Category Archives: MSA

Gait and Balance Webinar Notes (5-28-09)

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Did anyone attend this webinar on gait and balance webinar on May 28, 2009 and take notes (especially during the Q&A and when some photos were shown) that you can share?  It was at 2am in the timezone where I was that day, so I was unable to call in.

Though CurePSP was the organizer of this webinar, it applied to all the disorders in our group.

Today, as a registered attendee of the webinar, I received a copy of the slides.  I don’t think these have been posted yet to the CurePSP website (psp.org).

It seems from the slides that it was a very good webinar.  I’ve copied below the text from some of the slides shown during the webinar.

Here are some highlights from my perspective:

  • Assistive Device Golden Rule:  If you find yourself reaching out to touch walls, furniture, friends or care partners (or they are reaching to you) while walking–you are in need of an assistive device.
  • If falls continue no matter what we do, then safety gear may be the answer:  helmets; HipSaver®; chair or bed alarm; wheelchair; geriatric recliner chair.
  • How can PT and OT help?  Assess & treat functional mobility and ADL abilities.  Perform home safety assessments.  Develop new and safer ways to perform activities.  Train care-partners to safely assist.  Recommend appropriate adaptive equipment for the home.  Recommend appropriate mobility devices.

 

Robin

—————————————————————————-

Gait & Balance Questions Answered
by Heather Cianci, PT, MS, GCS, Penn Neurological Institute
5-28-09 CurePSP Webinar

Causes of Gait Changes & Falls
..Rigid/Stiff Muscles and Joints
..Shuffling steps
..Narrow base of support (feet too close)
..Slowed or absent balance reactions
..Slowness of Movement
..Visual Changes
–Difficulty with scanning your surroundings
–Double vision
..Loss of Coordination
..Freezing
..Blurred vision
..Orthostatic hypotension –sudden blood pressure drop
..“Alien limb”
..Myoclonus–quick, jerky movements
..Sensation changes
..Impaired safety judgment

Common Places Falls Occur
..Walking
..Turning
..On Stairs or Curbs
..While Reaching
..Transferring:
–In & out of bed
–In & out of the shower/bath tub
–In & out of the car
–Up & down from a chair or the toilet

Tips for Walking Safely
..Slow down & concentrate
–You must now “tell”your body what to do
–If you are unable to do this, it is important to have a care partner remind you
..To avoid shuffling
–Focus on landing with the heel hitting down first, not the toes
..Do one thing at a time
–Do not reach for the refrigerator door while walking toward it
..Keep your hands free
..Be aware of changes in the floor surface
–Tilt your head down to look at the floor
–Stop before the surface change and step over the threshold

Assistive Device Golden Rule
If you find yourself reaching out to touch walls, furniture, friends or care partners (or they are reaching to you) while walking–you are in need of an assistive device.

Getting Out of Bed
..Bend up knees
..Roll completely on your side
..Grab rail
..Allow your legs to drop off of the side of the bed…
..While you push yourself up sideways into sitting
..If help is needed, care partner can lift under shoulder & push down at outer thigh

Getting In & Out of the Car
To Safely Transfer
–Slide seat back
–Back into car & then slide legs in, reverse to get out
–Plastic bag on seat can make moving on fabric seats easier
–Place cushion on low seats to help with standing

Handy Bar (for gettign in and out of the car)
Allows for a safe place to hold
www.handybar.com, 888-738-0611

Sit to Stand Transfer
..Scoot to front of chair
..Open legs wide
..Pull feet back under the knees
..Lean forward –“Nose over Toes”
..Push forward until butt lifts off of chair
..Then push up to stand

Protection Products/Devices
If falls continue no matter what we do, then safety gear may be the answer…
Helmets
•http://www.cpsc.gov/CPSCPUB/PUBS/349.pdf(US Consumer Safety Product Commission brochure)
•www.headsaver.com.au
•Local sporting goods store
HipSaver®(shorts with hip and tailbone pads)
•www.hipsaver.com
•www.alimed.com or 800-225-2610
Chair or Bed Alarm
•Posey Sitter II®Alarm Unit
•www.posey.comor 800-447-6739
•Features different alarms or voice recording when person moves from chair or bed
Wheelchair or Geriatric Recliner Chair
•Contact local PT/OT or Rehabilitation Center to find a “Seating Clinic”or Wheelchair specialist

Rehabilitation Strategies
Current Research is Supporting
..Balance exercises
..Balance exercises along with eye movement training
..Treadmill and weight-supported treadmill training
..Group exercise programs
Exercise needs to be ongoing throughout the course of the disease. Delays in the need for wheelchairs, less falls, improvements in gait & balance, and reduced gait changes are possible.

