Category Archives: PSP

Improvement in gaze control after training/exercises in PSP

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This is a neat little study out of the University of Minnesota where 19 people with possible or probable progressive supranuclear palsy (PSP) were part of a randomized controlled trial.  Some received “balance training complemented with eye movement and visual awareness exercises” and others received balance training alone.  Gaze control was assessed after week 1 and week 5.  Gaze control significantly improved for the first group (who received the balance training and eye movement exercises).  Presumably the goal is to combat downward gaze palsy that many with PSP have.  An improvement in eye movement perhaps could lead to safer walking, easier reading, and an easier time finding food on one’s plate.
The abstract is copied below.
Robin
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Archives of Physical Medicine & Rehabilitation. 2009 Feb;90(2):263-70.

Improvement of gaze control after balance and eye movement training in patients with progressive supranuclear palsy: a quasi-randomized controlled trial.

Zampieri C, Di Fabio RP.
Department of Physical Medicine and Rehabilitation, University of Minnesota, Minneapolis, MN.

OBJECTIVE: One of the main oculomotor findings in progressive supranuclear palsy (PSP) is the inability to saccade downward. In addition, people with PSP have difficulty suppressing fixation, which may contribute to vertical gaze palsy. The objective was to investigate the effectiveness of a rehabilitation intervention tailored to enhance suppression of fixation and gaze shift in participants with PSP.

DESIGN: Controlled trial with a quasi-randomized design. Measures occurred at week 1 and 5. Researchers assessing participants were blind to the group assignments.

SETTING: Movement disorders assessment laboratory.

PARTICIPANTS: Nineteen adults with possible or probable PSP who were ambulatory for short distances and had far visual acuity of 20/80 and a Folstein Mini-Mental State score of more than 23.

INTERVENTIONS: Balance training complemented with eye movement and visual awareness exercises was compared with balance training alone.

MAIN OUTCOME MEASURES: Gaze control was assessed using a vertical Gaze Fixation Score and a Gaze Error Index.

RESULTS: Gaze control after the balance plus eye exercise significantly improved, whereas no significant improvement was observed for the group that received balance training alone.

CONCLUSIONS: These preliminary findings support the use of balance and eye movement exercises to improve gaze control in PSP.

PubMed ID#: 19236979  (see pubmed.gov for the abstract only)

“Core body temperature rhythm is altered” in PSP

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I don’t think this is a surprise to anyone. With the autonomic dysfunction that can come with PSP, body temperature regulation can malfunction as well. This research is out of Japan; the Japanese are world-class researchers of autonomic dysfunction.
Robin

Clinical Autonomic Research. 2009 Feb;19(1):65-68.

The core body temperature rhythm is altered in progressive supranuclear palsy.

Suzuki K, Miyamoto T, Miyamoto M, Hirata K.
Dept. of Neurology, Dokkyo Medical University, Tochigi, Japan.

We measured circadian rhythmicity in rectal temperature change and found that the amplitude of body temperature was significantly reduced in patients with progressive supranuclear palsy compared to those with Parkinson disease.

PubMed ID#: 19224326 (see pubmed.gov for this short abstract)

Using eye saccade velocity to distinguish PSP-P and PD

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This interesting research out of Germany looked at 12 cases of the RS (Richardson’s Syndrome) form of PSP, 5 cases of the PSP-P (PSP-Parkinsonism) form, 27 cases of Parkinson’s Disease, and 23 healthy controls. (It was just published on PubMed though the journal is dated Dec ’08 and the epub is dated 1/14/09.)

At the bottom of this post I’ve copied an abstract of an ’05 journal article explaining the terms RS and PSP-P. In short, “Cases of RS syndrome [make] up 54% of all cases, and [are] characterized by the early onset of postural instability and falls, supranuclear vertical gaze palsy and cognitive dysfunction.” “A second group of [32% are] characterized by asymmetric onset, tremor, a moderate initial therapeutic response to levodopa and were frequently confused with Parkinson’s disease (PSP-P).”

Because PSP-P and PD are so similar in symptoms, clinicians need a way to differentiate the two disorders. The German researchers conclude that video-oculography (VOG) can be used because of the “clear-cut separation between PSP-P and [PD] obtained by measuring saccade velocity.” Typically, a neuro-ophthalmologist has the equipment to conduct a VOG.

