Be on the lookout for Harlequin ... known as a wily character, this disorder can be tricky to diagnose and determine an underlying cause
About a third of patients also had pupillary abnormalities and abnormal reflexes ... consistent with Ross syndrome 👁️ 🔨
Post surgical patients looked similar to other patients other than onset.
Other than where a causes was suspected (patient with cancer) imaging was rarely useful.
Over 26 yrs, 51 patients presented with Harlequin.
🔹median age 52
🔹76% women
Causes:
▶️17 % occurred as post-op complications
⏯️Other causes included: neoplasm, MS, trauma, small fiber neuropathy, pure autonomic failure, autoimmune
⏭️ causes was deemed idiopathic in 37%
Sympathetics to the face:
🧠 Descending from the hypothalamus
⬇️intermediolateral cell column of the SC
⤴️second order neurons exit T1-T3 to the stellate ganglion
⬆️ third order neurons form a plexus around carotids to face 🙂↕️
Damage anywhere along this pathway can lead to Harlequin
First, what is Harlequin syndrome?
Unilateral facial anhidrosis leads to contralateral flushing and hyperhidrosis. While the flushed side is often concerning to the patient- the problem is the anhidrotic side which leads to compensatory sweating😓
TST: 🟣 sweating 🟡 is anhidrosis
In Clinical Autonomic Research:
pubmed.ncbi.nlm.nih.gov/40721556/
Curious about Harlequin syndrome- the neurologic disease named after the Italian theatrical performer from commedia dell'arte who is often depicted wearing a half mask?
The phenomenal Mayo med student Hannah Padilla shares new insights in Harlequin in our recent publication 🚨
5/ Three Actionable Takeaways from Dr. Block’s summary of our conversation:
2. Well-being initiatives must go beyond surface-level fixes: Yoga and mindfulness have value, but true solutions require structural changes like reducing unnecessary tasks and fostering social connections.
Movement + Autonomics go together like...
Peanut butter and jelly 🥜
Chocolate and vanilla 🍦
Socks 🧦
Join us for the #movement #autonomic section showcase #AANAM @AANmember Sunday 3-4
See you in San Diego!
@DrISubramanian @PaulBeachDOPhD @amahajanmd.bsky.social
Thanks, Paul! Yes, the natural history paper is fantastic.
PAF is fascinating. I also think we are missing so many patients... since there are no motor or cognitive features, they never get to neurology
This 'pure' group is important.
It's a #synucleinopathy with autonomic involvement and many have central involvement (RBD) yet don't develop motor or cognitive impairment leading to #MSA #PD or #DLB
And many thanks to my amazing colleagues & mentors @MayoClinicNeuro
Timeline was interesting with most symptoms clustering around OH onset.
Median time to development of DEB was 7 years after OH but note the variability
In patients that remain PAF, most developed widespread autonomic failure... bladder, bowels, sexual and thermoregulatory dysfunction.
Dream enactment behavior was documented in 52% - likely an undercount due to the study timeline for PAF diagnosis (1998-2012)
Let's dive in...
In our cohort of 202 PAF patients with over a year to follow-up, 65 % remain PAF. We called patients to assess for symptom timeline and phenoconversion.
This was a follow-up to our study focused on risk factors for phenoconversion.
pubmed.ncbi.nlm.nih.gov/32546656/
PAF is defined by neurogenic OH. For many, the term PAF means a prodromal state to a synucleinopathy with motor or cognitive impairment.
PAF➡️ MSA
PAF➡️DLB or PD
Yet many studies have shown the majority remain PAF.
So what is their natural history?
link.springer.com/article/10.1...
Sometimes all that glitters is gold 💫
In thinking of PAF as a prodromal state, we miss that the majority of patients remain PAF during their lifetime.
This is an important group and we studied their clinical and autonomic profiles
🚨 Hot off the press #CAR
Congratulations to our Mayo Clinic Neurology Class of 2029! I can't get over these incredible future leaders in neurology 🌟 Happy Match Day everyone! #neuromatch
1/ Welcome to Edition 243 of West’s Well-Being Wednesday! All of March will be dedicated to our new paper describing the @mayoclinic.org model and strategic plan for employee well-being #wellbeingwednesday #wellbeing #burnout #medtwitter #meded #medsky
www.mayoclinicproceedings.org/article/S002...
Her birth was at the start of WWI and WWII altered her professional career by leading to training in neurology with van Boegart. She later served her Belgian community through multidisciplinary neurologic care.
Interesting to see the eponym persist #historyofmedicine
Louis-Bar was interesting. Despite a very productive start to an academic career and training under Ludo van Bogaert, she was seemingly an outcast from the scientific community.
Here are the ocular telangiectasia photos from Louis-Bar's original 1941 description.
B shows what was likely the first description of ataxia-telangiectasia syndrome in 1926 although Henner and Syllaba described a dystonic syndrome rather ataxia
It's #BlackHistoryMonth! Feb 3 is National Women Physician's Day - the bday of Elizabeth Blackwell, the 1st woman to get an MD in the U.S. (1849).
Rebecca Crumpler was the 1st Black woman to get an MD (1864).
There are no photos of Crumpler! but you'll see photos, so let's talk about them. 1/10
An honor to work with the legend Dr. Wijdicks on the review of Janice Nimura's book on the Blackwell sisters
hekint.org/2021/07/15/b...
In honor of National Women Physicians Day, one of my favorite Elizabeth Blackwell quotes:
"I wish I could devise some good way of maintaining myself but the restrictions which confine my dear sex render all my aspirations useless."
So grateful that she persevered
Great work, Jeff!
Excited to share our recent paper with the results and analysis of the latest @aanmember.bsky.social survey of #Neurology Program Directors!
www.neurology.org/doi/10.1212/...
It's a good day to share S. Josephine Baker (1873-1945), a pediatrician featured in Endowed Chairs: Pediatrics, a card game created with @zachlondon.bsky.social
...and how the simple idea of feeding children led to an incredible decrease in mortality in NYC and America in the early 1900s 1/🪑
As we wind down interview season for #neuromatch 2025, I reflect on the misperceptions about @MayoClinic
My favorite is how many people have thought we drive around with chains on our tires!
No chains here... but we will take some more snow ❄️
Hello!
It's great to see so many of you here on Bluesky! I'm excited to be part of the #neurology #autonomic #movement #medEd #historyofmedicine communities.
Happy New Year! 🧠 🎊 🧠