How Can I Get More Help with Finding Assistive Devices?
RESNA (Rehabilitation Engineering & Assistive Technology Society of North America)
..www.resna.org
..“Projects”
..“Statewide AT Program”
..“State AT Program”or “Device Loan”
NATTAP (National Assistive Technology Technical Assistance Partnership)
..Locate programs available in each state
..703-524-6686

How Can I Find Someone to do Home Modifications?
Certified Aging-In-Place Specialist (CAPS)
..Certified specialist through The National Association of Home Builders
..Remodelers, Contractors, Interior Designers, Therapists
..800-368-5242
..www.nahb.org
–Resources –Online Directories –Find a CAPS

How Can PT and OT Help?
..Assess & treat functional mobility & ADL abilities
..Perform home safety assessments
..Develop new & safer ways to perform activities
..Train care-partners to safely assist
..Recommend appropriate adaptive equipment for the home
..Recommend appropriate mobility devices

Locating a PT or OT
..American PT Association
–1-800-999-2782
–www.apta.org
–“Find a Certified Specialist (Neurologic or Geriatric)”
..American OT Association
–301-652-2682
–www.aota.org
..LSVT®BIG
–888-438-5788
–www.lsvtglobal.com
–“Find a Clinician”
..Call Local Outpatient Rehab Centers
–Generally those associated with Hospitals (Regular & Rehabilitation), & non-sports oriented centers
–Ask if they have therapists who have worked with PSP or those who deal with neurological & balance problems
..We Move
–www.wemove.org
–“Find a Doctor”–Physical Medicine & Rehab
..National Parkinson Foundation
–800-327-4545
–www.parkinson.org
–Allied Team Training (ATTP)
–List of Graduates

Recent developments in MSA (dementia?)

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This looks like an important paper on MSA, written by Gregor Wenning, one of Europe’s authorities on MSA.

To me, the most interesting sentences were about whether dementia can occur in MSA.  The authors say:

“The revised consensus criteria regard dementia as a non-supportive feature of MSA [50]. However, recent evidence suggests that dementia can occur in some patients otherwise satisfying the MSA criteria [77]. Further studies are required to assess the frequency and profile of dementia in MSA. These data may support a broader phenotype of MSA that also includes cognitive dysfunction.”

In personal correspondence, Dr. Wenning confirmed that there are no post-mortem confirmed cases of MSA with dementia….so far.  He believes we need to “keep an open mind.”

I’ve copied the abstract below.

Robin

—————————–

Journal of Neurology. 2009 May 27. [Epub ahead of print] Recent developments in multiple system atrophy.
Wenning GK, Stefanova N.
Section of Clinical Neurobiology, Department of Neurology, Innsbruck Medical University, Anichstrasse 35, 6020, Innsbruck, Austria,Gregor.Wenning@i-med.ac.at.

Multiple system atrophy (MSA) is a rare late onset neurodegenerative
disorder which presents with autonomic failure and a complicated motor syndrome including atypical parkinsonism, ataxia and pyramidal signs. MSA is a glial alpha-synucleinopathy with rapid progression and currently poor therapeutic management. This paper reviews the clinical features, natural history and novel diagnostic criteria for MSA as well as contemporary knowledge on pathogenesis based on evidence from neuropathological studies and experimental models. An outline of the rationale for managing symptomatic deterioration in MSA is provided together with a summary of novel experimental therapeutic approaches to decrease disease progression.

PubMed ID#: 19471850

 

Sleep Disorders Affect Majority of Elderly (Mayo Rochester)

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This press release out of Mayo Rochester (MN) is about a new study in one Minnesota county that showed that “59 percent of 892 people age 70-89 had signs of at least one recognized sleep disorder other than insomnia. The most common disorder, reported by 32 percent of study participants, was sleep-related leg cramps. … Obstructive sleep apnea–characterized by breathing pauses during sleep–occurred in 17.6 percent of participants. … A movement disorder known as REM sleep behavior disorder (RBD) occurred in about 9 percent of participants. This happens when sleepers appear to act out their dreams. … Restless legs syndrome–an irresistible urge to move legs associated with uncomfortable sensations–was suggested in about 8 percent of the study group.”

