Welcome to the first of many editions of the Cortical Ribbon, the brain-child of Lindsay and John, two neurology residents at the University of Pennsylvania. We quickly realized in our JAR lives that there is a paucity of reading time. We felt a newsletter or blog highlighting important people as well as cases and tid-bits of knowledge acquired on the various services would help keep us all up to date and in the loop. Our goal is to intertwine things all residents should absolutely know with things we never need to know (but may help us impress someone at a dinner party some day) in a way that is both insightful and humorous.
Image of the Week – A Pain in the Neck
A middle aged female with no previous medical history presents with a two week history of right sided burning neck and shoulder pain not resolved with analgesics. She had also developed progressively worsening right arm worse than leg weakness, paresthesias, and sensitivity in the right arm and leg. She denies left sided symptoms and had no visual complaints. She had never experienced symptoms like this before. She reports mild saddle anesthesia but denies bowel or bladder incontinence. Her exam is notable for hyperesthesia in right neck and shoulder as well as moderate RUE and RLE weakness, and right sided hyperreflexia. Her lumbar puncture demonstrated no pleocytosis, normal protein, and 3 oligoclonal bands. It was negative for VZV PCR and cyto/flow in CSF. Serum studies were notable for normal ANA, West Nile, HIV, RPR, Lyme Ag, SSA/SSB, ANCA, ACE, B12. She was treated with 5 days of IV solumedrol followed by a slow prednisone taper with moderate improvement at time of discharge. After discharge, her serum studies return with a positive NMO antibody titer of 1:2000 and she will begin Rituximab infusions soon.
Pain in the Neck? – Yes, it’s a central demyelinating syndrome, but nonetheless pain can be a presenting symptom in NMO.
Friends but Not Family – NMO is a unique entity from Multiple Sclerosis. MS Drugs such as interferon beta, natalizumab and fingolimod may actually be make NMO worse.
Watchful Waiting – Bad idea in NMO. 90% of NMO patient’s not on treatment have repeat attacks within 3 years. Keep working on those therapeutic alliances young padawans!
NMO Toolbox – Initial treatment of exacerbation includes high dose steroids vs PLEX. Rituximab is the mainstay of treatment, but there is currently no published evidence regarding Ocrelizumab.
Weekly Word Salad
What to say when that Tinder date got real weird real fast…
They probably had Witzelsucht syndrome. Witzelwhat? This in fact is a real thing. Witzelsucht refers to a set of rare neurological symptoms, manifesting with a near constant tendency to pun, make poor jokes, and tell pointless stories in inappropriate situations. Additionally, people tend to make inappropriate sexual jokes or display hypersexuality. The best part is that people have no idea that their behavior is abnormal. This is most commonly seen with frontal lobe damage and can be referred to as the “Joker syndrome.” Swipe left next time kids.
The Price is Rite Review
Oculomasticatory Myorhythmia: A Pathognomonic Finding in Whipple’s Disease
If you thought you were done with Whipple’s disease the day you ended your step exams, think again. We all remember the uncommonly seen but always tested clinical syndrome of Whipple’s disease. Characteristically, patients present with malabsorptive symptoms, weight loss, diarrhea and joint pain. It turns out that the CNS can also be affected in a wide variety of ways, including confusion, coma, delirium, cognitive impairment, memory loss, or even abnormal movements. The most pathognomonic of these is oculomasticatory myorhythmia (say that five times fast). I could explain it to you in words, but check out this video first.
As you can see, this condition presents with pendular convergent and divergent oscillations of the eyes, in addition to a supranuclear palsy. Patients can also have myoclonus, hypothalamic dysfunction and dementia. Additionally, the muscles of mastication also have this notable contraction. It’s important to note that the actual palate muscles are not involved.
You’re probably asking yourself, didn’t my attending pimp me on some triangle with some weird mouth movements that kind of sounds like this? Yes, he did, but he was looking for a similar but unrelated condition of palatal myoclonus, localizing to the Guillan Mollaret Triangle (aka dentatorubro-olivary pathway). Basically, it’s a pathway connecting the dentate nucleus (pons) to red nucleus (midbrain) to inferior olive (medulla). Brainstem stroke = bad = watch for GMT problems. More learning here.
This is your Brain on Poetry – Weekly Neurology Haikus
Real bad nystagmus?
Normal HINTS – BPPV!
Still get MRI.