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PSP: Lumping Or Splitting? MDS-0110-224 VOLUME 15, ISSUE 1 • 2 0 1 1 • EDITORS, DR. CARLO COLOSIMO, DR. MARK STACY PSP: Lumping or Splitting? Lumping Splitting —­Andrew­Kertesz­MD­FRCP(C),­London,­ON,­Canada ­­—David­Williams,­MBBS,­PhD,­FRACP,­Associate­Professor,­Monash­ University,­Melbourne,­Australia The syndrome of bradykinesia, axial rigidity, dystonia, falls, dysphagia, and vertical gaze palsy with subcortical pathology was One of the enduring satisfactions in Clinical Neurology is providing described as progressive supranuclear palsy (PSP) by Steele, an early and accurate diagnosis in a patient with a rare or exotic Richardson and Olszewski1, and not disease. Much of this satisfaction comes much later the clinical syndrome of from being able to execute carefully unilateral rigidity, prominent apraxia, developed clinical skills. Sadly more reflex myoclonus and alien hand syn- procedural specialists will never experience drome as ������������������������������corticodentatonigral degenera- that same satisfaction, with technicians tion,2 ���������������������������������relabelled corticobasal degenera- and computers giving diagnoses that tion (CBD). Rebeiz and coworkers3 always trump clinical assessment. The best recognized the resemblance of the Neurologists, and this is particularly pathology to Pick’s disease (PiD). Even pertinent for Movement Disorder Special- before that there have been several case ists, are those who have seen a wide variety descriptions of PiD where the patients of clinical signs and symptoms in patients had prominent unilateral rigidity and over a number of years. It is in this context Parkinsonism.3 that the subtleties of Clinical Neurology The cognitive impairment was initially reach their full potential and carefully underestimated in the CBD syndrome considered diagnoses can be made. When (now called CBDS or CBS), but later it the specialist says the patient has a tic, was recognised that the most common then the patient has a tic. When the presenting symptom was “dementia”.4 specialist says the patient has PSP, then the A significant overlap was documented patient has PSP. There are no simple tests between CBDS and the syndromes of that can say that the doctor is wrong. FTD/Pick complex in the majority of However diagnosis is not the end-game. cases5. A corollary to this overlap is that The best Clinical Neurologists will see CBD pathology can produce focal such diagnoses as the beginning of a long cortical syndromes such as aphasia or term relationship with a patient. Predict- disinhibited behaviour without the ing pathology is therefore only half the movement disorder. PSP, although game, the diagnosis must help the patient, initially also described as a movement and other caregivers about the disease, disorder, became the prototype for treatment and prognosis. “subcortical dementia”, characterised by profound slowness of Progressive supranuclear palsy is such a condition where clinical mentation, impaired memory retrieval and personality changes diagnosis is easiest for the experienced neurologist and where referring (mainly apathy with some outbursts of irritability). Cognitive doctors are often unclear about the cause of the symptoms. Clifford phenomena, such as aphasia and apraxia, initially considered Richardson described the clinical syndrome in 1963, highlighting the unusual for PSP, are now seen as a rule. progressive disability, early falls, vertical supranuclear gaze palsy and The clinical overlap of CBD with PSP has been increasingly mild cognitive changes, and the absence of early parkinsonism. When recognized. Many CBD patients also have vertical gaze palsy, falls, faced with this clinical scenario within the first year of disease in a axial rigidity and some have symmetrical extrapyramidal syn- patient over 40, the diagnosis is extremely accurate. CONTINUED ON PAGE 16 CONTINUED ON PAGE 17 www.movementdisorders.org E d i t o r i a l inside this issue It’s about time to meet up again with our international colleagues and friends. The main annual event of our society, the 15th Controversy 1,16-17 International Congress of Parkinson’s PSP: Lumping or Splitting? Disease and Movement Disorders, will take place in Toronto, ON, Canada in Editor’s Section 2 Carlo Colosimo, MD and June. As you will read in this issue, the Mark Stacy, MD Congress Scientific Program Committee, chaired by Drs. Goetz and Fox, has made President’s Letter 3 tremendous efforts to plan an exciting Philip Thompson, MB, BS, PhD, FRACP, President scientific program. The final program is a fine blend of lectures on classic aspects of International Congress 4 movement disorders and new cutting- Carlo­Colosimo,­MD­and­Mark­Stacy,­MD Website 5 edge topics. The same is true for the speakers, who will include recognized opinion leaders but also a selection of newcomers, including a selection Society Announcements 6-7 of young and innovative researchers. This year the main Congress will be preceded for the first time by the European Section 8-9 Basic Movement Disorders