A Mayo Rochester neurologist who is also a sleep expert, Dr. Brad Boeve, had these comments about the study findings: “Perhaps the biggest surprise of the study is the high frequency of probable RBD in these participants.” The only study on the epidemiology of RBD in the population shows a prevalence of 0.5 percent, whereas these findings suggest a frequency of 9 percent in the 70-89 age range, he says. The frequency matters, he adds, because earlier research by our and other groups has suggested that those with RBD have an increased likelihood of developing a neurologic disorder such as Parkinson’s disease or Lewy body dementia in the future. “One ultimate goal is that once medications are developed that affect the biology of Parkinson’s disease and Lewy body dementia, we hope that they may be used in appropriately-identified individuals with RBD to delay the onset or prevent the development of symptoms associated with those disorders,” Dr. Boeve says.

In our local support group, many people with MSA and LBD have RBD. RBD can be confirmed with a polysomnogram, or a special sleep study that measures eye, leg, and other movements and records breathing and brain activity.

Here are two online video resources about this story:

http://www.youtube.com/watch?v=Kx9aDxv7YyI –> YouTube video of Dr. Jennifer Molano speaking about the study (2.5 minutes)

http://newsblog.mayoclinic.org/2009/04/ … nic-study/ –> scroll down to the Journalists section where you’ll find .wmv and .mp3 files on a study overview, study findings, and patient message

The press release is copied below.

http://www.mayoclinic.org/news2009-rst/5256.html

Sleep Disorders Affect Majority of Elderly Participants in a Large Mayo Clinic Study
Tuesday, April 28, 2009
Mayo Clinic Rochester Press Release

SEATTLE — Sleep disorders are common in the elderly, say researchers from Mayo Clinic who studied a large number of people in this age group in one Minnesota county.

At the 2009 American Academy of Neurology Annual Meeting in Seattle on April 28, the researchers report that 59 percent of 892 people age 70-89 had signs of at least one recognized sleep disorder other than insomnia. The most common disorder, reported by 32 percent of study participants, was sleep-related leg cramps.

The researchers say that their study is one of the first to look at a broader spectrum of sleep disorders in a community’s elderly, and understanding the prevalence of these problems may lead to increased diagnosis followed by beneficial treatment.

“All of these sleep disorders can disrupt a person’s quality of life, because they affect sleep,” says the study’s lead researcher, Jennifer Molano, M.D., a behavioral neurology fellow in the Department of Neurology. “But if these problems are recognized, an accurate diagnosis could lead to successful treatment.”

The research is part of Mayo Clinic’s Study of Aging, a multidisciplinary effort to understand age-related diseases and cognitive functioning. The bed partners of study participants in Olmsted County, Minn., home of Mayo Clinic, answered a questionnaire about how well their partners slept.

The researchers identified these other commonly reported disrupters of sleep:

Obstructive sleep apnea–characterized by breathing pauses during sleep–occurred in 17.6 percent of participants. Men were four times more likely to have features of obstructive sleep apnea compared to women.

Periodic, involuntary movements in the legs or arms during sleep were experienced by 17.4 percent.

A movement disorder known as REM sleep behavior disorder (RBD) occurred in about 9 percent of participants. This happens when sleepers appear to act out their dreams. In this study, men were twice as likely to exhibit recurrent dream enactment behavior as women. The disorder was also seen more often in people age 80 or older who had worsening cognitive impairment or dementia.

Restless legs syndrome–an irresistible urge to move legs associated with uncomfortable sensations–was suggested in about 8 percent of the study group.

Only 0.2 percent of participants were found to walked in their sleep.