It should be noted that none of the patients had pathologically-confirmed diagnoses, so the results may change based upon that.

Robin

Journal of Neurology. 2008 Dec;255(12):1916-1925. Epub 2009 Jan 14.

Differential diagnostic value of eye movement recording in PSP-parkinsonism, Richardson’s syndrome, and idiopathic Parkinson’s disease.

Pinkhardt EH, Jürgens R, Becker W, Valdarno F, Ludolph AC, Kassubek J.
Dept. of Neurology, University of Ulm, Ulm, Germany.

Vertical gaze palsy is a highly relevant clinical sign in parkinsonian syndromes. As the eponymous sign of progressive supranuclear palsy (PSP), it is one of the core features in the diagnosis of this disease.

Recent studies have suggested a further differentiation of PSP in Richardson’s syndrome (RS) and PSP-parkinsonism (PSPP).

The aim of this study was to search for oculomotor abnormalities in the PSP-P subset of a sample of PSP patients and to compare these findings with those of (i) RS patients, (ii) patients with idiopathic Parkinson’s disease (IPD), and (iii) a control group. Twelve cases of RS, 5 cases of PSP-P, and 27 cases of IPD were examined by use of video-oculography (VOG) and compared to 23 healthy normal controls.

Both groups of PSP patients (RS, PSP-P) had significantly slower saccades than either IPD patients or controls, whereas no differences in saccadic eye peak velocity were found between the two PSP groups or in the comparison of IPD with controls.

RS and PSP-P were also similar to each other with regard to smooth pursuit eye movements (SPEM), with both groups having significantly lower gain than controls (except for downward pursuit); however, SPEM gain exhibited no consistent difference between PSP and IPD.

A correlation between eye movement data and clinical data (Hoehn & Yahr scale or disease duration) could not be observed.

As PSP-P patients were still in an early stage of the disease when a differentiation from IPD is difficult on clinical grounds, the clear-cut separation between PSP-P and IPD obtained by measuring saccade velocity suggests that VOG could contribute to the early differentiation between these patient groups.

PubMed ID#: 19224319 (see pubmed.gov for this same abstract)

Here’s an abstract of an important paper on PSP in 2005 that introduced the terminology RS and PSP-P:

Brain. 2005 Jun;128(Pt 6):1247-58. Epub 2005 Mar 23.

Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism.

Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, Kilford L, Holton JL, Revesz T, Lees AJ.
The Queen Square Brain Bank for Neurological Disorders, University College London, UK.

The clinical diagnosis of progressive supranuclear palsy (PSP) relies on the identification of characteristic signs and symptoms. A proportion of pathologically diagnosed cases do not develop these classic features, prove difficult to diagnose during life and are considered as atypical PSP. The aim of this study was to examine the apparent clinical dichotomy between typical and atypical PSP, and to compare the biochemical and genetic characteristics of these groups. In 103 consecutive cases of pathologically confirmed PSP, we have identified two clinical phenotypes by factor analysis which we have named Richardson’s syndrome (RS) and PSP-parkinsonism (PSP-P). Cases of RS syndrome made up 54% of all cases, and were characterized by the early onset of postural instability and falls, supranuclear vertical gaze palsy and cognitive dysfunction. A second group of 33 (32%) were characterized by asymmetric onset, tremor, a moderate initial therapeutic response to levodopa and were frequently confused with Parkinson’s disease (PSP-P). Fourteen cases (14%) could not be separated according to these criteria. In RS, two-thirds of cases were men, whereas the sex distribution in PSP-P was even. Disease duration in RS was significantly shorter (5.9 versus 9.1 years, P < 0.001) and age at death earlier (72.1 versus 75.5 years, P = 0.01) than in PSP-P. The isoform composition of insoluble tangle-tau isolated from the basal pons also differed significantly. In RS, the mean four-repeat:three-repeat tau ratio was 2.84 and in PSP-P it was 1.63 (P < 0.003). The effect of the H1,H1 PSP susceptibility genotype appeared stronger in RS than in PSP-P (odds ratio 13.2 versus 4.5). The difference in genotype frequencies between the clinical subgroups was not significant. There were no differences in apolipoprotein E genotypes. The classic clinical description of PSP, which includes supranuclear gaze palsy, early falls and dementia, does not adequately describe one-third of cases in this series of pathologically confirmed cases. We propose that PSP-P represents a second discrete clinical phenotype that needs to be clinically distinguished from classical PSP (RS). The different tau isoform deposition in the basal pons suggests that this may ultimately prove to be a discrete nosological entity.

