Monthly Archives: December 2008

Ask the Doctor – diseases mistaken for PSP

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Here’s the “Ask the Doctor” section of the Fall ’08 CurePSP Magazine, which I received in the mail on 12/1/08: (not yet available on psp.org)

Ask the Doctor
Lawrence I. Golbe, MD
Director of Research and Clinical Affairs, CurePSP
CurePSP Magazine, Fall ’08

Q: I’ve heard that some people with a diagnosis of PSP actually have corticobasal degeneration. Should I worry about that and get another opinion on my diagnosis?

A: No. PSP and corticobasal degeneration (CBD) are very similar and some experts think they’re even two versions of the same basic disease. They are treated similarly, start at the same average age, have the same long-term prognosis and even share a specific genetic variant as a contributing factor. The difference is that in CBD, there is less falling than in PSP but more difficulty in using the limbs for fine movement. CBD also tends to develop minor jerking movements and abnormal postures of the limbs. The limb problems of CBD are typically highly asymmetric — that is, far worse on one side than the other. All of the symptoms of both PSP and CBD are treated “palliatively,” which means that if the symptom appears, the doctor treats it as a symptom. Some day, if we are able to cure or slow the progression of one but not the other, or if the cures for the two disorders are different, then it will be important to differentiate between the two diagnostically.

Q: OK, so are there any diseases that have cures or very useful treatments that can be mistaken for PSP?

Parkinson’s disease (PD) is sometimes mistaken for PSP when it fails to respond to levodopa, a drug that replaces insufficient brain dopamine. Often, this is simply the result of insufficient dosing. Very often patients with PD have difficulty looking in the upward direction. The combination of this with a much greater-then-average need for levodopa can fool an inexperienced physician into diagnosing PD as PSP. But PD would not display difficulty looking down, the cardinal feature of PSP, and differs in most other respects as well.

Another condition that can be mistaken for PSP and requires special treatment is multiple small strokes. Aggressive management of lipids and blood pressure, administration of blood thinners, repair of narrowed arteries feeding the brain and treatment of heart problems can all reduce the risk of future strokes.

Finally, a very rare brain infection called Whipple’s disease can superficially mimic PSP. However, it usually has rhythmic movements of the eyes and jaw that are absent in PSP. It is diagnosed via MRI with contrast and a spinal tap and treated with antibiotics. In 20 years as a PSP specialist, I have never seen a case.

Brigadier Michael Koe writes a book about his wife Sara Koe who had PSP

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My online friend Joe Blanc posted this to the Society for PSP’s Forum recently. It’s about Brigadier Michael Koe writing a book about his wife Sara, who had progressive supranuclear palsy (PSP). The Koes established the PSP Association in the UK, which is a wonderful organization.

Here’s an excerpt from the newspaper article:

“Mr Koe…said one of the reasons he decided to write the book was to help him deal with the experience of watching a loved one deteriorate from a condition which robs people of their balance, movement, speech, eyesight and ability to swallow. He said: ‘It’s also difficult in that the symptoms can make it seem like the sufferer has been drinking. Friends of Sara’s became less friendly. It was a pretty traumatic time and a pretty bad experience and I think writing it down has helped.'”

Robin

Book tells of tragic death of wife 

Brigadier Michael Koe with the book Charity begins at Home, which wrote after the death of his wife

Published Date: 01 December 2008
By Nick Spoors
A former soldier who set up a leading charity after losing his wife to a little-known brain disease has published an account to raise awareness.

Brigadier Michael Koe and his wife Sara started the progressive supranuclear palsy (PSP) association in 1994 after she was diagnosed with the condition. However, just eight months later she died from complications arising from the illness.

Mr Koe, of Wappenham, near Towcester, said one of the reasons he decided to write the book was to help him deal with the experience of watching a loved one deteriorate from a condition which robs people of their balance, movement, speech, eyesight and ability to swallow.