“Perhaps the biggest surprise of the study is the high frequency of probable RBD in these participants,” says Bradley Boeve, M.D., a Mayo Clinic neurologist and one of several study investigators.The only study on the epidemiology of RBD in the population shows a prevalence of 0.5 percent, whereas these findings suggest a frequency of 9 percent in the 70-89 age range, he says. The frequency matters, he adds, because earlier research by our and other groups has suggested that those with RBD have an increased likelihood of developing a neurologic disorder such as Parkinson’s disease or Lewy body dementia in the future. “One ultimate goal is that once medications are developed that affect the biology of Parkinson’s disease and Lewy body dementia, we hope that they may be used in appropriately-identified individuals with RBD to delay the onset or prevent the development of symptoms associated with those disorders,” Dr. Boeve says.

The researchers hope to follow up these results with a validation study using a polysomnogram (or sleep study), which measures eye, leg, and other movements and records breathing and brain activity.

The study was funded by grants from the National Institute on Aging, the Robert H. and Clarice Smith and Abigail Van Buren Alzheimer’s Disease Research Program of the Mayo Foundation. Researchers at Mayo Clinic campus in Florida assisted in the study.

###
About Mayo Clinic

Mayo Clinic is the first and largest integrated, not-for-profit group practice in the world. Doctors from every medical specialty work together to care for patients, joined by common systems and a philosophy of “the needs of the patient come first.” More than 3,300 physicians, scientists and researchers and 46,000 allied health staff work at Mayo Clinic, which has sites in Rochester, Minn., Jacksonville, Fla., and Scottsdale/Phoenix, Ariz. Collectively, the three locations treat more than half a million people each year. To obtain the latest news releases from Mayo Clinic, go to www.mayoclinic.org/news. For information about research and education visit www.mayo.edu. MayoClinic.com is available as a resource for your health stories.

Progression of dysarthria + dysphagia in PSP, CBD, etc

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I’ve posted some of the info in this article previously and the PubMed ID# but have never posted these excerpts.

This 2001 study is of dysarthria and dysphagia in autopsy-confirmed cases of DLB, CBD, MSA, PSP, and PD. According to the article, all atypical parkinsonism patients develop either dysarthria or dysphagia within one year of disease onset. (I thought dysarthria meant slurred speech. But, according to this article, it can also mean hypophonic speech or monotonic speech.)

Here’s the citation and abstract:

Archives of Neurology. 2001 Feb;58(2):259-64.

Progression of dysarthria and dysphagia in postmortem-confirmed parkinsonian disorders.

Muller J, Wenning GK, Verny M, McKee A, Chaudhuri KR, Jellinger K, Poewe W, Litvan I.
Cognitive Neuropharmacology Unit, Bethesda, MD

BACKGROUND: Dysarthria and dysphagia are known to occur in parkinsonian syndromes such as Parkinson disease (PD), dementia with Lewy bodies (DLB), corticobasal degeneration (CBD), multiple system atrophy (MSA), and progressive supranuclear palsy (PSP). Differences in the evolution of these symptoms have not been studied systematically in postmortem-confirmed cases.

OBJECTIVE: To study differences in the evolution of dysarthria and dysphagia in postmortem-confirmed parkinsonian disorders.

PATIENTS AND METHODS: Eighty-three pathologically confirmed cases (PD, n = 17; MSA, n = 15; DLB, n = 14; PSP, n = 24; and CBD, n = 13) formed the basis for a multicenter clinicopathological study organized by the National Institute of Neurological Disorders and Stroke, Bethesda, Md. Cases with enough clinicopathological documentation for the purpose of the study were selected from research and neuropathological files of 7 medical centers in 4 countries (Austria, France, England, and the United States).

RESULTS: Median dysarthria latencies were short in PSP and MSA (24 months each), intermediate in CBD and DLB (40 and 42 months), and long in PD (84 months). Median dysphagia latencies were intermediate in PSP (42 months), DLB (43 months), CBD (64 months), and MSA (67 months), and long in PD (130 months). Dysarthria or dysphagia within 1 year of disease onset was a distinguishing feature for atypical parkinsonian disorders (APDs) (specificity, 100%) but failed to further distinguish among the APDs. Survival time after onset of a complaint of dysphagia was similar in PD, MSA, and PSP (15 to 24 months, P =.7) and latency to a complaint of dysphagia was highly correlated with total survival time (rho = 0.88; P<.001) in all disorders.