PubMed ID#: 15788542 (see pubmed.gov for abstract only)

If you want to read the full paper published in Brain ’05, it’s available online for free here:
http://brain.oxfordjournals.org/cgi/reprint/128/6/1247
I have found that articles that are available for free are not always left online for free access. So if you want to save a copy of this article on your hard drive, do so now.

“Speech, Swallowing, and Mealtime” – Notes from 2/18/09 Webinar

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CurePSP (psp.org) hosted a webinar today on the topic of “Speech, Swallowing and Mealtime Questions.”  The speaker was expert speech therapist Laura Purcell Verdun.

Though many of the slides shown during today’s webinar are specifically about PSP and CBD, most of the slides were not limited to these two disorders. In addition, speech and swallowing problems occur in all the atypical parkinsonism disorders.

Here are the notes I took during the web-based conference call.  Please share your notes as you may have picked up different points than I did.

Robin

Robin’s Notes from

Speech, Swallowing and Mealtime Questions
CurePSP Webinar
February 18, 2009
Presenter:  Laura Purcell Verdun, SLP-CCC
([email protected], 703/573-7600 ext 1414)

Definitions:
Dysphagia:  difficulty swallowing
Aspiration:  food or liquid going into the lungs
Silent aspiration:  aspiration without clinical indication (cough, choke)

PSP swallowing difficulties:
difficulty looking down at plate of food
mouth stuffing or rapid drinking (frontal lobe problems)
poor self-feeding due to tremor or rigidity
restricted head and neck posture
hyperextension of head is especially a problem in PSP (food/liquids can go straight down to the lungs)
impaired coordination between swallowing, breathing, and eating
lack of awareness of swallowing

CBD swallowing difficulties:
slow or impaired chewing
apraxia
slowed swallowing movements

Swallowing management:
early evaluation of swallow
frequent monitoring of swallowing function
what are the patient’s goals?  (caloric intake, enjoyment, etc)

Swallowing evaluation:
patient/family should bring to meeting info on swallowing/feeding history

Common clinical questions:
do you have trouble swallowing?
do you have excess saliva in your mouth?
do meals take longer to eat?
does food stick to roof of mouth?
do you cough or clear your throat when you drink water or other liquid?
do you have trouble taking medication (pills)?

Warning signs:
drooling
food collecting in mouth
increased effort in swallowing
trouble talking
coughing and choking with a red face (showing it’s a stressful event)
wet voice (gurgly, sounds like someone is talking underwater) – the concern is that saliva is sitting on vocal cords
key:  do you cough or choke during mealtimes than during other times of the day?

VFSS = MBSS
Videofluroscopic Swallowing Study = Modified Barium Swallow Study
xray video of swallowing mechanism
important:  needs to replicate home feeding environment  (example: does patient hold cup at home?)
identify safe swallowing strategies

Oral hygiene – one treatment option:
need scrupulous dental care to get bacteria out of mouth
avoid alcohol, caffeine, and smoking
use club soda or sparkling water to help cut through secretions
Biotene (biotene.com) and Oasis – two good over-the-counter product lines
Plak-Vac oral suction toothbrush  (800/325-9044)

Drooling
use prescription anticholinergics such as Scopolamine patch, Robinul, atropine drops
speak with MD about botox injections.  Find an MD with experience doing these injections as there is a chance that the injections can worsen the swallow mechanism.

How will swallowing strategies impact the caregiver or family?  There can be changes to meal preparation.

Mealtime strategies:
sit upright
limit distractions
clear secretions from mouth prior to eating
put food plate in line of vision
experiment with different plates, utensils, straws, cups, etc.  (Sometimes straws can be useful.)
keep chin down
slow, steady rate of ingestion:  small bite followed by a swallow
alternate liquid and food swallows
take liquids by teaspoons
no Jello
be sure everyone knows the Heimlich maneuver
supervision during mealtimes
find other ways to nurture person with PSP/CBD

Diet modifications:
stick with moist, tender foods (eg, dark meat chicken, fish, casserole)
blend multiple consistency items
avoid textured, particulate, and dry foods (eg, nuts, cereal)
thickening liquids to slow rate of transit.  Problems:  may lead to reduced fluid intake; may be harder for lungs to tolerate thickening agents if aspirated