He said: “It’s also difficult in that the symptoms can make it seem like the sufferer has been drinking. Friends of Sara’s became less friendly.

“It was a pretty traumatic time and a pretty bad experience and I think writing it down has helped.”

The couple set up the PSP Association because they were frustrated at the lack of knowledge about PSP, even among medical professionals.

Since then, the charity has grown to a position where it now employs 21 people with nine volunteers, has helped 3,000 people and invested more then £2 million in research.

Mr Koe was awarded an OBE for his charity work.

Charity Begins At Home can be purchased from the PSP Association for £20 plus £3.50 postage and packaging with proceeds after costs going to the charity.

Cheques can be sent to PSP House, 167 Watling Street West, Towcester, Northamptonshire, NN12 6BX.

The charity can also be contacted via www.pspeur.org, by email on [email protected] or on 01327 3224110.

Voluntary, spontaneous and reflex blinking in PSP

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As we’ve heard, blink rate is “profoundly sparse in PSP.” This recently-published Italian research looks at 11 patients with probable PSP and records eyelid movements during three types of blinking — voluntary, spontaneous and reflex blinking. Blinking was found to be abnormal in all three types.

Brain. 2008 Nov 29. [Epub ahead of print]

Voluntary, spontaneous and reflex blinking in patients with clinically probable progressive supranuclear palsy.

Bologna M, Agostino R, Gregori B, Belvisi D, Ottaviani D, Colosimo C, Fabbrini G, Berardelli A.
Department of Neurological Sciences and Neuromed Institute, Sapienza, University of Rome, Italy.

Patients with progressive supranuclear palsy (PSP) often have blinking abnormalities.

In this study we examined the kinematic features of voluntary, spontaneous and reflex blinking in 11 patients with PSP and healthy control subjects. Patients were asked to blink voluntarily as fast as possible; spontaneous blinking was recorded during two 60 s rest periods; reflex blinking was evoked by electrical stimulation of the supraorbital nerve. Eyelid movements were recorded with the SMART analyzer motion system.

During voluntary blinking the closing and opening phases lasted longer in patients than in healthy subjects. Furthermore, the peak velocity of the closing phase of voluntary blinking was lower in patients than healthy subjects.

During spontaneous blinking the blink rate was markedly lower in patients than in control subjects. Patient’s recordings also showed kinematic abnormalities of spontaneous (reduced peak velocity of both closing and opening phases) and reflex (reduced peak velocity and increased duration of the opening phase) blinking.

Recordings during reflex blinking disclosed an enhanced excitability of the interneuronal pool mediating the closing and opening blink phases.

Finally, the pause, a neurophysiological marker of the switching processes between the closing and opening phases, was prolonged in all the three types of blinking.

The abnormal kinematic variables correlated with patients’ clinical and kinematic features. Abnormal voluntary, spontaneous and reflex blinking in patients with PSP reflects the widespread cortical, subcortical and brainstem degeneration related to this disease.

PubMed ID#: 19043083 (see pubmed.gov for abstract only)

From TheFreeDictionary:
kinematics – dealing with the study of the motion of a body

“Tools for Meal Time” – good article on dysphagia

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The Fall 2008 CurePSP Magazine (psp.org) has a good article on swallowing problems that may be of interest to everyone — not just those dealing with PSP (progressive supranuclear palsy).

I’ve copied the full text below.  At some point, the newsletter will be available on psp.org; I received it in the mail this week.  You can sign up online at psp.org to receive a copy of future newsletters in the mail.

Robin

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Tools for Meal Time
Laura Purcell Verdun, MA, CCC
Speech-Language Pathologist
Otolaryngology Associates, PC
Fairfax, VA

CurePSP Magazine
Fall 2008 Issue

Difficulty swallowing, or dysphagia, is commonly associated with many neurodegenerative diseases, specifically PSP or CBD.  In fact, difficulty swallowing may be one of the early symptoms of this disorder.  Given that complications related to difficulty swallowing are one of the most common causes of mortality, attention needs to be directed towards optimizing the ease and safety of swallowing.

