This recently-published German research is about transcranial sonographic findings when PSP patients are examined. The findings correspond to which type of PSP the patients have — Richardson’s Syndrome (RS) or PSP-Parkinsonism (PSP-P).

Reading between the lines, it seems that the findings for those with PSP-P are similar to the findings of those with Parkinson’s Disease (eg, a hyperechogenic substantia nigra). So, transcranial sonography may not be a good tool to differentiate between PSP and PD.

Robin

Movement Disorders. 2010 Mar 2. [Epub ahead of print]

Substantia nigra echogenicity in progressive supranuclear palsy.

Ebentheuer J, Canelo M, Trautmann E, Trenkwalder C.
Paracelsus-Elena-Klinik, Center of Parkinsonism and Movement Disorders, Kassel, Germany.

A normoechogenic substantia nigra (SN) is a typical finding in transcranial sonography in patients with progressive supranuclear palsy (PSP), whereas in patients with Parkinson’s disease a hyperechogenic SN is characteristic.

A recent classification scheme recommends the differentiation of PSP patients into those with Richardson’s syndrome (RS) and those with PSP-Parkinsonism (PSP-P).

We investigated 34 PSP patients (27 RS, 7 PSP-P) with ultrasound of the substantia nigra in search of differentiations in the two groups.

We found that most of the PSP-P patients, according to recently published criteria, had a hyperechogenic SN (6 of 7): right (cm(2)) median 0.22, 25% percentile 0.21 and 75% percentile 0.36 (cm(2)); left (cm(2)) median 0.21, 25% percentile 0.20 and 75% percentile 0.30 and a normal third ventricle (mean mm) +/-SD: 7.1 +/- 2.43).

In RS patients a normoechogenic SN (26 of 27) and an enlarged third ventricle (mean mm) +/-SD: 10.3 +/- 2.41) was found.

These differences may elucidate the pathological differences of RS and PSP-P.

PubMed ID#: 20198715 (see pubmed.gov for this abstract only)

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

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

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.