Thickening agents:
commercial thickeners
tofu
potato flakes

VitalStim:
clinical efficacy and utility of this therapy is unproven

Possible indications for alternate nutrition

Things to consider regarding a feeding tube:
discussions should take place sooner rather than later
don’t wait for a crisis!
discussions should be repeated
gastric contents and saliva can be aspirated
no clinical trials to know if feeding tubes are beneficial

Speech:
Change in speech may occur earlier in PSP than CBD
People with PSP and CBD may lose the ability to speak in late stages

Speech terms:
Dysarthria:  trouble pronouncing sounds; consistent articulation errors
Dysphonia:  difficulty generating a clear, strong voice
Apraxia:  inconsistency of errors; speech disorder
Oral apraxia:  the inability to perform a task upon command
Progressive non-fluent aphasia (PNFA):  simplified formation of sentences

PSP speech:
hypokinetic, spastic dysarthria
palilalia  (repeat your own words)

CBD speech:
apraxia of speech and oral apraxia
hypokinetic, spastic dysarthria
often has a component of PNFA
yes-no reversal

PSP speech strategies:
take a good breath before starting to speak
speak up and be deliberate
keep sentences short
repeat entire sentence if necessary, not just one word
use gestures
say one sentence at a time without immediate repetition
LSVT may help  (lsvt.org)

CBD speech strategies:
short phrases and simpler language
it may help to use written communication
use gestures
investigate using communication board

Strategies for the listener to utilize:
eliminate distractions, including background noise
face the speaker
keep questions and comments brief
ask one question at a time
stick with one topic at a time
provide choices to ease decision-making  (eg, ask “do you want coffee or tea?” vs. “what do you want to drink?”)

Robin’s note about resources:  I didn’t take these down because they are largely the same as appeared in a 2008 article authored by Laura Purcell Verdun.

Questions and Answers:

No medication can help with swallowing.

Laura personally does not use VitalStim for any patient population.  There is no research on VitalStim in treating neurodegenerative diseases (PD, PSP, etc).  Make sure your expectations aren’t misplaced.  VitalStim doesn’t address either of Laura’s two priorities in treatment.

Her priorities in treatment are oral hygiene and mealtime management.

Personal voice amplifiers can work if the voice is quiet but the speech is still clear.  Could even use a portable Karaoke machine.  Other machines:  Spokesman, Chattervox (more expensive).  [Robin’s note:  I couldn’t find the Spokesman or Spoke Man device any place on the web.  Hopefully someone can find it.  I will email Laura about the correct name.]

Augmentative or assisted communication device.  These are machines that are used to communicate for someone.  Most devices are computer-based or electronic.  How will you access this device?  Pointer, eye piece, etc.  Are there cognitive problems precluding the use of such devices?

Swallowing problems may occur later in CBD than PSP.  Hard to say.

Question:  Litvan published a paper in ’01 that showed that on average those with PSP died 18 months after the onset of dysphagia while those with CBD died 49 months after the onset of dysphagia.  Is this roughly your experience with survival time as well?
Laura’s answer:  those with PSP have dysphagia problems sooner than those with CBD but survival time is probably longer than 18 months for PSP.  She hasn’t kept track of survival time.

As soon as a person is diagnosed with one of these disorders, there should be a conversation about whether a feeding tube is desired in the future.  This is a very personal decision.  Not all MDs are comfortable bringing up this topic.  What does the person hope to accomplish in placing a feeding tube?  This conversation needs to be repeated later.

A soft, cervical collar may help keep the head up.

Exercises can be of benefit.  Apraxia can be aided in speech tasks (repeating words) and in non-speech tasks (blowing out candle, sticking out tongue).  She said that apraxia is especially a problem in PSP.  (I think she misspoke; she meant to say CBD.)

 

PSP/CBD Update – Diagnosis, Genetics, Treatment (Litvan ’07)

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Larry in southern California (whose wife has PSP) emailed several of the Yahoo!Groups yesterday about this August ’07 Irene Litvan article, asking if anyone knew anything about “transcranial sonography (TCS),” which is a diagnostic method mentioned in the article. Turns out that only a short paragraph of the Litvan article was on TCS. Dr. Irene Litvan is one of the top experts in the world on PSP. She’s written quite a bit on CBD and MSA as well.