What changes in eating and swallowing should we look for?

There are actually fairly typical behavioral changes with eating in PSP and CBD.  These often include difficulty looking down at the plate, mouth stuffing and rapid drinking, difficulty with self-feeding because of tremor or stiffness, restricted head and neck posture, and occasional difficulty opening the mouth.  These behaviors can make it more difficult to swallow and often contribute to decompensation of the swallowing mechanism resulting in coughing and choking.  Caregivers need to look out for these behaviors, because the person with PSP or CBD often is not aware of these changes.

What are some tools that we can use to help make meal times more successful?

1.  Use a suction machine, toothette swab (a pink or green sponge on a lollipop-type stick), or mouth rinse prior to meals to clear out secretions which may interfere with ease of swallowing.

2.  A firm chair with arms to support sitting upright for proper swallowing alignment is most ideal.

3.  Keep the plate in the line of vision, by placing the plate on top of a book or something similar, or substituting reading glasses for bifocals.

4.  Try experimenting with different plates and utensils.  Plates with higher edges and bottom grips enhance independence with eating.  Downsize eating utensils to limit how much food is being put in per mouthful.

5.  Experiment with different cups and glasses.  Some are just easier to drink out of than others, depending on a handle or even the thickness and shape of the lip.  Straws are generally not preferred because it results in acceleration of the liquid into the throat before it’s ready.

6.  Use a blender or food processor.  The goal is not to eliminate foods necessarily, but consider how they could be prepared difficultly to enhance ease and safety of swallowing.  Multiple consistency items such as fruit cocktail and broth based soups generally should be avoided, so blend them.

7.  A teaspoon can be used to restrict the amount of liquid placed in the mouth for each swallow.

Though clearly not a comprehensive list, here are some specific products that may be of benefit at meal times.  Look for other products available on these web sites as well:

Flexi-Cut Cup allows for drinking without extending the head and neck backwards, 3 sizes available (800/225-2610, www.alimed.com).

Provale Cup restricts the volume of liquid allowed per swallow (800/225-2610, www.alimed.com).

Maroon Spoons have a narrow shallow bowl to restrict how much food is placed on the spoon and in the mouth, come in 2 sizes (800/897-3202, www.proedinc.com). [Robin’s note:  that’s the correct website!  You can also find these spoons at AliMed.]

Less Mess Spoon is designed with holes to keep food on the spoon, or drain away liquid from a multiple consistency food item (800/257-5376, www.theraproducts.com).

Scooper Plate with Non-Skid Base has a high curve to help scoop food onto a utensil (913/390-0247, www.bindependent.com).

Skidtrol Non-Skid Bowl is a melamine bowl with non-skid base (972/628-7600, www.maddak.com).

Are there any cookbooks that may give us some ideas regarding meal preparation for people with trouble swallowing?

Achilles E and Levin T.  The Dysphagia Cookbook.  2003.  Cumberland House Publishing.

MEALS for Easy Swallowing.  2005.  Muscular Dystrophy Association Publications.  [Robin’s note:  the correct link to this book is www.als-mda.org/publications/meals/ The full contents of this book, including the recipes, are available online.]

Weihofen D, Robbins J, Sullivan P.  Easy-to-Swallow Easy-to-Chew Cookbook.  2002.  John Wiley & Sons, Inc.

Wilson JR and Piper MA.  I Can’t Chew Cookbook: Delicious Soft Diet Recipes for People with Chewing, Swallowing, and Dry-Mouth Disorders.  2003.  Hunter House, Inc.

Woodruff S and Gilbert-Henderson L.  Soft Foods for Easier Eating Cookbook: Recipes for People who have Chewing and Swallowing Difficulties.  2007.  Square One Publishers.

If you have any concerns regarding swallowing, be sure to discuss this further with your neurologist and speech pathologist.