Course. Alfredo Berardelli, MD, This course has been designed as a basic but comprehensive introduction to the fascinating field which is the MDS-ES Chairman area of interest of MDS. This course serves as a pilot for the Basic Movement Disorders Curriculum, which Asian and Oceanian 10-13 will eventually be available globally as a stand-alone course. Section In this issue, you will find the usual regular features including the President’s letter, reports from regional Bhim Singhal, MD, FRCP, MDS-AOS Chairman sections chairs and MDS-supported meeting organizers, and a summary of the activities of the MDS education committee. We also feature an interesting controversy on the relationship between progressive supranuclear Pan-American Section 14-15 palsy and corticobasal degeneration. We really hope you will enjoy the two contenders’ thorough and articulate Jorge Juncos, MD and pieces as we did. Irene Litvan, MD Finally, this time we asked for pictures from our readers who are also musicians for fun, and the response was quite enthusiastic as you can see on page 7. Next issue we decided to call for pictures taken during travelling, especially if the images are somewhat amusing or peculiar (or taken in unusual locations!). PA G E 2 M o v i n g A l o n g • I s s u e 1 , 2 011 P r E s i d E n t ’ s l E t t E r In June 2011, The Movement who will represent the Society. This year’s Nominating Disorder Society (MDS) will hold Committee included Warren Olanow, Stanley Fahn, OFFICERS its biennial election at the Annual Joseph Jankovic, Andrew Lees, Marcelo Merello, Wer- Business Meeting during the 15th ner Poewe and Hiroshi Shibasaki. The criteria they President International Congress of Parkin- used for assessing potential nominees for leadership Philip D. Thompson, MB, BS, PhD, son’s Disease and Movement Disor- positions in the MDS included: FRACP ders in Toronto, Canada. 1. Geographic diversity President-Elect The election process is an essential component in the 2. Gender diversity Günther Deuschl, MD success of our Society. It is our responsibility to iden- 3. Special expertise and knowledge Secretary tify leaders who promote advances in our field and also 4. Early as well as late career experience Matthew Stern, MD provide vision and oversight of our Society to ensure its 5. Previous service in the MDS leadership (for Officer Secretary-Elect continued growth and success. positions, a general guideline is that individuals Cynthia Comella, MD serving at this level have served previously as com- The election procedure is a multi-step process: mittee, task force and/or regional section members, Treasurer January as chairs of one or more of these groups, and as Oscar Gershanik, MD The Nominating Committee prepares a slate of candi- members of the IEC. In the case of President-Elect, Treasurer-Elect dates for each open Officer and International Executive these individuals may also have had previous experi- Nir Giladi, MD Committee position in January of the election year. ence serving as an MDS Officer, i.e., Secretary or Past-President February-April Treasurer) Anthony E. Lang, MD, FRCPC A Call for Nominations is sent to all MDS members in 6. Leadership/management skills February with the proposed slate of candidates. Each 7. Discipline diversity (e.g. neurology, neurosurgery, INTERNATIONAL EXECUTIVE member has the opportunity to submit nominations for basic science, etc.) COMMITTEE any additional individual he or she would like to stand This election process ensures that MDS continues to be Giovanni Abbruzzese, MD for election. All additional nominations must be re- guided by a diverse group of individuals prepared to Alim Benabid, MD, PhD ceived by April 8, 2011 and accompanied by a mini- devote their efforts working for the betterment of the Kailash Bhatia, MD, DM, FRCP mum of twenty-five letters of support from paid Society and the field of Movement Disorders. I hope David John Burn, MD, FRCP members of the Society. you will participate in the election process. Your in- Ryuji Kaji, MD, PhD April-May volvement is vital for the selection of an effective and Irene Litvan, MD By April 20, 2011, the Nominating Committee and innovative leadership which will contribute to the Serge Przedborski, MD, PhD membership nominations will be combined to form the ongoing development and achievement of the Society. Cristina Sampaio, MD, PhD final ballot. Should you not have an opportunity to A. Jon Stoessl, MD, FRCPC attend the Business Meeting during the International Congress on Thursday, June 9, 2011 to vote, you can vote by Proxy Ballot, which has a return deadline of May 18, 2011 and will be mailed to you with your final ballot. Philip Thompson, MB, BS, PhD, FRACP The Nominations Committee carefully considers MDS Pressident 2009 – 2011 many factors when selecting the slate of candidates MDS Past-President, Anthony Lang, Receives Order of Canada MDS Past-President, Anthony E. Lang, recently was named Officer to the Order of Canada, the country’s highest honor for a lifetime of outstanding achievement.
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