CONCLUSIONS: Latency to onset of dysarthria and dysphagia clearly differentiated PD from the APDs, but did not help distinguish different APDs. Survival after onset of dysphagia was similarly poor among all parkinsonian disorders. Evaluation and adequate treatment of patients with PD who complain of dysphagia might prevent or delay complications such as aspiration pneumonia, which in turn may improve quality of life and increase survival time.

PubMed ID#: 11176964

These results were the most interesting (and depressing):

“Dysarthria or dysphagia within 1 year of disease onset was a distinguishing feature for atypical parkinsonian disorders (APDs).”

“Median dysarthria latencies were short in PSP and MSA (24 months each), intermediate in CBD and DLB (40 and 42 months), and long in PD (84 months).”

“Median dysphagia latencies were intermediate in PSP (42 months), DLB (43 months), CBD (64 months), and MSA (67 months), and long in PD (130 months).”

“Survival time after onset of a complaint of dysphagia was similar in PD, MSA, and PSP (15 to 24 months).”

On this last point, here are the CBD and PSP data from the article:

Survival Time After Onset of Dysphagia, months
CBD 49 (25-89…..including a single patient with dysphagia but without dysarthria)
PSP 18 (6-96)

Here are some excerpts from the article’s Comment section:

“(Early) dysarthria and perceived swallowing dysfunction are not features of PD.”

“Dysarthria) as a presenting symptom has been described in clinical series of CBD, MSA, and PSP. In PD and DLB, hypphonic/monotonous speech represented the most frequent type of dysarthria, whereas imprecise or slurred articulation predominated in CBD, MSA, and PSP.”

“In a clinical study of CBD, Rinne et al described dysarthria as one of the initial symptoms in 11% of the patients, which is close to our findings. At follow-up, on average 5.2 years, dysarthria was diagnosed in 70% of the patients… According to our findings, dysarthria occurred in almost every patient with CBD.”

“In both PSP and MSA, progressive dysarthria is believed to represent a manifestation of brainstem and cerebellar involvement. In fact, PET studies revealed marked hypometabolism in the cerebellum and brainstem of patients with MSA, which correlated with dysarthria.”

“In our study, dysphagia was associated with concomitant dysarthria in all parkinsonian patients except one. This sequence of dysphagia following dysarthria has also been reported in clinical studies of PD, MSA, and PSP.”

“…Golbe et al reported dysphagia after a median of 1 year after the onset of dysarthria in PSP.”

In all these disorders, “bronchopneumonia has been reported as a leading cause of death, which may be subsequent to silent aspiration resulting from dysphagia.”

“Most of our patients with MSA and PSP complained of a swallowing dysfunction, in contrast to patients with PD, CBD, and DLB… Impaired lingual proprioception is hypothesized to contribute to the unawareness of swallowing difficulties in PD and might in part explain significantly longer latencies to dysphagia in our PD cases. In contrast, patients with PSP were reported to be keenly aware of swallowing problems, including those with cognitive impairment.”

“(The) similarly short remaining survival time in PD and PSP after the onset of perceived dysphagia suggests that this symptom represents a reliable marker for the onset of functionally relevant swallowing abnormalities in both disorders.”

Robin

MRI and MRS in PSP, MSA-P, PD (Brazilian study)

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This small Brazilian study looked at 11 patients with PSP, 7 patients with MSA-P, 12 patients with PD, and 10 controls. Everyone was studied with magnetic resonance imaging (MRI) and spectroscopy by MRI (MRS).

The authors concluded:
“(1) Patients with PSP and MSA-P presented increased motor and cognitive impairment in the scales used, correlating with decrease in NAA/Cr in lentiform nucleus and NAA/Cho in midbrain in the PSP group;
(2) Cerebral and cerebellar atrophy were more prevalent and severe in PSP and MSA-P groups;
(3) Linear hypersignal in the lateral portion of the putamen, hypersignal in midbrain and in pons, all suggest the diagnosis of PSP or MSA-P;
(4) Midbrain or pons atrophy suggests atypical parkinsonism, the former PSP, and the latter MSA-P;
(5) Comparing the two methods, MRI and MRS, the former had better applicability.”