This review article is an update for neurologists as to what advancements have been made in the area of diagnostic tools, genetics, and treatments for PSP, CBD, and MSA. The rest of this post is a summary of what I learned from the article. If you want more details, read the abstracts of the articles on PubMed (pubmed.gov – enter in the ID#). (A few of the full articles are available online for free. It’s mostly the harder-to-comprehend papers that are free!)

Dr. Litvan, writing in August ’07, concludes the article by saying that the field of atypical Parkinsonian disorders — diagnostic tools, genetics, and treatment — has “significantly advanced over the past year.” From a patient/family perspective, it is hard to agree with her.

DIAGNOSIS. From the article, I learned about recent studies of four diagnostic methods using “ancillary tools”:

1. TCS (transcranial sonography): TCS may help distinguish PD vs. atypicals, PD vs. MSA/PSP, MSA/PSP vs. CBD, and perhaps PSP vs. CBD, if I’m reading this correctly. PSP can be differentiated from CBD because the dilation of the third ventricule of the brain has so far only been described in PSP. This TCS study was done in Europe. TCS cannot be performed in up to 20% of patients. The study had nothing to say about LBD or about differentiating MSA from PSP. Of all the papers referenced in her review article, this is the only one labeled as “of outstanding interest” by Dr. Litvan. (PubMed ID#17189043)

2. Diffusion-weighted MRI: This method may help distinguish PSP and MSA-P. (PubMed ID#17089396)

3. T2-weighted MRI: This method may help differentiate MSA and PD. (PubMed ID#17361340)

4. Saccade tasks: This sort of diagnostic test (of saccade latencies and directional errors) would be performed by a neuro-ophthalmologist. This test may help distinguish PSP vs. CBD/PD. (PubMed ID#17124191)

GENETICS. From the article, I learned some things about genetics that I was unaware of:

* The location of a second genetic risk for PSP was identified in 2007. (This utilized brain tissue at the Mayo Jax PSP Brain Bank. PubMed ID#17357082; very challenging reading)

* The LRRK2 genetic mutation, which can be a factor in PD and DLB, is “not associated with MSA or with sporadic PSP.” (This is the genetic mutation that was discussed in the Frontline program last week on PD, “My Father, My Brother, and Me.”)

TREATMENT. And here’s what I learned about treatment:

* Because of the success (“significant gait and postural balance benefits”) of an Italian study of DBS in two locations of the brain in advanced PD patients, Dr. Litvan believes that DBS of the pedunculopontine nucleus (PPN) “may be useful in treating the balance and gait disorder in the atypical parkinsonian disorders, particularly in patients with PSP and MSA.” In fact, CurePSP is funding a study of DBS of the PPN in those with PSP in Toronto. (PubMed ID#17251240)

* Mayo Rochester is studying respiratory dysfunction in MSA. (PubMed ID#17235127; very challenging reading)

* Transgenic mice models are being developed for PSP, CBD, and MSA.

What follows are the abstract of the article.

Robin

Current Opinion in Neurology. 2007 Aug;20(4):434-7.

Update of atypical Parkinsonian disorders.

Litvan I.
Department of Neurology, University of Louisville, Louisville, Kentucky.

PURPOSE OF REVIEW: This timely update discusses novel diagnostic approaches, recently identified genes, and innovative experimental symptomatic treatments for these devastating disorders.

RECENT FINDINGS: Differential patterns in the basal ganglia transcranial sonography, magnetic resonance diffusion-weighted imaging regional apparent diffusion coefficients in the brainstem, basal ganglia T2-weighted gradient echo sequences combined with fluid attenuated inversion recovery, or saccades error rates in single and mixed-task blocks could help differentiate the various parkinsonian disorders. In addition to the familial tauopathies (frontotemporal dementia associated with chromosome 17) presenting with an atypical parkinsonian phenotype, ‘TDP-43opathies’ and ‘tataboxbinding or ataxinopathies’, depending on the protein deposited in the brain, widen the scope of the familial atypical parkinsonian disorders. Recent identification of novel deep brain stimulation targets such as the pedunculopontine nucleus may help treat the balance and gait disorder in atypical parkinsonian disorders in the near future.

SUMMARY: These new findings are important for diagnosis, help better understanding of the nosology of these disorders, and will likely in the near future impact our clinical practice.

PubMed ID#: 17620879 (see pubmed.gov for abstract only)