “PSP Rating Scale” was used as part of the clinical investigation. These researchers described that scale as follows: “This scale comprises 28 items in six categories: daily activities (by history), behavior, bulbar, ocular motor, limb motor and gait/midline, and has been shown to be sensitive to disease diagnosis and progression.” The researchers found the PSP Rating Scale better at assessing subtle cognitive deficits in PSP than the MMSE or the FAB (Frontal Assessment Battery).

You can also find links to the scale itself and to Dr. Golbe’s research article about the scale) here:
http://forum.psp.org/viewtopic.php?t=2468 (see my post from 11/19/09)

I glanced over the full article today. I found these statements about postural instability very clear:

“It is obvious that postural instability cause falls. Postural stability may include sensory organization, a motor adjustment process, and the background tone of the muscles. Cognitive processes such as attention and learning are also important resources required for postural stability. In PSP, the visual system (due to gaze palsy) and motor adjustment processes are known to be defective, and cognitive slowing is regularly and early observed.”

The abstract is copied below. It’s quite good, compared to other abstracts.

Robin

Movement Disorders. 2010 Feb 3. [Epub ahead of print]

Clinical and dual-tasking aspects in frequent and infrequent fallers with progressive supranuclear palsy.

Lindemann U, Nicolai S, Beische D, Becker C, Srulijes K, Dietzel E, Bauer S, Berg D, Maetzler W.
Department of Clinical Gerontology, Robert-Bosch-Hospital, Stuttgart, Germany.

Progressive supranuclear palsy (PSP) is a rare neurodegenerative disease with no sufficient therapeutic options to date. Falls are the most devastating feature. The causes of these falls are not well understood.

To test the impact of PSP-associated motor and cognitive features on falls, 26 PSP patients were prospectively recruited and divided into frequent fallers (> one fall/month, 18 patients) and infrequent fallers (</= one fall/month, 8 patients). Further parameters were assessed by clinical investigation and biomechanical gait and balance analysis with and without dual-task paradigms. Physical activity was measured through an ambulatory device.

Frequent fallers scored higher on the total PSP rating scale and the subscales “history,” “mental,” “bulbar,” “supranuclear ocular motor,” and “gait/midline exam” but not on disease duration, the subscale “limb exam,” the UPDRS motor score and the sway analysis.

Frequent fallers also showed an increased probability of an altered walking pattern with shortened step lengths and increased cadence under a dual-task situation.

It is concluded that the occurrence of falls in PSP seems strongly associated with the deterioration of bulbar function, but not relevantly with typical parkinsonian features like rigidity and bradykinesia. The decreased ability to deal with distraction of attention under a dual-task situation points to a relevant impact of cortical and subcortical dysfunction on the frequency of falls.

PubMed ID#: 20131396 (see pubmed.gov for this abstract only)