The abstract and lots of excerpts follow. The English-language article is available for free online here:
http://www.scielo.br/pdf/anp/v67n1/a02v67n1.pdf –> PDF form
http://www.scielo.br/scielo.php?script= … so&tlng=en –> HTML form

For me, the most interesting parts of this article were the three figures with captions, indicating what percentage of the patient groups had particular MRI or MRS findings, including the hot cross bun sign in MSA. (You’ll have to go online to see the figures.) And the Discussion section was worthwhile reading.

Robin

Arqivos de Neuropsiquiatria. 2009 Mar;67(1):1-6.

Neuroimaging in Parkinsonism: a study with magnetic resonance and spectroscopy as tools in the differential diagnosis.

Vasconcellos LF, Novis SA, Moreira DM, Rosso AL, Leite AC.
Hospital dos Servidores do Estado, Rio de Janeiro, RJ, Brazil.

The differential diagnosis of Parkinsonism based on clinical features, sometimes may be difficult. Diagnostic tests in these cases might be useful, especially magnetic resonance imaging, a noninvasive exam, not as expensive as positron emission tomography, and provides a good basis for anatomical analysis. The magnetic resonance spectroscopy analyzes cerebral metabolism, yielding inconsistent results in parkinsonian disorders.

We selected 40 individuals for magnetic resonance imaging and spectroscopy analysis, 12 with Parkinson’s disease, 11 with progressive supranuclear palsy, 7 with multiple system atrophy (parkinsonian type), and 10 individuals without any psychiatric or neurological disorders (controls). Clinical scales included Hoenh and Yahr, unified Parkinson’s disease rating scale and mini mental status examination.

The results showed that patients with Parkinson’s disease and controls presented the same aspects on neuroimaging, with few or absence of abnormalities, and supranuclear progressive palsy and multiple system atrophy showed abnormalities, some of which statistically significant. Thus, magnetic resonance imaging and spectroscopy could be useful as a tool in differential diagnosis of Parkinsonism.

PubMed ID#: 19330200

Here are excerpts from the full article:

“Magnetic resonance imaging (MRI) and spectroscopy by MRI (MRS) are noninvasive tools helping the physician to establish a more accurate diagnosis. MRI offers an adequate analysis of abnormalities in the basal nuclei, midbrain, pons, medulla, and cerebellum, which are impaired in atypical” parkinsonism.

“Method
We designed a prospective, case-control, double-blind, 24 months study. The MRI was performed in a GE machine, 1.5 Tesla Sigma Horizon model, the sequences analyzed were T 1, T 2, flair, diffusion, axial-oblique in T 2 in Fast Spin-Echo (FSE) and Proton Density (PD) and T 2 in Spin-Echo (SE). In addition to 5 mm slices, we included 3 mm slices in the lentiform nucleus. The MRS was single voxel (8 cc), PRESS technique (TR/TE=1500/50) bilaterally in lentiform nucleus, midbrain, white matter of frontal lobe and hippocampus.” …

“Forty individuals were included in this study (age range: 50 to 85 years), 30 with Parkinsonian syndrome and 10 without any neurological or psychiatric disorders.”

“All individuals were examined by the same neurologist, and 26 patients met the criteria for probable Parkinson’s disease (PD) [n=10], (Gelb et al.), progressive supranuclear palsy (PSP) [n=10], (Tolosa et al.), and multiple system atrophy-parkinsonian type (MSA-P) [n=6], (Gilman et al.). For clinical assessment, the scales adopted were Hoehn-Yahr stage, unified Parkinson’s disease rating scale (UPDRS) Part III and mini-mental status examination (MMSE). The patients performed the Tilt Table test for evaluation of dysautonomia.”

“Results …
Dysautonomia was documented in 20% of PD and 100% of MSA-P.

In the motor scales (UPDRS and Hoehn and Yahr), the results showed higher scores in PSP and MSA-P than in PD. There was statistical significance in PD versus MSA-P, and a trend to statistical significance in PD and PSP.

Patients with PSP presented lower scores in MMSE, followed by MSA-P and PD, and there was statistical significance in the three groups comparing to controls (Table 1).

Image variables demonstrated cerebral atrophy in all cases of PSP and MSA-P, having statistical significance in PD versus PSP, PD versus MSA-P, controls versus PSP, and controls versus MSA-P. Cerebellar atrophy was more common in MSA-P and PSP, with statistical significance in PD versus MSA-P, controls versus PSP and controls versus MSA-P. We observed a higher prevalence of white matter alterations in atypical [parkinsonism] with no statistical significance. Signal change in the lentiform nucleus was observed more commonly in MSA-P and PSP, but no statistical significance was documented (Figs 1­3).”

Fig 1. Hyposignal in the lentiform nucleus (found in 67% of MSA-P group), and hypersignal in the pons (found in 33% of the MSA-P group) and the midbrain on T2, flair or DP sequences (found in 70% of PSP group).

Fig 2. Posterolateral linear hypersignal in the lentiform nucleus with asymmetric symptoms, T2 sequence (found in 50% in MSA-P group).

Fig 3. Transverse signal (“hot cross bun sign”) in the pons, T2 sequence (found in 33% of MSA-P group).” …

“DISCUSSION
…Parkinsonian signs may be seen in different medical conditions, having variable course, treatment and prognosis so it is important to determine an accurate diagnosis as soon as possible. Based only in clinical data, especially in the early stages of the disease, physicians may not establish a correct diagnosis.

… One study conducted in a movement disorders specialized center, showed that the positive predictive value of PD was 98.6%, and to atypical parkinsonism 71.4%, confirming that the diagnosis of atypical [parkinsonism], even in specialized centers, is sometimes difficult to establish.

… We included the three [parkinsonism syndromes] that most frequently lead to misdiagnosis: PD, MSA-P, and PSP, all compared to control group. …

We used three clinical scales: motor part of UPDRS, Hoehn and Yahr and MMSE. These scales showed increased motor impairment (higher scores in UPDRS and Hoehn-Yahr) in the MSA-P, followed by PSP, and increased cognitive impairment (lower scores of MMSE) in PSP, followed by MSA-P. We did not observe a correlation between the duration of the symptoms with MRS abnormalities, but with the clinical diagnosis of patient.

MRI variables demonstrated that some are helpful to differentiated [parkinsonism] syndromes, as the presence of cerebral and cerebellar atrophy and signal enhancement of some encephalic structures (lentiform nucleus, midbrain and pons), more common in atypical [parkinsonism].

The decreased signal enhancement in the lentiform nucleus may be observed in normal aging, so in our study we only considered it as ‘abnormal’ if the hypointensity was moderate to severe. Our data showed that moderate to severe decrease hypointensity in lentiform nucleus was observed more frequently in MSA and PSP, with no difference between PD and control groups and when it was associated with posterolateral linear hypersignal in putamen, suggested the diagnosis of atypical [parkinsonism] (more frequently in MSA group).

The most useful measurement of encephalic diameter in our study was the midbrain, as it had been shown by Warmutth et al. Values below 15 mm in the midbrain suggested PSP or MSA-P, with lower values seen in PSP.

Some values of MRS had statistical significance, the most useful were from the lentiform nucleus, hippocampus, and midbrain, depending on the diagnosis, indicating a severe neuronal impairment (neuronal death). There are few studies in which the brainstem is evaluated by MRS, due to technical difficulties (bone proximity). In our study we demonstrated that it is feasible, but we had to repeat the exam, in some cases several times, to achieve a consistent chart. The study done by Watanabe et al. demonstrated the usefulness of MRS of the pons in MSA patients. As the midbrain is the most affected area in PSP, we analyzed it by MRS. We have found NAA/Cho decrease in midbrain of PSP group with statistical significance, indicating neuronal loss.

Based on our data we concluded that:
(1) Patients with PSP and MSA-P presented increased motor and cognitive impairment in the scales used, correlating with decrease in NAA/Cr in lentiform nucleus and NAA/Cho in midbrain in the PSP group;
(2) Cerebral and cerebellar atrophy were more prevalent and severe in PSP and MSA-P groups;
(3) Linear hypersignal in the lateral portion of the putamen, hypersignal in midbrain and in pons, all suggest the diagnosis of PSP or MSA-P;
(4) Midbrain or pons atrophy suggests atypical parkinsonism, the former PSP, and the latter MSA-P;
(5) Comparing the two methods, MRI and MRS, the former had better applicability.

Our study showed that anatomical analysis through MRI and MRS of some areas could be useful in the differential diagnosis of PD and atypical [parkinsonism], helping physicians to establish a more accurate diagnosis of [parkinsonian syndromes].”